S aureus: eradicated in - Asociacion Medica de Puerto Rico
Transcripción
S aureus: eradicated in - Asociacion Medica de Puerto Rico
(In vitro data; clinical significance unknown. Full course of therapy is complete in 7 days.)1,2 n ZYMAR® ophthalmic solution rapidly eradicates key pathogens in vitro, including: S aureus: eradicated in 15 minutes1,* S epidermidis: eradicated in 30 minutes1,* S pneumoniae: eradicated in 10 minutes2,* H influenzae: eradicated in 5 minutes2,* * Time to reach kill threshold. 10 CFU/mL is the lower limit of detection and is indistinguishable from complete kill. ZYMAR® ophthalmic solution is indicated for the treatment of bacterial conjunctivitis caused by susceptible strains of the following organisms: Corynebacterium propinquum,† Staphylococcus aureus, Staphylococcus epidermidis, Streptococcus mitis,† Streptococcus pneumoniae, and Haemophilus influenzae. (†Efficacy for this organism was studied in fewer than 10 infections.) Important Safety Information: NOT FOR INJECTION. ZYMAR® ophthalmic solution should not be injected subconjunctivally, nor should it be introduced directly into the anterior chamber of the eye. As with other antiinfectives, prolonged use may result in overgrowth of nonsusceptible organisms, including fungi. If superinfection occurs, discontinue use and institute alternative therapy. Patients should be advised not to wear contact lenses if they have signs and symptoms of bacterial conjunctivitis. ® The most frequently reported adverse events occurring in approximately 5% to 10% of the overall study population were conjunctival irritation, increased lacrimation, keratitis, and papillary conjunctivitis. Please see brief prescribing information on adjacent page. 1. O’Brien TP. Antimicrobial efficacy of ZYMAR® and Vigamox® against Staphylococcus species. Refract Eyecare Ophthalmol. 2003;7(12):15-18. 2. Novosad BD, Callegan MC. Killing of Streptococcus pneumoniae, methicillin-resistant Staphylococcus aureus (MRSA), and Haemophilus influenzae ocular isolates by fourth-generation fluoroquinolones. Poster presented at: 78th Annual Meeting of the Association for Research in Vision and Ophthalmology; April 30-May 4, 2006; Fort Lauderdale, FL. ©2009 Allergan, Inc., Irvine, CA 92612 www.allergan.com ® marks owned by Allergan, Inc. ZYMAR® is licensed from Kyorin Pharmaceutical Co., Ltd., Tokyo, Japan. APC50TC09 803807 B LETÍN 3 MENSAJE DEL PRESIDENTE Asociación Médica de Puerto Rico CONTENIDO Rolance G. Chavier Roper, MD Original Articles (Artículos originales) 5 CARACTERISTICAS PSICO-SOCIALES DE UNA MUESTRA INICIAL DE CENTENARIOS PUERTORIQUEÑOS José R. Rodríguez-Gómez MD, Alfonso Martínez-Taboas PhD, Abdiel Cruz MS 39 COLOBOMA AND CHARGE ASSOCIATION IN PUERTO RICO Javier Jardón BS, Natalio J. Izquierdo MD 43 EFFICACY AND SAFETY OF LAPAROSCOPIC SPLENECTOMY: REVIEW OF 14 ADULT CASES USING THE LATERAL APPROACH Ramón K. Sotomayor-Ramírez, MD Review Articles (Articulos de Reseña) 12 OBESITY PREVALENCE ODDS RATIOS IN FOUR 50 ANEMIA AND INFECTIONS IN MULTIPLE MYE- Ada M. Laureano EdD, José Rodríguez-Gómez MD, Rosa Janet Rodríguez PhD, Juanita Centeno EdD MPH, Judith Rodriguez, PhD William Cáceres MD, Karen Santiago MD, Liza Paulo MD, Jaime Román MD PUERTO RICAN TOWNS: A PILOT STUDY 17 EPIDEMIOLOGICAL CHARACTERIZATION OF PE- DIATRIC PATIENTS WITH STATUS ASTHMATICUS ADMITTED TO INTENSIVE CARE UNIT Mariel Silva MD, Luis Munoz MD, Tania Diaz MD, Weldon Mauney MD, Vylma Velazquez MD 21 EOSINOPHILIC ESOPHAGITIS AND ALLERGIES IN PEDIATRIC POPULATION OF PUERTO RICO Vylma Velazquez MD, Carlos Camacho MD, Alfredo E. Mercado-Quiñones MD, Jadira Irizarry-Padilla MD 23 IS BARIUM ENEMA AN ADEQUATE DIAGNOSTIC TEST FOR THE EVALUATION OF PATIENTS WITH POSITIVE FECAL OCCULT BLOOD? Carlos Ramos MD, Joel De Jesús-Caraballo MD, Doris H. Toro MD, Algia Ojeda MD, Jaime Martínez-Souss MD, Maria Isabel Dueño MD, Marcia Cruz-Correa MD 29 ANTERIOR SKULL BASE CEREBROSPINAL FLUID FISTULAS IN PUERTO RICO: TREATMENT AND OUTCOME Yadiel A. Alameda MD, José M. Busquets MD, Juan C. Portela MD 34 EARLY OUTCOMES OF REVERSE TOTAL SHOUL- DER ARTHROPLASTY Luis Marrero MD, Gabriel Garcia MD, Ivan Pacheco MD LOMA: SUPPORTIVE THERAPY 53 A PROPOSED APPROACH FOR THE SELECTION OF THE PROPER SURGICAL THERAPY TO OBTAIN AN ADEQUATE MARGIN OF RESECTION IN LOCALLY ADVANCED ULTRA-LOW RECTAL CANCER AFTER MODERN PREOPERATIVE CRX MANAGEMENT Ignacio Echenique MD, Fernando Cabanillas MD, Vangie Texidor BA, Janice Cáceres MD, Gerald Isenberg MD, Carlos Claudio MD, Roberto Ayala MD, Frank Madera MD Case Reports (Reporte de Casos) 56 DOUBLE CYSTIC DUCT IN A CHILD WITH VAC- TERL ASSOCIATION: A CASE REPORT Humberto Lugo-Vicente MD, Maria Correa MD, Hector Brunet MD 59 SMITH-MAGENIS SYNDROME IN PUERTO RICO: A CASE REPORT Javier Jardón BS, Natalio J. Izquierdo MD Historical Articles (Artículos Históricos) 62 EL DR. LUIS IZQUIERDO MORA: UNA VIDA EN- TERA DEDICADA AL SERVICIO DEL PUEBLO Natalio J. Izquierdo-Encarnación MD CME 72 CME Questions and answers Catalogado en Cumulative Index e Index Medicus Listed in Cumulative Index and Index Medicus No. ISSN-0004-4849 Registrado en Latindex -Sistema Regional de Información en Línea para Revistas Científicas de América Latina, el Caribe, España y Portugal Foto de Cubierta: Dr. Luis Izquierdo Mora Diseño Gráfico e Ilustración digital de cubierta realizados por Juan Carlos Laborde en el Departamento de Informática de la AMPR E-mail: [email protected] BOLETIN - Asociación Médica de Puerto Rico Ave. Fernández Juncos Núm. 1305 P.O.Box 9387 - SANTURCE, Puerto Rico 00908-9387 Tel.: (787) 721-6969 - Fax: (787) 724-5208 e-mail:[email protected] Web site: www.asociacionmedicapr.org Web site para el paciente: www.saludampr.org JUNTA DE DIRECTORES Dr. Rolance G. Chavier Roper Presidente Dra. Ilsa Figueroa Dr. Eduardo Rodríguez Vazquez Presidente Distrito Este Presidente Saliente Dr. Gustavo Cedeño Quintero Dr. Pedro Zayas Santos Presidente Distrito Noreste Secretario Dr. Roberto Perez Nieves Dr. Benigno López López Presidente Distrito Sur Tesorero Dra. Hilda Ocasio Maldonado Dra. Mildred R. Arché Matta Vicepresidente Presidente Distrito Central Dr. Raúl Castellanos Bran Dra. Wanda G. Velez Andujar Vicepresidente Presidente Consejo de Educación Medica Dr. Raúl A. Yordán Rivera Dr. José C. Román de Jesus Vicepresidente Presidente Consejo Ético Judicial Dr. Arturo Arché Matta Dr. Edgardo Rosario Burgos Presidente Cámara Delegados Presidente Consejo Relaciones y Servicios Públicos Dr. Juan Rodríguez del Valle Dr. Modesto Gonzalez del Rosario Vicepresidente Cámara de Delegados Presidente Consejo Servicios Médicos Dr. Gonzalo González Liboy Dr. Jaime M. Diaz Hernandez Delegado AMA Presidente Consejo Salud Pública y Bienestar Social Dr. Rafael Fernández Feliberti Dr. Rafael Fernández Soltero Delegado Alterno AMA Presidente Consejo Política Pública y Legislación Dr. Ricardo Marrero Santiago Dr. Rafael Fernández Feliberti Delegado Alterno AMA Presidente Comité Asesor Presidente Dr. Julio de la Cruz Presidente Comite de Finanzas JUNTA EDITORA Humberto Lugo Vicente, MD Presidente Luis Izquierdo Mora, MD Juan Aranda Ramírez, MD Melvin Bonilla Félix, MD Francisco J. Muñiz Vázquez, MD Carlos González Oppenheimer, MD Walter Frontera, MD Eduardo Santiago Delpin, MD Mario. R. García Palmieri, MD Francisco Joglar Pesquera, MD Raúl Armstrong Mayoral, MD Yocasta Brugal, MD José Ginel Rodríguez, MD 3 Mensaje del Presidente Rolance G. Chavier Roper, MD Estimados colegas medicos: N uevamente me dirijo a ustedes con otro excelente ejemplar de nuestra publicación BOLETÍN de la Asociación Medica de Puerto Rico. Con esta revista , producimos nuestro segundo número del 2009 y tercero desde que asumimos las riendas de nuestra querida Asociación Médica de Puerto Rico. Debo, humildemente, agradecer a nuestra Junta Editora y a su Presidente por tan exitosa y productiva gesta. En este BOLETÍN, se tratarán diversos temas, que incluyen múltiples ramas de la medicina y es por eso que hemos decidido dedicarle la portada a un verdadero Salubrista y Padre de la Especialidad de Medicina de Familia en Puerto Rico: el Dr. Luis Izquierdo Mora . El Dr. Izquierdo Mora, quien sirvió como Presidente de nuestra Asociación Medica de Puerto Rico. en el 1967 y ha continuado laborando incansablemente en beneficio del pueblo de Puerto Rico. a través de su servicio a nuestra institución, encabezó un grupo de ilustres médicos a fines de la década del 60 que promulgaron que las enmiendas a la ley del seguro social aplicaran en Puerto Rico. a la vez que en el resto de los Estados Unidos. A esta gesta, se le llamó “operación igualdad”. El Dr. Izquierdo ha permanecido activo en nuestra organización ininterrumpidamente y ha sido, en múltiples ocasiones, asesor principal de otros Presidentes de la Asociación. Siempre ha estado disponible para cooperar y ayudar, sin importar posiciones o títulos. Más adelante en su carrera , incursionó en la política partidista y fue electo Senador. Llegó a ser Presidente de la poderosa comisión de Hacienda del Senado. Fue distinguido. posteriormente. con el honor de ser seleccionado Secretario de Salud del Estado Libre Asociado de Puerto Rico. Ha sido asesor de subsiguientes Secretarios de todos los partidos políticos y su lema siempre ha sido que “la salud no tiene colores”. Además de su gesta política, hay muchas otras dimensiones en la personalidad de este gran puertorriqueño. Es importante señalar que el Dr. Izquierdo ha sido Profesor de las cuatro Escuelas de Medicina de Puerto Rico. Ha sido Director del Departamento de Medicina de Familia en varias de ellas y actualmente sigue activo en su rol como maestro y, sobretodo, inspiración para la nueva generación de galenos, quienes ven en él ejemplo de lo que realmente debe ser un médico que está comprometido con sus pacientes y que realmente estudió esta profesión por vocación. En adición a todo esto, debemos mencionar que este ilustre médico ha sido fundador de todas las residencias de Medicina de Familia de Puerto Rico y su labor ha sido reconocida y elogiada por la Academia de Médicos de Familia de Puerto Rico. Luego de todos estos logros que hemos mencionado y, créanme, que faltarían muchos más por mencionar, es fácil imaginarse que el Dr. Izquierdo hubiese escogido establecer una lucrativa práctica médica en algún prestigioso Instituto del área metropolitana, para ejercer su sub especialidad como Geriatra. Otros, tal vez, hubiesen optado por presidir alguna Empresa de Servicios de Salud u Hospital, especialmente una persona con los “boards” del NCQA, como lo es él. Sin embargo, el Dr. Izquierdo optó por mantenerse en el barrio Capetillo de Rio Piedras, sirviéndole a la gente humilde, quien siempre fue su razón de ser. Allí lleva laborando por mas de 49 años y aun hoy, atiende a sus pacientes, quienes se han convertido en sus amigos y hasta en su familia extendida. En la actualidad, es el medico con mayor “fanaticada” en el centro donde labora y los pacientes abarrotan su consultorio para buscar bienestar, confianza, tranquilidad y sobre todo, Salud Espiritual, que es su más reciente enfoque en la práctica. Su lema: “no sólo es importante ser un buen médico , sino un médico bueno”, ha calado profundo en sus alumnos y compañeros de trabajo. De mi maestro, mentor, amigo entrañable y “padre en la medicina”, quien, hace ya muchos años, me trajo por primera vez a la Asociación Médica, sólo puedo decir que es una verdadera inspiración y que laborar junto a él es un verdadero privilegio. Que el Todopoderoso le siga bendiciendo con salud y felicidad junto a su hermosa familia, que ha sido siempre el principal proyecto de su vida. Dr. Rolance G. Chavier Roper Presidente, Asociación Medica de Puerto Rico. Boletín de la Asociación Médica de Puerto Rico - Volumen 101 - Núm. 2 Abril - Junio 2009 5 RESUMEN Este es el primer estudio realizado en Puerto Rico con una población centenaria puertorriqueña. El propósito de esta investigación es explorar las condiciones socio-psicológicas en una muestra de centenarios puertorriqueños. Los hallazgos de esta muestra permiten comenzar a establecer un perfil de centenarios puertorriqueños, los cuales demuestran pocas alteraciones en sus capacidades cognitivas y psicológicas según evaluadas por varios instrumentos. La muestra estuvo constituida por 5 mujeres y 6 varones, cuyas edades fueron: tres de 100 años, uno de 101, dos de 102, dos de 103, uno de 105 y dos de 108 años; todos los/as participantes son viudos/as, católicos y reportan escasos recursos económicos. Los instrumentos utilizados fueron: el listado de síntomas SCL-90-R, el Inventario de Depresión de Beck (BDI-II), la Escala de Suicidio de Beck (BSS), la Escala de Desesperanza de Beck (BHS), el Inventario de Ansiedad de Beck (BAI) y el Mini-Mental de Folstein. Los resultados obtenidos sugieren poca o ninguna sintomatología depresiva o ideación suicida. La población centenaria es una con demandas o esquemas cognitivos de desesperanza evidentes según identificados en este estudio. Los niveles de ansiedad encontrados fueron por debajo de índices encontrados en poblaciones más jóvenes realizadas en previas investigaciones epidemiológicas en Puerto Rico, aun cuando se identifica en esta muestra más sintomatología de desesperanza y ansiedad según el BHS y BDI-II. Sugerimos más investigaciones en esta población. Palabras claves: Centenarios puertorriqueños, ancianos, cernimiento en salud mental Original Articles (Artículos originales) CARACTERISTICAS PSICO-SOCIALES DE UNA MUESTRA INICIAL DE CENTENARIOS PUERTORIQUEÑOS José R. Rodríguez-Gómez MD * Alfonso Martínez-Taboas PhD * Abdiel Cruz MS * * Universidad de Puerto Rico, Recinto de Río Piedras, Puerto Rico, y Universidad Carlos Albizu, Recinto de San Juan, Puerto Rico. Autor Principal: Dr. José R. Rodríguez, Universidad de Puerto Rico, Recinto de Río Piedras, Facultad de Ciencias Sociales, Dept. Multidisciplinario de Ciencias Sociales, P.O. Box 23345 San Juan, PR 00931-3345. E-mail: [email protected]. INTRODUCCION L a vejez es un concepto relativo y dinámico. Es relativo a cómo lo define una determinada sociedad y al momento histórico dónde se vive. Es dinámico porque los procesos sociales cambian, lo que impacta, por ejemplo, la expectativa y calidad de vida de los viejos. Como medida elemental, la vejez puede ser identificada en función de la edad cronológica. La etapa de vida en la que se encuentra toda persona con 65 años de edad o más, puede establecerse en términos sociales o legales como el umbral de la vejez. Se tomó como base esta frontera, ya que en Puerto Rico es usual considerar/identificar a una persona de 65 años o más como una persona vieja. Debido a la diversidad de características de esta población, una posible clasificación, consiste en definir tres cohortes o grupos para describir cada subconjunto de envejecidos/as sin generalizaciones entre los grupos de edad. Estos subconjuntos constituyen la etapa de vida que forma la adultez tardía. Se reconoce dentro de la categoría al “viejojoven”. Es decir, a las personas cuya edad es mayor de 65, pero menos de 75 años. De manera similar, se considera “viejo de mediana edad” a la persona cuya edad es mayor de 75, pero menor de 85 años. Por último, Atchley 8 considera “viejo-viejo” al que cuenta con 85 años o más. Al analizar los datos censales del 2000 en Puerto Rico9 para los grupos ancianos, se desprende que de los tres grupos mencionados, el grupo de “viejos-jóvenes” presentan la mayor concentración agrupando un total de 240,951 personas. Dicho número representa el 6.3% de la población. El segundo cohorte incluyó a los “viejos de mediana edad” con un total de 136, 480 personas. Ese número representó el 3.6% de la población. El grupo de los “viejos-viejos” incluyó 47,706 personas. Es decir, el 1.3% poblacional según el U.S. Bureau of the Census9. En Puerto Rico, al igual que en los países industrializados, la población envejecida exhibe el ritmo más rápido de crecimiento. Según el ‘U.S. Bureau of the Census’9 , la población envejecida constituía aproximadamente el 11.2% del total de la población de Puerto Rico En cuanto a los componentes de la población envejecida, nótese que una estructura demográfica suele considerarse como viejos si los grupos de 65 años y más sobrepasan el 10% del total de habitantes de un país. Esta premisa parte del dato de que a medida que la expectativa de vida aumenta, por razones tecnológicas, científicas y económicas, las sociedades se vean forzadas a retrasar la edad del retiro de la fuerza trabajadora. 6 Al comparar el porcentaje de la población de edad superior a los 65 años con el observado en 1990, que fue de 9.7%, se hace evidente un patrón de crecimiento sustancial. El cambio porcentual observado en esta década es de 24.7%9. Es decir, un promedio anual simple de 2.5% aproximadamente que es más del doble que el registro por la población total. El incremento en la población envejecida tiene importantes implicaciones sociales, entre las que se destacan la prolongación del contacto inter-generacional. Este se produce, por ejemplo, a través del apoyo familiar en el cuido de niños, el apoyo emocional y financiero. Además, la sociedad confronta ciertas necesidades de forma colectiva. Por ejemplo, proveer accesibilidad a servicios de salud, trasportación y vivienda a los ancianos. Los números reflejan que, al compararse con los datos históricos, hay una tendencia a aumentar en los componentes extremos de la estructura demográfica del país. El extremo superior de la pirámide poblacional gana 20,000 personas de edades superiores a los 59 años. Mientras tanto, la base de la pirámide sufrió una pérdida en la población menor de los 19 años10. Se proyecta que para el año 2010 el total de personas mayores de 65 años en Puerto Rico alcance las 563,516. Es decir, el 14% de la población, y que la tendencia coloque dicha proporción en un 18.6% para el 2020 y en un 30% en el 2050. La División de Población del Negociado del Censo Federal publicó una estimación de población por edad y sexo al 1 de julio de 2006 que incluye Estados Unidos, sus 50 Estados y Puerto Rico. El informe presenta la estimación de población para grupos de edad seleccionados11. Según los datos presentados, la población de 65 años y más, aumentó a 503,884 personas, lo que representa el 12.8 por ciento de la población total. Esto representa un aumento de 78,747 personas de 65 años y más, adicionales al Censo del 2000 o 1.6 puntos porcentuales adicionales evidenciándose el envejecimiento de la población en la Isla. Al comparar con los 50 Estados Unidos, Puerto Rico supera en el número de población ancianos a 26 estados. Algunos indicadores del envejecimiento de la población son: una proporción de población de 65 años y más de 10.0 por ciento o mayor, una mediana de edad de 30 años o más y una proporción menor a 30.0 por ciento para la población menor de 15 años con respecto a la población total. Estos datos confirman, sin lugar a dudas, que la población de Puerto Rico está envejeciendo. La mediana de edad al 1 de julio de 2006 es de 32.9 años para los varones y de 36.4 años para las mujeres o una diferencia por sexo de 3.5 años. El 11.5 por ciento de los varones y el 14.0 por ciento de las féminas al 1 de julio de 2006 tienen 65 años de edad o más. En relación a la población menor de 15 años, ésta representa el 22.8 por ciento de los varones y el 20.1 por ciento de las mujeres11. También se reporta por el Negociado del Censo de los Estados Unidos 2000 que en uno de cada tres hogares puertorriqueños habita una persona de 60 años o más9. El 74.1% de los envejecidos viven en hogares de familia, en donde dos o más personas están relacionadas por sangre, adopción o matrimonio. De esos, 99,702 (23.5%) personas de 65 años o mas viven con una o más personas no relacionadas por sangre, adopción o matrimonio. El 2.5% vive en hogares sustitutos o instituciones. Dentro de ese grupo debemos mencionar que hay 7,035 personas recluidas en instituciones correccionales, hogares de convalecencia, hospitales, programas de hospicio para condiciones terminales y en hospitales psiquiátricos12. Con el aumento de la población envejecida, se ha incrementado también los problemas que experimentan los viejos. Es de conocimiento general que el envejecimiento afecta muy especialmente los sentidos. Sin lugar a dudas las personas centenarias pudiesen tener una mayor prevalencia de limitaciones sensoriales debido a su edad. Investigadores han comprobado que el 35% de las personas centenarias son sordas y el 20% de los octogenarios sufren sordera social y problemas visuales de tal magnitud que impiden la lectura13. Lo anterior puede aumentar la marginación social en esta población causando a su vez serios problemas, incluyendo problemas de salud mental. La necesidad de explorar como los ancianos centenarios puertorriqueños se encuentran en términos de algunas variables de salud mental seria de utilidad para comenzar a conocer esta subpoblación de ancianos y proveer para sus necesidades. Estudios realizados en Puerto Rico Al realizar una revisión de la literatura sobre investigaciones realizadas con centenarios utilizando varias fuentes de búsqueda en los últimos 20 años (i.e., Pubmed, Medline, Psylit), adicional a la búsqueda en bibliotecas académicas de Puerto Rico, no encontramos que se hayan publicado estudios científicos dirigidos a identificar, explorar y describir dicha población. Este hallazgo da base a la realización de este estudio como uno piloto para compensar esta deficiencia. Estudios realizados fuera de Puerto Rico La búsqueda de estudios o investigaciones relacionadas a ancianos centenarios se concentró en revistas científicas, bases de datos científicos, libros y tesis doctorales. El tema de ancianos centenarios es uno relativamente virgen, ya que existen pocos estudios exploratorios-descriptivos o inferenciales publicados en revistas profesionales que tengan arbitraje de pares. Sin embargo, uno de los pocos estudios realizados en Latinoamérica con poblaciones centenarias expone sobre el incrementado de la población anciana en las ultimas décadas; y hoy la “Región de las Américas”, cuenta con alrededor de 2,228,900 personas de 90 años o más, de las cuales, 90,400 son centenarias14. Para mediados del siglo, estas cifras habrán ascendido a cerca de 13,903,000 y 689,000, respectivamente. Se han realizado otros esfuerzos investigativos a nivel mundial, fundamentalmente en países desarrollados, refutando la hipótesis de que las personas de 100 años o más son dependientes, dementes o discapacitadas en su gran mayoría14,15. Estos estudios han proporcionado datos científicos demostrando que existen centenarios saludables y que constituyen el mejor ejemplo de envejecimiento satisfactorio. De hecho, en PR según las Estadísticas Vitales del Departamento 7 de Salud de Puerto Rico, 2005 (últimas publicadas) la totalidad de ancianos sobre los 85 años es de 22,229; lamentablemente no se pueden identificar cuantos tienen 100 o más años por la forma en que se reportan, ya que la categoría de reporte solo indica “grupo de 85 años en adelante”. Sin embargo, el Negociado del Censo Federal, para 1ero de julio 2007 (ultimas disponibles), estima que en Puerto Rico hay aproximadamente 916 personas de 100 años en adelante, divididas en 295 varones y 621 mujeres. Por otro lado, un elemento a considerar cuando estudiamos a los centenarios lo son los cuidados paliativos de éstos 15. Desde esta perspectiva se ha estudiado el avance en el cuidado de la salud y los cambios demográficos y epidemiológicos presentes en el plano mundial, encontrándose que han llevado a que se produzca un envejecimiento progresivo de la población, con las implicaciones en el campo social y económico, en los sistemas de educación y salud, y en la seguridad social en términos de costos asociados a su cuido 15. Como resultado de lo anterior, va a ser mucho más frecuente encontrar ancianos que tengan patologías crónicas discapacitantes en estadios avanzados. Debido a la complejidad de este tipo de pacientes, se requiere de la implementación de estrategias de intervención que permitan valorar los aspectos médicos, psicológicos, sociales, funcionales y contextuales, de tal manera que se pueda realizar un diagnóstico manejo y seguimiento adecuado con un margen de tiempo intervención rápida. Lo anterior se logra mediante la evaluación geriátrica multidimensional, a través de un trabajo efectuado en equipo interdisciplinario. Es muy probable, pues, que comencemos a tener cada vez mas ancianos centenarios. Como se reconoce, el envejecimiento es un proceso universal que al parecer afecta a todos los seres vivos y que está concebido como una sucesión de modificaciones morfológicas, fisiológicas y psicológicas de carácter irreversible, que se presentan y dan el aspecto de anciano16. Sin lugar a dudas con un nivel de cuidado responsable y accesible podemos continuar mejorando nuestra expectativa de vida. Continuando con algunos estudios sobre centenarios identificados en nuestra revisión, uno de los pocos, lo fue el que estudiaba a ancianos italianos y grados de demencia. Ese estudio es uno de tipo epidemiológico, el cual identifica algunos componentes genéticos y como esto afecta la prevalencia de demencia en dicha población 17. Un segundo estudio con centenarios fue realizado en la cuidad Tokio, Japón. En este se indago el estatus funcional de los centenarios. La muestra de este estudio constó de 65 hombres y 239 mujeres residentes de Tokio, Japón. El estatus funcional incluía áreas de funcionamiento sensorial, físicos y cognitivos. En dicho estudio se clasificaron los participantes en cuatro categorías, acorde al estatus de su funcionamiento. El 2% obtuvo una puntuación de excepcional, el 18% se clasificó como normal en su funcionamiento cognitivo y fisiológico, el 55% fue clasificado como frágil con un deterioro en las áreas cognitivas o fisiológicas y el 25% fue clasificado con un deterioro significativo 18. Como notamos hay una carencia de investigaciones, muy particularmente en población Hispana, por lo que se hace imperativo comenzar a estudiar a este tipo de ancianos. Patologías más comunes en la ancianidad: Depresión y Conducta Suicida De las patologías mas comunes en la población de envejecidos están la depresión y la conducta suicida. Según el Negociado del Censo para el 2030, este grupo constituirá el 23% de la población total de los Estados Unidos, con estimados similares para Puerto Rico11. Cabe señalar que se ha reportado que uno de los diagnósticos más comunes encontrados, y que se identifica como de alto riesgo, para cometer suicidio, es la depresión clínica, tanto en Puerto Rico como Estados Unidos19. El suicidio es un problema de salud pública y de importancia creciente tanto en jóvenes como ancianos. Según la literatura, el suicidio es un acto que se comete con el propósito de quitarse la vida 20, 21. Investigadores puertorriqueños definen la conducta suicida como una conducta o conjunto de conductas que, llevadas a cabo por el sujeto que desea quitarse la vida21. En una de las primeras investigaciones con sobrevivientes de actos suicidas en Puerto Rico identifican varios factores de riesgo para el envejecido. El ser hombre, blanco, viudo(a), divorciado(a), separado(a), vivir solo(a), socialmente aislado(a), tener dificultades económicas y salud física pobre o deteriorada se consideró entre los mayores factores de riesgo21. Es necesario reafirmar el problema del suicidio como uno serio de salud mental sobretodo en población anciana puertorriqueña, puesto que ya hay múltiples estudios que la identifican como una población a muy alto riesgo de cometer suicidio en comparación con otras subestratas de edad más joven 22. Otro estudio realizado en Puerto Rico identificó algunos factores que podrían ser de alto riesgo para el suicidio22. Estas son: la falta de interés en actividades que usualmente disfrutaba, la reducción en interacción social, perdida de roles sociales importantes, las quejas en relación a la disminución en energía, o fatiga, quejas de dolores crónicos que no mejoran con tratamiento, conductas auto-destructivas (tales como no tomar medicamentos o negarse a comer), la pérdida de personas significativas, el sentimiento de desesperanza e inutilidad, el regalar objetos preciados, realizar cambios en testamento y despedirse. Es, pues, vital explorar la posibilidad de dicho acto en población anciana, junto a la identificación de los factores de riesgo asociados. Justificación. Ante la falta de datos socio demográficos y de información psicológica, se deben realizar nuevas vías investigativas dirigidas a explorar la población de centenarios puertorriqueños. Es por ello que este estudio pretende dar los primeros pasos, describiendo elementos socio demográficos y explorando algunos componentes de disfunción psicológica entre esta población. Método Muestra La muestra está compuesta por 11 sujetos escogidos por disponibilidad. La metodología de búsqueda incluyó el uso de guía telefónica, consulta a la prensa escrita y televisiva, llamadas a la Oficina del Seguro Social, y consultas a la Procuraduría de Personas de Edad Avanzada de Puerto Rico. 8 La muestra se compone de (5 mujeres y 6 varones). Los criterios de inclusión fueron: (1) que cada participante tenga la edad de 100 años o que esté cerca de cumplirlos, (2) que pudieran ver y escuchar, (3) que el pensamiento fuera lógico y coherente, (4) que se encontrara orientado en tiempo, lugar y persona, (5) que el cuidador y el participante acepten entran al estudio de manera voluntaria y (6), que no se encontrase en un hogar de cuido extendido. La frecuencia de edad fue: cuatro de 100 años, uno de 101, dos de 102 años, dos de 103 años y dos de 108 años Todos los participantes nacieron en Puerto Rico, al igual el su estado civil es de viudo/a. En el área de residencia, nueve viven en área rural y dos en área urbana. Todos los participantes viven con algún familiar. Seis participantes reportaron no haber asistido a la escuela y 5 llegaron a la escuela elemental. En cuanto al ingreso mensual sólo uno tiene ingreso entre 100 a 200 dólares, dos tienen entre 201 a 300 dólares de ingreso mensual. Tres entre 301 a 400 dólares, cuatro entre 401 a 500 dólares y uno entre 1, 300 o más de ingreso mensual. Nueve participantes se definieron como católicos, uno como de la Iglesia Pentecostal y uno pertenece a la Iglesia Presbiteriana. Siete participantes consideraron que su estado de salud actual es regular. Sólo 5 considera su relación con la iglesia que asiste como regular y 6 considera que su iglesia presta adecuado apoyo. Seis participantes expusieron que asiste poco a la iglesia y cinco centenarios consideraron como buena la relación con su líder eclesial (Véase Tabla 1). Tabla 1: Variables Socio-Demográficas de la Muestra de Centenarios Genero 5 mujeres; 6 varones Edad Promedio 102.4 años Categoría de Educación mas prevalente Analfabeto Religión practicante mas prevalente Católica Categoría de Ingreso mas prevalente $401.00-$500.00 mensuales Auto-Reporte de Estado de Salud mas Prevalente Estado de salud Regular Área de Residencia Prevalente Rural ¿Con quien vive? Familiares (usualmente Hijas) Estatus Marital Prevalente Viudo/a PROCEDIMIENTO Se contacto a familiares, custodios o encargados legales de cuidar a los centenarios para informarles y orientarlos sobre el estudio. Los mismos otorgaron su consentimiento. Luego se obtuvo el consentimiento de los centenarios. Toda la muestra fue elegida por disponibilidad. Si se identificaba la existencia de alguna limitación severa sensorial, entiéndase auditiva, visual o motora, se proveía asistencia para contestar los instrumentos a utilizarse o se le solicitaba ayuda al cuidador. Se orientaba, tanto al cuidador como al envejecido sobre los procedimientos a realizarse y las pruebas a administrar. La mayor cantidad de información demográfica era provista por el cuidador. En dos de los participantes hubo que utilizar un sistema de audio que aumentara el nivel de sonido (una alta voz) ya que los centenarios presentaban dificultad a escuchar. Con una de las participantes se utilizo un sistema de escritura agrandada ya que tenía dificultad visual. La función del cuidador en la etapa de la administración de prueba era trascendental para la contestación de algunas pruebas. Luego se realizó un análisis de los hallazgos de cada prueba por sujeto para posteriormente realizar análisis estadísticos descriptivos e inferenciales tales como; análisis de frecuencia y de correlación, utilizando el Programa SPSS-X versión 14. INSTRUMENTOS Se utilizaron los instrumentos a continuación; el Symptom Checklist 90-R (SCL-90-R), es un cuestionario multidimensional autoaplicado, desarrollado por Derogatis1 . Está compuesto por 90 reactivos, diseñados originalmente para evaluar el grado de malestar en pacientes psiquiátricos, como también en sujetos no clínicos. El malestar psicológico está medido en 9 dimensiones primarias de síntomas (Somatización, Obsesión- Compulsión, Sensibilidad interpersonal, Depresión, Ansiedad, Hostilidad, Ansiedad fóbica, Ideación Paranoide y Psicoticismo). A partir de estas dimensiones se obtienen tres índices globales de malestar que son indicativos de diferentes aspectos del sufrimiento psicopatológico general: el primero es el índice global (GSI, Global Symptom Index) que indica el grado de malestar general e indiscriminado de la intensidad del sufrimiento psíquico y psicosomático global. De acuerdo con Derogatis1, el índice global de severidad (GSI) es el mejor indicador de angustia y debería ser usado cada vez que se necesite un solo índice de evaluación; el segundo es el total de síntomas positivos (PST) también llamado total de respuestas positivas; por último, el tercero, es el índice de angustia de síntomas positivos (PSDI) que relaciona el sufrimiento global con el numero de síntomas, y es por lo tanto, un indicador de la intensidad sintomática medida. Las dimensiones primarias de síntomas provee un perfil de las áreas de psicopatología, y los ítems individuales dan información del nivel discreto del síntoma. El tiempo estimado para su administración es de entre 40 minutos a 55 minutos. El Inventario de Depresión de Beck-II (BDI-II)2 fue otro de los instrumentos utilizados. Este es un instrumento autoaplicado de 21 ítems usados para evaluar la severidad de los síntomas depresivos. Según Beck2, los reactivos evalúan los síntomas a nivel cognitivo, motor, afectivo y somático. Por cada ítem el participante puede seleccionar entre cuatro posibles alternativas que van de 0 a 3 según la severidad del síntoma. La puntuación total se calcula mediante la suma de todos los ítems. Según esta versión, las puntuaciones obtenidas pueden clasificarse de forma orientativa en las siguientes categorías: Estado no Depresivo de 0 a 9; depresión leve o disforia de 10 a 18; depresión moderada de 19 a 29; y depresión severa de 30 a 63. El tiempo estimado para su administración es aproximadamente de 10 minutos. El Inventario de Ansiedad de Beck (BAI)5 también fue utilizado. Este evalúa la severidad de ansiedad en los pacientes. Esta consiste en 21 ítems que describe los estados de ansiedad en una escala de 0 a 3, donde el 0 es ausencia de síntoma y el 3 es un nivel severo. El tiempo aproximado de administración es de 20 minutos. La puntuación fluctúa entre; 0 a 7 lo cual se considera un estado mínimo de ansiedad; de 8 a 15 el cual indica un estado leve de ansiedad; las puntuaciones de 16 a 25 reflejan un estado moderado de ansiedad y puntuaciones de 26 a 63 indican un severo estado de ansiedad. El Inventario de Desesperanza de Beck (BHS)4 consta de 24 ítems que miden actitudes negativas relacionadas al futuro. Cada ítem es contestado con un “cierto” o “falso”. La suma total consta de un intervalo de 0 a 20 que indica el grado de desesperanza. El tiempo estimado de la administración oral es de 5 a 10 minutos. La escala de suicidio de Beck (BSS)3, evalúa la severidad de ideación suicida. Esta consiste en 21 ítems. Los primeros 19 miden actitudes, disponibilidad y plan suicida. Los otros ítems miden las ideaciones entre vida y la muerte. El tiempo estimado de administración oral es de 5 a 10 minutos. El mini-examen del estado mental de Folstein (MMSE)6 mide la severidad de incapacidad cognitiva. Esta consta de 11 ítems, tres de ellos con una puntuación máxima de 5, tres con una puntuación máxima de 3, uno con una puntuación máxima de 2 y cuatro con una puntuación máxima de 1 punto. La puntuación total máxima es de 30 puntos; de 21 en adelante sugiere una severidad de incapacidad cognitiva leve; de 10 a 20 puntos sugiere la severidad de capacidad cognitiva moderado; y de 0 a 9 una incapacidad cognitiva severa. 9 RESULTADOS A continuación los resultados por instrumento administrado. Para las puntuaciones del GSI, que indica el grado de malestar general e indiscriminado de la intensidad del sufrimiento psíquico y psicosomático global, sólo hubo 4 puntuaciones significativas. Los participantes con mayores patologías lo son el 9 y el 12, ambas mujeres. Los otros 8 participantes no presentaron baja intensidad de sufrimiento psicológico y emocional. Es decir, no hubo indicadores significativos de somatización, obsesión- compulsión, sensibilidad interpersonal, depresión, ansiedad, hostilidad, ansiedad fóbica, ideación paranoide y psicotismo en estos participantes. Cabe señalar que este cuestionario no tiene normas para la población de ancianos centenarios. La puntuación de mayor significancia en el SCL-90-R1 lo es el GSI, ya que indica el grado general de malestar de la intensidad de sufrimiento psíquico y psicosomático. Al observar dichas puntuaciones encontramos solo 4 puntuaciones significativas, aunque según la norma comparativa de la escala están dentro de los parámetros de normales-altos. En las puntuaciones de la Tabla II se nos da una idea por cada área medida en dicha prueba. En las áreas emocionales y psicológicas observamos puntuaciones significativas en las siguientes áreas: depresión, ansiedad y fobia. Al comparar las puntuaciones entre géneros en el GSI encontramos que las mujeres obtuvieron las puntuaciones más altas, aunque en el parámetro normal. En las otras áreas, vemos el mismo patrón de puntuaciones altas por parte de las centenarias. Tabla II: Puntuaciones obtenidas del Symptom Checklist 90-R (SCL-90-R)1 Participante I II III IV V VI VII VIII IX X XI GeneroGSI F 1.58 M 1.49 M 0.59 F 0.71 M 0.89 M 0.67 M 1.00 F 1.84 M 0.30 F 1.01 F 1.93 PSI 1.60 0.80 0.00 0.20 0.90 0.00 0.70 1.10 0.10 0.40 1.70 PSDI 2.79 2.10 1.71 1.50 1.78 1.73 1.67 2.02 1.50 1.78 2.14 SOM 1.75 1.83 1.00 1.00 1.42 1.50 1.25 2.25 1.08 1.33 2.50 OBS 0.70 2.00 0.90 1.12 1.00 1.10 0.44 2.10 0.60 1.60 2.00 INT 1.11 0.44 0.00 0.11 0.67 0.00 1.15 1.33 0.11 0.33 1.44 DEP 2.38 1.85 0.85 1.00 1.23 0.92 1.20 2.31 0.08 1.15 2.38 ANS 1.70 1.00 0.30 0.50 1.00 0.40 0.67 1.70 0.00 1.20 1.70 HOS 1.17 0.83 0.17 0.00 0.67 0.33 0.71 1.17 0.00 1.00 1.17 FOB 2.00 2.29 0.14 1.86 0.00 0.86 0.17 2.71 0.00 1.00 2.29 PAR 0.00 1.33 0.00 0.00 0.50 0.00 0.70 0.83 0.00 0.83 1.17 Los números en negrillas son consideradas por sobre el parámetro normal para la subescalas del SCL-90-R1 Para el BDI-II2, los resultados fueron los siguientes: dos participantes con ningún síntoma, tres con síntomas mínimos, cuatro con síntomas leves y dos con síntomas moderado. Solo dos participantes evidenciaron síntomas moderados de depresión y nueve obtuvieron ninguno o pocos síntomas (Ver Tabla III). Es necesario señalar que en este instrumento de medición existen reactivos pocos sensitivos a la población centenaria. En un análisis entre las escala BDI-II2 y la escala de depresión de la SCL-901, concluimos que la población de centenarios puertorriqueños no presentan síntomas significativos depresión. 10 Tabla III.: Puntuaciones obtenidas en el BDI-II2 Tabla V: Puntuaciones obtenidas en el BHS4 Participante Genero Participante Puntuación de BDI-II Genero Puntuación de BHS I. F 19 (moderado) I. F 10 (moderado) II. M 9 (mínimo) II. M 13 (moderado) II. M 5 (mínimo) II. M 6 (ninguno) IV. F 24 (moderado) IV. F 13 (moderado) V. M 12 (leve) V. M 4 (leve) VI. M 15 (leve) VI. M 8 (leve) VII. M 4 (mínimo) VII. M 9 (moderado) VIII. F 15 (leve) VIII. F 9 (moderado) IX. M 1 (mínimo) IX. M 10 (moderado) X. F 0 (ninguno) X. F 14 (moderado) XI. F 24 (moderado) XI. F 14 (moderado) En relación al BAI5, los resultados obtenidos los describimos en la tabla III. Al comparar las puntuaciones obtenidas en el BAI5 y la sub-escala de ansiedad del SCL90-R1 observamos puntuaciones similares, esto es, lo que se consideraría como un nivel de ansiedad moderado (Véase Tabla IV). Tabla IV: Puntuaciones obtenidas en el BAI5 Con relación al BSS3, al observar los resultados obtenidos no se halló puntuaciones significativas. Cuando comparamos estos hallazgos con la escala que mide la dimensión suicida de la SCL-90-R1, se encontró puntuaciones semejantes a la del BSS3. En ambas escalas se confirman que no existe ideación suicida. Sabemos que PR, como sociedad, se expone a una tradición religiosa fuerte que en alguna forma influye en sus vidas. La posibilidad de que la religión puede actuar como un factor preventivo para evitar el suicidio es ampliamente documentada en la literatura psico-social21. El dato de que 100% pertenece una denominación judeo-cristiana, implicando que el suicidio no es bien visto como una alternativa en estas religiones, puede ser un factor que influya en prevenir la condición, pues en términos generales, la acción es considerada como un pecado. Participante Genero Puntuación de BAI I. F 24 (moderado) II. M 29 (severo) II. M 8 (leve) IV. F 38 (severo) V. M 13 (leve) VI. M 20 (moderado) VII. M 7 (mínimo) VIII. F 15 (leve) Con relación al MMSE6, los resultados fueron: cinco obtuvieron índices de leve, cinco moderado y uno severo. Esto sugiere que diez de los participantes no padecen de problemas cognitivos ni demencias profundas. En esta área debemos comentar que uno de los elementos de exclusión para participar en la investigación lo era el no tener deficiencias cognitivas serias. IX. M 8 (leve) Interacción de las pruebas X. F 14 (leve) XI. F 32 (severo) En términos del BHS4, los resultados fueron: cuatro participantes con síntomas de desesperanza leve, y siete con síntomas moderados. Las puntuaciones de desesperanza fluctuaron entre sintomatología leve a moderada. El concepto desesperanza suele ser uno que mide la necesidad afectiva y bienestar en términos del futuro. Esta muestra tiende a tener mayores demandas sentimentales y afectivas, ya que requieren mayores cuidos y presentan una mayor dependencia para realizar las funciones diarias, cuido físico y emocional, entre otras necesidades requeridas (Véase Tabla V). Los resultados muestran puntuaciones similares en múltiples de las pruebas administradas en las patologías evaluadas. Al realizar un análisis inferencial entre la escala de depresión de la SCL-901 con la BDI2 encontramos que ambas escalas correlacionaron .80 a una significancia de p<.01. En la las escalas de ansiedad de la SCL-901 y la de BAI5 encontramos que hubo una correlación de .87 con una significancia de p<.01. CONCLUSION Según los hallazgos obtenidos podemos concluir que la muestra obtenida de centenarios puertorriqueños sufre de poca o ninguna sintomatología depresiva, aunque los índices de desesperanza son evidentes acorde con el BHS4. La posibilidad de que se requiera intervención, entiéndase la necesidad de estar acompañados por familiares o grupos de apoyo social o religioso sensibles a las realidades de los centenarios, puede servir para minimizar la sintomatología de desesperanza. Según nos demuestra el Symptom Checklist 90-R (SCL-90-R)1, es una muestra que, en su mayoría, presenta una baja intensidad de sufrimiento psicológico y emocional por lo que parece que están adaptados a sus ambientes. Es importante señalar que fueron las centenarias las que demostraron tener mayor desesperanza. Los niveles de ansiedad fueron prevalentes en esta muestra de centenarios, fluctuando nueve ancianos, entre moderado a severo. También podemos reportar que tienden a tener un estilo de personalidad poco hostil, aun cuando padecen de miedos fóbicos e irracionales, según identificados por el SCL-90-R1. El elemento cultural de superstición seria material de análisis y estudio en investigaciones futuras para conocer si las creencias que tienen influyen en el desarrollo de sus miedos y a su vez en el experimentar depresión, ansiedad y desesperanza. A tenor con esto sugerimos el que se desarrollen estudios cualitativos para explorar dichas áreas. En términos generales se concluye que nuestra muestra de centenarios puertorriqueños es una que goza de, en términos generales de una salud psicológica dentro de los parámetros de normalidad ( a excepción de lo descrito sobre sus síntomas de ansiedad y desesperanza”. Es interesante observar que a pesar de su edad, todavía se puede notar un deseo por continuar viviendo, esto a pesar de haber atravesado circunstancias históricas trascendentales como el cambio de gobierno español a uno americano, severos huracanes, terremotos, tsunamis (como el de Mayagüez), depresiones económicas, falta de educación, condiciones infrahumanas de vivienda e higiene, malnutrición y otros males sociales. Sin lugar a dudas se les deben considerar “Héroes de Nuestro Tiempo”. También cabe señalar cualitativamente, que esta muestra ejemplifica un nivel de resiliencia alto ante las adversidades y limitaciones que le imponen su avanzada edad. Deseamos reconocer que, como todo estudio, este tiene unas limitaciones metodológicas que deben de subsanarse en futuras investigaciones. La más importante es que nuestra muestra centenaria fue una por disponibilidad y que requería, para ser incluida en nuestro estudio, demostrar un funcionamiento razonablemente normal en términos físicos y cognitivos. Es posible que si se incluyera una muestra representativa de centenarios puertorriqueños, encontraríamos un perfil de mayores dificultades psicológicas y psicosociales. Otra limitación es que muchas de las escalas que utilizamos no tienen normas para una población centenaria. Aun cuando los Inventarios y Escalas de Beck (i.e., ansiedad, desesperanza, suicidio y depresión) han comenzado a validarse con población anciana puertorriqueña, su normalización no ha sido llevada a cabo para ésta población. Por lo tanto, los datos obtenidos tienen que ser ponderados dentro de esta limitación. Para finalizar es necesario puntualizar la necesidad de realizar nuevos estudios que utilicen una muestra mas extensa; además, sería de utilidad estudios que contrarresten y correlacionen variables como el sexo, zona de vivienda y otras sintomatologías psico-psiquiátricas. Deseamos enfatizar en la necesidad de auscultar, no solo patologías psico-psiquiátricas en esta población, sino también debemos auscultar elementos positivos dentro de la vertiente de una psiquiatría/psicología que sirva para identificar aquellas características que pueden actuar positivamente para lograr llegar a tan avanzada edad. REFERENCIAS 11 (1) Derogatis, L.S. (1997) Administration, Scoring and procedures Manual I for the revised version of the SCL-90. Baltimore John Hopkins University Press (2) Beck, A.T. (1967) Depression Clinical, Experimental and Theoretical Aspects. New York: Hoeber. (3) Beck, A. T. (1991) Suicide: Clinical, experimental and theorical aspects. New York: Hoeber. (4) Beck, A. T. 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San Juan: Estado Libre Asociado de Puerto Rico. (11) United States Bureau of the Census. (2007) Estimate of population of Puerto Rico by age, sexual race. Washington (DC): Government Printing Office. (12) Oficina del Gobernador para Asuntos de la Vejez (2000). Perfil demográfico de la población de edad avanzada en Puerto Rico. San Juan: Estado Libre Asociado de Puerto Rico. (13) Menchón, J., Crespo, J., & Antin, J. (2001). Depresión en Ancianos. Cursos de Formación Continuada en geriatría: disponible en http://psiquiatria. com. (14) Pérez, R. (2006). Longevidad. Reflexiones acerca del tema desde una perspectiva psicológica. Publicación de Gerontología y Geriatría, vol. 1, 3. (15) Campos, J. M. (2005). Evaluación Geriátrica Multidimensional Del Anciano en Cuidados. P&B. 9, 2: 46-58. (16) González, A.M. & Rodríguez, L. (2006). Centenarios y Discapacidad. Publicaciones de Gerontología y Geriatría, Vol. 1, 4. (17) Ravaglia, G., Forti, P., De Ronchi, Maioli, F., Nesi, B., Cucinotta, D. Bernardi, M., & Cavalli, G. (1999) Prevalence and severity of dementia among northern Italian centenaries. Neurology, 53, 416-417. (18) Gondo, Y. , Hirose, N., Arai, Y., H. Inagaki, H., Y. Masui, Y., Yamamura, K., Shimizu, K., Takayama, M., Ebihara, Y., Nakazawa, S. et al., (2006). J. Gerontol. A Biol. Sci. Med. Sci., 61, 305 - 310. (19) Rosado-Rodríguez, M., Rodríguez, J.R. & Martínez, M. (1997) Depresión en ancianos con y sin apoplejía(s) Cerebral(es) o Amputaciones que reciben tratamiento de Rehabilitación Física. Boletín Asociación Médica de P.R., 89, 8896. (20) Alvarado, A. (2000) Factores de riesgo socio demográfico relacionado al suicidio en Puerto Rico y estimación prospectiva de la prevalencia de suicidio en Puerto Rico para el 2005 (tesis doctoral). San Juan: Universidad Carlos Albizu. (21) Lugo, Y. & Rodríguez-Gómez, J.R. (1997) Estudio piloto sobre la ideación suicida en ancianos puertorriqueños en hospital de salud mental. Boletín de la Asociación Médica de Puerto Rico 89: 167-173. (22) Alsina, S. & Rodriguez-Gómez, J.R. (1995) El Suicidio en Ancianos Puertorriqueños. Revista Latinoamericana de Psicología 27: 263-282. ABSTRACT This is the first research done in Puerto Rico presenting a basic socio-demographic profile of a sample of Puerto Rican centenary elderly that also includes psychological characteristics. The sample consist of eleven subjects, divided in five women and six men (mean age sample =102.4 years) evaluated with a battery of psychological instrument that include the Symptom Checklist 90-R (SCL-90-R); the Beck Depressive Inventory (BDI-II); the Beck Suicide Scale (BSS); the Beck Hopelessness Scale (BHS); the Beck Anxiety Inventory (BAI) and the Folstein Mini-Mental. Main findings demonstrate that, with the exception of anxiety and hopelessness symptomatology, this particular sample is with a good psychological health. It is recommended to continue this type of research with a bigger sample and include other psychological and psychiatric screening instruments. 12 OBESITY PREVALENCE ODDS RATIOS IN FOUR PUERTO RICAN TOWNS: A PILOT STUDY Ada M. Laureano EdD * José Rodríguez-Gómez MD ** Rosa Janet Rodríguez PhD £ Juanita Centeno EdD MPH ¥ Judith Rodriguez, PhD § From *the Expanded Food Nutrition Education Program (EFNEP), Agricultural Sciences College, University of Puerto Rico (UPR), Mayagüez Campus, Mayagüez, Puerto Rico, ** General Social Sciences Dept, Social Sciences Faculty, UPR Río Piedras Campus, San Juan, Puerto Rico, £ School of Health Professions UPR, Medical Sciences Campus (MSC), San Juan, Puerto Rico, ¥ Allied Health Professional College, Dietetic Internship UPR, MSC, San Juan, Puerto Rico, and § Dept. Public Health, Brooks College of Health, UNF, Jacksonville, Florida. Address reprints to: Dr. Ada M. Laureano, Professor of Nutrition, University of Puerto Rico, Mayagüez Campus, Agricultural Extension Service P. O. Box 9269, Caguas, Puerto Rico, 00936. E-mail: [email protected]. ABSTRACT Purpose: Obesity is a major public health problem associated with major mortality causes. It is the second leading cause of preventable diseases in the Caribbean island of Puerto Rico (PR). The purpose of the study is to estimate the Obesity Prevalence Odds Ratios in Four Puerto Rican Towns. Obesity prevalence in PR has been estimated by the Behavioral Risk Factor Surveillance System (BRFSS) in 2005 (23%) for both sexes. The obesity estimation of the BRFSS was used in order to relate it with our sample and compare results. Method: This is a descriptive-observational cross sectional study, based on a sample of 801 subjects selected from four major Puerto Rican municipalities (Loíza, Río Grande, Luquillo and Canovanas). A valid questionnaire with main questions to obtain health/nutrition and sociodemographic information were administered. An obesity prevalence odds ratio (OPOR) was calculated. Results: The obesity prevalence of the whole sample under study was higher (mean prevalence rate 42%) than the reported prevalence rate in the 2005 BRFSS. Canóvanas have the highest obesity prevalence (49%) followed by Loíza (41 %), Rio Grande (39 %) and Luquillo (37 %). Luquillo and Canovanas are the areas with higher possibility for obesity taking into consideration waist circumference as a risk factor for obesity (OPOR= 1.49 and 1.36, respectively; 95% CI). Conclusions: The sample under study has a higher OPOR as related to the BRFSS. These individuals may have a higher risk for chronic diseases associate to obesity. Future studies needs to address lifestyle prevention modifications and socioeconomic disparities in addition to evaluate intervention(s) that modify environmental factors in order to prevent obesity. Index words: obesity, prevalence, odds, Puerto Rico, towns INTRODUCTION O besity is the result of an imbalance between consumption and the expenditure of energy 1, 2, 3. The World Health Organization defines obesity as a high proportion of body fat. Some of the risk factors for obesity are BMI ≥ 25, high fat and high carbohydrates food patterns, lowest physical activity patterns, familiar history of chronic diseases, waist circumference measure >35 for women and >40 for men and genetics 4, 5, 6. Obesity, especially long term obesity, contributes to diabetes, cardiovascular disease, some cancers, as well as other chronic diseases 5, 7. The Centers for Disease Control (CDC) recognized the epidemic of obesity as a strong risk factor for many chronic diseases. Chronic diseases—such as cardiovascular disease (primarily heart disease and stroke), cancer, and diabetes—are among the most prevalent, costly, and preventable of all health problems in the United States and Puerto Rico8, 9, 10. United States has one of the highest obesity rates in the world and is the first nation to have obese poor people11. Despite extensive efforts in the improvement of diagnosis and treatment of subjects, more than 65 percent of U.S. adults (119 millions) are overweight or obese, and nearly 31 percent (61 million) are obese 11, 12. Data from the Puerto Rico’s Health Department reveals that the five leading causes of death in Puerto Rico are heart disease, cancer, diabetes, hypertension, and chronic lower respiratory disease 7. For all these death causes, obesity represents a directly related risk. According to statistics from the Behavioral Risk Factor Surveillance System (BRFSS) for Puerto Rico in 1996, the prevalence of obesity was 16.8 percent to 22 percent and 39.2 percent of the population were overweight. These higher rates are reflected in practically all age groups of the BRFSS, with the exception of those 50 to 64 years, for whom the prevalence was over 75 percent; and persons 18 to 34 years who have a lower prevalence than the population 35 years and older. The rate of obesity is similar for both sexes in 2002, with 22% 3, 13. In 2005 the BRFSS report an obesity prevalence rate of 23% 13 Diagnosis of obesity Earlier standards methods developed for diagnose obesity involved tables of desirable weights at various heights that were derived by life insurance companies. These tables were based on illnesses and death rates 4. During the last decades a standard was developed that closely correlates with body fat and the metabolic complications of obesity named the Body Mass Index ("BMI") 1, 14. For clinical diagnosis, the body mass index measurement (BMI) is calculated, using the following mathematical formula, BMI = wt (kg)/ ht (m2), weight in kilograms divided by height in square meters 2, 4. The International Obesity Task Force establishes the following standard classification for adults: overweight refers to subjects with a BMI = 25.0 – 29.9 kg/m2; subjects with a BMI ≥ 30 kg/m2 are considered obese 15. These municipalities were selected according to Puerto Rico Health Department Vital Statistics, demonstrating that they have the third highest prevalence rate of chronic diseases on the island. They include type 2 diabetes, heart attack, stroke, cancer and hypertension, which are usually related to obesity. In addition, the area has the staff and programmatic structure to support this type of research from the Expanded Food Nutrition Education Program (EFNEP). We identified food consumption patterns and consumer practices in the whole sample (Graph 3). Prevalence odds ratio for each town sample group was calculated using SPSS-14 for Windows. Descriptive statistics, such as frequencies, percentages, means, and standard deviations was computed to get an adequate picture of the data dispersion. The BMI has been subject to fundamental criticism, as it ignores fat distribution in the body, as central obesity, and the fat/muscle proportion in total body weight. This means, for instance, that a very fit athlete with little fat but highly developed, and therefore heavy muscles, could be classified as obese. Some researchers see as a more relevant and simpler measure to measure the waistline circumference, as this can highlight the dangerous location of body fat close to vital organs 2. The sample was enrolled within the four towns under study (Canóvanas, Loíza, Luquillo and Rio Grande), for a total of 801 subjects, as shown in Graphic 1. Table 1, reflect that the subjects’ mean age was 47.62 years; they had low income, mean monthly income of $846.27, and a high school education level. Since subjects were selected by convenience, age values were atypical distributed as compare to mean age population town sample. The subjects are mainly women, 76.8 percent, as it is expected in Puerto Rican social programs participation levels, like the Expanded Food and Nutrition Education Program (EFNEP), Food Stamps Program and Women Infant Children Program (WIC). Puerto Rico’s obesity prevalence data depends on the Behavioral Risk Factor Surveillance System (BRFSS) from the CDC, a state-based telephone survey of U.S. adults conducted in all states and several territories as Puerto Rico. The BRFSS conducts a range of surveillance and epidemiologic studies related to weight control practices, micronutrients, obesity, and nutritional risk factors for chronic diseases such as cancer, heart disease, osteoporosis, and hemochromatosis. The BRFSS collect their data by subjects’ perceptions about health status. Also, it has the potential bias of the exclusion of subjects who have none home telephone service, as persons who used only cell phones and those at working place. Thus, the BRFSS just bring a general trend and do not describe the real obesity problem in Puerto Rico, this type of studies have not been done yet in Puerto Rico, The purpose of this study was to determined the obesity prevalence odds ratio (POR) among four major municipalities of Puerto Rico (Loíza, Rio Grande, Luquillo and Canovanas) and use the BRFSS reported obesity rate to related it with the POR obtained at the municipalities. METHOD A descriptive cross-sectional study using quantitative methodology to collect anthropometric measurements and nutritional information was performed. Participants were a sample of volunteers’ adult, ages 18 to 74, from each of the four municipalities under study. The total sample was 801 persons selected from October to December 2006. To reach the people with main disparities and to address them more efficiently and facilitate future follow-up and intervention, we chose to contact the subjects using Puerto Rico government social services programs such as: Expanded Food Nutrition Educative Program (EFNEP), Food Stamps Program and the Women Infant Children Program (WIC). The towns selected for the study were in the eastern Puerto Rico area (Canovanas, Loíza, Luquillo and Río Grande). RESULTS Table 2 shows in the last column the expected nutritional consumption for each food group. For this analysis it is considered the Food Frequency Questionnaire (FFQ), identified from the extensive questionnaire a standard food for each group as for instance; rice for grains (culturally related to Puerto Ricans and a best choice mix consume with legumes as meat substitute); broccoli for vegetables and legumes (as an important complex B source and as phitochemical heart disease prevention source); orange juice for fruit group (due to their easy availability and as an important prevention factor on cancer and heart diseases due to their vitamin C concentration); olive oil for fat (an excellent monosaturated source); 2% low fat milk, for milk group, due to a fat reduction nutrient; and fish for meat group because we want to enforce their consumption. Although, the subjects consumed these food groups, the percentage of adequacy ingestion (according to RDA standards) are low for all of them. Graphic 3 present data of the 24 hours recall, where we found a low consumption for each previously mentioned nutritional group, except for the fat group. The excess consumption from vegetables and fruit groups did not attempt to the validity of this research since they had fewer calories and provided a higher nutritional density as compared with other Food Guide Groups Pyramid. Graphic 4 allows to identify the principal co-morbidities diagnoses in these subjects such as: Diabetes mellitus Type 2 (22%); 38% had Hypertension, 24% High Cholesterol; 13% High Triglycerides; 12% Cardiovascular Diseases and 3% Cancer. Only 6% of the sample was identified as healthy. A logistic regression model was performed emerging 4 14 Graphic 1: Subjects Distribution by Town 210 200 201 25.2% 201 25.1% 195 190 191 23.8% 185 180 Town Loiza Canovanas Río Grande Luquillo 050 040 030 020 Gende r Mal e Graphic 3: Twenty four (24) Hours Food Groups Consumption 7 0 Dm T2 HBP HChol HTg CD CA None Health Condition Legend: Legend: Dm T2=Diabetes mellitus HTg=High Triglycerides HBP=Hypertension CD=Cardiovascular Disease Hchol=High Cholesterol CA=Cancer Town Subjects N Educational Level % E JH H U Canóvanas 191 4.7 11 41.4 42.9 Age Monthly Mean Income Mean 45.9 $937.53 Loíza 201 8 12.5 59.2 20.4 45.1 $538.54 Río Grande 208 13.9 11 44.2 24 51.5 $836.08 Luquillo 201 5 9.5 36.2 48.1 47.8 $1,082.26 Total 801 7.9 11 45.3 33.9 47.6 $846.27 Adequate Consumption (%) * 15.8 11.8 36.9 14.7 38.2 11.9 *Adequate consumption should be 100% consumption for each group. ** Food used as representative of the entire group. Table 3: Obesity Prevalence Comparison with the 2005 BRFSS 6 5 4 3 2 1 0 51 05 Pyramid Subject’s Food Consumption Groups (n) Grains (rice**) 793 Vegetables & Legumes 400 (broccoli**) Fruits (orange juice**) 48 Fat (olive oil**) 231 Milk & Substitutes 368 (2% fat**) Meat & Substitutes 578 (fish**) 70 060 Femal e 52 01 Table 2: Weekly FFQ Food Groups Consumption Graphic 2. Gender Distribution 010 0 0 4 03 53 02 Table 1: Socio-demographic subjects’ distribution by town 208 25.9% 205 Graphic 4: Self reported health history Percentage of participants predictive variables for obesity in both sexes as shown in Table 4. These variables are the followings: health history (i.e., Type 2 diabetes, hypertension, high cholesterol, and high triglycerides), income, foods stamps participation and food consumption (i.e., fruits and vegetables consumption) was identify as risk factors. In order to predict obesity in men we needed to take into consideration in the equation three main variables: weight, health history and waist circumference. For women, although food stamps recipient and diet (i.e., fruits and vegetables consumption) variables do not obtain statistical significance, if we eliminate these confounding variables, the logistic regression model became not so efficient/significant do perform at an adequate statistical level the required predictions. Grains Vegetables Fruits Fat Food Groups Pyramid Consumed Milk Meat Recommended Towns Canóvanas Loíza Río Grande Luquillo All Study Prevalence 49 41 39 37 42 Puerto Rico’s BRFSS Prevalence 23.7 23.7 23.7 23.7 23.7 Data gathered was used to calculate the obesity prevalence odds ratio for each town and compared it with the 2005 unpublished obesity prevalence rate of the Behavioral Risk Factor Surveillance System which is presented in table 3. The obesity prevalence rate was calculated with contingency tables. Canóvanas had the highest obesity prevalence rate with 49%; followed by Loíza with 41 %; next was Rio Grande with 39 %; and the lowest was Luquillo with 37 %. From this analysis it is possible to infer that the obesity prevalence rate was higher with a mean prevalence rate of 42%, as compare to the reported prevalence rate of 23% in the BRFSS 2005. Table 5 presented confidence intervals that were more than 1, meaning that weight is a risk factor for the four towns. This was an important finding, and support the idea that BMI when used to establish the obesity prevalence, as done by the BRFSS, may act as a confounding variable, creating serious confusion when includes it in the analysis. Since the BMI is a significant variable that is part of the standards considered for diagnoses we need to be cautious when interpreting such findings. 15 Table 5: Prevalence Odds Ratios (POR) and 95% Confidence Intervals (CI) for Obesity with Weight as a Risk Factor Using a Simple Logistic Regression Model Towns N Loíza 171 Río Grande 185 1.09 (1.05 – 1.13) < 0.05 Canóvanas 158 1.09 (1.04 – 1.13) < 0.05 Luquillo 181 1.07 (1.05 – 1.11) < 0.05 All 695 1.19 (1.09 – 1.29) < 0.05 Table 4: Sample subjects’ profile Average Food Stamps Enrollment 32% Mean High Triglyceride 28% Mean High Cholesterol Prevalence 48% Mean Hypertension prevalence 37% Mean Diabetes Mellitus Prevalence 24% Fruits Consumption 37% Vegetables Consumption 12% Mean BMI 30 Mean Waist circumference (inches) 38 Mean Height (inches) 63 Mean Body Weight (pounds) 173 Mean Age (years) 48 p-value 1.12 (1.08 – 1.17) < 0.05 Table 6: Prevalence Odds Ratios (POR) and 95% Confidence Intervals (CI) for Obesity with Waist Circumference as a Risk Factor Using a Simple Logistic Regression Model Towns Luquillo Table 6 suggests that waist circumference was the Canóvanas most significant obesity risk factor in this study. Never- Loíza theless, the variable was not significant for Loíza and Rio Río Grande Grande towns. When we ran the logistic regression model, it is adequate to predict obesity for men but not for women. We hypothesized that this happen due to the effect of some unknown’s variable(s) at this research. These variables could be related to gender, as for instance; hormones, hip size proportion related to waistline, parity and children number, and breastfeeding. These findings promote future research initiatives that address gender disparities. POR (95% CI) All N POR (95% CI) p-value 181 158 171 185 1.49 (1.25 – 1.77) 1.36 (1.08 – 1.73) 0.95 (.82 – 1.11) 1.16 (.99 – 1.35) < 0.05 < 0.05 < 0.05 < 0.05 695 RESUMEN Propósito: La obesidad se considera un problema serio de salud pública asociado a las causas mas serias de mortalidad en Puerto Rico. Este estudio pretende estimar el índices de razón de los productos cruzados (odds ratios-OR) en términos de la prevalencia de obesidad en una muestra de cuatro municipios de Puerto Rico y compararlos con el estimando de prevalencia de obesidad realizado en el “Behavioral Risk Factor Surveillance System (BRFSS) del 2005 el cual fue de 23% para ambos sexos. Método: Este es un estudio descriptivo transversal basado en 801 sujetos seleccionados por disponibilidad de 4 municipios de Puerto Rico ( Loíza, Rio Grande, Luquillo y Canóvanas) durante un periodo de 3 meses al cual se les administro un cuestionario validado para obtener información sociodemográfica y nutricional. Resultados: El índice promedio de la prevalencia de obesidad fue de 42% mucho mayor que el índice promedio reportado en el BRFSS (23%). Los municipios de Canóvanas, Loíza, Río Grande y Luquillo reportaron un índice de prevalencia de obesidad de 49%, 41% 39% y 37% respectivamente. Luquillo y Canóvanas reportan los índices mas altos de prevalencia de obesidad (OPOR=1.49 y 1.36 respectivamente; 95% CI). Conclusiones: La muestra bajo estudio tiene una prevalencia de obesidad mayor que la reportada en el BRFSS. Lo anterior debe preocupar puesto que dichos individuos pueden estar a mayor riesgo de patologías asociadas a la obesidad. Se sugieren futuros estudios para conocer el impacto que puedan tener intervenciones preventivas en la modificación de factores de riesgo para prevenir la obesidad. ABSTRACT Asthma morbidity and mortality have increased in the past two decades; Puerto Rican children have the highest prevalence of asthma in the United States. An asthma admission to PICU is a marker of asthma severity. This study describes the profile of the pediatric population admitted with status asthmaticus during a three year period to PICU at Hospital Episcopal San Lucas. An ambispective cohort chart review of 46 cases was performed; there was a mean average age of 6.2 years, male predominance (70%) and a strong family and personal history of asthma and allergies. 48% were classified as persistent asthmatics, only 19% of these received preventive mediation regimens. 72% of patients were covered under Puerto Rico’s Health Care Reform and 28% had private insurance. Of the patients covered by Puerto Rico’s Health Care Reform, classified as persistent asthmatics, 79% did not receive preventive treatment medication compared to 46% in private insurance. Background asthma management remains suboptimal in children needing hospitalization. Lack of preventive medication appears to be related to the type of health insurance. Index words: epidemiology, astma, pediatrics, status, asthmaticus EPIDEMIOLOGICAL CHARACTERIZATION OF PEDIATRIC PATIENTS WITH STATUS ASTHMATICUS ADMITTED TO INTENSIVE CARE UNIT 17 Mariel Silva MD * Luis Munoz MD * Tania Diaz MD * Weldon Mauney MD * Vylma Velazquez MD ** From the * Pediatric Residency Program, Hospital Episcopal San Lucas and Ponce School of Medicine Consortium, Ponce, Puerto Rico, and ** Allergist-Immunologist Department, Program Director of Pediatric Department Hospital Episcopal San Lucas, Ponce Puerto Rico. Address reprints requests to: Vylma Velázquez MD – Director Pediatric Department and Residency Program, San Lucas Episcopal Hospital, Suite #807, Avenida Tito Castro 909, Ponce Puerto Rico 00716-4725. Email <vvalmodovar@ yahoo.com>. INTRODUCTION A sthma is a significant public health problem in the pediatric population. The burden of asthma on the pediatric population as measured by asthma prevalence, ambulatory visits, and mortality increased dramatically during the past 2 decades (1). The childhood asthma hospitalization rate increased more slowly but was still rising during a period when pediatric hospitalization rates for other causes were declining (1). The factors behind the increasing asthma burden remain unclear. Several studies have shown that Puerto Rican children and adults bear an increased burden of the disease (2-3). According to Lara et al(2), Puerto Rican children had a higher lifetime prevalence of asthma (25.8%) than did white (12.7%), black (15.8%), Mexican (10.1%), Cuban (14.9%), and Dominican (14.9%) children living in the mainland United States. In addition, mainland Puerto Rican children had increased odds of an asthma attack in the past 12 months. Another aspect of asthma management is the availability of care for these patients. Risk factors for increased morbidity and mortality in this population include number of hospital admissions, prior ER visits, history of PICU admissions and prior intubation history as well as derangements in care from the established guidelines.(1). Our study objective was to identify the characteristics of children admitted to our institution’s PICU with diagnosis of status asthmaticus, determine the pediatric population that is at risk for severe asthma attacks and evaluate the relationship between type of health insurance and adherence to established asthma management guidelines. METHOD This is an ambispective cohort study. Charts were reviewed for patients admitted to PICU at Hospital Episcopal San Lucas in Ponce, Puerto Rico during January 2003 to December 2005. The study was approved by the Board Review Board of Ponce School of Medicine and Hospital Episcopal San Lucas. A total of 46 charts were reviewed. The evaluated parameters were age, sex, past medical history, family history of asthma or allergies, medications utilized including preventive therapy, seasonal variability, length of stay in hospital, radiologic findings and type of insurance coverage. Bivariate statistical analyses were performed utilizing chisquare, Fisher’s exact test, Odds ratio, confidence interval 95% (IC), T Student and Mann-Whitney test. 18 Male 6 yr (SD=4.2) Female 6.6 yr (SD=4.7) Total 6.2 yr (SD=4.3) 30% 70% Figure 1: Demographic distribution RESULTS A total of 46 cases were reviewed. 70% of cases were males with an average age of 6 years (SD4.2). Although there were fewer females in the study, the average female age was 6.6 years (SD4.7). (Fig. 1) Most of the patients reported a personal history of asthma (94%) and allergies (40%). Approximately two thirds had a family history of asthma and almost one third had a family history of allergies (Fig 2). 70% of patients had visited the ER on previous occasions and 30% had a prior PICU admissions. 24% of cases had a history of both (Fig 3). 100 90 94% 74% 80 70 60 50 40% 40 30 15% 20 10 0 Asthma Allergies FHx Asthma FHx Allergies Figure 2: Past and Familial History of Asthma and Allergies 70 70% 60 Male Female The established guidelines for classification of asthma were reviewed and patients’ records evaluated for availability of physician classification. 41% of patients admitted to our PICU did not have a physician classification for their asthma. 11% were classified as having intermittent asthma, 13% as mild persistent asthma, and 17.5% were classified as having moderate persistent and severe asthma each (Figure 5). 70% of the cases, as a whole, did not have any type of preventive medication regimen (Data not shown). According to established guidelines, 48% of the classified cases should have been on a preventive medication, however only 19% had documented preventive medication regimens. In the 41% of cases that did not have any documented physician classification, only 4 patients had a documented preventive medication regimen (Figure 6). 50 45 40 35 30 25 20 15 10 5 0 41% Intermittent 17.5% 13% 11% Mild Per 17.5% Moderate Per Sev ere Non Class. Figure 5: Asthma Classification 50 40 30% 30 24% Preventive Non-preventive 20 10 0 ER PICU Both Figure 3: Previous Hospital Visits A seasonal pattern of admissions was noted. There was a steady rise in cases from March to July, followed by a moderate decline in cases until September, and highest frequency from October until December. The months with least amount of cases were January through March. (Fig 4) 16 14 12 16 14 12 10 8 6 4 2 0 15 5 4 1 5 3 1 5 3 Inter Mild PerMod Per Severe 4 Noclass. Figure 6: Prevention and Asthma Classification 10 8 6 4 2 0 Jan feb m ar april m ay june july aug sep oct nov dec Figure 4: Seasonality Variability The patients treatment at home, prior to hospitalization was reviewed. The vast majority (89%) had used albuterol nebulizer therapy for their symptoms; other medications used were oral steroids (10%), inhaled steroids (11%), antitussive medications (6%) and antibiotics (4%) (Figure 7). 19 cost effectiveness of preventive medicine use versus hospital care. However this is beyond the scope of this study. 89% 90 80 70 60 50 40 30 20 6% 10 0 Albuterol Antitus 10% 11% 4% Steroids ICS ATB Figure 7: At Home Treatment Medical insurance provider was recorded for every case and divided into two categories, either private insurance or Puerto Rico’s Health Insurance Reform (“La Reforma”). 72% of cases were covered by Puerto Rico’s Health Reform and 28% had private insurance (data not shown). When comparing health care coverage and preventive medication regimens, 79% of patients covered by Puerto Rico’s Health Reform who required preventive medication, according to the established guidelines, did not receive preventive treatment, compared to 46% of patients of privately insured patients (Figure 8). 100 80 79% 40 54% 20 0 No prevention Prevention 21% Reforma To our knowledge this is the only study in Puerto Rico in which characteristics of patients admitted to PICU for status asthmaticus were measured as well as treatment modalities, in particular preventive medication regimens, and compared these with different types of health care providers. CONCLUSIONS 46% 60 There are some limitations to our findings. It could be argued that data was collected at a teaching hospital and a referral center for patients from parts of central and southern Puerto Rico and hence we are prone to receiving sicker patients who are medically indigent as compared to other private hospitals, therefore skewing the sample towards patients covered under the state health reform. It would be interesting to compare PICU admissions at other non teaching private hospitals in the area and look at their patients’ characteristics. Another limitation could be that clinical outcomes of patients in this particular study are not well defined. Although length of stay in the hospital and radiological findings were studied, confounding factors such as co-morbid conditions were not recorded and morbidity and mortality outcomes where not measured. I t would also be interesting to be able to follow these patients and compare at home treatment pre and post PICU admission. Private Figure 8: Prevention Regimen and Medical Insurance Provider DISCUSSION Two very important findings emerge from our study. One is that the great majority of children who are classified in the persistent asthma categories are not receiving the adequate preventive treatment as recommended in the published guidelines and despite these guidelines almost half the patients admitted to our PICU did not have an established classification of their illness. Since derangement of care from the established guidelines is a risk factor for increased morbidity and mortality (1) perhaps a better continued education of primary care physicians concerning these guidelines should be encouraged. The second is the fact that most of the children needing preventive medications are covered by the medical insurance provided by the state; Puerto Rico’s Health Reform (“La Reforma”). Pediatricians may be burden with the issues of health care reform including low reimbursement, and lack of medications coverage. While it has been proven that controlling asthma symptoms adequately reduces recurrent attacks, frequent visits to the emergency department and leads to fewer hospitalizations, pediatricians care in the health care system may be affected with financial burden of asthma treatment. Further studies should be done to analyze In our study, 46 patients were admitted with diagnosis of status asthmaticus from January 2003 to December 2005 to the pediatric intensive care unit (PICU). All charts were reviewed in order to determine the characteristics of the population admitted. Sex, age, family history and seasonal variability results are in accordance with other studies published. The fact that only 19% of patients, who required preventive medication treatment regimens in accordance with the established guidelines, received it was surprising. These were patients that were appropriately classified by their physicians and yet did not receive the standard of care management for their disease. It is perhaps more alarming when comparing the treatment received with the provider of medical insurance. 72% of our patients were part of Puerto Rico’s health reform plan and 79% of those required preventive treatment regimens and did not receive it, compared to a total of 28% of patients with private insurance who more than half received preventive therapy. Therefore lack of preventive therapy appears to be associated to the type of health insurance carriers. REFERENCES 1. Akinbami LJ, Shoendorf KC, (2002) Trends in childhood asthma: prevalence, healthcare, utilization and mortality. Pedaiatrics 110, 315-22. 2. Lara, M, Akinbami, L, Flores, G, et al (2006) Heterogeneity of childhood asthma among Hispanic children: Puerto Rican children bear a disproportionate burden. Pediatrics 117,43-53. 3. Robyn T Cohen, Canino G. et al (2007), Area of Residence, Birthplace and Asthma in Puerto Rican Children; Chest, 131:1331-1338. 20 RESUMEN La morbilidad y mortalidad del asma ha aumentado en las pasadas décadas. Los niños puertorriqueños tienen el índice de prevalencia de asma más alto en la nación norteamericana .La admisión por asma a la unidad de intensivo pediátrico es de por si un marcador de severidad de la condición. Este estudio describe el perfil de la población pediátrica admitida con estatus asmático al intensivo pediátrico durante un periodo de tres años al Hospital Episcopal San Lucas de Ponce Puerto Rico. Es un estudio de cohorte de revisión de expedientes donde se evaluaron 46 casos, cuya edad promedio fue de 6.2 años, el género masculino predominó (70%) y existía un fuerte historial familiar y personal de asma y alergias. 48% de la población fueron clasificados como asmáticos persistentes y solo un 19% recibieron medicamentos de prevención para su condición. 72% de muestra población tenían cubierta de salud de Reforma y 28% tenían plan privado de salud. De los pacientes asmáticos persistentes con reforma el 79% no recibió tratamiento de prevención solo el 46% de los de plan privado de salud lo recibió. El tratamiento de asma permanece sub-óptimo en los niños de nuestra población que necesitan hospitalización. La falta de medicamentos preventivos aparenta estar relacionada al tipo de cubierta médica de salud. ABSTRACT Several studies have been done trying to describe the clinical manifestation, histopathological appearance, and natural history of eosinophilic esophagitis, but no studies have been done in the pediatric population of Puerto Rico. Our objective is to describe the demographic characteristics, clinical presentation, and the atopic characteristics of pediatric patients diagnosed with eosinophilic esophagitis. We conducted a cross sectional study in which the 24 medical records of the patients with eosinophilic esophagitis were revised and the following data was collected: age, sex, hometown and presenting symptoms. In a 24 pediatric patients with EE, the female sex was prevalent with a 54.2% and the median age was seven years old. The most common presenting symptom was epigastric pain. Allergies to environmental allergens were more prevalent in pediatric patients with EE. In conclusion, our sample proves that EE is indeed present among the pediatric population in Puerto Rico. Index words: eosinophilic, esophagitis, allergies, pediatric, Puerto Rico EOSINOPHILIC ESOPHAGITIS AND ALLERGIES IN PEDIATRIC POPULATION OF PUERTO RICO 21 Vylma Velazquez MD * Carlos Camacho MD ** Alfredo E. Mercado-Quiñones MD ¥ Jadira Irizarry-Padilla MD ¥ From the *Allergist and Immunologist and Director of the Pediatric Department, Hospital Episcopal San Lucas, Ponce Puerto Rico, ** Pediatric Gastroenterologist, Hospital Episcopal San Lucas, Ponce Puerto Rico, and ¥ Third year Pediatric Resident, Pediatric Residency Hospital Episcopal San Lucas/ Ponce School of Medicine Program Address reprints requests to: Carlos A, Camacho MD /Vylma Velázquez MD FAAP Director Department of Pediatrics/Associated Director Pediatric Residency Program, Hospital Episcopal San Lucas Suite #807, Avenida Tito Castro 909, Ponce Puerto Rico 007164725. [email protected]. INTRODUCTION D uring the past years eosinophilic esophagitis (EE) has been recognized as a separate disease. Several studies have been done trying to describe the clinical manifestation, histopathological appearance, and natural history of the condition, but no studies have been done in the pediatric population of Puerto Rico. Eosinophilic esophagitis (EE) is a chronic inflammatory disease characterized by an isolated, severe eosinophilic infiltration of the esophagus manifested by gastroesophageal reflux like symptoms unresponsive to acid suppression therapy. In normal, healthy individuals, eosinophils are commonly visualized in almost all portions of the gastrointestinal tract except the esophagus. Initially, 2-4 eosinophils per high power field (hpf) in the esophageal mucosa were thought to be highly suggestive of acid exposure of the esophageal mucosa. EE became a very different entity after reports of several patients with eosinophilic inflammation of the esophagus that failed anti-reflux therapies. Markowitz and Liacouras found that 8% to 10% of their pediatric patients with GERD-like symptoms who were unresponsive to acid blockade actually had EE. The etiology of EE is still unknown, but the improvement of clinical symptoms and histology with elimination of the offending food allergen or with corticosteroid treatment support that EE represents an allergic process. In addition, approximately 50% of affected children also exhibit other allergic signs and symptoms including bronchospasm, allergic rhinitis and eczema, and frequently, there is a strong family history of food allergies or other allergic disorders. Mishra et al have proposed a role of aero-allergens in the pathogenesis of EE based on rodent studies after repeated aeroallergen challenges, or epicutaneous sensitization followed by aeroallergen challenge, induced EE. Eosinophilic esophagitis is a clinicopathologic diagnosis; patient symptomatology and clinical presentations are not sufficient to diagnose EE. Although, significant variability in diagnostic criteria for eosinophilic esophagitis exists, most of the studies report that more than 15 eosinophils per hpf in esophageal biopsy are required to make the diagnosis of EE. It is best defined as the presence of more than 15 eosinophils per HPF isolated strictly to the esophageal mucosa associated with typical clinical symptoms of vomiting, heartburn, feeding difficulties, and dysphagia unresponsive to acid suppression therapy. Eosinophilic esophagitis may be under diagnosed because of the similarity of symptoms between EE and GERD. Objectives Our objectives were to identify the existence of eosinophilic esophagitis in the pediatric population of Puerto Rico and to describe the demographic characteristics, clinical presentation and atopic characteristics of pediatric patients diagnosed with eosinophilic esophagitis 22 Study Population Our population consisted of 24 patients between the ages of 1 month to 18 years with gastrointestinal symptoms who had esophagogastroduodenoscopy with the diagnosis of eosinophilic esophagitis confirmed by histopathology of esophageal biopsies. These patients were diagnosed and followed by the pediatric gastroenterologist and pediatric allergist at their private office. We conducted a cross sectional study in which the 24 medical records of the patients with eosinophilic esophagitis were revised and the following data was collected: age, sex, hometown and presenting symptoms. Atopic evaluation was done using the patient history of allergies, skin prick test and immunoassays test for food and environmental allergens. RESULTS In a 24 pediatric patients with EE, the female sex was prevalent with a 54.2% versus male sex with 45.8%. The median age of the sample was seven years old. The largest group age was the group age of 5 to 10 years old with 46% of the pediatric patients with EE and it median age was seven years old. The most common presenting symptoms of these patients with EE were epigastric pain with 42%, followed by abdominal pain not specified with 33% and GER with 21%. In the group age of 0 to 4 years old, the most common presenting symptom was vomiting, whereas in the other group ages the most common symptom was epigastric pain. When evaluating the allergies in the 24 pediatric patients with EE, 83% presented at least one allergy. Thirtyfive percent (35%) of the allergies were to environmental allergens being the most common dust mite allergy (33%). Food allergies were present in 15% of the patients; allergies to egg, wheat and soybean (12% each one) were the most prevalent. 42 40 33 Percentage (%) 35 30 25 21 16 20 15 8 10 4 5 0 Ep ig a stri c p ai n Vo m it i n g G ER Na us e a Ab d o mi n a l p a i n 33 30 Percentage (%) 15 12 12 12 10 8 4 5 4 4 corn oat 0 milk egg wheat rice soybean apple 25 20 20 15 12 8 10 8 8 8 5 0 dust mite dust mosquitos fire ants roach dog/cat pediatric population in Puerto Rico. Most of these patients are female, as compared with the male predominance in the medical literature. When comparing our results to the pervious reported data, EE was present more often in the group age of 5 – 10 years, the most common presenting symptom was epigastric pain and most of the patients had at least one allergy. Also consistent with the national experience, environmental allergies were the most prevalent among patients; 33% of the patients were allergic to dust mite allergens. Although food allergens are the proposed offending agents involved in the pathogenesis of EE, only 20% of the pediatric patients with EE in our study presented any food allergy. Our main recommendation is that physicians evaluating pediatric patients with chronic gastrointestinal symptoms should consider the diagnosis of EE, particularly in patients with atopy. Allergists, gastroenterologists, pathologists, and pediatricians must not only be educated to be able to properly identify patients with EE but also be informed about the current treatment and management of these patients. Because of the variability in diagnosis and treatment options, future research should focus on optimizing the diagnostic approach and treatment options of EE. REFERENCES 1. A. Assa’ad, P. Putnam et al . Pediatric Patients with Eosinophilic Esophagitis: An 8-year Follow Up. Journal of Allergy and Clinical Immunology, 2007, 119: 731-738 2. Bax KC, Gupta SK. Allergic eosinophilic esophagitis. Indian Journal of Pediatric 2006;73:919-25 3. C. Blanchard, N. Wang, M. Rothenberg. Eosinophilic Esophagitis: Pathogenesis, genetics, and Therapy. Journal of Allergy and Clinical Immunology, 2006; 118: 1054-1059 4. Mishra A, Hogan SP, Brandt EB, Rothenberg ME. An etiological role for aeroallergens and eosinophils in experimental esophagitis. J Clin Invest 2001; 107 : 83-90 5. Liacouras, C. Eosinophilic Esophagitis in Children and Adults. Journal Pediatric Gastroenterology and Nutrition, 2003; 37: S23-S28 6. Liacouras, C. Eosinophilic Gastrointestinal Disorders. Practical Gastroenterology, 2007; 48: 53-64 FTT Figure 1. Percentage of presenting symptoms of patients with EE. 35 20 Figure 3. Percentage of food allergies in patients with EE. METHOD 45 Percentage (%) 20 grass Figure 2. Percentage of environmental allergies in patients with EE DISCUSSION Eosinophilic esophagitis is being diagnosed in both children and adults with increasing frequency in USA and our sample proves that EE is indeed present among the RESUMEN Se ha realizados varios estudios tratando de describir las manifestaciones clínicas, apariencia histopatológicas e historial natural de la Esofagitis Eosinifilica (EE) en pediatría, pero ninguno había sido realizado en la población pediátrica puertorriqueña. El objetivo de nuestro estudio fue describir las características demográficas, manifestaciones clínicas, y las características atópicas de pacientes diagnosticados con EE. Realizamos un estudio transversal en el cual participaron 24 pacientes con EE diagnosticada por endoscopia. En nuestra población 54% fueron niñas y la edad media fue de 7 años de edad. El síntoma más común de presentación fue el dolor abdominal. Se realizaron apruebas de alergias ambientales y de comida donde fueron mas frecuentes las reacciones a alérgenos ambientales. En conclusión presentamos el primer estudio de EE en una población pediátrica puertorriqueña con EE y alergias. 23 From the * VA Caribbean Healthcare System and ** University of Puerto Rico Comprehensive Cancer Center. Address reprints requests to: Doris H. Toro, MD, FACP, AGAF, VA Caribbean Healthcare System Medical Service, Casia Street #10 San Juan Puerto Rico, 00921-3201. Email: [email protected] ABSTRACT Background: This study aimed to assess the diagnostic accuracy of double contrast barium enema (DCBE) compared to colonoscopy among Hispanic patients with positive fecal occult blood testing (FOBT). Secondary objectives were to determine: the diagnostic accuracy according to adenoma location, size, and pathologic characteristics; and patient satisfaction with each procedure. Methods: Cross-sectional study comparing the accuracy of DCBE and colonoscopy in detecting adenomatous polyps and/or colorectal cancer in patients with positive FOBT. DCBE and a colonoscopy were performed in all patients. Polyps identified during colonoscopy were removed and classified by histology. Tolerability and patient’s satisfaction with study procedures was assessed. Results: Fifty patients were enrolled, mainly men with a mean age of 63 years old. Polyps were found in 40/50 (80%) patients in colonoscopy, compared to 19/50 (38%) in DCBE. Eighty-four percent of polyps were missed by DCBE. Sensitivity of DCBE was 45% and specificity 90% for all adenomas. Diagnostic accuracy of DCBE was 54% for any size adenomas, and 72% for ≥10 mm adenomas. Sensitivity increased from right to left colon while specificity decreased. Patients preferred colonoscopy over DCBE. Conclusions: This study supports the use of colonoscopy as the gold standard test for the evaluation of positive FOBT and was preferred over DCBE by the patients. Diagnostic accuracy of DCBE was inferior to colonoscopy, for all size polyps and large adenomas. Compared to colonoscopy, DCBE is a substandard test for colorectal cancer screening and may result in ineffective outcomes. Index words: Colon polyps, Fecal occult blood test, Double contrast barium enema IS BARIUM ENEMA AN ADEQUATE DIAGNOSTIC TEST FOR THE EVALUATION OF PATIENTS WITH POSITIVE FECAL OCCULT BLOOD? Carlos Ramos MD* Joel De Jesús-Caraballo MD* Doris H. Toro MD*, Algia Ojeda MD* Jaime Martínez-Souss MD* Maria Isabel Dueño MD* Marcia Cruz-Correa MD ** INTRODUCTION C olorectal cancer is the second most common newly diagnosed cancer in Hispanics, and currently constitutes 11% of new cancer cases in men and 9% in women [1]. It constitutes the second leading cause of cancer death in men and the third in females [1]. Overall rates of cancer incidence vary considerably among racial and ethnic groups [2]. Among Puerto Rican Hispanics, colorectal cancer is the second most common cause of cancer in both men and women accounting for 14% of all cancers in the island, and is the second leading cause of death among women (Puerto Rico Central Cancer Registry, 2004). Despite evidence that 5-year survival is 90% when colorectal cancer is diagnosed at an early stage, less than 40% of cases are diagnosed when the cancer is still localized [3]. The importance of screening for colorectal cancer has been established, but the decision about which test to use for each patient may seem less clear. Randomized controlled trials using fecal occult blood testing (FOBT) as the screening strategy have shown a reduction in incidence and mortality due to colorectal cancer[4,5]. However, colorectal cancer differs from other cancers in that a variety of screening tests (FOBT, sigmoidoscopy, double contrast barium enema, DNA stool testing, CT colonography and colonoscopy) have been approved and recommended by experts [6]. The advantages and disadvantages of different screening tests have been the subject of intense debate, however, estimates of cost-effectiveness suggest that, from a societal perspective, compared with no screening, all methods of colorectal cancer screening are likely to be as cost-effective as many other clinical preventive services—less than $30,000 per additional year of life gained. 24 Single positive FOBT is the lowest sensitive test for colon cancer screening (13%-50%) [5], but is less expensive than the others, thus is used widely by primary care physicians. When positive it is recommended to have a complete evaluation of the colon with colonoscopy. Even though colonoscopy is the gold standard for the evaluation of a positive screening test, DCBE may be a reasonable alternative to no evaluation or delayed evaluation. DCBE is considered an option because it offers an alternative (albeit less sensitive) means to examine the entire colon, it is widely available, has less complications and it detects about half of large polyps, which are most likely to be clinically important. Furthermore DCBE could be an alternative diagnostic test, with acceptable confidence in: high risk patients, those with incomplete colonoscopic examination, in accordance to patient’s preferences and where colonoscopy is not accessible or available. The primary objective of this investigation was to assess the diagnostic accuracy of DCBE compared to colonoscopy (as the gold standard) in the evaluation of Hispanic patients with positive FOBT. Our secondary objectives were: (1) to examine location, number, size and pathologic characteristics of the lesions found in colonoscopy and/or DCBE, and (2) to determine patient satisfaction with both tests. METHODS We conducted a cross-sectional study of consecutive patients referred to an academic gastrointestinal practice [San Juan, VA Caribbean Healthcare System (VACHS), Endoscopy Unit]. The study evaluated the diagnostic accuracy of DCBE compared to colonoscopy (gold standard) in detecting adenomatous polyps and/or colorectal cancer in a cohort of Hispanic patients with positive FOBT. Patients were identified from a list of all patients with FOBT positive [Hemoccult Sensa II (Beckman Coulter, Inc., Fullerton, CA, USA)] provided by VACHS laboratory between October 2003 and September 2004. Study was approved by the local Institutional Review Board. Subjects. Inclusion criteria: Asymptomatic patients older than 50 years of age, with at least one positive FOBT (out of 3 consecutive separate samples), without history of colon cancer and/or adenomas, gross gastrointestinal bleeding or prior colonoscopy or DCBE, and who agreed to participate, were included in the investigation. Exclusion Criteria: Patients with poor cardio-respiratory condition, coagulation deficiency, use of anticoagulants or other drugs affecting coagulation, severe renal or liver disease, hospitalized patients and palpable abdominal or rectal mass were excluded from the study. The initial evaluation consisted of a complete clinical history, medication history including use of aspirin or other non steroidal anti inflammatory drugs (NSAIDs), anticoagulants or other drugs affecting coagulation (ticlopidine, clopidogrel, etc), physical examination and basic laboratories (blood cell count, coagulation profile and comprehensive metabolic panel) DCBE and Colonoscopy evaluation. DCBE and colonoscopy were performed in all patients within 6 weeks of completion of the FOBT. Bowel preparation for DCBE consisted in the standard barium enema preparation kits, which consisted of 480 mL of sodium citrate and bisacodyl, as a 10 mg suppository and 4 oral tablets. Colonoscopy was performed with standard preparation (polyethylene glycol solution) and sedation (benzopiazepines and narcotics), using standard Olympus video colonoscopes. Colonoscopy was performed within 4 weeks after the barium enema. The endoscopist documented the extent of the colon that was visualized and the quality of the bowel preparation. Completion of colonoscopy up the cecum with adequate visualization of the colonic mucosa was required. The size and location of any lesions were recorded. The colonoscopic examination and pathology reports were used to determine histological diagnosis. A single experienced staff radiologist (with over 10,000 BE examinations) interpreted all DCBE. These results were compared with those found at colonoscopy performed by experienced gastroenterologists in the Gastroenterology Section at the VACHS. For a patient to be included in the analysis both a complete DCBE and a complete colonoscopy up to the cecum were required. Findings in DCBE and colonoscopic examination were tabulated according to location (rectum, sigmoid colon, descending colon, splenic flexure, transverse colon, hepatic flexure, ascending colon or cecum); size (mm), histology, quality of preparation (see table 1) and completeness of each procedure [7]. The endoscopist was blinded to the results of DCBE. Upon retrieval of the instrument at the level of the rectum, one of the members of the procedure team revised the DCBE report and informed the endoscopist the results. In case of discrepancies between the DCBE and the colonoscopy findings the scope was advanced and the area revised. All polyps identified in colonoscopy were removed and then classified histologically into: tubular or villous adenomas, carcinomas or non adenomas (hyperplastic or normal mucosal “tags”). The polyps were then classified according to size in: ≤5 mm, 6-9 mm and ≥10 mm. Polyp size was estimated by the endoscopist using an open forceps during colonoscopic evaluation and by direct measurement in radiologic images. A scoring system was used by radiologists and gastroenterologists to grade colonic preparation (Table 1). Table 1. Grading system for the colonic preparation Grade Description Poor Large amounts of fecal residue, additio Fair Good Excellent nal cleansing required Enough feces or fluid to prevent a com pletely reliable examination Small amounts of feces or fluid not interfering with the examination No more than small bits of adherent fecal fluid Patient Satisfaction. At the end of the protocol patients were asked about tolerability and grade of satisfaction with the procedures using three questions that were graded by yes or no answers: (1) Did you feel pain in DCBE and/or colonoscopy?; (2) Did you feel discomfort in DCBE and /or colonoscopy?; (3) Would you repeat the DCBE/colonoscopy again? Statistical Analysis Study Outcomes. The primary outcomes of the study were the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy of DCBE for colonic adenomas and colorectal cancer. Diagnostic accuracy of DCBE was determined for all size polyps, polyps ≥ 10 mm, polyps 6-9 mm, and polyps ≤ 6 mm. Finally, we evaluated the patients’ satisfaction (DCBE vs. colonoscopy). Descriptive statistics were used to characterize the database including means, median, and percentiles. We calculated sensitivity [true positives/(true positives + false negatives)], specificity [true negatives/(true negatives + false positives)], PPV [true positives/(true positives + false positives)], NPV [true negatives/(true negatives + false negatives)], diagnostic accuracy [(true positive + true negative)/total]. Sample size was predetermined on the basis of the assumption that the DCBE tests had sensitivities for the detection of advanced neoplasia of at least 50%, and a prevalence of advanced neoplasia of 50%. Given this assumption, the enrollment of 50 subjects would provide the study with a statistical power of 80% to detect a significant difference at a two-sided α level of 0.05. Statistical analysis was performed using STATA 9.0. RESULTS General findings. Between October 2003 and September 2004, 50 average-risk asymptomatic patients with a positive FOBT were invited to enroll in this investigation and all agreed to participate. All 50 patients underwent a DCBE, a colonoscopy, and a clinical questionnaire. Most of the patients were men (49/50). The average age was 63.8 years (range 49-84 yrs old, SD ± 9.48). 25 Colorectal polyps. A total of 102 polyps were detected during the study (Table 2). Polyps were found in 40/50 (80%) patients during colonoscopy compared to 19/50 (38%) in DCBE. The number of polyps increased distally; with more than half of the polyps found in the left colon and approximately one fourth of them at the rectum (Table 2). Approximately 20% were of ≥ 10 mm, of which 68% were adenomas. Two out of three polyps (66%) were smaller than 5 mm. Overall, DCBE missed eighty-four percent (86/102) of the polyps, most of them [71% (61/86)] were adenomatous, of which 17% (15/86) were ≥ 10 mm in size. Most of the missed adenomatous polyps ≥ 10 mm were located at the left side of the colon, been most commonly located at sigmoid region [40% (6/15)] and the rectum [20% (3/15)]. DCBE did not identify additional adenomatous polyps, not diagnosed during colonoscopy. Histology. A total of sixty-six percent (66%) of all polyps were tubular adenomas. Most polyps were sessile, adenomatous, less than 5 mm in size and located in the left colon. Four colon cancers (adenocarcinomas) were identified in the evaluated population, three in the rectum and one at the cecum. Three of the lesions were localized while one was metastatic at the time of diagnosis. Refer to Figure 1 for histological distribution of the lesions. All cancers were identified by both DCBE and colonoscopy. Performance Characteristics. DCBE had a sensitivity of 45%, a specificity of 90%, and an overall diagnostic accuracy of 54% for colonic neoplasia (adenomas/cancer) of any size. The sensitivity of the DCBE increased from the right to left colon as specificity decreased. The DCBE had a perfect sensitivity for detection of colorectal cancer (all four cancers were identified by colonoscopy and DCBE). The overall (right and left sided) positive predictive value and negative predictive value were: PPV 94.7% vs. NPV 29%. Refer to Table 3 for DCBE performance in a per-patient analysis. Table 2: Distribution of polyps by size, location and histology in the 50 patients with positive fecal occult blood test LOCATION <5 MM 6-9MM ≥10 MM ANY SIZE OF POLYPS ADENOMATOUS (%) Cecum 9 2 1 12 8 (66) 6 1 1 8 5 (63) 5 0 0 5 4(80) Transverse Colon 10 0 2 12 10 (83) Splenic Flexure 5 1 2 8 7 (88) 8 3 2 13 11(85) Sigmoid 7 3 8 18 13 (73) Rectum 17 3 6 26 14 (54) 67 (65.7) 13 (12.7) 22(21.6) 102 (100) 72 (71) Ascending Colon Hepatic Flexure Descending Colon Total (%) 26 Table 3. Performance characteristics of DCBE for adenomatous polyps according to size and location (right vs. left colon) Sensitivity(%) Specificity (%) PPV(%) NPV(%) Accuracy(%) Any size polyps Any location 45 90 95 29 54 Right colon 20 96 83 54 58 Left colon 42 96 50 58 58 Polyps ³ 10 mm Any location 32 97 86 70 72 Right colon 50 98 67 96 94 Left colon 25 94 67 73 72 Polyps 6-9mm Any location 40 98 80 87 86 Right colon 33 98 50 96 94 Left colon 38 97 75 89 88 Polyps ≤ 5 mm Any location 14 93 83 30 36 Right colon 5 97 50 58 59 Left colon 17 92 67 54 56 *Right colon=ascending, hepatic flexure, transverse colon; Left colon= splenic flexure, descending, sigmoid, and rectum. **Per patient analysis (N=50). Bowel preparation and Tolerability. Regarding bowel preparation, there were no significant differences in the bowel preparation for both studies, with all examinations rated good or excellent for both colonoscopy and DCBE (p = 0.55). In terms of tolerability, most of the patients felt no pain with either procedure, with 98% (49/50) pain free during the colonoscopy vs. 80% (40/50) during the DCBE (chi square test, p= 0.20). There was not a statistically significant difference between DCBE and colonoscopy regarding discomfort of the procedure. However, patients preferred the colonoscopy over DCBE (60% vs. 92% would repeat DCBE and colonoscopy, respectively). DISCUSSION The main purpose of this study was to explore the performance characteristics and diagnostic accuracy of two conventional methods for the detection of colorectal neoplasia in asymptomatic Hispanic patients with positive fecal occult blood test. This study revealed that the DCBE has a good sensitivity for colorectal cancer detecting 100% of colon cancers, but a limited diagnostic yield in the evaluation of patients with colonic adenomas. By using the DCBE, a significant number of adenomatous polyps >10 mm, and most of the polyps less than 5 mm were missed. The sensitivity of DCBE was not as good as colonoscopy for the evaluation of polypoid lesions of any size, as such clinicians should be aware that they can be easily missed during a routine diagnostic study. Nonetheless, the sensitivity and concordance for the detection of carcinoma were equal for the colonoscopy and DCBE, as reported in previous studies [8]. There have been very limited studies that have evaluated the diagnostic accuracy of DCBE compared to colonoscopy in patients with positive FOBT [8]. The detection of adenomas by barium enema is size dependent. In the National Polyp Study, the barium enema detection rate of adenomas smaller than 5 mm was 32%, for adenomas 6 to 10 mm was 53%, and for those larger than 10 mm 48% [9, 10]. Attributed factors for the decreased sensitivity included: inadequate cleansing of the colon which leads to inadequate mucosal coating, diverticulosis and/or redundant bowel, which results in a 10% false-negative rates. Because of these issues the use of barium enema has been less favored. The large demand for colonoscopy usually overwhelm the capacity of gastroenterological services to perform timely colo- noscopy, therefore DCBE could be a reasonable alternative for those patients, who otherwise would remain without a timely evaluation. Hence, the use of DCBE has been included as a viable alternative for colorectal cancer screening [6]. Percentage (%) The high percentage of missed polyps larger than 10 millimeters observed in this study was unexpected. Most of these polyps had potential for carcinoma since an adenomatous component was found. Most were located at the left colon, being the sigmoid the most common region. There was no clear reason for such a high rate since inter-observer variations were eliminated by having a single expert radiologist interpreting all studies. In addition, bowel preparation during the DCBE study was graded either good or excellent in most of the patients. Comparable missed rates were also described on previous large studies including those reported 70 60 50 40 30 20 10 0 27 was unaware of the DBCE results. The major limitations of this study were probably the small sample size and the fact that most patients were male (as expected in a VA population). However, we calculated sample size based on reported diagnostic accuracy of DCBE and statistically significant differences in detection of colorectal polyps were observed between colonoscopy and DCBE [15, 16]. While considering patient tolerability using both procedures, there were no statistically significant differences among the discomfort. However, most patients preferred the colonoscopy over the DCBE. In summary, our study supports the use of colonoscopy as the gold standard test for the evaluation of Hispanic patients with positive FOBT and remained the preferred diagnostic tool by the patients. DCBE appears to have a good 64.7 28.4 1.9 Adenom a Hyperplastic Serrated 1 High Grade 3.9 Cancer Histology Figure: Histology of colon polyps removed during colonoscopy. on the “National Polyp Study” in where only 48% of adenomas greater than 10mm in size were detected [9, 10]. A total of 8% of the asymptomatic FOBT positive patients had colon cancer, while 80% had adenomatous polyps or advanced lesions. This finding is concordant to conventional guidelines where FOBT positive patients can have a higher index of mucosal related neoplastic lesions. The predictive value of a positive test for colorectal cancer was similar to those reported in previous studies [11, 12, 13, 14]. Three out of the four patients with colorectal cancer had localized disease at the time of diagnosis, again highlighting the benefit of screening in asymptotic individuals with occult blood test. The average risk Hispanic patient evaluated in this study with a positive FOBT had an increase incidence of polyps at the left side and a high proportion of distal colon cancers. The number of patients with colon polyps was higher that expected as compared to large studies. Perhaps our observations are related to genetic, ethnic and/or demographic characteristics of our study population, as most of the study patients were Hispanic male with an advanced mean age. Our study design has several strengths including its design, were all patients underwent both procedures, a single radiologist interpreted all the DCBE, and the endoscopist sensitivity for colorectal carcinoma, but its diagnostic accuracy for colorectal polyps of all sizes limits its efficacy as a diagnostic and screening method for colorectal cancer. In view of our observations, we believe DCBE is a suboptimal method and should be used in clinical scenarios were there is limited access to colonoscopy. Newer methods such as CT colonography may provide an alternative to DCBE and are currently under investigation. REFERENCES 1. Cancer Facts and Figures for Hispanics 2006-2008. American Cancer Society http://our.cancer.org/docroot/STT/stt_0_2006.asp?sitearea=STT&level=1 2. Greenlee RT, Hill-Harmon B, Murray T, et al: Cancer statistics 2001. CA Cancer J Clin 51:15, 2001. 3. Parkin DM, Pisani P, Ferlay J: Global cancer statistics. CA Cancer J Clin 49:33, 1999. 4. Ries L, Eisner M, Kosary C, et al: SEER Cancer Statistics Review, 1973–1977. Series. Bethesda, MD, National Cancer Institute, 2000. 5. Mandel JS, Bond JH, Church TR, et al. Reducing mortality from colorectal cancer screening for fecal occult blood. Minessota Colon Cancer Control Study. N Engl J Med 1993;328:1365-71. 6. Levin B, Lieberman DA, McFarland B, et al. Screening and Surveillance for the Early Detection of Colorectal Cancer and Adenomatous Polyps, 2008: A Joint Guideline from the American Cancer Society, the US Multi-Society Task Force on Colorectal Cancer, and the American College of Radiology. CA Cancer J Clin 2008;5:(in press). 7. U.S Preventive Services Task Force. Screening for colorectal cancer: recommendations and rationale. Ann Intern Med 2002;137:129-131. 28 OFICINAS ADMINISTRATIVAS SUBSCRIPCIONES Y ANUNCIOS Asociación Médica de Puerto Rico PO Box 9387 • SANTURCE, Puerto Rico 00908-9387 Tel 787-721-6969 • Fax: 787- 724-5208 Email: [email protected] ANUNCIOS EN BOLETIN Y WEB SITE [email protected] Web Site: www.asociacionmedicapr.org El “Boletín” se distribuye a todos los médicos y estudiantes de medicina de Puerto Rico (12.000 ejemplares) y se publica en versión digital en www.asociacionmedicapr.org. Todo anuncio que se publique en el Boletín de la Asociación Médica de Puerto Rico deberá cumplir con las normas establecidas por la Asociación Médica de Puerto Rico y la Asociación Médica Americana. La Asociación Médica de Puerto Rico no se hace responsable por los productos o servicios anunciados. La publicación de los mismos no necesariamente implica el endoso de la Asociación Médica de Puerto Rico. Todo anuncio para ser publicado debe reunir las normas establecidas por la publicación. 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Box 9387 San Juan, PR 00908-9387 Dirección Física: 1305 Fernández Juncos Ave. SANTURCE, PR 00908 Email: [email protected] "POSTMASTER" If you do not receive this magazine in your desk or you changed your address, send changes to: Boletín / Asociación Médica de Puerto Rico 1305 Fernández Juncos Ave. P.O. Box 9387 San Juan, Puerto Rico 00908-9387 8. McGrath JS ; Ponich TP ; Gregor JC Screening for colorectal cancer: the cost to find an advanced adenoma. Am J Gastroenterol. 2002; 97(11):2902-7 9. Winawer SJ The National Polyp Study. Design, methods, and characteristics of patients with newly diagnosed polyps. The National Polyp Study Workgroup. Cancer - 1-SEP-1992; 70(5 Suppl): 1236-45 10. Winawer SJ, Stewart ET, Zauber AG, et al: A comparison of colonoscopy and double-contrast barium enema for surveillance after polypectomy. N Engl J Med 2000; 342:1766. 11. Baig N; Myers RE; Turner BJ; Grana J; Rothermel T; Schlackman N; Weinberg DS Physician-reported reasons for limited follow-up of patients with a positive fecal occult blood test screening result. .Am J Gastroenterol. 2003; 98(9):2078-81 12. El Sayed AM. A randomized single-blind trial of whole versus splitdose polyethylene glycol electrolyte solution for colonoscopy preparation. Gastrointest Endosc - 01-JUL-2003; 58(1): 36-40 13. Don Rockey, MD, Johannes Koch, MD; Judy Yee, MD; Kenneth McQuaid, MD; Robert A. Halvorsen, MD. Prospective comparison of air-contrast barium enema and colonoscopy in patients with fecal occult blood: a pilot study. Gastrointenstinal Endoscopy; 2004 60 (6); 953-958. 14. O’Brien MJ, Winawar SJ, Zauber AG et al: The National Polyp Study. Gastroenterology 98; 371, 1990. 15. Winawer SJ, Stewart ET, Zauber AG, et al: A comparison of colonoscopy and double-contrast barium enema for surveillance after polypectomy. N Engl J Med 342: 1766, 2000. 16. Allison JE, Feldman RF, Tekawa IS: Hemoccult screening in detecting colorectal neoplasm: Sensitivity, specificity and predictive value. Long term Follow-up in a large practice setting. Ann Intern Med 112: 328; 1990. RESUMEN Propósito: Este estudio buscaba determinar la certeza diagnostica del enema de bario de doble contraste (EBDC) comparado con colonoscopía en la evaluación de pacientes hispanos con pruebas de sangre oculta positivas. Los objetivos secundarios eran: determinar la certeza diagnostica de acuerdo a la localización del adenoma, el tamaño y las características patológicas; tanto como la satisfacción del paciente. Métodos: Estudio cruzado comparado la certeza diagnóstica de EBDC y colonoscopía en detectar adenomas y/o cáncer colorrectal en pacientes con pruebas de sangre oculta positivas. A todos los pacientes se les hizo tanto EBDC seguido por colonoscopía. Los pólipos que se identificaron durante la colonoscopía se removieron y se clasificaron histológicamente. Se determino la tolerancia y satisfacción del paciente a ambos estudios mediante un cuestionario. Resultados: Cincuenta pacientes participaron, mayormente hombres con una edad promedio de 63 años. Se identificaron pólipos en 40 de los 50 pacientes (80%) durante colonoscopía comparado a 19/50 (38%) mediante EBDC. El 84% de los pólipos no se detectaron en EBDC. EBDC tuvo una sensitividad de 45% y una especificidad de 90% para adenomas. La certeza diagnóstica del EBDC fue de 54% para adenomas de cualquier tamaño, mientras que para adenomas de ≥10mm fue de 72%. La sensitividad aumento del lado derecho del colon hacia el izquierdo, mientras que la especificidad disminuyo. Los pacientes prefirieron la colonoscopía al EBDC. Conclusión: Este estudio apoya el uso de colonoscopía como la prueba diagnóstica de elección para la evaluación de pacientes con pruebas de sangre oculta positiva y fue esta también la prueba preferida por los pacientes. La certeza diagnóstica de EBDC fue inferior a colonoscopía para pólipos de cualquier tamaño al igual que para adenomas grandes. Comparado con colonoscopía, EBDC es una prueba inferior para el cernimiento y evaluación de cáncer de colon y puede llevar a resultados ineficaces. 29 ABSTRACT Background: The surgical management of anterior skull base cerebrospinal fluid (CSF) fistulas has evolved throughout the last decade. Endonasal endoscopic surgery of the anterior skull base has become the standard procedure for the repair of most these fistulas. Objectives: To describe the presenting symptoms, etiology, treatment, and long-term outcomes of patients with anterior skull base CSF fistulas treated endoscopically at our institution. Material/ Methods: Retrospective analysis of 25 patients with CSF fistulas treated endoscopically at the University of Puerto Rico Hospital from November 2004 to August 2008. Result: The etiology was spontaneous leak in 10 patients, menigoencephalocele in 7 patients, previous sinus surgery in one patient, and trauma in one patient. The most common location of leak was the cribiform plate, followed by the ethmoid roof. An overlay technique was used for repair in 61% of the procedures versus 39% for the underlay technique. The mean follow up was 23 months. Our overall initial rate of closure was 94% with 100% after a second procedure. Conclusions: Endoscopic repair of anterior skull base CSF fistulas has a high success rate and lower morbidity and mortality when compared with open approaches. Index words: Cerebrospinal Fluid Fistulas, Anterior Skull Base, Endoscopy ANTERIOR SKULL BASE CEREBROSPINAL FLUID FISTULAS IN PUERTO RICO: TREATMENT AND OUTCOME Yadiel A. Alameda MD* José M. Busquets MD* Juan C. Portela MD* From * the Department of Otolaryngology-Head and Neck Surgery, University of Puerto Rico, San Juan, Puerto Rico. This study was presented at the Raffucci Research Forum, ACS Annual Meeting 2008, La Concha Resort, San Juan. Address reprints requests to: Yadiel A. Alameda, MD, Department of Otolaryngology-Head and Neck Surgery, Medical Science Campus, PO Box 365067, San Juan PR 00936-5067. Email: [email protected] INTRODUCTION T he surgical management of anterior skull base cerebrospinal fluid (CSF) fistulas has evolved throughout the last decade. The endonasal endoscopic approach has recently become the preferred technique in the surgical treatment of most anterior skull base CSF fistulas. These may arise as a complication of endoscopic sinus surgery or neurosurgical procedures, trauma, meningoencephalocele, or spontaneously without any identifiable cause1,2,3. Almost all of these patients experience unilateral rhinorrhea, but others may present with episodes of meningitis. Studies have shown that cerebrospinal fluid leak is a well-known cause of ascending bacterial meningitis, with reported risk of 10% annually4. Additionally there is a 1% risk of annual mortality5. It has been shown that closure of skull base defects with and without active CSF leak in patients with prior ascending bacterial meningitis provides excellent long-term results6. Pnemocephalus and intracranial abscess are also potentially fatal complications of an anterior skull base fistula. Successful endoscopic repair relies on precise site identification, accurate graft selection and placement, and postoperative management, with modest variations between the published series. Several factors have been studied to predict success of the procedure including location, size of defect, and etiology. The success rates reported are over 90% in most series,7,8,9. Low morbidity and mortality is widely reported from this procedure, compared to traditional open approaches. Two main techniques have been used for the closure of anterior CSF fistulas, the underlay (graft placed between dura and skull base) and the overlay (graft placed below the skull base). The overlay technique carries less potential damage to the neurovascular structures and is less technically demanding than the underlay technique. The purpose of this study is to report the presenting symptoms, etiology, treatment modality, and outcomes of patients with anterior skull base CSF fistulas treated endoscopically at our institution. MATERIAL AND METHODS This is a retrospective chart review of all patients that underwent endoscopic repair of anterior skull base CSF fistulas at our institution from November 2004 to August 2008. Routinely, the diagnosis was confirmed with a pertinent history, physical exam, nasal endoscopy, high-resolution CT scans, and b-2 transferrin measurement of nasal drainage. A total of 25 patients were operated during this period by the two senior authors. Six patients were excluded from the analysis due to a short follow up period. Data collected from 19 patients included age, gender, presenting symptoms, etiology, location of defect, size of defect, type of surgical closure (underlay vs. overlay), lumbar drainage use, graft materials, and use of image-guided surgery (IGS). 30 At the beginning of the procedure, 0.05mL of 10% fluorescein diluted in 10mL of CSF was injected intrathecally to aid in the localization of the defect. Current standard endoscopic sinus surgery equipment and approaches were used for all the procedures. Surgery began with standard maxillary antrostomy and total ethmoidectomy, with sphenoid and frontal sinusotomies performed when necessary for exposure. After identification of the bony defect, wide exposure was obtained and the defect explored. The mucosa surrounding the defect was curetted, followed by placement of an appropriate graft in a layered fashion, with support of a fibrin sealant (Tisseel, Baxter Pharmaceuticals, Illinois). All surgically treated patients remained at the surgical ward with strict bed rest until discharge at postoperative day 4-5, and after removal of the lumbar drainage. In selected cases where elevated intracranial pressure was suspected, acetazolamide, a diuretic known to reduce CSF production, was used for 3 weeks in the postoperative period. Patients were followed up in the outpatient clinics. Postoperative complications, rate of recurrence, and need for revision surgery were determined. This study had local IRB approval. The size of the defect was more than 10mm in 3 patients (16%), between 3mm and 10mm in 10 patients (53%), and less than 3mm in 5 patients (26%). Associations between the CSF leak etiology and location are seen in Table 2. The most common location for spontaneous leak was the cribiform plate in four patients followed by the ethmoid roof in 3 patients, and sphenoid sinus in one patient. In the meningoencephalocele group, 4 of 6 were located in the cribiform plate, whereas 2 were in the sphenoid sinus, and one in the ethmoid roof. Four of seven patients with empty sella had spontaneous leaks, located at the cribiform plate in 3 patients and one in the ethmoid roof. The other two patients with empty sella had a meningoencephalocele located at the cribiform plate (Table 3). The surgical data is listed on Table 4. All patients underwent endoscopic repair using graft materials that included mucoperichondrium, mucosa, fat, cartilage, and muscle/fascia. Mucoperichondrium was used in 4 patients (22%), mucosa in 11 patients (61%), fat in 8 (44%), cartilage in 9 patients (50%), and muscle/fascia in 5 patients (28%). In most cases (89%), two or more grafts were used. Table 1. Demographic Data Mean age Sex 56 years 16 female (84%) rhinorrhea 19(100%) Symptoms / Signs Range(17-76) 3 male (16%) headaches 5 (26%) Etiology Site Size spontaneous 10 cribriform plate 9 >10mm 3(16%) (53%) (47%) meningoencephalocele 7 ethmoid roof 5 3-10mm 10 (37%) (26%) (53%) iatrogenic 1(5%) sphenoid sinus 3 <3mm 5 (26%) meningitis 5 (26%) (16%) Rosenmuller fossa 1 not identified 1 (5%) (5%) not identified 1 (5%) fever 3 (16%) Trauma 1 (5%) empty.sella 7 (37%) RESULTS Four postoperative complications were observed, including one mortality. This latter patient developed a hypertensive crisis and subarachnoid hemorrhage on her fourth postoperative day, and died of pneumonia and sepsis four weeks later. One patient experienced recurrence of the CSF leak (5%), one patient developed a mucocele (5%), and one presented with paresthesia of the maxillary branch of the trigeminal nerve. The demographic data of the 19 patients is listed in Table 1. There were 3 males (16%) and 16 females (84%) with the mean age of presentation being 56 years (range, 1776 years). Clinical findings at presentation included: rhinorrhea, fever, headaches, meningitis, and empty sella. Rhinorrhea was seen in all patients (100%), headaches in 5 patients (26%), meningitis in 5 patients (26%), fever in 3 patients (16%), and empty sella in 7 patients (37%). The etiology of the leak was spontaneous leak in 10 patients (53%), presence of meningoencephalocele in 7 patients (37%), trauma in one patient (5%), and previous sinus surgery in one patient (5%). The location of the defect was the cribiform plate in 9 patients (47%), ethmoidal roof in 5 patients (26%), sphenoid sinus in 3 patients (16%), and the fossa of Rosenmuller in one patient (5%). For the purpose of this analysis the cribiform plate was defined as the area of the skull base from the attachment of the middle turbinate to the nasal septum medially. The ethmoid roof was defined as the area lateral to the middle turbinate attachment. The location of the CSF leak could not be identified in one patient. Grafts were placed using either the underlay or overlay technique, with overlay used in 11 patients (61%), and underlay in 7 patients (39%). A lumbar drain was left in place postoperatively in 17 patients (89%). IGS was employed in 15 patients (79%). Patients’ follow-up ranged from 13 to 42 months, and the average follow-up was 23 months. DISCUSSION Dandy10 first described intracranial repair of CSF fistulas in 1926 using a bifrontal craniotomy. Even though success rates for open repair in the literature are over 80%, this approach is associated with significant morbidity inclu- 31 Table 2. Association between etiology and location of CSF leak Site Etiology cribiform plate ethmoid roof sphenoid sinus Rosenmuller fossa not seen Spontaneous 4 3 1 1 1 meningoencephalocele4 1 2 - - Iatrogenic - 1 - - - Trauma 1 - - - - Table 3. Association between location of CSF leak in patients with empty sella Site Etiology cribiform plate ethmoid roof Spontaneous 3 1 meningoencephalocele 2 - Table 4. Surgical Data Several factors have been used to predict success of the procedure including location, size of defect, and etiology. However, recently, Zweig et al16 found that regardless of its location or size, adequate exposure of the defect was the essential step for a successful repair. Furthermore, Mirza et al9 found that six of their eight failures occurred in the spontaneous CSF leak group, and seven occurred in those cases with evidence of raised intracranial pressure. They agree that elevated intracranial pressure appears to be a feature of spontaneous CSF leaks and would explain the higher failure rate. An association between spontaneous CSF leaks and empty sella has also been reported by Schlosser and Bolger17. Surgical closure Graft Lumbar Drainage IGS Complications Overlay 11 (61%) mucosa 11 (61%) used 17 (89%) used 15 (79%) recurrence leak 1 (5%) Underlay 7 (39%) cartilage 9 (50%) not used 2 (11%) not used 4 (21%) mucocele 1 (5%) fat 8 (44%) death 1 (5%) muscle/fascia 5 (28%) V2 parethesia 1 (5%) mucopericondrium 4 meningitis 0 (0%) (22%) anosmia 0 (0%) headaches 0 (0%) ding prolonged hospital stay, anosmia, external scarring, potential intracerebral hemorrhage, and frontal lobe retraction injury11,12,13. In 1981, Wigand14 first reported the use of a nasal endoscope to repair CSF rhinorrhea. In 1990, Matox and Kennedy15 described the transnasal approach in detail. Since then, high success rates with low morbidity associated with this procedure have been reported. We performed endonasal endoscopic surgery for anterior skull base CSF fistula in 19 patients with a success rate comparable to published rates7,8,9. Our overall initial rate of closure was 94%, and 100% after a second procedure. Five of the eighteen patients in our study presented with meningitis preoperatively. None of them have developed another episode of meningitis after a mean follow-up of 36 months postoperatively for this subgroup. Published reports indicate that untreated CSF leaks may lead to bacterial meningitis with reported risk of 10% annually4. Bernal-Sperkelsen et al6 showed that closure of CSF leaks in patients with prior ascending bacterial meningitis provides excellent long-term results with no recurrence of meningitis. These leaks are commonly located in cribiform plate and sphenoid sinus. Correspondingly, 4 out of 7 of our patients with empty sella had spontaneous leaks, three of them located at the cribiform plate. Interestingly, none of our patients with empty sella or with other objective signs of increased intracranial pressure presented with recurrence at last follow-up. In our series, one patient had a recurrence of the leak after repair. She presented with a lateral sphenoid sinus wall defect. Even though a limited pterygomaxillary fossa approach was performed, the exposure at this location was suboptimal. Since the size of the defect was considered small (5mm), the sphenoid sinus was obliterated with fat. However, the leak recurred 5 weeks postoperatively. The patient underwent revision surgery through an extended pterygomaxillary fossa approach. Adequate exposure was attained at this time and the defect repaired with a layered reconstruction using muscle/fascia, cartilage, and free mucosa in an overlay fashion. No recurrence has been noted on follow-up for 24 months. 32 Interestingly, we could not identify the site of the defect in one of our patients, even with use of intrathecal fluorescein, complete exposure of the skull base, and maneuvers to increase intracranial pressure. In this case, the decision was made to strip the mucosa of the entire skull base from the posterior edge of the frontal recess to the sphenoid rostrum and the entire area was packed with a large free mucosal graft and fibrin sealant. No leak recurrence has been reported so far at 20 months of follow-up. Several authors12,18 have suggested that graft choice and operative closure technique (underlay vs. overlay) should be selected according to the size of the defect. Recently, other authors have advocated that the choice of graft and technique should depend solely on availability of materials and adequate surgical exposure7,16. In our experience we prefer the use of autologous free grafts (mucosa, fat and cartilage) instead of pedicle composite grafts. The former are easily harvested, do not alter nasal anatomy and do not add significant donor site morbidity. We chose among grafts based on the size and location of the defect. Hegazy et al8, however, found no statistically significant difference among the different grafting materials. The underlay technique carries more potential damage to the neurovascular structures and is more technically demanding than the overlay technique. We employed the overlay technique in 61% of our patients. This is consistent with the literature. A meta-analysis of CSF rhinorrhea8 studies from 1990 to 1999 reported that the overlay technique is more commonly employed than the underlay technique. However, Lee et al.7 reported no significant differences in success rates between both techniques. There is no clear indication in the literature for the use of lumbar drainage to repair CSF fistulas. Although Anand11 used it in all his patients, recent publications by Lee7 and Lindstrom19 recommend its use only in selected cases. In our institution it is used in most patients for two reasons. First and foremost, it provides access to the intrathecal space for fluorescein injection, which aids in the localization of the defect intraoperatively. In our experience, this technique has been very helpful and is routinely employed in all of our cases. Using a combination of pre-operative CT scan and intra-operative fluorescein, we have successfully identified the site of leak, in 95% of our patients, with no need for other ancillary studies. There were no fluorescein-associated complications. Secondly, lumbar drainage also allows decompression of the subarachnoid space after reconstruction to facilitate the initial adhesion process of the graft. Several authors3,18 have used the initial spinal tap pressure reading to establish a correlation with increased intracranial pressure as a possible etiology. Given that most of our patients had high flow leaks, the spinal tap pressure was not measured since it might be inaccurate because of a possible decompression effect through the skull defect. Tabee et al20 recently studied the use of image-guided surgery (IGS) in endoscopic repair of CSF fistulas. Their study did not detect a significant difference in the rate of complications or successful closure in patients in where IGS was utilized. In our experience, the use of IGS offers a great benefit when dealing with cases of distorted anatomy from prior sinus surgery or trauma, and also in some cases with meningoencephalocele. We routinely employ the use of IGS, as it is readily available to us, involves minimal morbidity, and is an excellent tool for correlating anatomy in a teaching institution. Only one patient experienced leak recurrence. We ascribe this failure to suboptimal exposure on the initial procedure. The same patient developed paresthesia of the maxillary branch of the trigeminal nerve from a pterygomaxillary fossa approach. The paresthesia improved without treatment. A frontal mucocele was found in another patient that had previous history of a traumatic frontal sinus fracture. One patient developed a hypertensive crisis and subarachnoid hemorrhage on her fourth postoperative day. Unfortunately, she had a complicated hospital course and died of pneumonia and sepsis four weeks later. CONCLUSIONS Endoscopic closure of anterior skull base CSF fistula is a less invasive procedure with high success rates and low morbidity and mortality as compared with traditional intracranial approaches. This approach should be considered as the initial management in the treatment of most anterior skull base CSF fistulas. REFERENCES 1. Schlosser RJ, Bolger WE. Nasal Cerebrospinal fluid leaks. J Otolaryngology 2002; 31 (Suppl): s28-s37. 2. Har-El G. What is “spontaneous” cerebrospinal fluid rhinorrhea? Classification of cerebrospinal fluid leaks. Ann Otol Rhinol Laryngol 1999; 108: 323-326. 3. Lopatin AS, Kapitanov DN, Potapov AA. Endonasal endoscopic repair of spontaneous cerebrospinal fluid leaks. Arch Otolaryngol Head Neck Surg. 2003 Aug; 129 (8): 859-63 4. Eljamel MS. The role of surgery and beta-2-transferrin in the management of cerebrospinal fluid fistula (MD thesis). Liverpool: University of Liverpool, 1993: 207-219. 5. Jones NS, Becker DG. Advances in the management of CSF leaks. BMJ . 2001 Jan 20; 322 (7279): 122-123. 6. Bernal-Sprekelsen M, Alobid I, Mullol J, et al. Closure of cerebrospinal fluid leaks prevents ascending bacterial meningitis. Rhinology 2005 Dec; 43 (4): 27781 7. Lee TJ, Huang CC, Chuang CC, et al. Transnasal endoscopic repair of cerebrospinal fluid rhinorrhea and skull base defect: Ten year experience. Laryngoscope. 2004 Aug; 114: 1475-1480. 8. Hegazy HM, Carrau RL, Snyderman CH, et al. Transnasal endoscopic repair of cerebrospinal fluid rhinorrhea: A meta-analysis. Laryngoscope. 2000 July; 110: 1166-1172. 9. Mirza S, Thaper A, McCkelland L, et al. Sinonasal cerebrospinal fluid leaks: management of 97 patients over 10 years. Laryngoscope. 2005 Oct; 15 (10): 1774-7. 10. Dandy WE. Pneumocephalus (intracranial pneumatocele or aerocele). Arch Surg 1926; 12: 949-982. 11. Anand V, Murali RK, Glasgold MJ. Surgical decisions in the management of cerebrospinal fluid rhinorrhea. Rhinology. 1995 Dec; 33 (4): 212-8. 12. Burns JA, Dodson EE, Gross CW. Transnasal endoscopic repair of craniofacial fistula: a refined technique with long term follow-up. Laryngoscope. 1996; 106: 1080-3. 13. Dodson EE, Gross CW, Swerddloff JL, et al. Transnasal endoscopic repair of CSF rhinorrhea and skull base defects: A review of 29 cases. Otolaryngol Head Neck Surg. 1994; 111: 600-6. 14. Wigand ME. Transnasal ethmoidectomy under endoscopic control. Rhinology 1981; 19: 7-15. 15. Mattox DE, Kennedy DW. Endoscopic management of cerebrospinal fluid leaks and cephaloceles. Laryngoscope 1990; 100:857-862. 16. Zweig J, Carrau RL, Celin SE, et al. Endoscopic repair of cerebrospinal fluid leaks to the sinonasal tract: Predictors of success. Otolaryngol Head Neck Surg. 2000 Sep; 123(3): 195-201. 17. Schlosser RJ, Bolger WE. Significance of empty sella in cerebrospinal fluid leaks. Otolaryngol Head Neck Surg. 2003; 128: 32-38. 18. Casiano R, Jassir D. Endoscopic cerebrospinal fluid rhinorrhea repair: is a lumbar drain necessary? Otolaryngol Head Neck Surg. 1999 Dec; 121 (6): 745-50. 19. Lindstrom DR, Toohil RJ, Loehrl TA, et al. Management of cerebrospinal fluid rhinorrhea: the Medical College of Wisconsin experience. Laryngoscope. 2004 Jun; 114 (6): 969-74. 20. Tabaee A, Kassenoff TL, Kacker A, et al. The efficacy of computer assisted surgery in the endoscopic management of cerebrospinal fluid rhinorrhea. Otolaryngol Head Neck Surg. 2005 Dec; 133 (6): 936-43. 33 RESUMEN El manejo quirúrgico de las fístulas de líquido cefalorraquídeo (FLC) en la base de cráneo anterior ha evolucionado en la última década. La cirugía endoscópica endonasal de la base de cráneo anterior se ha convertido en el procedimiento de elección para reparar las mayorías de estas fístulas. Objetivos: Describir los síntomas, etiologías, tratamientos y resultados de pacientes con FLC tratados endoscópicamente en nuestra institución. Sujetos y Métodos: Un análisis retrospectivo de 25 pacientes con FLC tratados endoscópicamente en el Hospital Universitario de la Universidad de Puerto Rico desde noviembre 2004 a agosto 2008. Resultados: La etiología fue fuga espontánea en 10 pacientes, menigoencefalocele en 7 pacientes, cirugía previa de senos paranasales en un paciente y trauma en un paciente. La localización más común de la fuga fue el plato cribiforme, seguido por el techo etmoidal. La técnica de enchapado por encima (injerto puesto entre la dura y la base del cráneo) se utilizo para reparar 61% de los pacientes versus 39% para la técnica de enchapado por debajo de la base del cráneo. Los pacientes se siguieron por un periodo promedio de 23 meses. Se obtuvo cerrar exitosamente a 94% de los pacientes con fístulas en el procedimiento inicial y a 100% después del segundo procedimiento. CONCLUSION: La reparación endoscópica endonasal de las FLC de la base de cráneo anterior es altamente exitosa, con poca morbilidad y mortalidad comparado con los abordajes abiertos. 34 EARLY OUTCOMES OF REVERSE TOTAL SHOULDER ARTHROPLASTY Luis Marrero MD* Gabriel Garcia MD* Ivan Pacheco MD* From the * Department of Orthopedics, UPR School of Medicine, Rio Piedras, Puerto Rico. Address reprinsts request to: Luis Marrero MD, Department of Orthopedics, 9th Floor UPR School of Medicine, Rio Piedras, PR 00936. [email protected] INTRODUCTION T he utilization of different reconstructive techniques for rotator cuff arthropathy, complex fractures of the proximal humerus and pathologies that involve the glenohumeral joint, has become a controversial issue in orthopaedic surgery nowadays. Published reports have indicated a large variation in the benefits of shoulder hemiarthroplasty from patient to patient. After this procedure, the patients usually have pain-free shoulders, but reduced motion and strength, especially those with rotator cuff tears (1,2,3). Hettrich et al.(4) reported, that alone shoulder hemiarthroplasty has been shown to yield inferior functional results when the glenoid surface is compromised . On a recent study, it was reported that six out of eighteen patients with preoperative elevation of less than 90° had an unsatisfactory result; also pain relief and function were less predictable (5). In 1991 Grammont and Baulot, presented a reversed total shoulder prosthesis which reported not only good pain relief, but also a marked improvement in function (6). The design of the implant leads to a medialised and distalised centre of rotation of the glenohumeral joint and, subsequently, to an increased lever arc and presentation of the deltoid muscle which causes better functional results (7). In the present, there exist up to eight approved indications for this procedure which include complex shoulder reconstruction problems such as, revision arthroplasty, tumor resection and rheumatoid arthritis (8). Recent results have been satisfactory especially in addressing the problem of function and range of motion even on a joint that no longer has a rotator cuff (8). Due to the absence of long-term results, reverse shoulder total prostheses are mainly recommended for elderly patients and for the treatment of severe shoulder ABSTRACT The utilization of different reconstructive techniques for rotator cuff arthropathy, complex fractures of the proximal humerus and pathologies that involve the glenohumeral joint, has become a controversial issue in orthopaedic surgery nowadays. The purpose of the current study was to evaluate early outcomes of reverse total shoulder arthroplasty for primary osteoarthritis with a rotator cuff tear, rotator cuff arthropathy three and four part humerus fractures and proximal comminuted displaced humerus fractures in a group of Latin-American patients. Methods: Between July 2006 and February 2008, fourteen patients underwent reverse total shoulder arthroplasty with the use of Delta III shoulder prosthesis (Depuy France, Saints Priest, France) at the Hospital Buen Samaritano in Puerto Rico. All patients were evaluated by an independent examiner who performed a clinical pre-operative and post-operative evaluation with the use of the Constant & Murley (ref) and the UCLA (ref) scores, as well as measuring active shoulder range of motion. Results: All fourteen patients were seen at clinics. The mean duration of follow up was 9.5 months [+/- 6 S.D.] with a range of 1 month to 20 months of follow up Discussion: In our study we have shown that the reverse total shoulder replacement is a successful surgery, the mean improvement in the outcome scores have been significant in all patients, been the greatest improvement in the arthropathy groip. (Table II and Table III). Index words: shoulder, reverse, arthoplasty, oucomes dysfunction (9). Wall et al. presented the largest series for this procedure up to date, which consists of 199 patients divided in a wide spectrum of etiologies with a two year follow up(8). They found that the reverse total shoulder arthroplasty prosthesis can produce good results with high patient satisfaction, when used for the treatment of a number of other complex shoulder problems in addition to cuff tear arthropathy. The purpose of the current study was to evaluate early outcomes of reverse total shoulder arthroplasty for primary osteoarthritis with a rotator cuff tear, rotator cuff arthropathy three and four part humerus fractures and proximal comminuted displaced humerus fractures in a group of Latin-American patients. MATERIAL & METHODS Between July 2006 and February 2008, fourteen patients underwent reverse total shoulder arthroplasty with the use of Delta III shoulder prosthesis (Depuy France, Saints Priest, France) at the Hospital Buen Samaritano in Puerto Rico. This prosthesis is based on the Grammont design with medialization of the center of rotation. The procedures were performed by a single surgeon (senior author) all in the same hospital. The patients included ten women and four men. The mean age at the time of surgery was 71.3 Years old [+/- 6.2 S.D] (Range 57 to 80). Four left shoulders and ten right shoulders were included. The prosthesis was implanted due to arthropathy in eight shoulders, three humerus fractures, and three massive rota- tor cuff. The procedure was carried out by a single suregeon with a fellowship in upper extremity, and previous experience with this type of procedures. The utilized surgical approach delto-pectoral in all shoulders. All glenoid implant were uncemented. However humerus implants could de cemented or uncemented. The study was approved by IRB (University of Puerto Rico) and all subjects provided informed consent to allow their information to be used in the study. All patients were evaluated by an independent examiner who performed a clinical pre-operative and post-operative evaluation with the use of the Constant & Murley (ref) and the UCLA (ref) scores, as well as measuring active shoulder range of motion. Subjective results were graded by asking the patients to rate their personal satisfaction with surgical outcome. Radiographic evaluation was collected pre-op and post-op, including shoulder series, CT-scans and shoulder MRI. Extremity EMG studies were also performed in all patients before surgery. 35 range of 1 month to 20 months of follow up. There was one patient that had a dislocation, this patient was a schizophrenic with diagnosed psychiatric condition, the dislocation was reduced with the patient under anesthesia, and three days after discharge , the patient re-dislocated, but this time he underwent an open reduction, patient was followed up with no additional complications six months later. No other complications were reported. Table III. Clinical Results UCLA , Constant Score and ROM Patient No. FF Post ABD Post ER Post 1 140 100 50 2 125 100 60 3 180 110 35 4 180 110 60 5 130 100 60 Table I. Clinical Results for Constant Score and UCLA. 6 115 100 30 Score Pre – Op Post – Op p 7 95 84 20 Constant 33 49 0.05* 8 110 110 50 UCLA 9 22 <0.001* 9 110 100 40 F-FLE 94 115 0.62 10 75 55 0 ABD 68 90 0.32 11 95 60 30 ER 31 20 0.39 12 100 65 25 13 110 100 30 14 100 80 35 Table II. Clinical Results for Post operative Range of Motion Patient Constant Score Constant Score UCLA UCLA No. Pre Post Pre Post 1 17 67 6 25 2 20 56 4 21 3 82 76 24 35 4 8 67 4 35 5 38 66 7 33 6 18 54 5 24 7 41 55 20 19 8 81 39 5 11 9 43 46 13 27 10 4 15 4 8 11 10 40 6 15 12 15 14 4 4 13 67 44 5 26 14 15 45 6 23 RESULTS All fourteen patients were seen at clinics. The mean duration of follow up was 9.5 months [+/- 6 S.D.] with a DISCUSSION The length of follow up appeared to be sufficient to allow assessment of functional recovery. Mechanically, the intact rotator cuff has been found to be essential for the dynamic stability of the gleno-humeral joint through passive tension of the muscle bulk, compression of joint surfaces, and tightening of the static capsuloligamentous constraints (10). Destruction of the rotator cuff was the common handicap in the majority of the operated shoulders. The reverse total shoulder arthroplasty was used instead of an anatomic prosthesis for biomechanical considerations. The reverse implant medializes and lowers the center of joint rotation, thereby increasing the lever arm and deltoid muscle efficiency (11). Currently the indications for reverse total shoulder arthroplasty include a classic rotator cuff tear arthropathy, a massive irreparable rotator cuff tear with chronic loss of elevation of more than 6 months of duration that failed to respond to treatment with physiotherapy; posttraumatic glenohumeral arthritis with rotator cuff compromise; primary osteoarthritis with rotator cuff compromise and with severe glenoid bone loss and static posterior instability that prevented insertion of an unconstrained glenoid component; rheumatoid arthritis with rotator cuff compromise; an acute comminuted displaced proximal humerus fracture in an elderly patient; a shoulder girdle tumor requiring resection of all or a portion of the rotator cuff, creating rotator cuff compromise; and revision arthroplasty with rotator cuff compromise. The initial symptoms are mainly pain and loss of movement which results in a major functional deficit (12). 36 In our study we have shown that the reverse total shoulder replacement is a successful surgery, the mean improvement in the outcome scores have been significant in all patients, been the greatest improvement in the arthropathy groip. (Table II and Table III) The literature, acknowledges poorer results of anatomic total shoulder replacement when cuff deficiencies exist, especially when they are beyond reconstruction (13). Usually, pain relief is good after anatomic total shoulder replacement with a non-reconstructible rotator cuff, whereas success rates for functional improvements are lower (13), as we have demonstrated in our study. Epidemiblogical data reported a marked female preponderance of rotator cuff tears and a more frequent occurrence in the dominant shoulder (14). Based in UCLA and Constant scores, the irnjrovement in functionality and pain scores has been significant, as well as range of motion, but we did not find significant differences between them, Sanchez-Sotelo et al (15) showed in their comparative study between hemiarthroplasty and reverse total shoulder prosthesis, hemiarthroplasty provided satisfactory relief from pain in 75% of patients but only a moderate gain in movement after five year of follow up. By contrast, the gain in active forward flexion and abduction was better with the Grammont prosthesis than with hemiarthroplasties and bipolar prostheses. In the majority of studies, the range of active forward elevation was compatible with us where the average was ovet 1100 at follow-up in 70% of the patients. Other studies conducted in Europe have reported similar results in the short and medium term with use of a reversed or inverted shoulder implant. A multicenter study performed in Europe in which seventy-seven patients (eighty shoulders) with glenohumeral osteoarthritis and a massive rupture of the cuff were treated with the Delta-Ill prosthesis, described an improvement in the mean constant appear to be excellent, and the level of patient satisfaction also appears to be high. Overall, patients in that series had 37 points of improvement in terms of the Constant score and 510 of improvement in terms of active elevation, which are gains that are comparable with the findings described in previous reports (12). The same study showed that patients who were managed with a reversetotal shoulder arthroplasty for the treatment of posttraumatic arthritis or for a revision arthroplasty fared worse than patients with cuff tear arthropathy, primary osteoarthritis associated with a massive rotator cuff tear, or a massive rotator cuff tear alone. On the other hand, patients of the posttraumatic group had worse preoperative Constant scores and worse active elevation as compared with the other groups of patients. The external rotation score decreased slightly although this was not significant, but the other ranges of movement remained satisfactory. Using the rverse arthroplasty in acute trauma, Cazeneuve and Cristofari obtained anterior elevation of more than 1200 (6) Observation of the cases with the longest follbw up suggests that reverse arthroplasty in elderly patients sustaining a complex fracture of the upper humerus is an interesting option, because, like conventional replacement of the head, it provides excellent relief from pain and may also offer better and easier functional recovery than conventional arthroplasty in patients over 75 years of age (18). The present study had limitations. The retrospective design did not allow for a direct comparison between reverse total shoulder arthroplasty and other type of treatments like hemiarthroplasty. Also this is a short term report, which did show some improvement in addressing the problem of function and range of motion even on a joint that no longer has a rotator cuff (8). Due to the absence of long-term results, reverse shoulder total prostheses are mainly recommended for elderly patients and for the treatment of severe Table IV. Differences between outcomes by diagnostics. Outcome Diagnostic Mean S.E Sig. FF-Post Arthropathy Humerus Fracture Traumatic Cuff 134.38 90 106.7 31.2 13.2 7.8 .059 ABD post Arthropathy Humerus Fracture Traumatic Cuff 101.7 60 93.3 8.7 5 11.5 .000 ER post Arthropathy Humerus Fracture Traumatic Cuff 46 18.3 35 15.4 16 5 .048 Constant Post Arthropathy Humerus Fracture Traumatic Cuff 66 23 45 11.4 14 1 .001 UCLA post Arthropathy Humerus Fracture Traumatic Cuff 8.5 9 25 11 5.6 2 .018 score of 42 points, an increase of 650 in forward flexion, and minimal or no pain in 96% of the patients. However, fortynine patients (63.6%) were noted to have medial component encroachment and scapular notching without evidence of loosening (16). Bouttens and Nerot also reported excellent pain relief and good active mobility in 39 patients with rotator cuff tear arthropathy with an average 5 year follow up (17). In a study performed by Wall et al (12), the short term functional results of reverse total shoulder arthroplasty shoulder dysfunction (9). Wall et al. presented the largest series for this procedure up to date, which consists of 199 patients divided in a wide spectrum of etiologies with a two year follow up(8). They found that the reverse total shoulder arthroplasty prosthesis can produce good results with high patient satisfaction, when it used for the treatment of a number of other complex shoulder problems in addition to cuff tear arthropathy. The purpose of our study was to evaluate early outcomes of reverse total shoulder arthroplasty in primary osteoarthritis with either a rotator cuff tear, rotator cuff arthropathy three and four part humerus fractures and proxi. 37 mal comminuted displaced humerus fractures in a group of Hispanic population REFERENCES 1. Hawkins RJ, Switlyk P. Acute prosthetic replacement for severe fractures of the proximal humerus. Clin Orthop 1993; 289: 156-60 2. Goldman RT, Koval KJ, Cumomo F, Gallagher MA, Zuckerman JD. Functional outcome after humeral head replacement for acute three and four-part proximal humeral fractures. J Shoulder Elbow Surg 1995; 4: 81-6 3. Zyto K, Wallace WA, Frostick SP, Preston BJ. Outcome after hemiarthroplasty for three- and four- part fractures of the proximal humerus. J Shoulder Elbow Surg 1998; 7: 85-9 Early Outcomes of Reverse Total Shoulder Arthroplasty 10 4. Hettrich CM, Weldon EJ 3rd, Boorman RS, Prasons IM 4th, Matsen FA 3rd. Preoperative factors associated with improvements in shoulder function after humeral arthroplasty. J Bone Joint Surg Am. 2004; 816: 1446-1445 5. Goldberg S, Bell J, Kim H, Bak S, Levine W, Bigliani L (2008) Hemiarthroplasty for the rotator cuff deficient shoulder. J Bone Joint Surg Am. 2008; 90: 554-9 6. Grammont PM, Baulot E (1993) Delta shoulder prosthesis for rotator cuff rupture. Orthopedics 16: 65-68 7. Berth A, Pap G (2007) Hemi- versus bipolar shoulder arthroplasty for chronic rotator cuff arthropathy. International Orthopedics DOI 10.1007/s00264-007-0394-x 8. Wall B, Nove-Josserand L, O’Connor D, Edwards B, Walch G (2007) Reverse total shoulder arthroplasty: A review of results according to etiology. J Bone Joint Surg Am 2007; 89:1476-85 9. Guery J, Favard L, Sirveaux F et al (2006) Reverse total shoulder arthroplasty. Survivorship analysis of eighty replacements followed for five to ten years. J Bone Joint Surg Am 88: 1742-1747 10. Hsu HC, Luo ZP, Cofield RH, An KN. Influence of rotator cuff tearing on glenohumeral stability. J Shoulder Elbow Surg 1997; 6: 413-22 11. Rittmeister M, Kerschbaumer F. Grammont reverse total shoulder arthroplasty in patients with rheumatoid arthritis and nonreconstructible rotator cuff lesions. J Shoulder Elbow Surg 2001; 10: 17-22 12. Tiggie HF, Inglis AE, Goldberg VM, Ranawat CS, Figgie MP, Wile JM. An analysis of factors affecting the long term results of total shoulder arthroplasty in inflammatory arthritis. J Arthroplasty 1988; 3: 123-30 13. Cazaneuve JF, Cristofari DJ. Grammont reversed prosthesis for acute complex fracture of the proximal humerus in an elderly population with 5 to 12 years follow up. Rev Chir Orthop Reparatrice Appar Mot 2006; 92: 543-8 14. Bufquin T, Hersan A, Hubert L, Massin P. Reverse shoulder arthroplasty for the treatment of three and four part fractures of the proximal humerus in the elderly. J Bone Joint Surg (Br) 2007; 89-B: 516-20 15. Sirveaux F, Favard L, Oudet D, Huquet D, Walch G, Mole D. Grammont inverted total shoulder arthroplasty in the treatment of glenohumeral osteoarthritis with massive rupture of the cuff. J Bone Joint Surg (Br) 2004; 86-B: 388-95 16. Sanchez-Sotelo J, Cofield R, Rowland C. Shoulder hemiarthroplasty for glenohumeral arthritis associated with severe rotator cuff deficiency. J Bone Joint Surg (Am) 2001; 83-A: 1814-22 17. Frankle M, Siegal S, Pupello D, Saleem A, Mighell M, Vasey M. The reverse shoulder prosthesis for glenohumeral arthritis associated with severe rotator cuff deficiency. J Bone Joint Surg (Am) 2005; 87: 1697-1705 RESUMEN La diversidad de tratamientos para patología de manguillo rotador, fracturas de humero proximal y patología de la articulación glenohumeral a creado controversia en cuanto cual es el mejor manejo ortopédico. Técnicas reconstructivas como la hemiartroplastia, artroplastia total y la artroplastia total con protesis reversa han demostrado resultados variados, dependiendo de la selección de los pacientes. El propósito de este estudio es evaluar los resultados clínicos al tratar paciente de osteoartritis con patología del manguillo rotador y fracturas de humero proximal con artroplastia total reversa. Desde julio 2006 a febrero 2008 se evaluaron 14 pacientes que se sometieron a tratamiento utilizando la artroplastia total reversa de hombro Delta III (DePuy France, Saints Priest, France). Todos los pacientes fueron evaluados para la función de hombro pre-operatoria y post-operatoria, utilizando las escalas de función de hombro Constant & Murley y la escala UCLA, además de medidas cuantitativas de arco de movimiento y fuerza. Todos los pacientes fueron vistos en un tiempo promedio de 9.5 meses. En este tiempo se reporto una sola complicación, una dislocación reducida en el quirófano. En el estudio se demostró que la artroplastia total reversa de hombro es una técnica reconstructiva con resultados clínicos satisfactorios, evidenciado por los aumentos en las escalas de función de hombros observadas y arco de movimiento en las tablas II, III y IV. Asóciate B LETÍN ASOCIACIÓN MÉDICA DE PUERTO RICO El “Boletín” acepta para su publicación artículos relativos a medicina y cirugía y las ciencias afines. ¡Igualmente acepta artículos especiales y correspondencia que pudiera ser de interés general para la profesión médica. 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ARTICLES SHOULD BE ACCOMPANIED WITH A "CD" *The above 'Instructions to Authors" are according to the format required by the international Committee of Medical Journal Editors in its “Uniform Requirements for Manuscripts Submitted to Biomedical Journals”. ABSTRACT Introduction: Previous studies have reported that patients with the CHARGE association have congenital anomalies including: Coloboma; heart defects; choanal atresia, retarded growth and development; genital hypoplasia; and ear anomalies. Ocular findings in patients with the association include: poor visual acuity; anisometropia; myopic astigmatism; strabismus; microcornea; cataracts; staphyloma; and reduced stereopsis. Methods: We conducted a non-concurrent prospective study of 13 patients with coloboma from Puerto Rico. Results: Seven patients out of the 13 patients (53.8%) had the CHARGE association. Age ranged from 10 to 84 (mean = 38 years). Ocular findings in all the patients with coloboma include: nystagmus; strabismus; poor visual acuity; refractive errors; microcornea; cataracts; glaucoma; and dry eyes. Patients in our study had systemic findings such as: heart defects; ear anomalies; and endocrine problems. Conclusions: To our knowledge this is the first case series of patients with Coloboma and the CHARGE association reported in Puerto Rico and the Caribbean basin. Index words: coloboma, CHARGE association, Puerto COLOBOMA AND CHARGE ASSOCIATION IN PUERTO RICO 39 Javier Jardón BS* Natalio J. Izquierdo MD** * From the * UPR School of Medicine, University of Puerto Rico, Medical Sciences Campus, Rio Piedras, Puerto Rico and ** and Pediatric Ophtalmogist. Address reprints requests to: Natalio Izquierdo, MD 369 De Diego Street, Torre San Francisco Suite 310, San Juan PR 00923 Email Address: [email protected] INTRODUCTION P agon and co-workers (1) have reported that ocular coloboma is a spectrum of phenotypes that ranges from iris coloboma to clinical anophthalmos (1). Previous studies report that coloboma can affect the iris;(2)(3) lens;(4) choroid; optic disc;(5) macula;(5)(3) and retina (3). Complications associated to colobomas include: poor visual acuity;(6) strabismus;(6) microcornea;(6) cortical lens opacities;(6) choroidal detachment;(3) retinal detachment;(6) subretinal neovascularization;(7) retrobulbar cyst;(6) and microphthalmia.(6) Patients with coloboma may have craniofacial (Abbruzzo-Erickson Syndrome) and systemic anomalies.(8) On the other hand, previous studies (9) have reported that patients with the CHARGE association have congenital anomalies including: coloboma; heart defects; choanal atresia, retarded growth and development; genital hypoplasia; and ear anomalies. Mc Main and co-workers (10) reported that patients with the association have ocular findings including: poor visual acuity; anisometropia; myopic astigmatism; strabismus; microcornea; cataracts; staphyloma; and reduced stereopsis. Davenport and co-workers (11) proposed that CHARGE association is not just an association but a recognizable syndrome. Further, Pagon and co-workers (12) reported that parents of a child with the CHARGE association appear to have an increased risk of having a similarly affected child. In 2004, Vissers and co-workers (13) found a gene mutation associated with the syndrome in the long arm of chromosome 8 (8q12), the so called gene CHD7, which encodes for a chromodomain helicase DNA binding protein. Then, Lalani and co-worker (14) reported the SEMA3E mutation in a patient with CHARGE syndrome, encoded in chromosome 7q21. Even though Kallen and co-workers (16) suggest that CHARGE association occurs in 1/12,000 of the population, to our knowledge patients with coloboma or the CHARGE association have not been previously reported in the Caribbean basin. We report on 13 patients with coloboma and seven patients with the CHARGE association in Puerto Rico. MATERIALS AND METHODS We conducted a non-concurrent prospective study of 13 patients with coloboma. Patients were examined by at least one of the authors (NJI). Patients had a comprehensive ophthalmic examination. Further, patients were evaluated for systemic associations as part of the CHARGE association. Patients with two out of the six clinical characteristics were diagnosed with the CHARGE association. CASES OBSERVATIONS In our study, a total of 13 patients had coloboma. There were 9 female and 4 male patients. Age ranged from 10 to 84 years old (mean = 38 years). Seven out of the 13 patients with Coloboma (53.8%) had the CHARGE association. Ocular findings are summarized in Table I. Visual acuities ranged from no light perception to 20/20. Refractive errors ranged from -6.00 to +3.00 spherical equivalent (average = -1.44 sphere) in the right eye; and from -6.00 sph to +3.75 spherical equivalent in the left eye (average = -0.55 sphere). Three patients had pendular horizontal nystagmus, with small amplitude and high frequency. Two patients had strabismus. 40 One patient had a left exotropia and the other had been surgically treated prior to evaluation. Five patients had microcornea, with horizontal diameters ranging from 7.0 to 12.0 mm (average = 9.3 mm). Four patients had microcornea in both eyes and one patient had microcornea in one eye. Five patients had bilateral colobomas; and eight patients had coloboma in one eye (of these, four patients had coloboma in the right eye and four in left eye). Four patients out of thirteen had lens opacities (31.0%); two patients out of the thirteen had ectopia lentis (15.0%); and one patient one out of thirteen had surgical aphakia (8.0%). Two patients had glaucoma. One patient had a bilateral retinal detachment. One patient had a tilted optic nerve. Three patients had dry eyes. Only one patient had a family history of coloboma. Systemic findings are summarized in Table II. Seven out of the 13 patients (53.8%) with coloboma, had systemic findings as part of the CHARGE association. Five patients had heart manifestations as part of the association including: mitral valve prolapse; aortic root dilatation; and ventricle hypertrophy due to pulmonary hypertension. Three patients had ear anomalies such as: conical-shaped pinna; microtia; and one patient had hearing problems. One patient had endocrine manifestations including hypocortisolism and hypothyroidism. DISCUSSION Previous studies (17) (8) suggest that ocular coloboma may occur isolated or in association to several multisystemic syndromes. Isolated ocular colobomas have been associated with other ocular findings such as: microcornea; (18) pigmentary glaucoma; (19) or mild hypertension.(19) In our study, six patients had isolated coloboma. These patients had coloboma with ophthalmic findings including: strabismus, ectopia lentis, cataracts, glaucoma, microcornea and retinal detachment. Findings in our patients are compatible with reports in previous studies. Patients with isolated ocular coloboma share ocular findings with patients who had the CHARGE syndrome. These findings suggest a common pathophysiologic or genetic relationship with patients with the CHARGE syndrome. However, coloboma and CHARGE syndrome have been linked to different loci in chromosome 8. (20) Previous studies have reported that patients with the CHARGE syndrome have coloboma, heart defects, choanal atresia, retarded growth and development, genital hypoplasia, and ear anomalies (12). McMain and co-workers (10) described ocular findings in patients with CHARGE including: poor visual acuity; microcornea; myopic astigmatism; cataracts; and either unilateral or bilateral colobomas. Further, Aramaki and co-workers (21) describe iris’ findings either as isolated iris defects, or a spectrum of combined iris, retina, and optic disc findings. In our study, seven out of the thirteen patients with coloboma (53.8%) had systemic findings compatible with the CHARGE association. In our study, the six patients diagnosed with CHARGE syndrome had poor visual acuity; microcornea; myopic astigmatism; cataracts; unilateral or bilateral coloboma of iris and optic nerve; and one patient had a bilateral retinal detachment. Three patients had pendular nystagmus; and three patients had dry eyes. One patient had ectopia lentis. One patient had glaucoma. Even though in our study, patients with the CHARGE syndrome had characteristics previously reported,(10) (19) to our knowledge findings such as dry eyes, and ectopia lentis, have not been previously reported in patients with the CHARGE association. Metlay and co-workers (22) reported that patients with the CHARGE syndrome have coloboma associated to cardiac and/or ear defects. In our study, five patients had heart defects. Two patients had mitral valve prolapse. One patient had aortic root dilatation. One patient had ventricular hypertrophy due to hypertension. One of the patients had a previous heart surgery. Three patients had ear anomalies. Findings in our patients correlate with previous reports of systemic anomalies in patients with the CHARGE syndrome. Vogt and co-workers (2) have reported an association between isolated coloboma and hypothyroidism. In our study, one of the patients with isolated ocular coloboma had hypothyroidism and hypocortisolism. To our knowledge, hypocortisolism has not been previously reported in patients with isolated colobomas. Both of these metabolic disorders may occur in patients with colobomas due to a congenital midline defect. On the other hand, previous studies have reported endocrine complications as part of the CHARGE syndrome that include: growth hormone insufficiency; (23) hypogonadotropic hypogonadism; (24) and hypothyroidism (21). In our study none of the patients with the CHARGE syndrome was found with these anomalies. Previous studies have reported genetic mutations leading to the CHARGE syndrome. (13, 14, 25) In our study, one patient had a family member with the CHARGE association. The genetic mutation reported by Vissers and coworkers (13) or any other mutation as SEMA3E (15) could had been the cause of our only patient with family history of coloboma. The prevalence of CHARGE association has been reported by Kallen and co-workers (16) to be one out of 12,000 in the general population. In our study, there were six patients with the CHARGE association out of a population of approximately 4,000,000 people in Puerto Rico. These findings may be due to patients being undiagnosed by primary physicians. Limitations to our study include: that we compare our patients with historical controls; and that there are was no genetic linkage analysis for the CHD7 mutation leading to CHARGE syndrome in our patients. Further studies will lead to genetic evaluation of patients with coloboma and the CHARGE syndrome in Puerto Rico. In conclusion, this is the first report of a group of patients with coloboma and CHARGE syndrome in the Caribbean basin. To our knowledge, we reported new ophthalmic findings in patients with CHARGE syndrome as: glaucoma; ectopia lentis; and dry eyes. Further, this is the first report to our knowledge of a patient with isolated coloboma with hypocortisolism. Table I. Ophthalmic findings in patients with coloboma Ophthalmic Findings Number of Patients Table II. Systemic findings in patients with coloboma Systemic Findings Number of Patients Coloboma 13/13 Endocrine Abnormalities 1/13 Microcornea 5/13 Heart Defects 4/13 Cataract 4/13 Ear Anomalies 3/13 Dislocated lens 1/13 Ectopia lentis 1/13 Glaucoma 2/13 Strabismus 2/13 Nystagmus 3/13 Dry eyes 3/13 Detached retina 1/13 REFERENCES (1) 1- Pagon RA.: Ocular coloboma. Surv Ophthalmol, 1981; 25: 223-36. (2) 2- Vogt G, Puhó E, Czeizel AE.: A population-based case-control study of isolated coloboma, J Med Genet 2004; 41: 881-91. (3) 3- Daufenbach DR, Ruttum MS, Pulido JS, Keech RV.: Chorioretinal colobomas in a pediatric population, Ophthalmology 1998; 105:1455-8. (4) 4- Khan AO, Al-Assiri A.: Lens coloboma associated with ciliary body cyst. Ophthalmic Genet, 2007; 28: 208-9. (5) 5- Satorre J, López JM, Martinez J, Piñera P.: Dominant macular colobomata, J Pediatr Ophthalmol Strabismus 1990; 27: 148-52. (6) 6- Berk AT, Yaman A, Saatçi AO.: Ocular and systemic findings associated with optic disc colobomas, J Pediatr Ophthalmol Strabismus 2003; 40: 272-8. (7) 7- Rouland JF, Constantinides G.: Retinochoroidal coloboma and subretinal neovascularization. Ann Ophthalmol 1991; 23: 61-2. (8) 8- Adegbehingbe BO, Olabanji JK, Adeoye AO.: Isolated bilateral upper lid coloboma-a case report, Niger J Med 2005; 14: 224-6. (9) 9- Hall BD.: Choanal atresia and associated multiple anomalies, J Pediatr 1979; 95: 395–8. (10) 10- McMain K. Ocular features of CHARGE syndrome, J AAPOS 2008; 12: 460-5. (11) 11- Davenport SL, Hefner MA, Mitchell JA.: The spectrum of clinical features in CHARGE syndrome, Clin Genet 1986; 29: 298-310. (12) 12- Pagon RA, Graham JM, Zonana J, Yong SL. Coloboma, congenital heart disease, and choanal atresia with multiple anomalies: CHARGE association, J Pediatr 1981; 99: 223–7. (13) 13- Vissers LE, van Ravenswaaij CM, Admiraal R, et al.: Mutations in a new member of the chromodomain gene family cause CHARGE syndrome, Nature Genetics 2004; 36: 955–957. (14) 14- Lalani SR, Safiullah AM, Fernbach SD, et al.: Spectrum of CHD7 mutations in 110 individuals with CHARGE syndrome and genotype-phenotype correlation, Am J Hum Genet 2006; 78: 303-314. (15) 15- Lalani SR, Safiullah AM, Molinari LM, Fernbach SD, Martin DM, Belmont JW.: SEMA3E mutation in a patient with CHARGE syndrome, J Med Genet 2004; 41: e94. (16) 16- Källén K, Robert E, Mastroiacovo P, Castilla EE, Källén B.: CHARGE association in newborns: a registry-based study, Teratology 1999; 60: 334343. (17) 17- Gregory-Evans CY, Williams MJ, Halford S, Gregory-Evans K.: Ocular coloboma: a reassessment in the age of molecular neuroscience, J Med Genet 2004; 41: 881-891. (18) 18- Tesser PM.: An iris coloboma preventing pigmentary glaucoma, Arch Ophtalmol, 2003; 121: 1055-6. (19) 19- Hornby SJ, Adolph S, Gilbert CE, Dandona L, Foster A.: Visual acuity in children with coloboma: clinical features and a new phenotype classification system, Ophthalmology 2000; 107: 511-520. (20) 20- Asai-Coakwell M, French CR, Berry KM, et al.: GDF6, a novel locus for a spectrum of ocular developmental anomalies, Am J Hum Genet 2007; 80: 306-315. (21) 21- Aramaki M, Udaka T, Kosaki R, et al.: Phenotypic spectrum of CHARGE syndrome with CHD7 mutations, J Pediatr 2006; 148: 410-4. (22) 22- Metlay LA, Smythe PS, Miller ME. Familial CHARGE syndrome: clinical report with autopsy findings, Am J Med Genet 1987; 26:577-587. (23) 23- Asakura Y, Toyota Y, Muroya K, et al.: Endocrine and Radiological Studies in Patients with Molecularly Confirmed CHARGE Syndrome, J Clin Endocrinol Metab 2008; 93:920-4. (24) 24- Pinto G, Abadie V, Mesnage R, et al.: CHARGE Syndrome Includes Hypogonadotropic Hypogonadism and Abnormal Olfactory Bulb Development, J Clin Endocrinol Metab 2005; 90: 5621-6. (25) 25- Jongmans MC, Admiraal RJ, van der Donk KP, et al.: CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene, J Med Genet 2006; 43: 306-314. 41 RESUMEN Introducción: Estudios previos han reportado que pacientes con la asociación de CHARGE tienen anormalidades congénitas incluyendo: Coloboma; defectos cardiacos; atresia de las coanas, retardación en el crecimiento y el desarrollo, hipoplasia genital, y anormalidades en los oídos. En estos pacientes se pueden encontrar características oculares tales como: pobre agudeza visual; anisometropia; astigmatismo miope; estrabismo; microcornea; cataratas; estafiloma y disminución en la estereopsis. Materiales y Métodos: Nosotros llevamos a cabo un estudio no-prospectivo de 13 pacientes con coloboma en Puerto Rico. Resultados: Siete pacientes de trece pacientes (53.8%) tenían la asociación de CHARGE. La edad de estos pacientes estaba entre los 10 a 84 años (media = 38 años). Los hallazgos oculares en todos los pacientes con Coloboma incluían nistagmos; estrabismo; pobre agudeza visual; problemas refractivos; microcornea; cataratas; glaucoma y ojos secos. Los pacientes en nuestro estudio presentaban con hallazgos sistémicos como defectos cardiacos, anormalidades en los oídos, y problemas endocrinológicos. Conclusión: Para nuestro conocimiento este es la primera serie de casos con Coloboma y asociación de CHARGE reportado en Puerto Rico y el Caribe asociacionmedicapr.org 43 ABSTRACT Background: Laparoscopic splenectomy has advantages over conventional surgery mainly related to the smaller incision, less postoperative pain and shorter recovery period. The aim of this study was to assess the outcomes of patients with diseases of the spleen managed by elective laparoscopic splenectomy. Methods: Retrospective review of medical charts was performed of patients who underwent laparoscopic splenectomy by a single surgeon during a three year period. Patient demographics, diagnosis and outcomes including operative time, weight of the specimens, blood loss, operative complications, length of stay, and long term outcome with platelet counts in Immune Thrombocytopenic Purpura (ITP) were reviewed. Data on the presence of an accessory spleens, and need for use of hand assist devices was reviewed. Results: Fourteen patients underwent laparoscopic splenectomy; 9 (nine) for Immune Thrombocytopenic Purpura(ITP); one for Evans Syndrome, one for splenic artery aneurysm, two for suspected lymphoma and one for suspected metastatic disease. Ages were 22 to 70 years (mean 46.6). All patients underwent surgery in a full lateral position. Eight specimens were morcellated for removal and 6 were removed intact; 4 using a hand assist device and two by extending one of the port incisions. In 10 patients, total laparoscopic splenectomy was completed; four patients required use of a hand assist device due to difficulty with the operation. Mean spleen weight was 127 gms; spleen weight for ITP specimens was 90 gms; for non ITP diagnosis mean weight was 230 gms. Accessory spleens were identified and excised in two patients. Mean operative time for all patients was 137 minutes. Mean operative blood loss was 202 ml; no patients were transfused as a result of perioperative bleeding. Time to start diet was from 1-2 days and length of stay was 2.9 days. Two patients had post op fevers requiring prolonged length of stay. There were two readmission for deep venous thrombosis, and one for brain toxoplasmosis six weeks post op. Two ITP patients older than 40 years relapsed during the first year. Conclusions: Laparoscopic splenectomy can be safely performed using the lateral approach with a high success rate, low rate of blood loss and a low rate of perioperative complications. Laparoscopic splenectomy should be offered to all patients undergoing surgery for ITP. Use of hand assist devices can facilitate surgery for larger spleens and avoid need of conversion to open surgery. Accessory spleens can be adequately identified and excised using laparoscopic splenectomy. Hematologic response in ITP is adequate with laparoscopic splenectomy. Index words: laparoscopy, splenectomy, adults, safety, lateral, approach EFFICACY AND SAFETY OF LAPAROSCOPIC SPLENECTOMY: REVIEW OF 14 ADULT CASES USING THE LATERAL APPROACH Ramón K. Sotomayor-Ramírez, MD From the * Department of Surgery Hospital Interamericano de Medicina Avanzada, Caguas Puerto Rico. Address reprints requests to: Ramón K. Sotomayor-Ramírez, MD - Director Oncology Surgery, Hospital HIMA San Pablo Caguas, # 500 Avenida Degetau, Suite 500, Caguas, Puerto Rico 00725. Email: [email protected] Poster presented during the 58th Annual Meeting American College of Surgeons, Puerto Rico Chapter, February 2008, La Concha Hotel, San Juan, PR. INTRODUCTION T he goal of laparoscopic splenectomy is to offer a minimally invasive approach that decreases postoperative pain, recovery period, wound complications and earlier return to normal activity. Laparoscopic splenectomy is the most commonly performed solid organ procedure and can be technically demanding (1-3). Exposure of the spleen in the left upper abdomen can be problematic because of its protected location, particularly in obese patients. Control of the blood supply of the short gastric vessels, polar vessels and the splenic hilum can be challenging. There is proximity to the stomach, colon and pancreas. There must be adequate exposure to mobilize and divide the attachments to the diaphragm. In addition, there is the issue of the size of the spleen; whereas surgery for normal sized spleens as are seen in Immune Thrombocytopenic Purpura (ITP) can be relatively straightforward, surgery in larger spleens can be very difficult due to the weight and size of the spleen, with increased probability of need for conversion to open surgery. As in all laparoscopic procedures, problems related to exposure, bleeding and inability to complete the procedure in the past were dealt with conversion to open surgery. Two developments have made this unnecessary in the majority of cases. One is the development of the lateral approach to splenectomy (4-7). The “Hanged Spleen Technique” takes advantage of gravity for exposure of the spleen, makes exposure of the colon, pancreatic tail, division of the short gastric vessels easier than the anterior approach; there is no need to pull on the spleen for exposure as it “hangs“ naturally from its splenophrenic attachments, and there is a direct approach to the splenic hilum. 44 46 Alternatively, the spleen may be removed intact by making a small subcostal incision or using a Hand Assist Device if it was used for the dissection (see Figure 4). Post operative care loss of 114 ml; and patients that required use of hand assist devices for dissection and completion of the operation had a mean EBL of 466 ml. No patients required transfusion due to perioperative blood loss. None of the patients required another procedure for a complication. Patients are admitted to the intermediate care unit overnight for monitoring. Nasogastric tubes are not routinely used, oral diet and ambulation is started on post op day 1; discharge is usually possible on post op day 2. Two patients had accessory spleens identified and excised; one in the splenocolic ligament and one in the hilum. Time to start diet was 1.5 days; mean length of stay was 2.9 days. Two patients had postoperative fever due to Figure 4- Relationship of the spleen in relation to the extraction incision shown in the background RESULTS There were 14 patients between the ages of 22 and 72. In all patients, laparoscopic dissection was planned, with hand assist devices available for backup. Most patients underwent surgery for ITP. Other diagnoses were Evans Syndrome (autoimmune hemolytic anemia with thrombocytopenia), one for a 2 cm splenic artery aneurysm, two for suspected lymphoma due to multiple lesions in the spleen. Final pathology exam in one patient was casseating granulomas; the second suspected lymphoma case underwent lap splenectomy for splenomegaly and a mass was found on CT and MRI; final path was negative for lymphoma (Table 1). One woman underwent surgery due to suspected metastatic disease from cervical cancer, after a PET scan showed an FDG avid lesion. Final pathology was negative. Eight of nine ITP spleens were morcellated and the other 6 patients had extraction of intact specimens. Table 2 lists the operative outcomes. Mean operative time was 137 minutes. ITP spleen weight was 90 grams as opposed to non ITP spleens whose mean weight was 230 grams. Mean estimated blood loss was 202 ml. Ten patients had a complete laparoscopic splenectomy and 4 patients required hand assistance. Patients in which complete laparoscopic excision was completed had a mean estimated blood atelectasis and bronchitis. There were three readmissions; two for deep venous thrombosis, and one 42 year old male who had a good response to splenectomy due to ITP was readmitted after 6 weeks for central nervous system symptoms, headache and fever. Workup showed brain toxoplasmosis due to undiagnosed Acquired Immune Deficiency Syndrome and died after a prolonged hospital stay. Table 3 details the profile of the patients who required use of a hand assist device. In three of the non ITP patients plans were to complete the dissection laparoscopically, and remove the intact specimen by extending one of the incisions. All proved more difficult mainly due to adhesions, difficulty with isolation of the pedicle, and bleeding. Most were larger than ITP spleens. Rapid use of a hand assists device (lap disc or Dextrus), facilitated completion of the operation, exposure of the pancreatic tail, control of the hilum prior to division and intact extraction. The one ITP patient which required HALS, was a 68 year old male with diabetes. Dissection was very tedious due to adhesions of the splenic capsule to the pancreatic tail; this was the patient with the highest blood loss. It was completed uneventfully with hand assistance with no adverse outcome. Long term results of laparoscopic splenectomy All nine patients with immune thrombocytopenic. Table1. Demographics and operative indications for laparoscopic splenectomy # Gender Male Female Age Diagnosis ITP* Evans Syndrome** Splenic artery aneurysm Lymphoma*** Suspected mets**** * ** *** **** 5 7 42.5 years (mean) ( 22 to 70 years) 6 pts <40 years; 8 pts> 40 years 9 1 1 2 1 ITP immune thrombocytopenic purpura; Evans Syndrome- autoimmune hemolytic anemia with thrombocytopenia dependent on plasmaphere sis; Suspected lymphoma, final path bening disease; Solitary lesion on PET scan history of cervical can cer 47 Table 2. Operative characteristics and immediate outcome of laparoscopic splenectomy. Operative time (minutes) Weight of specimens (grams) All Non ITP spleens (5) ITP spleens (9) Complete laparoscopy excision Hand Assist Device Required* Estimated blood loss (ml) All spleens Laparoscopy (8/14) HALS (4/14) Time to start diet (days) Length of postop stay (days) Accessory spleens identified and removed Complications w readmission After one year follow up, a 57 year-old-woman with ITP developed recurrent thrombocytopenia requiring steroids and two admissions for platelet transfusions. 127 (54-349) 230 (136-349) 90 (54-153) 10 patients 4 patients 202 114 466 1.5 2.9 (30-600) (30-250) (350-600) (1-3) (2-6) 2 patients Deep venous thrombosis 2 Brain abscess (HIV Toxoplasmosis)** ITP - immune thrombocytopenic purpura; HALS - hand assisted laparoscopic surgery; HIV - human immunodeficiency virus; * Profile of patiens in wich hand assist was required detailed in table 3; ** Adequate response to splenectomy, readmission 6 weeks with headache, CNS symptoms died of brain toxoplasmosis. purpura had adequate initial response to laparoscopic splenectomy, with platelet counts > 150,000 on office follow up and no readmissions to the hospital. The patient with Evans Syndrome was a 72 year old male with coronary artery disease, and previous history of non Hodgkin Lymphoma. He had been admitted on multiple occasions for blood transfusions and platelet transfusions. On his last admission, he had been dependent on plasmapheresis. After splenectomy, he has remained plasmapheresis and transfusion free after one year. Mean Range 137 (95-225) Work-up with liver spleen scans have been negative for accessory splenic tissue. The 68 year-old-male with ITP was lost to follow up, and readmitted 11 months later with recurrent thrombocytopenia, CNS bleeding and died. DISCUSSION In all ITP patients except one, surgery was uneventful and completed laparoscopically in less than two hours, with no need for conversion to open surgery, no procedure Table 3. Profile of patients undergoing laparoscopic splenectomy which required completiom with use of a hand assist device.* Diagnosis Age Spleen weight Reason for HALS Blood loss Outcome ITP 106 g Splenic capsule adhesions difficult 600 ml fever bronchitis 68 hilar dissection los**6d Evans Splenic capsule adhesions difficult hgb drop 7g 70 136 g 450 ml Syndrome hilar dissection no transfusion Aneurysm Difficult isolation aneurysm Post op fever; atelecta- 51 207 g 350 ml splenic artery sis - los 4 days r/o lymphoma 72 los 2 days 349 g Adhesions between spleen 250 ml and liver, difficult hilum * Dextrus disc utilized for hand assistance and extraction of intact specimen; ** los - length of stay 48 related complications and a short length of stay. ITP spleens had mean weight of 90 grams and surgery with meticulous technique was straightforward. ITP spleens usually have a normal size and consistency. This suggests that the ideal operation for ITP should be laparoscopic splenectomy instead of an open approach. Blood loss was relatively low; 114 ml for the ITP spleens, 466ml for the larger specimens, and 202 ml overall. This is difficult to compare to historical series of open surgery and in fact to laparoscopic surgery. However, a Cleveland Clinic study of 147 patients that looked at outcome based on hematologic indication found an estimated blood loss of 130 ml in ITP. In this same series, blood loss in non ITP diagnosis such as benign and malignant tumors was significantly, higher up to 650 ml (9). Our blood loss in larger more difficult cases was lower. This is supported by the fact that none of our patients required a blood transfusion in the perioperative or postoperative period. Recovery in all of the patients was dramatically shorter than the expected stay in open surgery, 2.9 days, with two patients developing respiratory complications such as atelectasis and bronchitis which made average los longer. While we did not directly compare length of stay to open surgery, average length of stay for open surgery varies from 4-7 days; laparoscopic splenectomy averages 2-4 days (3). All of the laparoscopic splenectomy patients left the hospital faster and were pain free on office follow-up. There were no wound related problems such as hematomas, seromas or wound infections which traditionally complicate open surgery. There were five other indications for splenectomy and all showed larger spleens. Most authors agree that as spleen size increases, so does the complexity of the surgery, with larger blood loss, longer operative times, and increase rate of conversion to open surgery (1-3). All non-ITP spleens in this series were about twice the size of ITP spleens, and surgery was more difficult. This was dealt with use of a hand assist device; which made the use of conversion to open surgery unnecessary. In the cases which required HALS, most of the procedure had been completed and HALS allowed completion of one or two final steps of the operation. In the splenic artery aneurysm, proximal dissection of the artery was completed with hand assistance; most of the dissection was already done, but it was difficult to place the stapler. In the only ITP patient which required HALS, the patient with Evans Syndrome, and one of the suspected lymphoma patients, the vascular pedicle was very thick, the vessels were large and it was not clear whether a stapler could be safely placed and fired. Isolation of the pedicle of the hilum with the left hand allowed safe placement of the vascular stapler and safe completion of the operation. Also, intact specimens were required, so these patients would have needed an incision anyway to extract the specimen. Our largest spleen was 349 grams. While the definition of splenomegaly is not clear, with some authors classifying splenomegaly as greater than 300 g and some greater than 500 g (7) , there seems to be consensus that hand assisted laparoscopic technique should be available and used to decrease the rate of conversion to open surgery (2). None of the patients underwent preoperative splenic artery embolization. One controversial issue in laparoscopic splenectomy is whether or not accessory spleens can be identified and excised laparoscopically (2, 7). Two of the nine ITP patients had accessory spleens identified and removed. The locations were the splenocolic ligament and one in the hilum of the spleen. Both were carefully dissected and excised without rupture or increased operative time. Search in the most common areas of accessory spleens; the splenic hilum, vascular pedicle, pancreatic tail and omentum can be performed laparoscopically; other unusual areas such as the small bowel mesentery and broad ligament are not usually explored with open surgery so there is no need to do this laparoscopically (3). In terms of long term outcome, if the spleen is excised intact there should be no difference in long term response to splenectomy, particularly as it relates to ITP. The main predictor of response in ITP is age with patients older than 40 years-old having a higher failure rate (8). Two of 5 patients older than 40 years relapsed in less than one year, while all 4 patients younger than 40 maintain an adequate platelet count and are steroid free. There are several controversies not addressed in this paper. No case was performed for massive splenomegaly (weight > 1000g), nor is the issue of the need for preoperative splenic artery embolization prior to splenectomy addressed. This has been advocated as a way of decreasing the volume of the spleen, but not routinely done. One potential limitation of the lateral approach is that it restricts the access to the rest of the abdomen in case another procedure is needed. Sickle cell and congenital spherocytosis cases may require a cholecystectomy. If the lateral approach is used, then the patient would require repositioning and re-draping prior to the second procedure. CONCLUSIONS Laparoscopic splenectomy using the lateral approach can be performed with a high success rate, low blood loss and low complication rate. In normal sized spleens, complete laparoscopic excision is possible in most cases, while larger spleens are more difficult. Laparoscopic surgery should be considered and offered to all patients undergoing splenectomy for immune thrombocytopenic purpura. In larger spleens, use of hand assist devices can facilitate completion of the operation and avoid need for conversion to open surgery. Accessory spleens can be adequately identified in most cases using a laparoscopic approach. REFERENCES 1. Brodsky JA, Brody FJ, Walsh RM, Malm JA, Ponsky JL. Laparoscopic Splenectomy. Experience with 100 cases. Surgical Endoscopy (2002) 16: 851854 2. Habermalz B, et al. Laparoscopic Splenectomy: The clinical practice guidelines of the European Association of Endoscopic Surgery (2008). Surgical Endoscopy 22: 821-848. 3. Glasgow R, Mulvhill SJ. Laparoscopic Splenectomy. World J. Surgery (1999) 23, 384-388. 4. Targarona EM Laparoscopic splenectomy for splenomegaly: anterior and posterior approach using the “hanged technique”. Epublication: Websurg.com May 2007:7(5). 5. TriasM, Taragarona EM, Balague C. Laparoscopic Splenectomy: an evolving technique. Surgical Endoscopy (1996) 10: 389-392. 6. Park, A. Lateral Approach to Laparoscopic Splenectomy. In Surgical Laparoscopy, 2nd Ed, (2001) Karl Zucker, Editor p 625-635. 7. TriasM, Targarona EM, Espert JJ, Balagué C. Laparoscopic surgery for splenic disorders, lessons learned from 64 cases. Surgical Endoscopy (1998) 12: 66-72. 8. Katkhouda S, et al. Predictors of response after laparoscopic splenectomy for immune thrombocytopenic purpura. Surgical Endoscopy (2001) 15: 484488. 9. Rosen, M et al. Outcome of laparoscopic splenectomy based on hematologic indication. Surgical Endoscopy (2002) 16: 272-279. RESUMEN Trasfondo: La esplenectomia laparoscópica ofrece ventajas sobre la cirugía abierta debido al uso de heridas más pequeñas, menos dolor post operatorio y un periodo más corto de recuperación. El propósito de este estudio es evaluar los resultados en pacientes con enfermedades del bazo, manejados con esplenectomía laparoscopica electiva. Método: Se llevó acabo una revisión retrospectiva de los archivos de hospitalización y de seguimiento en la clínica de los pacientes a quienes se les practicó esplenectomias electivas durante un periodo de tres años, por un solo cirujano. Se revisaron datos demográficos, diagnósticos y resultados incluyendo tiempo de cirugía, peso de especímenes, pérdida de sangre, complicaciones post operatoria y estadía en el hospital. El resultado a largo plazo en términos de contajes de plaquetas en pacientes de púrpura trombocitopénica idiopática fué evaluado, al igual que la habilidad para identificar y remover bazos accesorios y la necesidad de utilizar cirugía laparoscópica asistida con la mano. Resultados: Catorce pacientes fueron sometidos a esplenectomía laparoscópica; nueve por Purpura Tromocitopénica Idiopática (PTI), 1 por aneurisma de la arteria esplénica, dos por sospecha de linfoma, 1 por Sindrome de Evans, y dos por sospecha de enfermedad metastática. La edad promedio fué de 46 años. Todos los pacientes se operaron en posición lateral completa. Ocho especimenes fueron “morcelados” para remoción y 6 fueron removidos intactos. En 10 pacientes, la cirugía fue completada laparoscopicamente y en 4 se utilizó la técnica de laparoscopía asistida con la mano debido a dificultad con la cirugía. El peso promedio para los bazos con PTI fué de 127 gramos, y el de otros diagnósticos 230 gramos. Se identificaron y se removieron dos bazos accesorios. Tiempo operatorio promedio fué de 137 minutos. Pérdida promedio de sangre fué 202 ml; no hubo necesidad de transfusions debido a sangrado operatorio. Se comenzó dieta en 1-2 dias y la estadía promedio fué de 2.9 dias. Diez pacientes tuvieron estadias de 2 dias; 2 pacientes tuvieron fiebres postoperatorias requiriendo estadías más largas. Hubo dos readmisiones debido a tromboflebitis, y un paciente se readmitió con Toxoplasmosis luego de seis semanas de su operación. Dos pacientes con PTI mayores de 40 años tuvieron recurencia de su enfermedad en el primer año. Conclusiones: La esplenectomía laparoscópica se puede llevar a cabo de forma segura y efectiva utilizando el abordaje lateral con tiempo corto de cirugía, pérdida de sangre baja, una taza baja de complicaciones, al igual que una estadía corta en el hospital. El uso de la técnica laparoscopica asistida con la mano puede facilitar la cirugía en casos de bazos más grandes y así evitar la conversión a cirugía abierta. Bazos accesorios se pueden identificar y remover laparoscópicamente, y la respuesta hematológica en PTI es adecuada con cirugía laparoscópica. You don’t need a new one... ...you need a better one. Ahorre dinero y obtenga recompensas, mientras ayuda a subsidiar y presta apoyo a la Asociación Médica de Puerto Rico. Solicite la tarjeta de crédito de la Asociación Médica de Puerto Rico MasterCard® y obtenga una gran oferta introductoria baja de APR† recompensas WorldPoints® que le permiten obtener dinero en efectivo y mercancía, y un Certificado de Boleto Aéreo 2-en-1 luego de transacciones que reúnan los requisitos¨. Aplique en www.asociacionmedicapr.org 50 Review Articles (Articulos de Reseña) ANEMIA AND INFECTIONS IN MULTIPLE MYELOMA: SUPPORTIVE THERAPY William Cáceres MD* Karen Santiago MD* Liza Paulo MD* Jaime Román MD* From the * Hematology-Oncology Section, VA Caribbean Healthcare System, San Juan, Puerto Rico. Address reprint request to: William Caceres MD, VA Caribbean Healthcare System, 10 Calle Casia, San Juan , Puerto Rico 00921. E-mail: [email protected] ABSTRACT Patients with multiple myeloma (MM) have a high rate of infectious complications and symptomatic anemia. Emerging antineoplastic therapies are resulting in a better response, but still quality of life and potentially survival can be significantly affected by these complications. Multiple cytokines and an impaired immunologic system are involved in the development of these complications. Emerging therapies and guidelines will be reviewed taking in consideration the benefits versus risks of those strategies. The beneficial effect of recombinant human erythropoietin (EPO) and darbepoietin alpha in MM patients have been confirmed in several trials. Evidenced-based guidelines for the use of EPO in patients with cancer should be closely followed to ensure correct use of erythropoiesis stimulating agents. Infection, despite being the most common cause of morbidity and mortality, can be controlled by prompt recognition and proper prophylaxis.Supportive care is an important adjunct to cancer therapy. Adequate use of pharmacologic strategies to control anemia and prevent or treat infectious complications will result in benefit to MM patients. Index words: anemia, infections, myeloma, multiple, therapy INTRODUCTION M ultiple myeloma (MM) is a clonal B-cell disorder in which malignant plasma cells expand and accumulate in the bone marrow leading to cytopenias, bone resorption, and the production in most cases of a characteristic monoclonal protein (1). Multiple myeloma accounts for more than 10% of all hematologic malignancies and is the second most frequent hematologic cancer in the United States after NonHodgkin’s lymphoma, with approximately 15,000 new cases per year (2). The median age is approximately 65 years, although occasionally myeloma occurs in the second decade of life. More than two thirds of patients with multiple myeloma are affected by anemia with 35% of patients having an hemoglobin less than 9 g/dl on diagnosis (3). Anemia is associated with loss of quality of life and is a poor prognostic factor. The etiology of anemia in multiple myeloma is multifactorial with bone marrow replacement, relative endogenous erythropoietin deficiency, renal failure, disregulated cell apoptosis, vitamin B-12 and folate deficiency and rarely autoimmune hemolytic anemia as possible causes (4, 5). Bleeding complications, increased plasma volume and secondary development of a myelodysplastic syndrome are other attributed causes for anemia in these patients. Infection is the major cause of morbidity and mortality in multiple myeloma. Its major cause is impaired antibody production due to a decrease in uninvolved immunoglobulins but several other factors such as defective optimization, deficient complement activity, decrease in granulocyte adhesiveness, impaired leukocyte migration and treatment with corticosteroids contribute to this complication (6). We will discuss these two common complications in multiple myeloma and emerging therapies and controversies for their management. Pathophysiology of anemia in multiple myeloma In addition to bone marrow failure and decrease in endogenous erythropoietin, several cytokines have recently been described as contributing to low hemoglobin levels in MM patients. The overexpression of the Fas ligand, interleukin-6, tumor necrosis factor- related apoptosis inducing ligand (TRAIL), and macrophage inhibitory protein 1-alpha (MIP-1 alpha) in the bone marrow microenvironment trigger death of immature erythroblasts (7). Silvestris and others studied erythroblasts in 28 untreated MM patients and found that an abnormal upregulation of apoptogenic receptors by myeloma cells induced ineffective erythropoiesis and chronic exhaustion of the erythroid matrix. Also, there is an abundant production of interleukin-1B (IL-1B) and tumor necrosis factor beta (TNF-B) producing low erythropoietin levels relative to the degree of anemia (anemia of chronic inflammation) (8). Management of anemia The beneficial effect of recombinant human erythropoietin (EPO) and darbepoietin alpha in MM patients and in patients with lymphoid malignancies have been confirmed in several trials (9-11). EPO has shown to reduce transfusion requirements, improve quality of life and performance status in symptomatic anemic patients (Figure 1). The most common causes of failure to respond to EPO are functio nal iron 51 In general, treatment of anemia in MM patients must be individualized taking in consideration risks versus benefits. Reversible causes such as iron, B12 or folate deficiency must be corrected before the use of EPO. EPO has shown beneficial effects in patients with symptomatic anemia receiving chemotherapy but if a more rapid effect is needed, transfusion therapy is indicated. In advanced MM patients, the use of chemotherapy, steroids or IMiDs such as thalidomide and lenalidomide is highly effective resulting in an improvement in the hemoglobin level in responding patients Infectious complications in MM Figure 1. Kaplan-Meier plots of transfusion-free survival during 16 weeks' treatment with either epoetin beta or placebo in anemic, transfusion-dependent patients with MM, NHL, or CLL. P=0.0012 Walt x2 test (reference 10) deficiency, infection, surgery, and advanced disease with extensive plasma cell bone marrow involvement. However, due to recent data of adverse outcomes associated with erythropoietin-stimulating agents in patients with cancer (head and neck, metastatic breast and lung cancer), the FDA had a new mandated label for the use of EPO (12). Several of the trials, which had a target hemoglobin level over 12 g/ dl, demonstrated a shorter overall survival, higher progression and an increased risk of thrombotic events in patients using EPO. The FDA does not recommend use of EPO for patients receiving myelosuppressive chemotherapy if the anticipated outcome is cure and should not be initiated for an hemoglobin greater than 10 g/dl. An American Society of Hematology and Clinical Oncology consensus conference in year 2007 recommended to begin therapy with steroids and/ or chemotherapy for anemic patients with multiple myeloma (Stage III) and to observe hematologic outcomes before using EPO. The major concern about EPO use and thrombosis arose in the context of the immune modulatory drugs (IMiDs), thalidomide and lenalidomide However, the use of packed red blood cell transfusions have as potential adverse outcomes the development of transfusion reactions such as hemolysis , lung injury (TRALI), worsening of congestive heart failure, viral or bacterial contamination or iron overload. Both the transfusion of blood products and EPO improve fatigue and quality of life but the transfusion effect upon symptoms is more rapid. Mittelman and others demonstrated that EPO induces an antitumor effect in a multiple myeloma mouse model, presumably mediated by activation of CD8 - positive T cells (13). Although the erythropoietin receptor mRNA is expressed in lymphoid malignancies such as chronic lymphocytic leukemia, mantle cell lymphoma and multiple myeloma, there is no surface expression of the erythropoietin receptor in the malignant cells or stimulation in vitro (14). Data from animal studies and case reports suggest that EPO has antineoplastic properties in MM. Thus, EPO therapy likely is safe in patients with lymphoid malignancies. Investigators from the Cleveland Clinic, evaluated 257 MM patients from years 1997-2003, and the use of EPO resulted in an overall survival benefit (15). Infection is a major cause of morbidity and mortality in MM patients, especially in patients with more advanced/ relapsed disease or hospitalized for acute care. 15% of patients present with a bacterial infection and the rate of infection is higher within two weeks of initiation of treatment (6). The major cause of this complication is impaired antibody production due to a decrease of uninvolved immunoglobulins. Corticosteroids, a mainstay in the treatment, leds to transient T-cell sequestration, diminished synthesis of immunoglobulins and a decreased adherence and degranulation of neutrophils (3). Cytotoxic chemotherapy causes a variable decrease in number and function of T- cells, Bcells and granulocytes. Bortezomib, a highly active therapy which functions as a proteasome inhibitor, has shown a 13% rate of herpes zoster reactivation, for which prophylaxis is recommended (16). The most common pathogens are those that depend on humoral cytotoxicity, such as encapsulated organisms Streptococcus pneumonia and H. influenza. The respiratory tract is a common site of infection but also urinary tract infections with gram negative bacteria such as E. coli, Klebsiella sp ,Enterobacter and Pseudomonas are frequent, especially in patients undergoing active therapy. Other organisms, such as anaerobes, M. tuberculosis, fungal infections, are uncommon unless after transplantation. The use of antibiotic prophylaxis is controversial, but can be indicated in those patients at high risk like those that had started treatment (especially with high dose steroids) or have renal impairment. Routine use of penicillin prophylaxis increases resistance and generally is not recommended. The use of intravenous immunoglobulin replacement is generally not recommended except for recurrent life threatening infections in patients with documented hypogammaglobulinemia (17). Pneumococcal vaccination and the H. influenza vaccine are recommended, but many patients do not develop adequate antibody titers. Early diagnosis of infection and prompt initiation of broad spectrum antibiotics are critical in patients with MM. Third generation cephalosporins or extended spectrum penicillins are used most frequently. The antibiotic choice will be determined by the local flora and by the patterns of antibiotic resistance at each institution. CONCLUSIONS Patients with MM have a higher risk of infections and anemia is a predominant feature of the disease (Figure 2). Cytokines play a major role in the development of complications in addition to tumor burden. Supportive care is an important adjunct to cancer therapy. 52 Pharmacologic supportive care measures have also become a mainstay of therapy, but the current use of some of these agents such as erythropoietin stimulating agents have recently been brought into question because of concerns of safety. The American Societies of Hematology and Clinical Oncology have provided evidenced-based guidelines for the use of EPO in patients with cancer which should be closely followed for the benefit of MM patients. Judicious use of proper antibiotics and vaccines and prompt recognition of infections in MM patients is essential to decrease morbidity and mortality in these patients. Adequate use of these strategies will improve quality and quantity of life to these patients as more effective antineoplastic therapies are available to practicing physicians. Figure 2. Major clinical and laboratory features of multiple myeloma (reference 8). REFERENCES 1. Fonseca R, San Miguel J. Prognostic factors and staging in multiple myeloma. Hematol Oncol Clin N Am. 2007; 21:1115-1140. 2. Bartlogie B, Shaughnessy J, Epstein J, et al. Plasma cell myeloma In: Lichtman M, Beutler E, Kaushansky K. Williams Hematology:7th ed. McGrawHill Companies;2006:1501. 3. Bladé J, Rosiñol L. Complications of multiple myeloma. Hematol Oncol Clin N Am. 2007; 21:1231-1246. 4. Mecharchand J. Management of haematological complications of myeloma. In: Malpas JS, Bergsagel DE, Kyle RA ,et al. Myeloma:biology and management.2nd ed. Oxford: Oxford University Press:332-57. 5. Berguin Y, Yerna M, Loo M, et al. Erythropoiesis in multiple myeloma: defective red cell production due to inappropriate erythropoietin production. Br J Haematol 1992;82:648-53. 6. Kelleher P, Chapel H. Infections: principles of prevention and therapy. In: Metha J, Singhal S. Myeloma . London: Martin Dunitz Ltd; 2002:223-39. 7. Silvestris F, Cafforio P, Tucci M, Damacco F. Negative regulation of erythoroid maturation by Fas-L(+) highly malignant plasma cells: a major pathogenetic mechanism of anemia in multiple myeloma. Blood 2002;99(4):1305-13. 8. Tricot G. Multiple myeloma and other plasma cell disorders. In: Hoffman R, Benz E, Shattil S, et al. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa. Elsevier Churchill Livingstone; 2005:1511. 9. Garton JP, Gertz MA, Witzig TE, et al. Epoietin alfa for the treatment of anemia of multiple myeloma. A prospective,randomized, placebo-controlled, double-blind trial. Arch Intern Med 1995;155:2069-74. 10. Osterborg A. et al. Randomized, double-blind, placebo-controlled trial of recombinant human erythropoietin, epoetin Beta, in hematologic malignancies. J Clin Oncol 2002; 20:2486-2494. 11. Dammacco F, Castoldi G, Rodjer S. Efficacy of epoietin alfa in the treatment of anaemia of multiple myeloma. Br J Hematol 2001;113:172-9 12. Rizzo JD, Somerfield MR, Hagerty KL, et al. Use of epoietin and darbapoietin in patients with use of epoietin and darbapoietin in patients with cancer:2007 American Society of Hematology /American Society of Clinical Oncology clinical practice guideline update. Blood 2008;111:25-41. 13. Mittelman M, Neumann D, Peled A, Kanter P, Haran-Ghera N. Erythropoietin induces tumor regression and antitumor immune responses in murine myeloma models. Proc Natl Acad Sci USA 2001 ;98(9):5181-6. 14. Kokhaei P, Abdalla AO, Hansson L,et al. Expression of erythropoietin receptor and in vitro functional effects of epoetins in B-cell malignancies. Clin Cancer Res 2007;13(12):3536-44. 15. Baz R, Walker E, Choueiri TK, et al. Recombinant human erythropoietin is associated with increased overall survival in patients with multiple myeloma. Acta Haematol 2007;117(3):162-7 16. San Miguel JF, Schlag R, Khuageva NK, et al. Bortezomib plus melphalan and prednisone for initial treatment of multiple myeloma. N Engl J Med 2008; 359(9): 906-17. 17. Kyle RA, Rajkumar SV. Multiple myeloma. New Engl J Med 2005 ;351(18) :1860- 73. RESUMEN Los pacientes con mieloma múltiple (MM) tienen un aumento en complicaciones infecciosas y en anemia sintomática. Nuevas terapias antineoplásicas resultan en una mejor respuesta, pero todavía se afecta significativamente la calidad de vida y potencialmente la sobrevida por estas complicaciones. Múltiples citocinas y un sistema inmunológico deficiente están asociadas en el desarrollo de estas complicaciones. Terapias en desarrollo y guías de manejo serán discutidas teniendo en consideración los beneficios versus los riesgos de estas estrategias. El efecto beneficioso de la eritropoyetina recombinante humana (EPO) y la darbepoyetina alfa en los pacientes con mieloma múltiple ha sido confirmado en varios ensayos clínicos. Guías basadas en la evidencia médica del uso de EPO en pacientes con cáncer deben ser seguidas para garantizar el uso correcto de estos agentes estimuladores de la eritropoyesis. Las infecciones, aunque resultan en la causa más común de morbilidad y mortalidad en estos pacientes, pueden ser controladas por una sospecha temprana y profilaxis apropiada. El cuidado de apoyo es sumamente importante como un adyuvante a la terapia del cáncer. El uso apropiado de estrategias farmacológicas para controlar la anemia o tratar complicaciones infecciosas resulta de beneficio para los pacientes con mieloma múltiple. ABSTRACT Objective: We performed a retrospective review to identify objective factors that could facilitate the surgeon’s decision regarding the feasibility of an adequate resection with a margin of < 2 cm from the dentate line. We could not find clear guidelines for clinicians regarding the use of close margins for sphincter saving surgery following chemoradiation (CRX). We proposed what state of the art imaging tools are potentially useful to identify tumor downstage following preoperative CRX and aid in the development of guidelines. Methods: Reviewed of the literature on the subject and performance of current diagnostic imaging studies useful in identifying rectal tumor downstaging after preoperative CRX. Results: Without safe margins of resection an abdominoperineal resection (APR) is the operation of choice. All sphincter saving rectal cancer operations results for ultra-low tumors need to be as good as results from an APR. Performing frozen section for the ultralow rectal cancer margins is recommended. The Endorectal Ultrasonography (ERUS) data appear encouraging and suggest that we should evaluate TRUS earlier after CRX, before the desmoplastic reaction and scar tissue appears. It could turn out to be an objective and accurate method of evaluating tumor downstaging. Color Doppler evaluation has shown higher specificity than that of grey scale ultrasound in staging and differentiating scar from anal cancers. Similarly, PET scanning performed earlier and with modern PET-CT equipment is worth exploring. Conclusion: At this point with the information available from the literature, we suggest that patients with clinically advanced rectal cancer can have a distal margin resection of less than 2 cm if: 1- the tumor is not mucin producing, 2- the tumor is not high-grade, and 3- the response to preop CRX is adequate, however there exist no clear guidelines available to judge what is an excellent versus a moderate or poor response. Index words: approach, surgery, locally, advanced ultra-low, rectal, cancer INTRODUCTION T he ideal surgical approach for rectal carcinoma is Low Anterior Resection (LAR) with Total Mesorectal Excision (TME), and free radial and distal margins of resection after preoperative chemoradiotherapy (CRX).1-3 Traditionally, rectal cancer resection has been performed obtaining a minimum of 2cm distal margin, sphincter preservation, if possible. The difficulty presents in patients with very low rectal tumors with less than 2 cm distal resection margin from the anal verge. It is known that distal intramural spread (DIS) of tumor rarely exceeds more than 1-2 cm in rectal cancer and this forms the basis for the traditional 2 cm resection margin. However, with the use of modern preoperative CRX, it is likely that the traditional 2 cm margin may no longer be necessary because of the decrease in distal intramural spread after preoperative CRX. The American Society of Colorectal Surgeons (ASCRS) published practice parameters for the treatment of rectal cancer in 2005. The ASCRS supports a smaller than 2 cm margin, in some cases A PROPOSED APPROACH FOR THE SELECTION OF THE PROPER SURGICAL THERAPY TO OBTAIN AN ADEQUATE MARGIN OF RESECTION IN LOCALLY ADVANCED ULTRA-LOW RECTAL CANCER AFTER MODERN PREOPERATIVE CRX MANAGEMENT 53 Ignacio Echenique, MD* Fernando Cabanillas, MD* Vangie Texidor, BA** Janice Cáceres, MD* Gerald Isenberg, MD§ Carlos Claudio, MD* Roberto Ayala, MD* Frank Madera, MD* From *Auxilio Mutuo Hospital, Rio Piedras, PR, **University of Puerto Rico School of Medicine, and § Jefferson’s Medical School Philadelphia, PA, Address reprints to: Ignacio Echenique MD - Office 218, Torre Auxilio Mutuo, 715 Ave. Ponce de Leon, Rio Piedras, PR, 00918. E-mail<[email protected]> Poster presented during the 58th Annual Meeting American College of Surgeons, Puerto Rico Chapter, February 2008, La Concha Hotel, San Juan, PR reaching 1 cm in patients without adverse tumor histology.4 In order to perform sphincter preservation surgery, determination of the effectiveness of preoperative CRX and post CRX re-staging would be helpful in patients who need a distal resection margin under 2 cm. However, clinical determination of the effectiveness of preoperative CRX has been fraught with difficulties. Recently, several authors have described a relatively favorable clinical outcome after sphincter saving surgery with a distal margin of 1cm.2,5,6 We reviewed the literature to identify objective factors that could facilitate the surgeon’s decision regarding the feasibility of an adequate resection with a margin of < 2 cm. Based on this review 54 we will propose what state of the art imaging tools are potentially useful to identify tumor downstage following preoperative CRX. The goal of our study is to aid in the development of guidelines. METHOD We performed a retrospective review of the literature on this subject. We analyzed the performance of current diagnostic imaging studies used in the evaluation of tumor response to preoperative CRX. We could not find clear guidelines for clinicians regarding the use of close margins for sphincter saving surgery following CRX. RESULTS Published studies have shown that a good histological response in resected specimens has a direct correlation with survival with the best responders having prolonged survival.7 Hiotis et al. (2002) studied the relationship between clinical complete response and pathological complete response in colorectal cancer patients after CRX. All their subjects had T3, T4 tumors or cancer with positive nodes. After CRX, digital rectal exam (DRE) and proctoscopy were used to determine clinical response. The pathologic complete response rate among all patients was 10%. The correlation between clinical and pathologic complete response was 25%. Clinical complete response was a significant predictive factor of pathologic complete response. Three fourths of clinical complete responders had residual foci of tumor not detectable on DRE or proctoscopy.8 Thus, clinical complete response fails to adequately predict pathologic tumor response because it frequently overestimated and also underestimated the response to treatment. Therefore, DRE combined with proctoscopy cannot be used in order to determine if non-radical surgical therapy can be undertaken. This underestimation is probably a result of the desmoplastic reaction at the tumor site in response to CRX. Other studies have evaluated the use of proctoscopy, biopsies, Endorectal ultrasonography (ERUS), CT scan, and MRI as having limited accuracy for rectal cancer staging following CRX therapy.8-11 Kalff et al. studied the prognostic accuracy of 18FFDG PET after patients with T3/T4 rectal cancer underwent CRX before curative surgery. They found post CRX PET results to be directly related to overall survival duration. However, they didn’t evaluate the relationship with downstaging. Guillem et al (2004) examined rectal cancer response to CRX by performing FDG-PET before and 4-5 weeks after CRX. They measured the mean percentage decrease of standard uptake value (SUV) and total lesion glycolysis (dTLG). Recurrence-free patients had 69% mean decrease in SUV and over 69.5% dTLG, while patients with recurrence had a mean decrease of only 37%.12 Thus, metabolic activity measured in PET might be a good indicator of tumor regression after CRX. More recently they looked at the correlation between PET and pathological response post CRX and found that PET underestimated the pathological response probably because of the desmoplastic inflammatory reaction to treatment. At this point PET/CT is considered not to be sensitive enough to determine complete pathologic tumor response (CPR). However, the PET scans were done 4-5 weeks after completion of CRX at which time the degree of inflammation is probably at is peak. Furthermore the study was done 12 years ago using PET equipment that nowadays would be considered outdated. The literature describes the sensitivity of ERUS to be low after CRX and not reliable, when the ERUS is done 2 weeks after completing treatment.13 CRX leads to scar formation at the treatment site, making it making it almost impossible to distinguish between scar tissue and tumor with ERUS.9 However, all patients showed tumor regression and in 15 of 34 cases the T level changed. The accuracy of ERUS when compared with the postoperative histopathological findings after CRX increased from 18/32 before radiation to 25/32 after CRX.13. DISCUSSION When we don’t have a safe margin of resection then an Abdomino-perineal resection (APR) is the operation of choice. All sphincter saving rectal cancer operations results for the ultra-low tumors have to be as good as the results of an APR. Performing a frozen section for the ultralow rectal cancer margins is recommended. The ERUS data appear encouraging and suggest that we should evaluate TRUS earlier after chemoradiation (CRX), before the desmoplastic reaction and scar tissue appears. It could turn out to be an objective and accurate method of evaluating tumor downstaging. Color Doppler evaluation has shown higher specificity than that of grey scale ultrasound in staging14,15 and differentiating scar from anal cancers.16 Similarly, PET scanning performed earlier and with modern PET-CT equipment is worth exploring. To do or not to do a radical resection is not the goal of our discussion. We are addressing the issue of sphincter saving with adequate radial and distal margins of resection with Total Mesorectal Excision in Ultra Low Rectal cancer patients. Patients with poor prognostic factors such as highgrade tumors, bulky, tethered tumors, mucin production, and T4 currently should not be candidates for a less than traditional margin of resection.5,9,12 CONCLUSIONS At this point with the information available from the literature, we suggest that patients with clinically advanced rectal cancer can have a distal margin resection < 2 cm if: 1- 2- 3- The tumor is not mucin producing The tumor is not high-grade The response to preop CRX is adequate, however there are no clear guidelines available to judge what is an excellent versus a moderate or poor res- ponse. In view of the lack of clear guidelines, we are proposing guidelines to summarize and clarify in a friendly format the recommended adequate margins of resection according to the clinical response to preop CRX. These guidelines are provisional and need to be tested in a prospective study: 1-POOR RESPONSE: (2 cm margin recommended) No clinical or radiological downstaging 55 No decrease from a baseline T3-T4. 2- INTERMEDIATE RESPONSE- (1.5- 2 cm margin recommended) Clinical or radiological decrease in “T stage” by 1 level, i.e. preoperative downstaging from baseline T3[T2 or from T4[T3. 3- EXCELLENT RESPONSE – (1 cm margin recommended) Clinical or radiological decrease in T stage by 2 levels, i.e. preoperative downstaging from T3[ < T1 or T4[<T2 • • All patients who achieve a complete clinical response would also be classified as excellent response. (A tattoo may be needed in these patients before surgery in order to identify the original site of disease.) Performing a frozen section for the ultralow rectal cancer margins is recommended. Which imaging test is best to define downstaging is currently an unanswered question, which needs to be addressed. Newer ERUS and more sensitive PET scans seem to be the most promising imaging test to evaluate tumor downstage response. ERUS - Color Doppler may increase ultrasound specificity in differentiating tumor from fibrosis. Acknowledgements This research was made possible with the cooperation of The American Cancer Society, Puerto Rico Division. No conflict of interest. Respuesta Recomendacion Margen Distal Indicacion POBRE >2 cm Ningun cambio clinico o radiologico en estadio. Ningun cambio en T3-T4 o produccion de mucina, alto-grado, tumor grande. INTERMEDIO 1.5- 2 cm Disminucion clinica o radiologica en estadio T por 1 nivel, i.e. Disminucion preoperativa de estadio de un T3 a T2 o T4 a T3. EXCELENTE 1 cm Respuesta clinical completa; disminucion clinica o radiologica en estadio T por 2 niveles, i.e. Disminucion preoperativa de estadio de un T3 a <T1 o T4 a <T2. REFERENCES 1. Echenique LE, Cabanillas F, Freire V, Echenique IA. Outcome of Hispanics with colorectal cancer residing in Puerto Rico with access to adequate health care facilities: results are not inferior to USA or European Caucasians. Cancer Therapy 2008;6:413-20. 2. Kuvshinoff B, Maghfoor I, Miedema B, et al. Distal margin requirements after preoperative chemoradiotherapy for distal rectal carcinomas: are < 1 cm distal margins sufficient? Ann Surg Oncol, 2001;8(2):163–9. 3. Mohiuddin M, Marks GJ. High dose preoperative radiation and sphincter preservation in the treatment of rectal cancer. Int J Radiat Oncol Biol Phys. 1987;13(6):839-42. 4. Tjandra JJ, Kilkenny JW, Buie WD, et al. Practice parameters for the management of rectal cancer (revised). Dis Colon Rectum 2005;48:411-23. 5. Andreola S, Leo E, Belli F, et al. Adenocarcinoma of the lower third of the rectum surgically treated with a <10-mm distal clearance: preliminary results in 35 N0 patients. Ann Surg Oncol 2001;8:611-5. 6. Moore HG, Riedel E, Minsky BD, et al. Adequacy of 1-cm distal margin after restorative rectal cancer resection with sharp mesorectal excision and preoperative combined-modality therapy. Ann Surg Oncol 2003;10(1):80-5. 7. Kalff V, Duong C, Drummond EG, et al. Findings on 18F-FDG PET scans after neoadjuvant CRX provides prognostic stratification in patients with locally advanced rectal carcinoma subsequently treated by radical surgery. J Nucl Med 2006; 47(1):14-22. 8. Fleshman WJ, Myerson RJ, Fry RD, Kodner IJ. Accuracy of transrectal ultrasound in predicting pathologic stage of rectal cancer before and after preoperative radiation therapy. Dis colon rectum 1992;35(9):823-9. 9. Hiotis SP, Weber SM, Cohen AM, et al. Assessing the predictive value of clinical complete response to neoadjuvant therapy for rectal cancer: an analysis of 488 patients. J Am Col Surg 2002;194(2):131-5. 10. Kim CJ, Yeatman TJ, Cappola D, et al. Local excision of T2 and T3 rectal cancers after downstaging chemoradiation. Ann Surg 2001;234:352-8. 11. Vanagunas A, Lin DE, Stryker SJ. Accuracy of endoscopic ultrasound for restaging rectal cancer following neoadjuvant chemoradiation therapy. Am J Gastroenterol 2004;99(1):109-12. 12. Guillem JG, Moore HG, Akhurst T, et al. Sequential preoperative fluorodeoxyglucose-positron emission tomography assessment of response to preoperative chemoradiation: a means for determining longterm outcomes of rectal cancer. J Am Coll Surg 2004;199(1):1–7. 13. Houvenaeghel G, Delpero JR, Giovannini M, et al. Staging of rectal cancer: a postoperative study of digital examination and endosonography before and after preoperative radiotherapy. Acta Chir Belg 1993;93(4):164-8. 14. Heneghan JP, Salem RR, Lange RC, et al. Transrectal sonography in staging rectal carcinoma: the role of gray- scale, color-flow, and Doppler imaging analysis. Am J Roentgenol 1997;169:1247-52. 15. Ogura O, Takebayashi Y, Sameshima T, et al. Preoperative assessment of vascularity by color Doppler ultrasonography in human rectal carcinoma. DCR 2001;44:538-46. 16. Drudi F, Giovagnoriob F, Raffetto N, et al. Transrectal ultrasound color Doppler in the evaluation of recurrence of anal canal cancer. Eur J Rad 2003;47(2):142-8. 17. Guillem JG, Chessin DB, Cohen AM, et al. Long-term oncologic outcome following preoperative combined modality therapy and total mesorectal excision of locally advanced rectal cancer. Ann Surg 2005; 241(5):829–38. RESUMEN Objetivo: En este repaso sugerimos varios estudios de imágenes que son útiles para identificar una disminución en el estadio clínico de adenocarcinomas de recto después de quimo-radioterapia pre-operatoria. Nuestro objetivo principal es proponer unas guías para facilitar la decisión del cirujano en cuanto a si es posible obtener márgenes de resección distales menores de 2 cm en cáncer de recto bien bajo localmente avanzado. Revisamos la literatura científica disponible y no pudimos encontrar unas guías establecidas. Discusión: En el tratamiento de cáncer de recto, localmente avanzado, al presente se recomienda obtener 2 cm de margen distal. En cáncer de recto ultra bajo el resultado de una cirugía conservadora del esfínter anal debe ser tan bueno como el resultado de una resección abdomino-perineal. Cuando no hay márgenes de resección seguros en cáncer de recto bien bajo, se debe hacer una resección abdomino-perineal. Identificar una respuesta adecuada para poder decidir aceptar menos de 2 cm al presente no esta claramente establecido. Sugerimos que hacer el ultrasonido endo rectal y “PET scan” mas temprano luego de la quimo-radioterapia pre-operatoria, antes de que se desarrolle la respuesta inflamatoria y formación de tejido cicatricial en el área del tumor, puede mejorar la detección de disminución de estadio por estos estudios. El sonograma con color parece ser más específico en determinar estadio y en diferenciar tejido cicatricial que el ultrasonido sin color. Metodología: Repasamos la literatura acerca del tema y analizamos el rendimiento de estudios de imágenes que son útiles para identificar una disminución en el estadio de tumores de recto. Conclusiones: Proponemos que se debe intentar obtener un margen de resección distal menor de 2 cm si el paciente con cáncer de recto avanzado cumple con los siguientes parámetros: 1. El tumor no produce mucina, 2. El tumor no es de alto grado y 3. Si la respuesta a quimoradioterapia preoperatoria es adecuada. Sin embargo, no hay unas guías claras disponibles para determinar lo que es una respuesta adecuada, moderada o pobre. Proponemos la siguiente escala para ser probada y correlacionada con la respuesta de cáncer de recto a quimo-radioterapia. 56 Case Reports (Reporte de Casos) DOUBLE CYSTIC DUCT IN A CHILD WITH VACTERL ASSOCIATION: A CASE REPORT Humberto Lugo-Vicente, MD* Maria Correa, MD**, Hector Brunet, MD d From the *Section of Pediatric Surgery, d Department of Surgery, and ** Department of Pathology, U.P.R. School of Medicine, San Juan, Puerto Rico. Address reprints requests: Humberto Lugo-Vicente MD, PO Box 10426, San Juan PR 00922. [email protected]. INTRODUCTION A natomic variations of the biliary tree are common and an important source of injury during open and laparoscopic gallbladder procedures (1). Variants of the extrahepatic bile ducts are present in 10% of patients such as low insertion of the cystic duct into the common hepatic duct, emptying of the cystic duct into the right hepatic duct and a second order large branch draining into the cystic duct (2). One of the rarest congenital anomaly of the biliary tree is the presence of double cystic duct with less than 15 cases described in the world literature, all adults (3). We report the first pediatric case born with VACTERL association found to have double cystic duct during gallbladder surgery. CASE HISTORY A 6-year-old male child born with VACTERL association developed recent history of postprandial abdominal pain and vomiting. Abdominal ultrasound revealed cholelithiasis. Past history describes the child born with esophageal atresia, tracheoesophageal fistula, duodenal atresia, imperforate anus, atrial septal defect, patent ductus ateriosus, polydatilism and hypospadia. The esophageal, duodenal, anorectal, genitourinary and extremity defects were repaired during infancy satisfactorily, while the cardiac defects closed spontaneously. Due to symptomatic cholelithiasis the child was schedule for cholecystectomy. CASE HISTORY A 6-year-old male child born with VACTERL association developed recent history of postprandial abdominal pain and vomiting. Abdominal ultrasound revealed cholelithiasis. Past history describes the child born with esophageal atresia, tracheoesophageal fistula, duodenal atresia, imperforate anus, atrial septal defect, patent ductus ateriosus, defects ABSTRACT Double cystic duct is an extremely rare anomaly of the biliary tract not described in the pediatric literature. We report the first pediatric case born with VACTERL association found to have double cystic ducts during gallbladder surgery for symptomatic cholelithiasis. Description of the anatomic variability, cholangiography images, and pathologic findings along with review of the literature is included. Index words: double cystic duct, child, VACTERL association closed spontaneously. Due to symptomatic cholelithiasis the child was schedule for cholecystectomy. Laparoscopic cholecystectomy was intended but due to dense abdominal adhesions around the gallbladder the procedure was converted before reaching Calot’s triangle. The gallbladder was dissected from fundus toward porta hepatis. During dissection of a cystic duct entering the common hepatic duct bile leak occurred. Further dissection identified the bile leak to be a pinpoint injury produced near the junction of a proximal cystic duct entering the common hepatic duct while a second cystic duct entering the common bile duct distally was also identified (see Figure 1). Irrigation independently through both cystic ducts while occluding each other demonstrated patency with the main biliary system. Two titanium medium size clips were placed tangentially (parallel) occluding the small rent in the proximal “accessory” cystic duct - hepatic duct junction. The bile leak stopped and a cholangiogram performed through the second “main” distal cystic duct demonstrated patent intra- and extrahepatic biliary duct system with adequate bowel drainage and no leak (see Figure 2). The right hepatic radicals had a smaller lumen compared to the left side probably the effect of better drainage of the right hepatic system directly to the gallbladder. The second distal cystic duct was clipped and the single gallbladder removed. A single cystic artery was identified. A Jackson-Pratt drain was placed and removed two days later. Postoperative HIDA scan showed adequate bi-lobar hepatic-enteric drainage. The child made an uneventful recovery send home on postoperative day three. Pathology confirmed a single gallbladder with double cystic ducts and chronic erosive cholecystitis. Gross examination revealed two cystic ducts of 0.3 and 0.2 cm each; the smaller clamped by a surgical clip. On opening the gallbladder, a single cavity was identified communicating to the previously described ducts. Lumen septation or a smaller cavity to suggest a double or accessory gallbladder was not seen. The gallbladder mucosa was focally hemorrhagic and erosive with focal Rokitansky aschoff sinuses. Sections of the ducts revealed one epithelial lined larger duct and a smaller duct lumen with eroded mucosa (see Figure 3). DISCUSSION The anatomic variability of the biliary system has been well documented. Bile duct injury and biliary 57 leaks are very often the result of failure to recognize anomalous biliary anatomy. The double cystic duct anatomic variant presented in this case was recognized after bile leak occurred during open intraoperative dissection. On a few occasions postoperative bile leaks are the result of not recognizing the existence of such second cystic duct (4, 5). Double cystic duct is an extremely rare congenital anomaly that is usually associated with a double gallbladder, also known as gallbladder duplication. Only 20% of all reported cases of double cystic ducts have a single lumen gallbladder. Depending on the configuration of the ducts the anomaly has been categorized into three types: 1- The “Y” type where the two cystic ducts join to form a common channel which then enters the common hepatic duct, 2- the “H” type, exemplified in our patient, where the accessory cystic duct enter separately the right, left or common hepatic duct, while the main cystic duct enter the common bile duct, and 3- the trabecular type or cholecystohepatic duct wherein the accessory cystic duct either single or multiply enters directly the liver substance (6). The trabecular type described include drainage of the VI segment of the liver into the cystic duct, drainage of the right posterior sector into the cystic duct, drainage of the distal right posterior sector into the gallbladder neck, or drainage of the proximal part of the right posterior sector into the body of the gallbladder (7). Figure 1: While grasping the single gallbladder, the white arrow points toward the proximal cystic duct entering the common hepatic duct and the pinpoint injury cause of the biliary leak. The black arrow shows the distal cystic duct entering the common bile duct. The previous fifteen cases of double cystic duct published in the literature are all adults operated for symptomatic cholelithiasis or gallstone pancreatitis. This report is the first pediatric description of a double cystic duct in a child born with the VACTERL association. Cholelithiasis and biliary dysplasia has previously been reported in children with the VACTERL association (8, 9), but since they are so infrequent they are not considered extension of the association. Double cystic ducts are usually identified intraoperatively and as mentioned earlier 80% of all cases are associated with a duplicated gallbladder (10). On a few reports ERCP or MRCP has suggested the preoperative diagnosis helping the surgeon build confidence toward the expected anatomy (2, 11, 12,). When any of these anatomic variants are suspected intraoperatively, cholangiography is the next step in management to better define the surgical anatomy (13). Usually there is only one cystic artery that arises from the right hepatic artery and accompanies the primary cystic duct in double cystic ducts descriptions associated with single gallbladders (14). Several reports of double cystic ducts have completed the case laparoscopically without injuring the bile ducts (5, 12-15). Misidentification of the common bile duct for the cystic duct leads to the most common bile duct injury during laparoscopy. Two scenarios simulating duplication of the cystic ducts include the case with a short or absent cystic duct where vigorous traction on the gallbladder results in angulation and tenting of the common bile duct misleading the surgeon to believe two cystic ducts exist, and secondly, in the presence of scarring the right hepatic duct adherent to the gallbladder might be misidentified as a cystic duct (5). Figure 2: Cholangiogram performed through the distal cystic duct showing two clips placed in the proximal cystic duct – common hepatic duct junction (black arrow). Figure 3. Photomicrograph showing the two cystic ducts. The distal larger epithelial lined duct (1), and the proximal smaller duct (2) with eroded mucosa. AMPRnews 58 In conclusion, should suspicion arise during cholecystectomy dissection that there is an uncommon anatomic variation in the biliary tract intraoperative cholangiogram will delineate the anatomy and foresee whether the case can be completed laparoscopically or need conversion. The management of double cystic duct is either open or laparoscopic closure of each duct individually. REFERENCES l Sea El Segundo En Enterarse (El primero fue quien lo descubrió) Primeros en noticias médicas www.asociacionmedicapr.org 1- Lamah M, Dickson GH: Congenital anatomical abnormalities of the extrahepatic biliary duct: a personal audit. Surg Radiol Anat. 1999;21(5):325-7. 2- De Filippo M, Calabrese M, Quinto S, Rastelli A, Bertellini A, Martora R, Sverzellati N, Corradi D, Vitale M, Crialesi G, Sarli L, Roncoroni L, Garlaschi G, Zompatori M: Congenital anomalies and variations of the bile and pancreatic ducts: magnetic resonance cholangiopancreatography findings, epidemiology and clinical significance. Radiol Med. 2008; 113(6):841-59. 3- Paraskevas G, Papaziogas B, Natsis K, Spanidou S, Kitsoulis P, Atmatzidis K, Tsikaras P: An accessory double cystic duct with single gallbladder. Chirurgia (Bucur). 2007; 102(2):223-5 4- Momiyama T, Souda S, Yoshikawa Y, Kuratani T, Toda K, Koma M: Injury to a duplicated cystic duct during laparoscopic cholecystectomy. Surg Laparosc Endosc. 1996;6(4):315-7. 5- Ng JW, Yeung GH, Lee WM, Tse S: Isolated duplications of the cystic duct: case report and implications in laparoscopic cholecystectomy. Surg Laparosc Endosc. 1996;6(4):310-4. 6- Shivhare R, Sikora SS: Double Cystic Duct: A Rare Biliary Anomaly Encountered at Laparoscopic Cholecystectomy. J Laparoendosc Adv Surg Tech A. 2002;12(5):391-2. 7- Blumgart LH, Hann LE: Surgical and radiological anatomy of the liver and biliary tract. In Blumgart LH (ed): Surgery of the liver and biliary tract, ed. 3, Vol. 1. London: WB Saunders, 2000, pp 18-20. 8- Asabe K, Handa N: Infant cholelithiasis: report of a case. Surg Today. 1997;27(1):71-5. 9- Khoss A, Weninger M, Fr√ºhwirt I, Gherardini R, Zeitlhuber U: [Associated abnormalities in the VACTERL syndrome--case report with autopsy findings]. Klin Padiatr. 1985;197(1):1-4. 10- Charles K, Kloppel G: The bile duct system and its anatomical variatios. Endoscopy 1989; 21:300-308. 11- Kubota Y: Duplication of the Cystic Duct Detected by Endoscopic retrograde Cholangiopancreatography. Endoscopy. 1991;23: 308. 12- Tsutsumi S, Hosouchi Y, Shimura T, Asao T, Kojima T, Takenoshita S, Kuwano H: Double cystic duct detected by endoscopic retrograde cholangiopancreatography and confirmed by intraoperative cholangiography in laparoscopic cholecystectomy: a case report. Hepatogastroenterology. 2000; 47(35):1266-8. 13- Huston TL, Dakin GF: Double cystic duct. Can J Surg. 2008; 51(1): E9-E10. 14- Hirono Y, Takita Y, Nitta N, Hashimoto H: Double cystic duct found by intraoperative cholangiography in laparoscopic cholecystectomy. Surg Laparosc Endosc. 1997;7(3):263-5. 15- Lobo EJ, Herbella FA, Goldenberg A, Kobata MH, Triviño T: Laparoscopic cholecystectomy in a patient with a duplicated cystic duct. Surg Laparosc Endosc Percutan Tech. 2000;10(5):326-8. RESUMEN Ductos císticos dobles representan una anomalía congénita extremadamente rara del sistema biliar que no ha sido reportada en la edad pediátrica. Este reporte estudia el primer caso pediátrico de una paciente con ducto cístico doble asociado a la anomalía de VACTERL que debuto durante colecistectomía por piedras sintomáticas de la vesicular biliar. Este informe pretende demostrar la variabilidad anatómica, colangiograma y hallazgos patológicos junto a un repaso de la literatura de esta rara condición congénita. ABSTRACT Smith-Magenis syndrome (SMS) is characterized by deletions in the short arm of chromosome 17. Systemic findings in patients with the syndrome include: dysmorphic facies and skeletal deformities. Ophthalmic findings in patients with the SMS include: strabismus, refractive errors, microcornea, iris anomalies, microphthalmos, and coloboma. A 14-yearold boy with cytogenetic studies confirming the SMS underwent a comprehensive ophthalmologic examination. The patient has a history of strabismus surgery. Clinical findings in this patient include: developmental delay, facial dysmorphism, enamel hypoplasia, short broad hands, clinodactyly, and scoliosis. Ocular findings in our patient include: myopia, iris nodules, loose zonules, and ectopia lentis. To our knowledge this is the first reported case of SMS in the Caribbean basin and the first case that report ectopia lentis in SMS. There is a possibility that lens subluxation in our patient is due to self inflicted trauma. Key words: Smith-Magenis syndrome,Chromosome 17, ectopia lentis SMITH-MAGENIS SYNDROME IN PUERTO RICO: A CASE REPORT 59 Javier Jardón BS *, Natalio J. Izquierdo MD ** From the * UPR School of Medicine, University of Puerto Rico, Medical Sciences Campus, Rio Piedras, Puerto Rico and ** Pediatric Ophtalmogist. Address reprints requists to: Natalio J. Izquierdo MD, 369 De Diego Street, Torre San Francisco Suite 310, San Juan PR 00923. Email Address: [email protected] INTRODUCTION P revious studies have reported that patients with a deletion on the short arm of chromosome 17 (17p-) have the so called Smith-Magenis syndrome (SMS) (1-4). Patients with the syndrome have systemic findings including: neurological retardation, behavioral problems, developmental delay, facial dysmorphisms, skeletal anomalies, and sleep disturbances (2, 5-9). Ophthalmic findings in patients with the syndrome include: strabismus, iris abnormalities such as iris dysgenesis, doubled pupils, microcornea, bilateral microphthalmos with uveal and retinal coloboma, and refractive errors such as myopia(1, 10-12). Greenberg and co-workers suggested that the Smith-Magenis syndrome was associated with a deletion in a critical region of the short arm of chromosome 17 (del(17) (p11.2))(6, 7, 13, 14). Further, Girirajan and co-workers found evidence that the genes found in locus 17p11.2 contribute to the clinical findings in patients with the syndrome (13, 14). The SMS is mostly due to a chromosomal deletion of <4 Mb at the 17p11.2 locus, affecting different genes (15, 16). Girirajan and co-workers concluded that alterations in gene RAI1 lead to most of the phenotypic characteristics found in patients with the syndrome (13-15, 17, 18). We report on the systemic, ocular, and genetic findings in a Puerto Rican patient with the Smith-Magenis syndrome. CASE REPORT This is the case of a 14 years-old-boy who carries a diagnosis of Smith-Magenis syndrome. Patient has history of self mutilation, surgery for an undescended testicle, and strabismus. Patient has developmental delay and mental retardation. As depicted in Figure 1, clinical findings include: brachycephaly; a broad face with flat facies; a prominent forehead; a flat maxillary area; and a flat occiput. Patient has enamel hypoplasia; and scoliosis. As depicted in Figure 2, he has short broad hands; clinodactyly; and has evidence of self mutilation in his left hand close to the snuff-box. Figure 1. Patient’s head 60 Patient’s best corrected visual acuity is 20/20 in both eyes. He is ortophoric. Upon slit lamp examination, he has multiple nodules in the iris. Upon pupillary dilation patient has loose zonules and ectopia lentis in the left eye. Cycloplegic refraction was -8.50 +3.50 X 90 and -7.00 +2.50 X 90 in the right and left eye respectively. Upon indirect ophthalmoscopy patient has intact optic nerves; vessels; maculae; and peripheries. Upon genetic studies, our patient has an interstitial deletion of band 17p11.2 in all cells (46, XY, del (17)(p11.2p12), as depicted in Figure 3. DISCUSSION Previous studies have reported that patients with the SMS have developmental delay, craniofacial dysmorphisms and skeletal abnormalities (1). Our patient has mental retardation; a broad face, with frontal bossing and maxillary hypoplasia. Further our patient had scoliosis; enamel hypoplasia; and clinodactyly. Clinical findings in our patient are compatible with previously reported patients with the syndrome. Chen and co-workers (10), reported on the ocular findings of patients with the syndrome. Ophthalmic findings in our patient include: a history of strabismus surgery; iris nodules; and high myopia. These findings are compatible with their report. However, our patient does not have microcornea, nor coloboma. Further, our patient has loose zonules and ectopia lentis. To our knowledge, ectopia lentis has not been previously described in patients with the SMS. However, there is a possibility that lens subluxation in our patient is due to self inflicted trauma. Ectopia lentis in patients with the syndrome may contribute to their myopia. Greenberg and co-workers (6, 7, 13, 14, 17), reported that cytogenetic studies in patients with the syndrome show a deletion of the short arm of chromosome 17 (17p-). Girigan and co-workers (13, 14), suggested that a critical region within the locus is found at 17p11.2. As a matter of fact, Potocki and co-workers showed that 90% of patients with the SMS carry deletions for the same genetic markers in the 17p11.2 region (8). As depicted in Figure 3, our patient’s chromosome analysis showed an interstitial deletion of band 17p11.2 in all cells (46, XY, del(17)(p11.2-p12). This finding is compatible with previous reports on patients with the syndrome. In conclusion, to our knowledge, this is the first report of a patient with the SMS in the Caribbean basin. Further, ectopia lentis has not been previously reported in patients with the syndrome. REFERENCES 1. Patil SR, Bartley JA. Interstitial deletion of the short arm of chromosome 17. Hum Genet 1984 67: 237-238. 2. Smith AC, et al. Interstitial deletion of (17)(p11.2p11.2) in nine patients. Am J Med Genet 1986 24: 393-414. 3. Stratton RF, et al. Report of six additional patients with new chromosome deletion syndrome. Am J Med Genet 1986 24: 421-432. 4. Zori RT, et al. Clinical, cytogenetic, and molecular evidence for an infant with Smith-Magenis syndrome born from a mother having a mosaic 17p11.2p12 deletion. Am J Med Genet 1993 47: 504-511. 5. Moncla A, et al. Smith-Magenis syndrome: a new contiguous gene syndrome: report of three new cases. J Med Genet 1991 28: 627-632. 6. Greenberg F, et al. Molecular analysis of the Smith-Magenis syndrome: a possible contiguous-gene syndrome associated with del(17)(p11.2). Am J Hum Genet 1991 49: 1207-1218. 7. Greenberg F, et al. Multi-disciplinary clinical study of Smith-Magenis syndrome (deletion 17p11.2). Am J Med Genet 1996 62: 247-254. 8. Potocki L, et al. Circadian rhythm abnormalities of melatonin in SmithMagenis syndrome. J Med Genet 2000 37: 428-433. Figure 2. Patient’s hands 9. De Leersnyder H, et al. Inversion of the circadian rhythm of melatonin in the Smith-Magenis syndrome. J Pediat 2001. 139: 111-1162. Barnicoat AJ, et al. An unusual presentation of Smith-Magenis syndrome with iris dysgenesis. Clin Dysmorph 1996 5: 153-158. 10. Chen RM, Lupski JR, Greenberg F, Lewis RA. Ophthalmic manifestations of Smith-Magenis syndrome. Ophthalmology 1997 104:732-3. 11. Barnicoat AJ, et al. An unusual presentation of Smith-Magenis syndrome with iris dysgenesis. Clin Dysmorph 1996 5: 153-158. 12. Edelman EA, et al. Gender, genotype, and phenotype differences in Smith-Magenis syndrome: a meta-analysis of 105 cases. Clin Genet 2007 71: 540550. 13. Girirajan S, Elsas LJII, Devriendt K, Elsea SH. RAI1 variations in Smith-Magenis syndrome patients without 17p11.2 deletions. J Med Genet 2005 42: 820-828. 14. Girirajan S, et al. Genotype-phenotype correlation in Smith-Magenis syndrome: evidence that multiple genes in 17p11.2 contribute to the clinical spectrum. Genet Med 2006 8: 417-427. 15. Andrieux J, et al. Genotype-phenotype correlation of 30 patients with Smith-Magenis syndrome (SMS) using comparative genome hybridisation array: cleft palate in SMS is associated with larger deletions. J Med Genet 2007 44: 537540. 16. Lucas RE, et al. Genomic organisation of the ~1.5 Mb Smith-Magenis syndrome critical interval: transcription map, genomic contig, and candidate gene analysis. Europ J Hum Genet 2001 9: 892-902. 17. Seranski P, et al. RAI1 is a novel polyglutamine encoding gene that is deleted in Smith-Magenis syndrome patients. Gene 2001 270: 69-76. 18. Slager RE, et al. Mutations in RAI1 associated with Smith-Magenis syndrome. Nature Genet 2003 33: 466-468. RESUMEN El síndrome de Smith-Magenis es caracterizado por deleciones en el brazo corto del cromosoma 17. Las características sistémicas en estos pacientes pueden ser; caracteristicas faciales dismórficas y deformidades esqueléticas. Cambios oftalmológicos en estos pacientes que pueden ocurrir son estrabismo, errores de refracción, microcornea, anormalidades del iris, microoftalmos y coloboma. A un paciente de 14 años de edad con estudios previos de citogenética que confirmaban el diagnostico de SMS, a este paciente se le llevo a cabo un examen comprensivo oftalmológico. El paciente tenía en su historial una operación previa de estrabismo. Las características clínicas en este paciente incluían: retraso en el desarrollo, dimorfismo facial, hipoplasia del esmalte dental, unas manos cortas y anchas, clinodactilia y escoliosis. Hallazgos oculares en este paciente incluían; miopía, nódulos en el iris, zonulas sueltas y lente ectópico. Para nuestro conocimiento este es el primer caso reportado de SMS en el Caribe y el primer caso que reporta lente ectópico en este síndrome. Existe una posibilidad que la subluxación del lente se deba a un trauma causado por el mismo paciente. 61 Figure 3. Chromosome analysis Obtenga Créditos de Educación Médica Continua Jornadas Científicas - Boletín de la AMPR - Website Gratis para Asociados www.asociacionmedicapr.org 62 El Dr. Luis Izquierdo Mora: una vida entera dedicada al servicio del pueblo Natalio J. Izquierdo-Encarnación, M.D. C ierto día escuché en el pueblo de Río Piedras dos señoras hablando mientras esperaban la salida del carro público que las devolvería a sus respectivos hogares en Trujillo Alto. Una le preguntó a la otra: “¿De dónde vienes?” La segunda respondió: “de la oficina del Dr. Izquierdo” La primera le cuestionó: ¿y qué plan médico tú tienes? La segunda respondió: “el plan de Izquierdo-Mora”. La primera se puso curiosa y le volvió a preguntar: “¿y cuál es ese plan?” Yo puse más atención a lo que decían. La segunda se sonrió y dijo: “pues yo llego allí enferma, él me evalúa y no me cobra por sus servicios, me regala unas muestras de medicamentos para que me cure y me da un peso para pagar la transportación y llegar a mi casa de vuelta.” Esa anécdota es típica de la vida de tan ilustre galeno. La vida del Dr. Izquierdo Mora se resume con la frase que él acuñó: “se sirve sirviendo” y “el que no sirve, sencillamente no sirve”. El Dr. Izquierdo Mora es mi padre y me llena de orgullo escribir sobre su vida y algunas de sus obras. Permítanme que les cuente algo más íntimo de nuestra vida familiar. Pienso que el amor por servir lo fue aprendiendo poco a poco desde el seno de su hogar. Su padre, Don Luis Izquierdo-Galo sirvió a su pueblo desde su Cátedra en el Colegio de Mayagüez y más tarde culminó su servicio al pueblo cuando fue nombrado Secretario de Agricultura y Comercio en la década de 1930. En esa época, la agricultura era la principal industria isleña. Por otro lado, su madre Doña Jenara Mora-Ríos fue maestra de inglés. Cuando niño, este futuro médico invitaba todos los días a almorzar a su casa, compañeros de clase que eran pobres, de dos en dos, porque en esa época, no se servían almuerzos en las escuelas públicas de la isla. Más tarde, en la década del cincuenta, el Dr. IzquierdoMora se graduó de la tercera clase de la Escuela de Medicina de la Universidad de Puerto Rico, escuela que culminó los anhelos de un gobierno que supo servir a su pueblo empobrecido, como bien describía la canción del Lamento Borincano. En esa misma década el doctor se casó con Doña Rita Encarnación-Fas, entonces estudiante de farmacia. Juntos formaron una familia numerosa. Es menester mencionar que el suegro del doctor, el Licenciado Natalio Encarnación-Vega, también sirvió a su pueblo desde sus farmacias, su Droguería Encarnación de Mayagüez y su fábrica de medicamentos, llamada Western Pharmacal. Recuerdo que su Farmacia De Diego en la calle Post de Mayagüez, se le decía “la Farmacia de ricos y pobres”. Por otro lado, la suegra del doctor, Doña Fifa Fas de Encarnación, era un alma muy piadosa, que se desbordaba con obras caritativas a través de la Iglesia. Me parece que ellos también reforzaron el espíritu del servicio de mi señor padre. Después de su servicio militar en la Zona del Canal de Panamá, abrió las puertas de su oficina en Río Piedras, un 13 de agosto. Allí veía decenas de pacientes. Luego visitaba otros en los diferentes barrios de Río Piedras, Carolina y Trujillo Alto. A mediados de la década del 60, presidió la Asociación Médica de Puerto Rico. El lema de su mandato fue: “Operación Igualdad: mejor calidad de servicios y libre selección de su médico”. Recuerdo los carteles amarillos y azules que anunciaban dicha frase. Fue entonces cuando se dio a la gesta de que los puertorriqueños de la Isla también tuvieran los servicios de Medicare y Medicaid. Se presentó ante el presidente de los Estados Unidos, Hon. Lyndon B. Johnson, en compañía del Dr. Alvarez de Choudéns y de unas cajas llenas de 200,000 firmas de puertorriqueños, para solicitar la igualdad de los servicios para los ciudadanos americanos que vivían en la Isla. El resultado de ese cabildeo, fue el primer gran logro de mi padre, obra que perdura hasta la actualidad. Como sabemos, hoy día cientos de miles de puertorriqueños impedidos y envejecientes son beneficiarios de Medicare. Para la década del 70, mi padre fue electo Senador por Acumulación. Fue nombrado Presidente de la Comisión de Hacienda del Senado. Junto a otros legisladores médicos, presentó sendos proyectos de ley para beneficiar la salud del pueblo. Una de esas leyes creó las residencias de Medicina de Familia en la Isla. En esa década todavía los legisladores trabajaban a tiempo parcial. Mi padre veía pacientes en su oficina durante la mañana y en la tarde iba a su oficina en el edificio Anexo del Senado. Hace poco estuve en aquella oficina. Volvieron a mis recuerdos la época en que los legisladores atendían personalmente los ciudadanos y escribían cartas para ayudarlos a conseguir una vivienda, o cupones de alimentos. En esos años, se repartieron muchas viviendas públicas para los puertorriqueños de escasos recursos económicos. Ya en la década de los 80, el Dr. Izquierdo-Mora fue nombrado Secretario de Salud. Les cuento una anécdota de sus visitas a los hospitales. Él llegaba con mi madre a la Sala de Emergencia, por sorpresa, temprano de madrugada. Doña Rita se anotaba en la lista de espera para atenderse. Juntos esperaban su turno, observando el funcionamiento de la sala. Un día encontraron un paciente con dolor de pecho, sentado en la sala de espera. No había una camilla vacía disponible para atenderlo. Mi padre notó que el paciente estaba infartando y ordenó que lo acostaran y administraran un suero. La recepcionista le preguntó: “¿Quién se cree usted que es?” Mi papá le respondió: “Soy médico y además soy el Secretario de Salud. Si no hay camilla, saque la de la ambulancia y acueste este paciente, antes de que se muera. Es una orden.” Se podrán imaginar el “corre y corre” que se formó en aquella sala de Emergencia. Es menester recordar que durante su incumbencia en el Departamento de Salud, ocurrieron tres grandes tragedias en Puerto Rico: el deslizamiento que enterró el Barrio Mameyes de Ponce, el fuego del hotel Dupont-Plaza y la primera cresta de la epidemia de SIDA. Sospecho que fue entonces cuando a mi papá se le ocurrió el concepto de la “Medicina Pastoral”. Era una idea innovadora en la medicina. Formó unos grupos de apoyo para ayudar los familiares de los pacientes con asistencia médica, psicológica y espiritual. Reclutó psicólogos, rabinos, sacerdotes y ministros protestantes. Más tarde, el Venerable Juan Pablo II, habló de la Pastoral de la Salud, como un programa para ser asumido en todas las diócesis de la 63 Iglesia Católica. En ese sentido, me parece que mi padre y S.E.R. Cardenal Luis Aponte Martínez fueron visionarios en el servicio de los pacientes y sus familiares. Todavía se está trabajando para añadir la esfera espiritual a la definición de la salud. Creo que la historia se encargará de comprobar que la década del 80 fue la era dorada de la Salud en Puerto Rico. Los centros de salud de la Isla fueron mejorados y mucho convertidos en Centros de Salud Familiar. Mi padre se ocupó de que todos los centros académicos, hospitales, internados y residencias estuvieran acreditados, para servir al pueblo. Diría que también el Centro Médico llegó a su clímax convirtiéndose en el Centro Supra-Terciario del Caribe. En esa época se construyó el Centro Cardiovascular, se remodeló el Hospital Universitario, la Sala de Emergencias y se añadieron estacionamientos multi-pisos que perduran todavía, dos décadas más tarde. Fui testigo ocular de que la Sala de Emergencias permaneció como mi padre la dejó durante dos décadas. De hecho, fue remodelada recientemente. Logró todas estas hazañas gracias a la ayuda de mil voluntarios, cientos de profesionales de la salud y miles de empleados que como él, sirvieron al pueblo por amor a sus pacientes. Son muchas las anécdotas y poco el espacio. La oficina que un día fundó mi padre en Río Piedras, este mes ha cumplido 50 años. Desde allí, el Dr. Luis Izquierdo-Mora ha educado miles de estudiantes de medicina. Allí sigue sirviendo a su pueblo. Él dice que es el más “chongo” de todos los médicos y que “es un mediquito de un pueblo que ya no existe”. Es otra de sus múltiples bromas, muestra de su humildad sincera, porque en el servicio y la sabiduría, en la compasión y el liderazgo, yo no le encuentro par en la historia de la medicina puertorriqueña. Permítanme el honor de citar lo que dijo un día una de sus alumnas distinguidas, la Dra. Tiody Vda. de Ferré: “Los zapatos de mi viejo, no los llena ni un gigante.” asociacionmedicapr.org Preventing two of chemotherapy’s toughest side effects is possible... Don’t accept nausea & vomiting from chemotherapy. Stand up to it with the help of EMEND. Ask your doctor or nurse if adding EMEND to your treatment plan is right for you. Help prevent nausea and vomiting—before it starts! To learn more about EMEND, log on to standuptoit.com. EMEND, in combination with other medicines, helps prevent chemotherapy-induced nausea and vomiting (CINV). Important information about EMEND EMEND is only used to help prevent nausea and vomiting caused by chemotherapy. It is not used to get rid of nausea and vomiting after they start. Do not take EMEND if you are taking Orap (pimozide), Seldane (terfenadine), Hismanal (astemizole), or Propulsid (cisapride). Taking EMEND with these medicines could cause serious or life-threatening problems. EMEND may also affect some medicines, including chemotherapy, causing them to work differently in your body. Patients taking Coumadin (warfarin) may need to have blood tests after taking EMEND to check their blood clotting. Women who use birth control medicines during treatment with EMEND and for up to 1 month after using EMEND should also use a backup method of contraception to avoid pregnancy. if you have liver problems. Tell your doctor if you are pregnant or plan to become pregnant because it is not known if EMEND can harm your unborn baby. Tell your doctor if you are breast-feeding because it is not known if EMEND passes into your milk or if it can harm your baby. The most common side effects of EMEND are tiredness, nausea, hiccups, constipation, diarrhea, loss of appetite, headache, and hair loss. These are not all the possible side effects of EMEND. EMEND is available by prescription only. Please read the Patient Information for EMEND on the next page and discuss it with your doctor. Need help paying for your EMEND? Merck may be able to help. Please call 866-363-6379 (866-EMEND-Rx). You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088. Tell your doctor if you are taking or plan to take prescription or nonprescription medicines, vitamins, or herbal supplements, or EMEND is a registered trademark of Merck & Co., Inc. Other brands listed are the trademarks of their respective owners and are not trademarks of Merck & Co., Inc. emend.com Copyright © 2009 Merck & Co., Inc. All rights reserved. 20851971(1)(807)-EME Introducing the first new molecule in analgesia in over 25 years for the relief of moderate to severe acute pain in patients 18 years of age or older l HEALTH information technologies Manténgase informado Nuestro Portal Médico le brindará todo lo que Usted necesita saber www.asociacionmedicapr.org 72 4.0 CME Credits Boletin Vol 101 No 02, 2009 Questions from article: “CARACTERISTICAS PSICOSOCIALES DE UNA MUESTRA INICIAL DE CENTENARIOS PUERTORIQUEÑOS – Rodriguez-Gomez et al” 1. 1. Se proyecta que el cambio porcentual de la población de ancianos mayores de 65 años entre los años 2010 al 2050 será aproximadamente de: a. 15% b. 30% c. 40% d. 50% 2. En Puerto Rico, los centenarios/as, según las ultimas estadísticas del Negociado del Censo Federal son: a. aproximadamente 500 b. entre 550-700 c. aproximadamente 916 d. más de 2000 3. En la muestra de centenarios/as del estudio de Rodríguez, Martínez y Cruz (2009) se encontró que las condiciones psicológicas más comunes reportadas eran: a. Desesperanza y Ansiedad b. Ansiedad y Bipolaridad c. Narcolepsia y Desorden de Pánico d. Depresión y alcoholismo Questions from article: “OBESITY PREVALENCE ODDS RATIOS IN FOUR PUERTO RICAN TOWNS: A PILOT STUDY – Laureano et al” 4. La prevalencia de obesidad para ambos sexos en Puerto Rico en el Behavioral Risk Factor Surveillance System (BRFSS) para el año 2005 fue de: a. 50% b. 10% c. 35% d. 23% (√) 5. De las ciudades bajo estudio las que tuvieron una mayor prevalencia de obesidad fueron: a. Canóvanas y Loíza (√) b. Río Grande y Canóvanas c. Loíza y Luquillo d. Río Grande y Luquillo 6. El “International Obesity Task Force” establece la clasificación de un adulto con sobrepeso con un BMI de a. 15.7- 21.9 kg/m2 b. 25.0 - 29.9 kg/m2 (√) c. < 16.9 kg/m2 d. > 30 kg/m2 Questions from article: “EPIDEMIOLOGICAL CHARACTERIZATION OF PEDIATRIC PATIENTS WITH STATUS ASTHMATICUS ADMITTED TO INTENSIVE CARE UNIT – Silva et al” 8. En ocasiones las exacerbaciones de asma se asocian a un patrón en ciertos meses del año. En nuestro articulo los meses mas frecuentes de exacerbaciones fueron a. enero febrero y marzo b. abril, mayo, y junio c. julio, agosto y septiembre d. octubre, noviembre y diciembre e. ningún patrón 9. El manejo de asma persistente requiere de medicamentos de prevención. De los pacientes admitidos a la unidad de intensivo pediátricos que porciento de estos recibieron terapia de prevención a. 100% b .75% c. 50% d. 25% e. 10% Quesions from article: “EOSINOPHILIC ESOPHAGITIS AND ALLERGIES IN PEDIATRIC POPULATION OF PUERTO RICO – Velazquez et al” 10 . Cual de las siguientes es cierta acerca de la Esofagitis Eosinofilica a. ocurre solamente en adultos b. se caracteriza por un conteo absoluto alto de eosinófilos. c. se caracteriza por la presencia de 15 o mas eosinófilos en biopsia de esófago d. mejora con medicamentos anti-reflujo e. el síntoma característico es la diarrea 11. En nuestro estudio que porcentaje de los pacientes tuvieron alergia a las comidas a. 50% b. 35% c. 20% d. 15% e. 6% 12. Dentro de las alergias ambientales en nuestro estudio cual se identifico el alérgeno como la más prevalente a. grama b. polen de árboles c. ácaros del polvo d. hongos e. plumas 7 . El asma es un problema de salud pública importante en nuestra población. De las siguientes poblaciones quien tiene la prevalencia mas alta de esta enfermedad: Questions from article: “IS BARIUM ENEMA AN ADEQUATE DIAGNOSTIC TEST FOR THE EVALUATION OF PATIENTS WITH POSITIVE FECAL OCCULT BLOOD? – Ramos et al” a. Estados Unidos b. Cuba c. Puerto Rico d. Republica Dominicana e. México 13. In 2004, the Puerto Rico Central Cancer registry identified colorectal cancer as: a. The second most common cause of cancer in both men and women accounting for 14% of all cancers in the island. b. The second leading cause of death among women. b. The ninth cause of death among patients >80 years. c. a and b are correct. d. None of the above. ANSWERS 73 1- (A) (B) (C) (D) (E) 2- A) (B) (C) (D) (E) 14. Which of the following is not a screening tool for colorectal cancer in average risk patients: a. Yearly Fecal occult blood testing ( 3 samples) b. Colonoscopy c. Genetic testing d. Double-contrast barium enema e. Flexible sigmoidoscopy combined with FOBT 3- (A) (B) (C) (D) (E) 4- (A) (B) (C) (D) (E) 5- (A) (B) (C) (D) (E) 6- (A) (B) (C) (D) (E) 7- (A) (B) (C) (D) (E) 15. Which of the following statements is false regarding the performance of Double contrast Barium enema in this study: a. DCBE missed 84% of the polyps, most of them adenomatous. b. Seventeen (17%) of the polyps missed were ≥ 10 mm in size. c. DCBE had a sensitivity of 45%, a specificity of 90%. d. Overall diagnostic accuracy of 54% for colonic neoplasia (adenomas/cancer) of any size. e, When considering patient tolerability most patients preferred the DCBE over colonoscopy. 8- (A) (B) (C) (D) (E) 9- (A) (B) (C) (D) (E) 10- (A) (B) (C) (D) (E) 11- (A) (B) (C) (D) (E) 12- (A) (B) (C) (D) (E) 13- (A) (B) (C) (D) (E) 14- (A) (B) (C) (D) (E) 15- (A) (B) (C) (D) (E) Questions from article: “ANEMIA AND INFECTIONS IN MULTIPLE MYELOMA: SUPPORTIVE THERAPY – Caceres et al” 16. En el momento de diagnóstico, qué porciento de pacientes con mieloma múltiple poseen un valor de hemoglobina menor o igual de 9 gm/dl? 16- (A) (B) (C) (D) (E) 17- (A) (B) (C) (D) (E) 18- (A) (B) (C) (D) (E) 19- (A) (B) A. 100% B. 75% C. 35% D. 10% 17. La etiología de anemia en pacientes que padecen de mieloma múltiple incluye: Fill in the following information: Name __________________________________________ Licence No. ________________________ A. Afectación de la médula ósea B. Deficiencia relativa en eritropoyetina endógena C. Aumento en volúmen plasmático D. Todas las anteriores Postal Address____________________________________ 18. La complicación infecciosa más común en los pacientes con mieloma tratados con Bortezomib es: Telephone ___________________ Fax ________________ A. Streptococcus pneumonia B. Herpes zoster C. H. influenza D. Pseudomonas aeruginosa 19. La anemia de mieloma múltiple usualmente responde a administración exógena de eritropoyetina ________________________________________________ Email: __________________________________________ Cut along the dotted lines and send answers with check/money order for $20.00 payable to: Asociación Medica de Puerto Rico PO Box 9387 San Juan, PR 00908-9387 A. Cierto B. Falso You can download this Boletin and test, by free, from our website: www.asociacionmedicapr.org Besides, you can answer this test on-line. IMPORTANT TREATMENT CONSIDERATIONS PRISTIQ 50-mg Extended-Release Tablets are indicated for the treatment of major depressive disorder in adults. WARNING: SUICIDALITY AND ANTIDEPRESSANT DRUGS Antidepressants increased the risk compared to placebo of suicidal thinking and behavior (suicidality) in children, adolescents, and young adults in short-term studies of Major Depressive Disorder (MDD) and other psychiatric disorders. Anyone considering the use of PRISTIQ or any other antidepressant in a child, adolescent, or young adult must balance this risk with the clinical need. Short-term studies did not show an increase in the risk of suicidality with antidepressants compared to placebo in adults beyond age 24; there was a reduction in risk with antidepressants compared to placebo in adults aged 65 and older. Depression and certain other psychiatric disorders are themselves associated with increases in the risk of suicide. Patients of all ages who are started on antidepressant therapy should be monitored appropriately and observed closely for clinical worsening, suicidality, or unusual changes in behavior. Families and caregivers should be advised of the need for close observation and communication with the prescriber. PRISTIQ is not approved for use in pediatric patients. Contraindications • PRISTIQ is contraindicated in patients with a known hypersensitivity to PRISTIQ or venlafaxine. • PRISTIQ must not be used concomitantly with an MAOI or within 14 days of stopping an MAOI. Allow 7 days after stopping PRISTIQ before starting an MAOI. Warnings and Precautions • All patients treated with antidepressants should be monitored appropriately and observed closely for clinical worsening, suicidality, and unusual changes in behavior, especially during the first few months of treatment and when changing the dose. Consider changing the therapeutic regimen, including possibly discontinuing the medication, in patients whose depression is persistently worse or includes symptoms of anxiety, agitation, panic attacks, insomnia, irritability, hostility, aggressiveness, impulsivity, akathisia, hypomania, mania,or suicidality that are severe, abrupt in onset, or were not part of the patient’s presenting symptoms. Families and caregivers of patients being treated with antidepressants should be alerted about the need to monitor patients. • Development of a potentially life-threatening serotonin syndrome or Neuroleptic Malignant Syndrome-like reactions have been reported with SNRIs and SSRIs alone, including PRISTIQ treatment, but particularly with concomitant use of serotonergic drugs, including triptans, with drugs that impair the metabolism of serotonin (including MAOIs), or with antipsychotics or other dopamine antagonists. If concomitant use with a triptan is clinically warranted, careful observation of the patient is advised, particularly during treatment initiation and dose increases. Concomitant use of PRISTIQ with serotonin precursors is not recommended. • Patients receiving PRISTIQ should have regular monitoring of blood pressure since sustained increases in blood pressure were observed in clinical studies. Pre-existing hypertension should be controlled before starting PRISTIQ. Caution should be exercised in treating patients with pre-existing hypertension or other underlying conditions that might be compromised by increases in blood pressure. Cases of elevated blood pressure requiring immediate treatment have been reported. For patients who experience a sustained increase in blood pressure, either dose reduction or discontinuation should be considered. For the treatment of adults with major depressive disorder The start is just the beginning It’s not just about starting your adult patients with MDD on therapy; it’s about helping them toward their treatment goals. Patients should be periodically reassessed to determine the need for continued treatment.1 PRISTIQ 50 mg: • SNRI therapy with efficacy proven in 8-week clinical studies • One recommended therapeutic dose from the start • Discontinuation rate due to adverse events comparable to placebo in 8-week clinical studies1 • SSRIs and SNRIs, including PRISTIQ, may increase the risk of bleeding events. Concomitant use of aspirin, NSAIDs, warfarin, and other anticoagulants may add to this risk. • Mydriasis has been reported in association with PRISTIQ; therefore, patients with raised intraocular pressure or those at risk of acute narrow-angle glaucoma (angle-closure glaucoma) should be monitored. • PRISTIQ is not approved for use in bipolar depression. Prior to initiating treatment with an antidepressant, patients should be adequately screened to determine the risk of bipolar disorder. • As with all antidepressants, PRISTIQ should be used cautiously in patients with a history or family history of mania or hypomania, or with a history of seizure disorder. • Caution is advised in administering PRISTIQ to patients with cardiovascular, cerebrovascular, or lipid metabolism disorders. Increases in blood pressure and small increases in heart rate were observed in clinical studies with PRISTIQ. PRISTIQ has not been evaluated systematically in patients with a recent history of myocardial infarction, unstable heart disease, uncontrolled hypertension, or cerebrovascular disease. • Dose-related elevations in fasting serum total cholesterol, LDL (low density lipoprotein) cholesterol, and triglycerides were observed in clinical studies. Measurement of serum lipids should be considered during PRISTIQ treatment. • On discontinuation, adverse events, some of which may be serious, have been reported with PRISTIQ and other SSRIs and SNRIs. Abrupt discontinuation of PRISTIQ has been associated with the appearance of new symptoms. Patients should be monitored for symptoms when discontinuing treatment. A gradual reduction in dose (by giving 50 mg of PRISTIQ less frequently) rather than abrupt cessation is recommended whenever possible. • Dosage adjustment (50 mg every other day) is necessary in patients with severe renal impairment or end-stage renal disease (ESRD). The dose should not be escalated in patients with moderate or severe renal impairment or ESRD. • Products containing desvenlafaxine and products containing venlafaxine should not be used concomitantly with PRISTIQ. • Hyponatremia may occur as a result of treatment with SSRIs and SNRIs, including PRISTIQ. Discontinuation of PRISTIQ should be considered in patients with symptomatic hyponatremia. • Interstitial lung disease and eosinophilic pneumonia associated with venlafaxine (the parent drug of PRISTIQ) therapy have been rarely reported. Adverse Reactions • The most commonly observed adverse reactions in patients taking PRISTIQ vs placebo for MDD in short-term fixed-dose premarketing studies (incidence ≥5% and twice the rate of placebo in the 50-mg dose group) were nausea (22% vs 10%), dizziness (13% vs 5%), hyperhidrosis (10% vs 4%), constipation (9% vs 4%), and decreased appetite (5% vs 2%). Reference: 1. Pristiq® (desvenlafaxine) Prescribing Information, Wyeth Pharmaceuticals Inc. Please see brief summary of Prescribing Information on adjacent pages. For more information on PRISTIQ, please visit www.PristiqHCP.com. © 2009, Wyeth Pharmaceuticals Inc. Philadelphia, PA 19101 254222-01 El website de la asociación Médica para orientación del paciente información de enfermedades seguimiento de epidemias buscadores de médicos, farmacias, hospitales y laboratorios de análisis clínico. Esta publicación llega a Ustedes gracias al auspicio de: