S aureus: eradicated in - Asociacion Medica de Puerto Rico

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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. (1978) Hopelessness: Clinical, experimental and theorical
aspects. New York: Hoeber.
(5)
Beck, A. T. (1987) Anxiety: Clinical, experimental and theorical aspects. New York: Hoeber.
(6)
Folstein, M.F., Folstein, S.E. & McHugh, P.R (1975). Mini Mental State: A practical method for grading the cognitive state of patients for the clinician. J.
Psychiatry Res., 12: 189-198.
(7)
Canino, G., Bird, H., Shrout, P., Rubio,-Stiper, M., Bravo, M., Martínez,
R., Sesman, M. & Guevara, L. (1997). The prevalence of Specific psychiatry disorders in Puerto Rico. Archives of General Psychiatric, 46, 727-735.
(8)
Atchley, R. (1991) The social faces in later life. 7 ed. California: Wadsworth Publishing Company.
(9)
United States Bureau of the Census (2000). Estimate of population of
Puerto Rico by age, sexual race. Washington (DC): Government Printing Office.
(10)
Oficina del Gobernador para Asuntos de la Vejez (2003). Datos Estadísticos y perfil poblacional de la Población Anciana. 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 <[email protected]
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
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Asociación Médica de Puerto Rico
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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
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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.
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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, Frühwirt 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
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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
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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% (&#8730;)
5. De las ciudades bajo estudio las que tuvieron una mayor
prevalencia de obesidad fueron:
a. Canóvanas y Loíza (&#8730;)
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 (&#8730;)
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
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