Dra. Wanda G. Vélez Andújar - Asociacion Medica de Puerto Rico

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Adopte
a la
Asociación Médica
BOLETIN
CONTENIDO
Médico Científico de la Asociación Médica de Puerto Rico
Año 106 Número 4 - Octubre a Diciembre de 2014
3
Mensaje del Presidente
Dra Wanda G. Vélez Andújar
Original Articles/Articulos Originales
6
CERVICAL CYTOLOGY TRENDS IN A SAMPLE OF INFLAMMATORY BOWEL DISEASE PATIENTS IN PUERTO RICO: A Descriptive Study and Research of Current Literature
Ana Muñoz-Matta MD, Roberto Osorio MD, Valerie Cabrera-León BS, Helen Oquendo MD, Esther Torres MD, Josefina Romaguera
Para médicos, cónyuge
médicos y sus hijos.
No es obligatorio ser so
de la Asociación Médica
Case Report/Reporte de Casos
Actividades para todas
22
UNCOMMON PRESENTATION OF FOLLICULAR THYROID CARCINOMA: When Chronic edades
Back Pain Should
Raise
(incluso
Seniors
a Flag
Milliette Alvarado MD, Margarita Ramirez-Vick MD, Liurka Lopez MD, Maria J Marcos-Martinez MD, Fanor M. Saavedra
MD, Juan C. Negron-Rivera
Infórmese:
(787)721-696
MD, Marielba Agosto MD, Meliza Martinez MD, Rafael Gonzalez MD, Myriam Allende-Vigo MD
Sr. Laborde
27
ANAL LARGE CELL NEUROENDOCRINE CARCINOMA WITH TUBULOVILLOUS ADENOMA COEXISTENCE: A 11
FINGERTIP RECONSTRUCTION WITH THE “SHARK MOUTH” INCISION THENAR FLAP: Analysis of Outcomes in Pediatric Patients
Manuel García Aríz MD, Antonio Otero López MD, Ernesto R. Otero López MD, Beatriz García MD, Pablo A. Marrero Barrera MD, Samuel Fernández MS
17
THE RELATIONSHIP BETWEEN TYPE 2 DIABETES MELLITUS AND BODY MASS INDEX: Data from a Primary
Care Facility in Puerto Rico
Rolance G. Chavier-Roper MD, Sharlene Alick-Ortiz MS, Geraldine Davila-Plaza MS, Aixa G. Morales-Quiñones MS
Case Report
29
PRIMARY IMMUNE REFRACTORY THROMBOCYTOPENIA IN ASSOCIATION WITH SECONDARY DISORDERS: A
Case Report
31
GRANULOMATOSIS WITH POLYANGIITIS IN PUERTO RICO: Case Reports
35
THE ROLE OF NUCLEAR IMAGING IN TRAUMATIC HEPATOPLEURAL FISTULA: A Case Report
Juan C Negrón MD, Ralph Martin MD, Frieda Silva MD, Gary Ehlert MD
38
THE USE OF A FIBULAR STRUT ALLOGRAFT WITH DBM, CANCELLOUS CHIPS AND BMP FOR A 10 CM HUMER
AL SHAFT INFECTED NON-UNION: A Case Report
43
A VERY RARE NEOPLASM IN AN IMMUNOCOMPETENT PATIENT
49
A TRAUMATIC BILATERAL FEMORAL NECK FRACTURES IN A HEALTHY PREMENOPAUSAL FEMALE WITH
HYPOVITAMINOSIS D
53
SINDROME DE LYELL POTENCIALMENTE FATAL
Iván E. Aponte Aponte MD, William Cáceres MD
Iván E. Aponte Aponte, MD
Luis Aranguren MS, Natalio J. Izquierdo MD, Armando Oliver MD
Pedro J. Reyes Martínez MD, Pablo A. Marrero Barrera MD
Emmanuel O. González-Irizarry MD, Luis A Figueroa-Jiménez MD, Javier Ramos Rossy MD, Rodolfo Estremera MD, Javier Monserrate MD, Amy
Lee González-Márquez MD, Luis Negrón-Garcίa MD, Mόnica Santiago-Casiano MD, William Cáceres-Perkins MD
Giovanni Paraliticci, MD, David Rodríguez-Quintana MD, Ariel Dávila MD, Antonio Otero-López MD
Daniel Agustín Godoy MD, Erica Alvarez, Andrea Amigot, Mario Di Napoli MD, Luca Massoti
Review Articles/Artículos de Reseña
58
HEMATOMA SUBDURAL CRÓNICO: Fisiopatología y Manejo Quirúrgico
Hernando Raphael Alvis-Miranda, Gabriel Alcalá-Cerra, Andrés M. Rubiano, Luis Rafael Moscote-Salazar
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
OFICINAS ADMINISTRATIVAS:
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Ilustración digital de cubierta y diseño gráfico realizados por Juan Laborde-Crocela en la Oficina de Informática de la AMPR. Impreso en los talleres
gráficos digitales de la Asociación Médica de Puerto Rico - E-mail:[email protected]
Detail of restored stained glass
window St. Johns Anglican
Church Lunenburg Nova Scotia
Canada
Mensaje del Presidente
Asociacion Medica de Puerto Rico
Junta de Directores 2013/2014
A
Dra. Wanda G. Vélez Andújar
18 de diciembre de 2014
A nombre de los miembros de la Junta de Directores de la Asociación Medica de Puerto Rico les ofrezco un cordial saludo y
deseo hayan tenido unas muy Felices Pascuas.
Ya nos encontramos programando mi segundo año como presidente de esta honorable asociación, y espero que nuestras actividades sean para el agrado y disfrute de todos.
Dra. Wanda G. Velez Andujar
Presidente
Dr. Ricardo Marrero Santiago
Dr. Eliud López Vélez
Presidente electo
Dr. Natalio Izquierdo Encarnación
Presidente saliente
Dr. José R. Villamil Rodríguez
Tesorero
Vicepres. Cámara Delegados
Dr. Gonzalo González Liboy
Delegado AMA
Dr. Rolance G. Chavier Roper
Delegado AMA
Dra. Ilsa Figueroa
Dr. Luis A. Román Irizarry
Secretaria
Delegado Alt. AMA
Dra. Hilda Ocasio Maldonado
Vicepresidente AMPR
Dr. Rafael Fernández Feliberti
Dr. Raúl A. Yordán
Delegado Alterno AMA
Dr. Salvador Torrós Romeu
Vicepresidente AMPR
Pres. Distrito Este
Dr. Jaime M. Díaz Hernandez
Vicepresidente AMPR
Dr. Benigno López López
Dra. Mildred R. Arché
Pres. Distrito Central
Dr. Rubén Rivera Carrión
Pres. Cámara Delegados
Pres. Distrito Sur
Dr. Humberto Lugo Vicente
Presidente de la Junta Editora del Boletin
Es tan interesante observar cómo nos pasa el tiempo y cuantas
cosas de envergadura pueden ocurrir entre una edición y otra.
Estamos comenzando una era donde las relaciones entre Estados Unidos y Cuba han sido reinstaladas. ¡Fueron muchos
los años en que estuvieron rotas! En la medicina, el ebola nos
exigió estar más alerta al tomar el historial del paciente para incluir sus recientes viajes y personas de contacto. Vimos en las
noticias todos los contratiempos que le trajo a alguna Sala de
Emergencia en USA. El acortamiento de las distancias es impresionante y no podemos ignorar los problemas
de salud de otros países, no importa lo lejano que esté de nosotros. En cuanto a nuestro ámbito local, el virus
del Chikungunya, ¡ha sido terri-ble! Nuestros pacientes han sufrido tantas diferentes presentaciones clínicas que
pronosticarles el futuro es un gran reto para cualquiera de nosotros. Estos “retos”, nos han obligado a “regresar
al salón de clases de medicina”, para estudiar que esta ocurriendo. Esto es lo que me lleva a las “Educaciones
Medicas Continuadas” (CME por sus siglas en ingles).
Practicar la medicina en el siglo 21 va mucho más allá de graduarse de una excelente escuela de medicina, hacer
el internado y la especialidad en el mejor hospital posible; o en ser el mejor y más destacado estudiante y/o residente. Vivimos una era donde cada día tenemos que adquirir nuevos conocimientos además de terapéuticos y de
adelantos técnicos, también clínicos. Por lo tanto, todo esto nos lleva a convertirnos en ¡estudiantes perpetuos! Y, ¿cómo se logra? Con las Educaciones Médicas Continuadas.
Participar de CME no solo es un requisito para mantener nuestra licencia de médicos como nos lo exige la Junta
de Licenciamiento y Disciplina Medica de Puerto Rico. Esas 60 horas/crédito que cada tres años la JLDM pide
para reconocernos como médicos competentes, puede que se queden cortas ante los inminentes y rápidos cambios en nuestra profesión actualmente.
Las educaciones medicas continuadas son nuestra escuela de medicina tan pronto nos convertimos en los médicos activos del país. Los proveedores de las CME son aquellos que velan por brindarle a sus médicos participantes actividades educativas con las cuales puedan:
1. Obtener conocimiento (knowledge)
2. Conservar su competencia (competence) 3. Mejorar su ejecutoria (performance)
Todo esto es indispensable para que se logre la mejor atención médica para nuestros pacientes. Necesitamos
que nuestros médicos siempre ofrezcan una medicina de insuperable calidad en todos los sentidos.
La Asociación Médica de Puerto Rico tiene como norte cumplir con todos los requisitos de brindar una educación
médica continuada de máxima calidad. Este año, estaremos brindando CME mensualmente y en nuestra convención la cual será el 17 al 19 de abril de 2015.
2 | BOLETIN Médico Científico de la Asociación Médica de Puerto Rico
BOLETIN Médico Científico de la Asociación Médica de Puerto Rico | 3
Gracias a nuestros auspiciantes sin cuyo aporte no habría sido posible la
edición de este número de nuestro Boletín
Fundación Médica
de Puerto Rico
CERVICAL CYTOLOGY
TRENDS IN A SAMPLE
OF INFLAMMATORY
BOWEL DISEASE
PATIENTS IN PUERTO
RICO: A Descriptive
Study and Research of
Current Literature
Ana Muñoz-Matta MDa
Roberto Osorio MDb
Valerie Cabrera-León BSc
Helen Oquendo MDa
Esther Torres MDb
Josefina Romaguera MDa
Department of Obstetrics and Gynecology, University of Puerto Rico School of
Medicine.
b
Department of Medicine, University of
Puerto Rico School of Medicine.
c
University of Puerto Rico School of Medicine.
*Corresponding author: Josefina Romaguera MD - Medical Sciences Campus,
Dept. of Obstetrics and Gynecology,
GPO Box 36-5067, San Juan, PR 00927.
E-mail: [email protected].
a
I
INTRODUCTION
Inflammatory Bowel Disease (IBD) is a term that encompasses conditions characterized by recurrent inflammation of the
gastrointestinal tract (GI). In IBD, inflammation is caused by
an exaggerated immune response to normal components of
the GI tract targeted against gastrointestinal cells. The most
common and well-known IBD conditions are Crohn’s Disease
(CD) and Ulcerative Colitis (UC). In the United States, precise
incidence and prevalence of these conditions is limited due to
lack of gold standards for diagnostic criteria, inconsistent case
ascertainment and disease misclassification (1). However,
prevalence has been described at 263 per 100,000 for UC and
241 per 100,000 for CD in a large data group encompassing
approximately 12 million Americans (2). According to previous
studies, the prevalence rates of IBD in Puerto Rico vary around
5.9 (rural) per 100,000 to 62.2 (urban) per 100,000 in the early
2000’s (3,4). More recent studies describe the total prevalence
of IBD conditions in Puerto Rico according to insurance claims
filed by gastroenterologists being at 38.2 cases per 100,000
(14.9 cases for CD and 23.3 cases for UC respectively) (5).
As compared with older reports, an increasing trend has been
constantly described since the early 2000’s (3). Although gender variation is small, a female predominance has been described for CD when compared to UC. However this may vary
according to geographic location and age group. The mean
age of diagnosis for IBD ranges from 15-40 years, similar to
other immunologically mediated conditions. Additional data
shows a bi-modal diagnosis curve with another peak present
after 50 years and reaching until 80 years of age.
ABSTRACT
Objective of the study was to describe
cervical cytology trends in a sample of
patients with Inflammatory Bowel Disease (IBD) and to provide an overview of
aspects such as contraception and menstrual patterns. STUDY DESIGN: We
identified women with diagnosis of IBD
of ages between 21-49 years followed
at the IBD clinics of the Medical Sciences Campus from June 2012 to April
2014. A 15-minute questionnaire was
administered. Data was entered and analyzed calculating frequencies and percentages. RESULTS: Sixty-three subjects were recruited. After reviewing the
questionnaires, 52 subjects remained
for analysis. All women were between
21 and 49 years of age. Thirty (58%)
patients had a diagnosis of ulcerative
colitis (UC), while 22 (42%) patients had
Crohn’s Disease (CD). Twenty-seven
IBD patients (52%) were in remission.
Use of immunomodulators was reported in ten (33%) and seventeen (61%)
of patients for UC and CD respectively.
Sixty six percent (67%) of participants
specified having their cervical cytology
for cervical cancer screening done annually. Twenty patients (38%) reported
abnormal cytology including 13 patients
(43%) with UC and 7 patients (23%)
with CD. No significant relation was
found between the two conditions and
the self-reported history of abnormal
cervical cytology. Almost one-half of IBD
patients reported a menstrual cycle of
25-30 days (45%). Duration of menses
was described as normal (lasting between 4-6 days) in 45% of IBD patients
(95% CI 31.13-59.66). Patients with UC
were more likely to report regular menses than patients with CD. Eighty-six
percent of participants reported no use
of contraception. CONCLUSION: This
is the first descriptive report of gynecologic conditions in which Puerto Rican
patients with IBD have been studied.
It is imperative to continue with similar
larger studies to gain a broader idea of
what are the gynecological needs of this
population.
Index words: cervical, cytology, inflammatory bowel disease, Puerto
Rico
Among recommended treatments for both UC and
CD, immunomodulators are the most commonly used
long-term therapies for both UC and CD. Targownik
recently reported that the proportion of corticosteroid
use in patients within the first year of diagnosis was
35%, with an increase to up to 63% within 10 years
of diagnosis (6). This proportion increased continuously through the years and at 10 years after diagnosis. Agents against Tumor Necrosis Factor are also
used as standard therapy for this condition. In recent
years, immunomodulators have become one of the
most implemented therapies in an attempt to change
the natural course of the disease (7,8). However, the
use of these agents is not without risk, including serious infection and malignancy. The most commonly
used therapies are immunomodulators, such as thiopurines (azathioprine or 6-mercaptopurine) or methotrexate, and biologics including anti-tumor necrosis
factor agents, such as infliximab, adalimumab and
certolizumab pegol.
A strong interest in the gynecological complications
common to women with IBD is arising, as evidenced
by the emerging literature on the topic. Most studies
are limited due to the epidemiology of the conditions
as well as the more private aspect of the gynecological medical field. Nevertheless, IBD patients represent a group of patients that cannot be overlooked.
Sharing the abdominopelvic space, both gastrointestinal and reproductive systems can be closely influenced by one another by the local release of substances and the hostile environment they share.
Human Papilloma Virus (HPV) is a known cause of
cervical dysplasia and cervical cancer. Studies have
shown a direct effect of HPV infection on the cellular architecture of the cervix. Most recent guidelines
have incorporated HPV co-testing with cytology as
part of the cervical cancer screening. In April 2014,
the FDA approved the use of an HPV test as a single
cervical screening tool. The cobas HPV test® (Roche
Molecular Systems, Inc. Pleasanton, California) can
be used as an initial screening mechanism to dictate
whether or not more aggressive testing is needed
(9). These new recommendations are still waiting
for approval from cervical cancer screening authorities such as The American Cancer Society (ACA),
American Cancer Society for Colposcopy and Cervical Cytology (ASCCP), the U.S. Preventive Service
Task Force (USPSTF), and The American College of
Obstetricians and Gynecologists (ACOG). This new
information clearly shows the importance of the presence and HPV status of a patient regarding future
cervical dysplasia and/ or cancer.
The ability to clear HPV infections is dependent on
the immunologic system of each patient. Immunocompromised patients have a limited ability to clear
HPV infection. A large number of studies have reported a higher incidence of cervical cancer among
immunosuppressed patients, most commonly HIV
infected patients. Cervical cancer is an AIDS defining
condition and its relation to HPV has been well documented.
IBD patients represent an especially interesting
group to study as most have some degree of immunocompromise. In 2008, Kane et al. published a
study comparing the use of immunomodulators in a
sample of IBD patients. They found that not only did
IBD patients have a higher rate of abnormal cervical cytology but also that the grade of dysplasia increased with the amount of immunomodulators used
by each patient (10). An older study with a larger
sample revealed that although dysplasia was more
common in IBD women when compared to the general population, higher grades of dysplasia were not
associated to the use of immunomodulators (11). Adequate cervical screening practices have also been
studied in this population. A Canadian publication in
2011 presented cervical cancer screening practices
among IBD patients. This study evidenced that there
was no major difference between that of the general
population and the target population. However, this
difference increased with associated risks. For example, patients with CD living in low socioeconomic status and with exposure to immunosuppressants were
less likely to undergo cervical cancer screening (12).
In one of the most crucial reviews written about this
topic, Weber describes that IBD patients are more
likely to suffer from gynecologic conditions such as
vaginal discharge, infertility, gynecologic surgery and
menstrual abnormalities when compared to healthy
controls (13). Furthermore, new concerns have been
raised about the use and administration of contraceptives in IBD patients. A recent study about these
concerns reported a 25% non-contraceptive use
in this population during reproductive years. At the
same time, they were able to link effective and correct use of contraceptives with a higher severity of
the condition (14).
It is natural to wonder about the effect that IBD has in
female patients’ reproductive health as well as the effect of the current treatment modalities in the pathological evolution of certain gynecological conditions. Our study aims to describe cervical cytological trends
in a sample of IBD patients who have follow up at our
clinics as well as to give an overview of certain aspects such as contraception and menstrual patterns.
The University of Puerto Rico has been home to a
weekly multidisciplinary clinic dedicated to Inflammatory Bowel Disease for many years. The Clinic has
grown into a multidisciplinary center with an expertise that is unique in Puerto Rico and the Caribbean.
This clinic has provided first class directed care to
over 1000 patients from all over the island. The UPR
Center for IBD offers superb medical care, education, support and community service, and serves as
a training site for students, residents and fellows as
well as a research center for IBD.
Regarding use of contraceptive methods, 45 (86%) participants reported no
use of contraception. Among participants
that described the use of contraceptive
methods, condoms and coitus interruptus
were the only two methods reported. No
use of oral contraceptives, intrauterine
devices or depot medroxyprogesterone
acetate was reported. Although sexually
transmitted infections were not more frequent in the IBD population, 7 (13%) IBD
patients reported history of bacterial vaginosis (see Figure 4).
METHODS
A questionnaire was provided to female patients waiting to be evaluated at the IBD Clinics of the University of Puerto Rico School Of Medicine from June 2012
to April 2014. Participation was restricted to female
patients 21-49 years of age with an established diagnosis of Inflammatory Bowel Disease. Once objectives of the study were presented and informed consent was obtained, a 15-minute questionnaire was
provided to the patient. The questionnaire included
topics related to general gynecological health, overall
health, type of medications used for IBD and date of
diagnosis, among others. Most of the questions were
asked in a yes/no format and other answer choices
were presented in ranges. Research personnel were
available at hand to help with adequate question interpretation. The Medical Sciences Campus Institutional Review Board approved the study.
DISCUSSION
Figure 1: Inflammatory Bowel Disease Diagnosis of participants.
Data was entered and analyzed using Epi Info 7
(Centers for Disease Control and Prevention 1600
Clifton Rd Mail Stop E-91 Atlanta, Georgia 30333).
Frequencies and percentages were calculated using
this tool.
RESULTS
Sixty-three subjects were recruited. After reviewing
the questionnaires, one patient was provided the
questionnaire twice and 10 were wrongly labeled as
IBD patients. Fifty-two subjects remained for analysis. All women were between 21 and 49 years of age.
All participants were still in their reproductive years.
Thirty (58%) patients had a diagnosis of UC, while
22 (42%) had CD (see Figure 1). Disease activity
ranged from remission to active disease among participants for both conditions. For the UC group, 15
(50%) of participants were in remission according to
the Rachmilewitz Activity Index, of which 6 (20%) fell
under the moderate disease group. No participants
were described as having severe disease. Disease
activity in CD patients was calculated using the Harvey-Bradshaw Index. Twelve (55%) CD patients were
in remission, two (9%) had severe disease and moderate and low disease activity was equally reported in
four (18%). Immunomodulators use was reported in
10 (33%) and 17 (77%) of patients for UC and CD patients respectively. Anti-TNFα medication was used
in 24 (80%) patients with UC and 5 (23%) with CD
(see Figure 2).
In relation to cervical screening practices, 18 (82%)
patients with CD reported having had cervical cancer
screening during their lifetime. Of the four patients
without cervical screening, one was 21 years old and
the three others were all above 21 years. According
to the American Society for Colposcopy and Cervical
Pathology (ASCCP) cervical cancer screening should
begin at age 21. Therefore, all four patients (8%) were
not complying with the recommended screening practices (15). For the UC group, 28 (93%) of patients
Figure 2: Disease Activity among study participants according to
the Rachmilewitz Questionnaire for Ulcerative Colitis and the Harvey-Bradshaw Questionnaire for Crohn’s Disease.
stated having the screening done at least once in
their lifetime. Only one participant (5%) expressed
never having a screening done; the patient had also
recently turned 21. When asked how often they had
their cervical cancer screening done, 35 (67%) participants of both UC and CD specified having their
screening done annually.
History of abnormal cytology was reported by 20
(38%) patients; thirteen (43%) with UC and 7 (32%)
with CD. When cross tabulation was done between
frequency of screening and history of abnormal cervical cytology, no significant relation was found between the two of them.
Duration of menses was described as normal (lasting
between 4-7 days) in 23 (45%) IBD patients (95%
CI 31.13-59.66%). Fourteen (65%) CD patients
described their period as regular (95%CI 46.4980.25%). For UC patients, 21 (71%) participants described periods as regular (95%CI 44.04-89.69) (see
Figure 3).
Although recent literature has shown a
new scientific interest in the gynecological issues associated with IBD patients,
the amount of literature is limited, studies
are sparse and the number of subjects
in most studies is small. No previous reports were found in the current literature
regarding gynecological conditions in IBD
patients specific to the Puerto Rican population. The main focus of our study was
to describe the most common gynecologic conditions in our patient sample and at
the same time compare them to the current literature.
Figure 4: The described Gynecological conditions and Practices in our sample population.
Contraception use, as described in the
Canadian population, is low and the
methods reported by the patients are
not necessarily the most effective. Contraception is an important topic in these
patients as pregnancy has been shown to
Figure 3: Interval of Menstrual Cycles as reported by the patients.
alter disease activity. Although previous
studies have reported a lack of adequate
contraceptive use, it is important to take
into consideration that some of the IBD treatments (17), this is not seen in our sample. Conflicting results
such as methotrexate can adversely affect the grow- have been found in the literature with studies ranging fetus.
ing from large samples with lower controls to smaller
samples with tighter variable controls (7,10,14). CerPerception towards the use of contraception might be vical cytology patterns in immunocompromised painfluenced by patient location and culture. In the case tients might be influenced by HPV epidemiology and
of our study, the rate of contraceptive use seems to prevalence in certain geographical areas.
be low. When compared to the general population, a
national study published by the Center for Disease Menstrual irregularities are not present in our samControl regarding contraceptive use in the United ple nor seem to be influenced by presence of IBD
States and its territories showed a reported use of or severity of condition. Most patients reported their
oral contraception of 19% in Puerto Rican patients perception of the menstrual cycle as regular. This
at risk for pregnancy. The use of oral contraception perception was confirmed when analyzing menstrual
in the island represents the lowest percentage use patterns in day ranges also described by the patients.
in the U.S. and its territories. The only other contraceptive method more popular than oral contraceptive Our study limitations include a small sample size that
pills in the Puerto Rican population was surgical ster- might not be representative of the female population
ilization reported for 46% of women at risk of preg- with IBD in Puerto Rico. Another limitation is recall
nancy (16).
bias from the patients. Many patients may have forgotten specific diagnosis or occasions in which they
Although previous studies have suggested worsening were treated for a certain gynecological condition.
trends in cervical cytology associated to medications Also, the absence of cytology results represents a
limitation, as diagnosis could not be verified because
many of these patients receive their gynecological
care outside our institution.
This is the first descriptive study of gynecologic conditions in which Puerto Rican patients with IBD have
been studied. Our goal was to present a preliminary
report of the gynecological conditions that usually affect these patients.
It is evident that contraceptive education and recommendations are an important need in this group of
patients. It is imperative to continue with similar larger
studies to gain a broader idea of what are the gynecological needs of this population. Gynecologists and
gastroenterologists can then target their approach to
cover all the needs of the patient. Interaction and
discussion between the patient and her physicians
should be strongly encouraged.
Screening Guidelines for the Prevention and Early Detection of
Cervical Cancer, CA Cancer J clin. 2012 May-Jun; 62(3):147172. Accessed by July 2014 at: http://www.ncbi.nlm.nih.gov/pmc/
articles/PMC3801360/
16. Bensyl D, Juliano D, Carter M, Santelli J, Colley, Gilbert B.
Contraceptive Use---United States and Territories, Behavioral
Risk Factor Surveillance System, 2002. Surveillance Summaries. 2005. http://www.cdc.gov/mmwr/preview/mmwrhtml/ss54
06a1.htm. Accessed by June 12, 2014.
17. Lees CW, Critchley J, Chee N et al. Lack of association between cervical dysplasia and IBD: a large case-control study. Inflamm Bowel Dis. 2009; 15(11):1621-9.
Acknowledgement
The project described was supported by Award Number 8U54MD
007587-03 from the National Institute on Minority Health and
Health Disparities and Award Number 2U54MD007587 of the
Puerto Rico Clinical and Traslational Research Consortium of the
National Institute of Health. The content is solely the responsibility of the authors and does not necessarily represent the official
views of the National Institutes of Health.
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9. National institutes of health. FDA Panel Recommends HPV
Test as Replacement for Pap smear. http://www.nlm.nih.gov/
medlineplus/news/fullstory_145075.html. Accessed by June 12,
2014.
10. Kane S, Khatibi B, Reddy D. Higher incidence of abnormal
Pap smears in women with inflammatory bowel disease. Am J
Gastroenterol. 2008; 103(3):631-6.
11. Bhatia J, Bratcher J, Korelitz B et al. Abnormalities of uterine
cervix in women with inflammatory bowel disease. World J Gastroenterol. 2006; 12(38):6167-71.
12. Singh H, Nugent Z, Demers AA, Bernstein CN. Screening
for cervical and breast cancer among women with inflammatory bowel disease: a population-based study. Inflamm Bowel
Dis. 2011; 17(8):1741-50.
13. Weber AM, Ziegler C, Belinson JL, Mitchinson AR, Widrich T,
Fazio V. Gynecologic history of women with inflammatory bowel
disease. Obstet Gynecol. 1995; 86(5):843-7.
14. Gawron LM, Gawron AJ, Kasper A, Hammond C, Keefer L.
Contraceptive method selection by women with inflammatory
bowel diseases: a cross-sectional survey. Contraception. 2014;
89(5):419-25.
15.American Cancer Society,American Society for Colposcopy and
Cervical Pathology, and American Society for Clinical Pathology
RESUMEN
El objetivo de este estudio fue describir los patrones
en citología cervical de una muestra de pacientes
con enfermedad inflamatoria del intestino (EII) y los
aspectos relacionados a contracepción y patrones
menstruales en esta población. DISEÑO: Mujeres
entre 21 y 49 años de edad con enfermedad inflamatoria del intestino (EII) con seguimiento en nuestras
clínicas fueron identificadas en el periodo de junio
del 2012 a abril del 2014. Se les ofreció un cuestionario de duración de 15 minutos. Frecuencias y porcentajes fueron calculados utilizando estadística de
la data. RESULTADOS: Se reclutaron 63 mujeres,
de las cuales 52 permanecieron para análisis. Todas
las mujeres se encontraban entre las edades de 21
a 49 años. Treinta pacientes (58%) tenían diagnóstico de Colitis Ulcerosa (CU) y 22 (42%) enfermedad
de Crohn (EC). Veintisiete pacientes (52%) se encontraban en remisión. Los inmunomoduladores se
utilizaron en 33% de pacientes con CU y 61% de pacientes con EC. Sesenta y seis por ciento (67%) de
las participantes informaron hacerse tamizaje para
cáncer de cérvix anualmente. Cuarenta y un por
ciento de las pacientes con CU y 23% de pacientes
con EC refirieron haber tenido una prueba anormal.
Casi la mitad de las pacientes con EII reportaron ciclos menstruales de entre 25-30 días (45%) o 31-35
días (37%). Las pacientes de CU reportaron periodos menstruales más regulares que las pacientes de
EC. Ochenta y seis por ciento de las participantes
reportaron no haber utilizado un método anticonceptivo. CONCLUSIÓN: Este es el primer estudio de su
clase en describir condiciones ginecológicas características de pacientes puertorriqueñas con EII. Es
de suma importancia continuar estudiando esta población y sus necesidades para así poder establecer
guías de manejo que permitan un mejor tamizaje de
condiciones ginecológicas.
ABSTRACT
The thenar flap technique is a time-tested
method of fingertip reconstruction that has
been criticized due to possible finger flexion contractures and unsightly donor site
scars. Functional outcome data on thenar
flaps on the pediatric population is poor
in the medical literature. Method: In this
retrospective chart review we acquired
information from sixteen patients who underwent a “Shark Bite” incision thenar flap
reconstruction. Results: Patients ranged
in age from 0-17 years. Time to division
ranged from 16-30 days. All 16 patients’
affected finger ROM were measured in
DIP, PIP and MCP joints and compared
with corresponding finger on contralateral hand. A questionnaire measured subjective satisfaction in: sensibility, appearance, and function. Fingertip sensation
was preserved in both affected finger and
donor site. The most persistent patient
complaint involved fingertip contour, were
most patients rated it as “Fair”(43%) and
“Good”(56%). After statistical analysis of
the data we found no statistical difference
in range of motion (ROM) between affected finger and the same finger on the
contralateral hand on both PIP and MCP
joints (p=0.08, 0.06 respectively). Conclusions: The “Shark Mouth” incision thenar
flap is an effective strategy for fingertip
reconstruction. The results demonstrate
this technique has excellent functional
and aesthetic results and is not associated with flexion contractures, excessive
sensibility or pain in the pediatric population.
FINGERTIP
RECONSTRUCTION
WITH THE “SHARK
MOUTH” INCISION
THENAR FLAP:
Analysis of Outcomes
in Pediatric Patients
Manuel García Aríz MDa
Antonio Otero López MDa
Ernesto R. Otero López MDa
Beatriz García MDa
Pablo A. Marrero Barrera MDa*
Samuel Fernández MSa
Department of Orthopedic, UPR School of Medicine, Puerto Rico Health
Science Center.
*Corresponding author: Pablo A. Marrero Barrera MD - University of
Puerto Rico, Medical Sciences Campus, Department of Orthopedics PO
Box 365067, San Juan, Puerto Rico 00936-5067. E-mail: [email protected].
a
INTRODUCTION
T
The fingers serve a crucial role in the functioning of the
hand. They are an organ of manipulation and sensation and
are wrapped with countless sensory nerve endings, which
account for sensation of pain and an extremely sharp twopoint discrimination. The skin covering the pulp of the finger
is very durable and has a thick epidermis with deep papillary
ridges. It consists of fibrofatty tissue that is stabilized by fibrous septa extending from the dermis to the periosteum of
the distal phalanx. Unfortunately, the most common injuries
of the hand are fingertip injuries. These are injuries that ocIndex words: fingertip, reconstruction, cur distal to the insertion of flexor and extensor tendons (1).
shark, mouth, incision, thenar, flap, These injuries could be as insignificant as a fingertip fracture with no significant displacement or soft tissue injury, at
outcome
which point treatment is conservative, or could be as severe
as fingertip amputation with loss of distal fragment. In the latter case, even though preservation of the nail,
length and keeping a good appearance is important, the primary goal of treatment would be to maintain
good, strong, durable and sensate skin with no fingertip pain. Considerable dysfunction can occur when a
painful fingertip excludes the use of a digit, or even the hand from use. It is crucial to maintain sensation in
fingertips as not only does it help guide the finger along surfaces and aid in the overall functioning of the
whole hand, but it serves as a defense mechanism and protective sensation.
Treatment of fingertip injuries should be individualized to each specific wound’s characteristics. Various
methods of treatment are possible, including allowing wound to heal by secondary intention, shortening of
the bone and primary closure, skin grafting and coverage with a local and regional flap. It is important to
know whether there is loss of skin and pulp tissue and the size of the defect. Also knowing whether there is
exposed bone, bone loss or fracture of the distal phalanx tip is important for choosing the appropriate treatment. Primary closure of injury usually suffices for injuries with no skin or tissue loss. For those injuries with
loss of skin or pulp tissue with no exposure of bone, both skin grafting and allowing the wound to heal by
secondary intention are appropriate treatments. Several authors have reported few complications and
high patient satisfaction with the latter method (2,3),
specifically for wounds no larger than 1 cm2. Larger
wounds, however, when treated non-operatively heal
with a thin layer of epithelium that is relatively fragile and could be over-sensitive to the point of being
irritating and disabling to patients. Also, injuries that
involve exposed bone and tissue loss almost never
have enough tissue to close primarily, and treatment
by secondary intention after shortening exposed
bone below exposed tissue is associated with an unacceptable incidence of nail plate deformities (1). In
these cases the application of full thickness skin graft
should be considered. Full thickness skin grafts tend
to contract less than split-thickness grafts, are more
durable and less tender, and achieve better sensibility than split grafts (4).
One possible option for obtaining full coverage of
a fingertip injury is implementation of a thenar flap.
Thenar flaps are very advantageous in fingertip injuries with extensive tissue loss since they can provide a three-dimensional reconstruction that contains
similar skin to the original skin of the finger, has extensive sensory endings, and is functional, durable, and aesthetically pleasing. The caveats of flap
design are described by Beasley and Melone (5-7)
and state that the flap should be placed high on the
thenar eminence, based laterally, and adjacent to
the metacarpophalangeal joint crease. Placing the
incision too close to the mid-palm has been associated with debilitating donor-site tenderness (7). The
thenar flap can be used for fingers two to five, although problems may arise in thick hands were the
4th and 5th distal digits could have difficulty comfortably reaching the thenar eminence. Rinker described
that in a study with 15 patients who underwent thenar
flap reconstructions of fingertip injuries, 75% reported good or excellent results in terms of sensibility,
function and appearance (8). Dellon (9) reported a
small case series of five patients with thenar flaps
who had excellent sensory recovery. Barbato et al
(10), also reported a case series of 20 patients, who
also underwent thenar flap reconstruction, and also
resulted with excellent sensory recovery.
All the studies previously mentioned show that the
thenar flap is effective for sensory recovery of affected digit; however, functional outcome data is scarce
in medical literature. The technique has been criticized due possible flexion contractures of the proximal interphalangeal joint due to the mandatory digit
flexion. Barbato (10) reported in his thenar flap case
series a 25% rate of PIP flexion contracture requiring
extension splinting after flap division, but Rinker reports no significant flexion contractures observed in
his case series. He did however notice a measured
decrease in DIP joint motion of 13° compared to the
contralateral side that was statistically significant (8).
Recently, in the University of Puerto Rico School of
Medicine, pediatric orthopedic surgeons have been
implementing a modification to the thenar flap techniques currently described, specifically the shape of
the incision and flap. This new technique, termed
“Shark Mouth” incision thenar flap, has given excellent functional, sensory and aesthetic results in
this institution. In this retrospective chart review we
acquired information from 16 medical records of patients ages 0-17 years old, who underwent “Shark
Mouth” incision thenar flap reconstruction by two different surgeons after traumatic amputation at the level proximal to the middle portion of the nail bed with
loss of the pulp and exposure of bone from 20052011. The purpose of this study was to review the
experience of two pediatric surgeons with the “shark
mouth incision” thenar flap for composite fingertip
reconstruction. An objective assessment of sensory
recovery and range of motion was performed to confirm the approach in pediatric fingertip injuries in the
future. The main focus is to provide the best care,
in the adequate timeframe, and avoid unnecessary
or avoidable complications. Also, we wish to provide
the orthopedic community with a procedure that has
a small learning curve and excellent results.
MATERIAL & METHODS
Institutional Review
The present study was categorized as expedite and
received the approval of the participating institution’s
Institutional Review Board for research involving human subjects. This approval was given prior to beginning data collection.
Study Design
This is a retrospective study studying the medical records of pediatric patients ages 0-17, who underwent
thenar flap fingertip reconstruction with “shark mouth”
incision of distal 2nd, 3rd and 4th distal fingertip injuries. Patients underwent surgical treatment by two
separate pediatric orthopedic surgeons at the University Pediatric Hospital with follow-up at the Puerto Rico Trauma Center. A data sheet was created to
organize information extraction. This information included demographic data, mechanism of injury, type
of injury, timeline which included time to flap division,
complications that may have developed, and physical examination findings, specifically range of motion
of PIP, DIP and MP joints, and grip strength.
Participants
Inclusion Criteria
• Male and Female Patients
• 0 to 17 y/o
• Suffered traumatic amputation at the level proximal to the middle portion of the nail bed, with loss
of the pulp and exposure of bone in index, long
and middle fingers and underwent thenar flap reconstruction with at least 6 months of follow up Exclusion Criteria
• Patients older than 17 years old
• Multiple digit amputation
•
Patients who underwent other flap reconstruction Figure 1: Illustration demonstrating steps to follow to
or completion of amputation
perform the “Shark Mouth” incision thenar flap.
Procedure/Operative
Technique (see Figure 1)
The donor site was found by
taking the tip of the injured finger and placing it against the
thenar eminence. Two parallel
lines are traced along the lateral and medial borders of the affected finger. The finger is then
raised and the two lines are
joined distally by a curved line
(concave towards fingers and
convex proximally) creating an
angle greater than 60° between
straight and curved lines. The
wound is incised following the
sketched lines. The tip of the
wound incised is elevated and is
sutured to the respective dorsal
and volar fingertips and to each
other along the lateral margins
(see Figure 2). This method effectively advances the edges of
the donor defects to one another. The hand is immobilized with
the thumb in palmar abduction
and the MP joint of the affected
finger flexed to minimize flexion
at the PIP joint. Plaster is added
to the dressing along the dorsal
aspect of the finger to act as a
splint and maintain that position.
A thumb Spica is then placed
over the dressing to complete
immobilization. The digit is left
attached to the donor site for
approximately three weeks, and
the flap is then divided. This
can be accomplished by injecting local anesthesia. Skin flap
is approximated in the finger
with 3-4 sutures and the donor site is closed (see
Figure 3). An active range of motion program is initiated for both the affected finger and thumb. Cautious
wound hygiene is maintained along with dressing
changes to decrease risk of wound infection and graft
failure. Patients and parents are instructed on proper maintenance of cast and dressings; especially on
bathing techniques utilizing plastic bags to cover the
cast and prevent ideal conditions for infection. After
15 days after graft division, sutures are removed. Patients were followed up for at least 6 months (Figure
4) to assess function and any possible complication.
Sensory Assessment
Objective sensory assessment was made by the surgeon at follow-up clinics performing pinprick and light
touch to verify adequate sensory nerve regeneration.
Average follow up time was 14 months post
flap division. Semmes Weinstein Monofilament test
and two-point discrimination were not performed. Patients were subsequently given a questionnaire that
surveyed their opinions in terms of sensibility, color,
pain, appearance, and general motion. Patients were
asked to rate each category as poor, fair, good or
excellent, depending on how they perceived each
outcome. Data Analysis
Data gathered was analyzed utilizing descriptive statistics and a Wilcoxon Signed Rank Test to compare
between affected digit ROM and corresponding finger on contralateral hand.
Continued in page 14...
RESULTS
We identified 150 records with distal fingertip injuries. Of these, 37 were excluded by age and 86
did not undergo thenar flap graft. Of all records reviewed, we found 27 fingertip injuries that had thenar
flap technique with “shark mouth” incision performed.
Of these, 2 patients denied participation, and 9 were
unable to be contacted, leaving a final population
sample of sixteen patients who fit the inclusion criteria for this study. There were 14 males (87.5%) and
2 were females (12.5%), ranging in age from 0-17
years old with a median age of 9.8 years. Three fingers were affected, with 9 cases involving the index
finger (56%), 3 involving the long finger (19%), and
4 involving the ring finger (25%). The mechanisms
were mostly restricted to some kind of door crushing
the fingertip, being interior door the most common
observed mechanism of injury (56%), followed by exterior door (18%) (see Table 1).
Figure 2: Suturing of the thenar flap to distal fingertip defect of third finger.
Time from injury to reconstruction ranged from 1248 hours. Time to division ranged from 12 to 30 days
with a mean of 19.6 days. No infections were recorded and length of finger was successfully preserved
in all cases. Both fine and gross sensation, as well
as temperature and pain were preserved in affected
finger and donor site in all cases, as recorded in follow-up clinics after subsequent division of flaps. Revision was not necessary in any of the sixteen cases
reviewed and there were no reported complications. DISCUSSION
composition that allows restoration
of fine sensation as well as maintain
original fingertip function. Aesthetic appearance is secondary to the
previous goals but is nonetheless
important to maintain patient satisfaction and self-esteem. The results
of this study show that the thenar
flap done with the “Shark Mouth” incision is effective for treating these
types of injuries. This is reflected
in the questionnaire results were
100% of patients reported good or
excellent results in sensibility of affected finger and donor site, pain,
and general motion. Overall, patients reported satisfactory results
in terms of fingertip appearance after reconstruction. 88% of patients
believed that the flap color match
with original fingertip color was Figure 3: Post flap division and closing of flap donor site.
good or excellent. Contour, however, was the category that received
the worst results, reflected by 6 patients (43%) who reported fair results in this aspect; nonetheless, the
majority of patients (57%) believed
that the shape of the reconstructed
fingertip was good or excellent. The
fact that no patients reported poor
results in any category speaks favorably about this technique.
Fingertip injuries can produce composite loss of fingertip pulp and tactile skin. The skin on the palmar
aspect of the finger is specialized in that it has many
more nerve endings that in other parts of the body and
allow fine sensation. Extensive loss of palmar fingertip
skin must be replaced with a flap of similar
Barbato (10) reported in his thenar
flap case series a 25% rate of PIP
flexion contracture requiring extension splinting after flap division. The
results of this study show no clinically significant contractures of the Figure 4: Healed fingertip and donor site.
Range of motion (ROM) was measure in degrees in
PIP, DIP, and MP joints in both affected finger and
corresponding finger on the contralateral hand. MP
joint mean ROM was 100.3° in flexion with a standard deviation of 2.2°, PIP recorded at a mean of
89° with a standard deviation of 4.6°, and DIP had a
mean of 39.7° with a standard deviation of 7.9° (see
Table 2). ROM of corresponding finger on non-affected extremity was a mean of 102 ± 5.8° at MP joint,
100 ± 5.5° on PIP joint, and 55 ± 5° for DIP joint. A
Wilcoxon Signed Rank Test revealed no significant
differences in ROM between PIP (p=0.08) and MP
(0.06) joints when compared to mean ROM of the
corresponding digits PIP and MP joints of contralateral hand (Table 3). DIP ROM, however, showed to
be significantly less (p = <0.001) than corresponding joint on contralateral hand. On follow-up, there
were no functionally significant flexion contractures
or thumb adduction contractures. All 16 patients answered the questionnaire. All 16
patients reported resulting sensibility, pain and general motion in affected finger and donor site as good
or excellent. Two patients (12%) reported fair results
in color of skin on donor site and graft, and 14 (88%)
reported good or excellent results in this category.
Six patients (43%) reported fair results in fingertip
contour, while 10 patients (57%) reported contour to
be good or excellent. No patients reported poor results in any category (see Table 4).
digits. Both MP and PIP joints of affected finger
showed no statistically significant loss of ROM when
compared to corresponding finger on the unaffected
hand, showing only a 2° and 4° loss of ROM respectively. There was an average loss of ROM in affected
DIP joints of 15° that reached statistical significance;
however, the cause of this loss of motion cannot be
attributed to this technique. The specific cause for
these differences in DIP motion is unknown and
could have been easily caused by initial distal phalanx injury. Rinker (8) had similar results, finding no
significant difference in MP and PIP joints compared
to contralateral hand and statistically significant reduction in ROM in DIP joints of affected finger.
Weaknesses of this study include a low number of
participants and the unavailability of two-point discrimination and Semmes-Weinstein monofilament
testing sensory assessments. If a higher number of
patients were found, differences in ROM of affected
digits’ PIP and MP joints could have reached statistical significance. Future studies will aim to be prospective in nature, find a larger population and include more specific neurological/sensory testing on
patients with the “Shark Mouth” incision thenar flap
and compare them to current data on other commonly used composite graft techniques for distal fingertip
injuries.
The “Shark Mouth” incision thenar flap is an effective strategy for fingertip reconstruction. The results
demonstrate that this technique has excellent functional and aesthetic results and is not associated with
flexion contractures, excessive sensibility or pain in
the pediatric population. The technique is an excellent procedure with a small learning curve and will
most likely provide the patient with a fully functional
recovery.
THE RELATIONSHIP
BETWEEN TYPE 2
DIABETES MELLITUS
AND BODY MASS
INDEX: Data from a
Primary Care Facility
in Puerto Rico
RESUMEN
ABSTRACT
La técnica del colgajo tenar es un método de reconstrucción de la punta del dedo de la mano que
ha sido cercenada por la posible ocurrencia de
contracturas en flexión así como cicatrices no estéticas en el sitio donante. Método: En este análisis retrospectivo se adquirió información de 16
expedientes médicos de pacientes a los cuales
se le realizó una reconstrucción de colgajo tenar
tipo “Shark Bite” por dos cirujanos de nuestra
institución. Los pacientes sostuvieron una amputación traumática a nivel proximal de la parte
media del lecho ungal con pérdida de la pulpa y
exposición ósea entre los años 2005 y 2011. Resultados: El rango de edad fue de 0-17 años. El
tiempo de división del colgajo fluctuó entre 16 y
30 días. El arco de movimiento del dedo afectado
fue medido en las articulaciones DIF, PIF Y MCF
y comparado con el dedo correspondiente en la
mano contralateral. A base de un cuestionario
se midió la satisfacción del paciente en cuanto a
sensibilidad, apariencia y función. La sensación
de la punta del dedo se preservó tanto en el dedo
afectado así como en el sitio donante. La queja
principal de los pacientes incluía la forma residual
del dedo donde los pacientes la catalogaban de
adecuada (43%) y buena (56%). En el análisis
de la data no se encontró diferencia estadística
con arco de movimiento entre el dedo afectado y
el contralateral en las articulaciones PIF y MCF
(p=0.08, 0.06). Conclusión: El colgajo tenar tipo
“Shark Mouth” es una estrategia efectiva para la
reconstrucción de la punta del dedo. Los resultados demuestran que esta técnica provee una
función y una apariencia excelente y no está asociada a contracturas de flexión, sensibilidad excesiva o dolor en la población pediátrica
Obesity is a major risk factor in the development of Diabetes Mellitus (DM). Body
Mass Index (BMI), an estimation based on
the persons weight and height, helps identify patients at risk to develop DM. We report the relationship between DM and BMI
using data from a primary care facility in
Puerto Rico. Method: Patients were chosen at random with the only requirement
to be included in this study was age over
50. A population of 200 patients was obtained and each participant was categorized by gender, weight, height, BMI and
their status as a known diabetic or not.
Results: In respect to the diabetic populaRolance G. Chavier-Roper MDa*
tion identified, which totaled 67 patients, 1
Sharlene Alick-Ortiz MSb
out of 67 (1%) were underweight, 14 out of
Geraldine Davila-Plaza MSb
67 (20%) were normal, 28 out of 67 (42%)
Aixa G. Morales-Quiñones MSb
were overweight, and 24 out of 67 (36%)
obese. Conclusion: 78% of the diabetic
population fell in the categories of either
overweight or obese. BMI increases the aFrom ‘Centro de Medicina de Familia y Geriatría de Rio
Inc.’, San Juan, Puerto Rico.
incidence of Diabetes Mellitus in patients Piedras,
b
Antigua School of Medicine, University of Health Sciences,
older than 50 years of age. REFERENCES
1. Fassler, Paul R., M.D, Fingertip Injuries: Evaluation and Treatment, J Am Acad Orthop Surg 1996;4:84-92.
2. Louis DS, Palmer AK, Burney RE: Open treatment of digital tip
injuries. JAMA 1980;244:697-698.
3. Chow SP, Ho E: Open treatment of fingertip injuries in adults.
J Hand Surg [Am] 1982;7:470-476.
4. Schenck RR, Cheema TA: Hypothenar skin grafts for fingertip
reconstruction. J Hand Surg [AM] 1984,9:750-753.
5. Beasley RW. Reconstruction of amputated fingertips. Plast
Reconstr Surg 1969;44:349 –352.
6. Beasley RW. Local flaps for surgery of the hand. Orthop Clin
North Am 1970;1:219 –225.
7. Melone CP, Beasley RW, Carstens JH. The thenar flap—an
analysis of its use in 150 cases. J Hand Surg 1982;7:291– 297.
8. Rinker, B. Fingertip reconstruction with the lateral based thenar flap: Indications and long term functional results. HAND
2006;1:2-8
9. Dellon AL. The proximal inset thenar flap for fingertip reconstruction. Plast Reconstr Surg 1983;72:698 –704.
10. Barbato BD, Guelmi K, Romano SJ, et al. Thenar flap rehabilitated: a review of 20 cases. Ann Plast Surg 1996;37:135
–139.
Antigua.
Index words: relationship, type 2,diabe- *Corresponding author: Rolance G. Chavier-Roper MD 1107 William Jones Street, San Juan, Puerto Rico, 00925.
tes, mellitus, body, mass, index
Email: [email protected]
INTRODUCTION
D
Diabetes Mellitus is a chronic condition in which glucose levels are high due to decreased production
of insulin or resistance to insulin in the muscle, fat
and liver. The body does not yield sufficient insulin to
meet its needs. Normally, glucose in the bloodstream
would stimulate the production of insulin in order to
convert glucose to energy or to be stored as glycogen. In Type I Diabetes Mellitus, the pancreatic β-cell
is destroyed by the immune system, presumably in
response to possibly a viral infection, recurring pancreatitis, or any disorder that damages the pancreas
prior to 30 years of age, although its cause is essentially unknown. These patients produce little to no
insulin and are therefore known as insulin-dependent
requiring daily injections of the hormone.
In Type 2 Diabetes Mellitus, although the pancreatic β-cell produces insulin, there is insulin resistance.
This means that muscle, fat and liver cells do not
adequately respond to insulin and therefore are impaired to absorb glucose from the circulation. The
immediate consequence of this is the need of higher
levels of insulin to help glucose enter cells. These
conditions cause stress in the β-cell of the pancreas,
which may eventually fail to keep up with the bodies
increased need for insulin (1). This type of Diabetes
Mellitus is also known as adult-onset Diabetes Mellitus. The Diagnosis of Diabetes Mellitus is made by
yielding a HgA1C≥ 6.5%, Fasting blood sugar test
≥126mg/dL on two separate occasions, Oral glucose
tolerance test (75g) ≥ 200mg/dL after 2 hours and a
random blood glucose test ≥200 mg/dL (2). According to the Merck Manual (2013), “About 27%
of people older than 65 have type 2 diabetes”. There
are modifiable risk factors such as being overweight
or obese, physical inactivity, smoking, high blood glucose in pre-diabetes, and abnormal lipid metabolism
(3). A good indicator to take into consideration is the
individual Body Mass Index (BMI), which is estimation based on the persons weight and height. Individuals that yield a BMI of 25 to 29.9 are considered
overweight and individuals with a BMI of 30 or higher are considered obese (4). These two categories
of BMI have the highest risk of developing Diabetes
Mellitus. According to Merck Manual (2013), “Obesity is the chief risk factor for developing type 2 diabetes, and 80 to 90% of people with this disorder are
overweight or obese. Because obesity causes insulin
resistance, obese people need very large amounts
of insulin to maintain normal blood glucose level” (3).
An example of obesity as a major risk factor in the
development of diabetes can be seen in a research
conducted by the National Institute of Diabetes and
Digestive and Kidney Diseases (NIDDK, 2002) on the
Pima Indians of Arizona. “One-half of adult Pima Indians have diabetes and 95% of those with diabetes are
overweight” (5). “The highest rates of T2D are found
among Native Americans, particularly the Pima Indians who reside in Arizona in the US, and in natives of
the South Pacific islands, such as Nauru” (6).
According to the national survey “Behavioral Risk
Factor Surveillance System”, the prevalence in Puerto
Rico has increased over the years (7). In 1996 it was
10.8% while in 2010 it was 12.8%. During all those
years (1996-2010), Puerto Rico has been in the top
position among the states and territories of the United
States, and has only been surpassed by the state of
West Virginia in 2004 and 2006 and Alabama in 2010.
In 2003 along with Mississippi, Puerto Rico shared the
first position. In 2007, among people aged 20 years or
more, the incidence was 12.8 per 1,000 inhabitants.
Diabetes is the third leading cause of death in Puerto
Rico (7).
Figure 1: Total Population.
Figure 3: Total BMI.
Figure 2: Diabetics versus Non Diabetics.
Figure 4: Underweight Population.
Figure 6: Overweight Population.
Figure 5: Ideal or Normal Body Weight Population.
Figure 7: Obese Population.
The Institute of Statistics of Puerto Rico has had prior studies concerning the prevalence of diabetes in
people 18 years of age and older in Puerto Rico by region and tendencies of mortality. However, there is not
enough available statistical data. The purpose of this
study was to establish how the Body Mass Index increases the incidence of Diabetes Mellitus in patients
older than 50 years of age in a random population of
200 patients managed in a Primary Care Physician
facility in the San Juan metropolitan area.
MATERIALS & METHODS
The clinical study was conducted in the office of a Primary Care Physician at the ‘Centro de Medicina de
Familia y Geriatría de Rio Piedras, Inc.’ located in San
Juan, Puerto Rico. Patients were chosen at random.
The only requirement to be included in this study was
to be over the age of 50. Each patient was measured
their height and weight to calculate their Body Mass
Index (BMI). A population of 200 patients was obtained and each participant was categorized by gender, weight, height, BMI and their status as a known
diabetic or not diabetic.
d Body Mass Index (see Figure 3).
After establishing our target population of 200 patients, the following results were obtained. Out of the
200 patients, there were 128 females (64%) and 72
males (36%)(see Figure 1). Of the total number of patients, 133 (67%) were not diabetic and 67 patients
(33%) were known diabetics (see Figure 2).
In reference to the underweight population, 4 out of
5 were not diabetic (80%) and 1 out of 5 (20%) was
a known Diabetic (see Figure 4). In reference to the
normal BMI population, 38 out of 52 (73%) were not
diabetic and 14 out of 52 (27%) were known diabetics
(see Figure 5). In reference to the overweight population, 52 out of 80 (65%) were not diabetic and 28 out
of 80 (35%) were known diabetics (see Figure 6). In
reference to the obese population, 39 out of 63 (62%)
were not diabetic and 24 out of 63 (38%) were known
diabetics (see Figure 7).
Of the total population, 5 out of 200 (3%) were underweight, 52 our of 200 (26%) were of ideal body weight,
80 out of 200 (40%) were overweight, and 63 out of
200 (32%) were obese, according to the calculate
In reference to the known diabetic population only,
which totaled 67 patients, 1 out of 67 (1%) were underweight, 14 out of 67 (20%) were normal, 28 out of 67
(42%) were overweight and 24 out of 67 (36%) were
RESULTS
THE RELATIONSHIP BETWEEN TYPE 2 DIABETES MELLITUS AND
BODY MASS INDEX: Data from a Primary Care Facility in Puerto Rico
obese (see Figure 8). In reference to
the non-diabetic population, 4 out of
133 (3%) were underweight, 38 out of
13 (29%) were ideal body weight, 52
out of 133 (39%) were overweight and
39 out of 133 (29%) were obese (see
Figure 9).
RESUMEN
DISCUSION
The study demonstrates that there is an
alarming prevalence of Diabetes in the
age group that participated in the study.
One out of every three patients, over
the age of 50 had Diabetes Mellitus.
Two thirds (67%) of the patients who
participated in this study had no knowledge they had diabetes. It is important
to emphasize that this is a primary care
setting and not an endocrinology prac- Figure 8: Known Diabetic Population versus BMI.
tice. We also demonstrates that out of the
total population, only 26% had normal
or ideal body weight and 72% were
either overweight or obese in both the
male and female populations.
La obesidad es un factor de riesgo importante en el desarrollo de la diabetes. El Índice de Masa
Corporal (IMC ) , que es una estimación basada en el peso y la altura de las personas, ayuda a
identificar pacientes a riesgo de desarrollar diabetes. Reportamos la relación entre diabetes y IMC
utilizando data clínica de pacientes manejados en una clínica primaria de salud. Métodos: Los
pacientes fueron escogidos al azar con el único requisito para ser incluidos en este estudio que
fuesen mayor de 50 años de edad. Se obtuvo una población de 200 pacientes y cada participante
se clasifico por sexo , peso, estatura, índice de masa corporal y si padecían o no de la condición
de diabetes. Resultados: En referencia a sólo a la población diabética conocida, que ascendieron a
67 pacientes, 1 de cada 67 (1%) tenían bajo peso, con 14 de 67 (20%) fueron normales, 28 de 67
(42%) tenían sobrepeso y 24 de 67 (36%) eran obesos. Conclusión: 78% de los diabéticos cayó en
las categorías de sobrepeso u obesidad. El índice de masa corporal elevada aumenta la incidencia
de diabetes mellitus en pacientes mayores de 50 años de edad.
In addition and most important with
respect to the diabetic population, we
can say that 78% of Diabetics fell in the
categories of either overweight (42%)
or obese (36%). Also, the patients who
fall into the category of the non-diabetic
population show a pretty even distribution between obesity, overweight and
ideal body weight.
As previously discussed, we have
demonstrated elevated BMI increases
the incidence of Diabetes Mellitus in patients older than 50 years of age managed at a primary care center in Puerto Figure 9: Non-Diabetic Population versus BMI.
Rico.
REFERENCES
(1) National Diabetes Information Clearinghouse (2014). Insulin
resistance and Pre-diabetes Retrieved October 15, 2014, from
http://diabetes.niddk.nih.gov/dm/pubs/insulinresistance/
(2) American Diabetes Association (2014). American Diabetes
Association (ADA) 2014 Guidelines Summary Recommendations from NDEI. Retrieved October 15, 2014, from http://www.
ndei.org/uploadedFiles/Common/NDEI/Treatment_Guidelines/
NDEI%20org%20summary%20recommendations%20ADA%20
2014%20guidelines---012314%20FINAL.pdf
(3) Kishore, P. (2013, July 1). Merck Manual. Diabetes Mellitus.
Retrieved October 10, 2014, from http://www.merckmanuals.
com/home/hormonal_and_metabolic_disorders/diabetes_mellitus_dm/diabetes_mellitus.html
(4) Center for Disease Control and Prevention (2014). Body
Mass Index. Retrieved October 15, 2014, from http://www.cdc.
gov/healthyweight/assessing/bmi/Index.html
(5) National Diabetes Information Clearinghouse (2002). Obesity Associated with High Rates of Diabetes in the Pima Indians.
Retrieved October 13, 2014, from http://diabetes.niddk.nih.gov/
dm/pubs/pima/obesity/obesity.htm
(6) Wild, S., Roglic, G., Green, A., et al. Global prevalence of
diabetes. Diabetes Care, 2004. 27: 1047-1053.
(7) Centro de Diabetes en Puerto Rico (2011). Diabetes en Puerto Rico. Retrieved October 13, 2014, from http://www2.pr.gov/
agencias/diabetes/Documents/Diabetes/DIABETES%20EN%20
PUERTO%20RICO.pdf
Continued in page 21......
UNCOMMON
PRESENTATION OF
FOLLICULAR
THYROID
CARCINOMA: When
Chronic Back Pain
Should Raise a
Flag
Milliette Alvarado MDa
Margarita Ramirez-Vick MDa*
Liurka Lopez MDbc
Maria J Marcos-Martinez MDbc
Fanor M. Saavedra MDd
Juan C. Negron-Rivera MDe
Marielba Agosto MDa
Meliza Martinez MDa
Rafael Gonzalez MDa
Myriam Allende-Vigo MDa
a
Endocrinology, Diabetes and Metabolism Section, Internal Medicine Department, University of Puerto Rico, School of Medicine.
b
Department of Pathology and Laboratory Medicine, University
of Puerto Rico, School of Medicine.
c
Puerto Rico Medical Services Administration.
d
Neurosurgery Section, University of Puerto Rico, School of
Medicine.
e
Nuclear Medicine Section, University of Puerto Rico, School of
Medicine.
*Corresponding author: Margarita Ramirez-Vick MD - University
of Puerto Rico Medical Sciences Campus, PO Box 365067, San
Juan, PR 00936-5067. E-mail: [email protected]
Poster Presentation at the American Association of Clinical
Endocrinologists 23rd Annual Scientific and Clinical Congress,
2014.
I
INTRODUCTION
In recent years, the incidence of differentiated thyroid cancer (DTC) has increased dramatically, more
than any other malignancy, as the cases of follicular thyroid carcinoma (FTC), accounting for almost
10% of cases in iodine sufficient areas (1,2). Cases
of thyroid cancer presenting with aggressive latestage disease with distant bone metastases have
also risen. Although DTC had in general been associated with good prognosis and survival, in cases
where distant disease is present, the overall survival
decreases significantly (3). Bone metastases have
the potential to cause severe morbidity, including
pain, neurologic deficit, spinal cord compression and
paraplegia (4). We present a patient that developed
spinal metastatic FTC causing cord compression
ABSTRACT
Follicular thyroid carcinoma is the second most
common type of thyroid cancer, and its incidence
has increased dramatically in recent years. Although it typically presents as a thyroid nodule,
it can spread to distant sites via hematogenous
dissemination. Spinal cord compression complicating thyroid carcinoma is rare with only few
cases reported in the literature. This case illustrates a minimally invasive follicular carcinoma
that showed such an aggressive behavior, and
thus the importance of considering metastatic
thyroid carcinoma in the differential diagnosis of
chronic back pain, which may possibly progress
to spinal cord compression carrying severe morbidity.
Figure 1: T1 contrast enhanced sagittal (left) and axial (right) images of the thoracic spine showing a mass centered at the left
aspect of T3 vertebral body extending to posterior elements, also
involving left posteroinferior aspect of T2 vertebral body and left
adjacent paraspinal muscles, causing compression of the thecal
sac and obliteration of CSF column. T2-T3 and T3-T4 neuroforamina are obliterated by the mass.
Index words: follicular, thyroid, carcinoma,
chronic, back, pain
after an initial minimally invasive follicular carcinoma. Case History
A 56 year-old-female patient with arterial hypertension, chronic low back pain, multinodular goiter and
hypothyroidism on thyroid hormone replacement, underwent left hemithyroidectomy on March 2006 due to
the presence of a left sided solid thyroid nodule measuring 1.6 x 1.3 x 1.5 cm. Prior fine needle aspiration
(FNA) of this lesion was consistent with follicular lesion of thyroid. Pathologic diagnosis of left hemithyroidectomy was reported as follicular adenoma. Patient continued her routine medical care at another
institution. Approximately six and a half years later,
the patient developed a worsening left sided back
pain initially believed to be herniated disk disease,
for which she sought multiple treatments without any
pain relief. A spinal mass was found on an MRI and
she was referred for Neurosurgery evaluation. The
pain became unbearable and debilitating, prompting
an emergent visit to emergency department, where
imaging study revealed an aggressive mass centered at T3 vertebral body with severe spinal canal
stenosis causing compression of spinal cord (see
Figure 1); no evidence of lymph node enlargement or
further structural involvement. Patient underwent T3
vertebrectomy with internal fixation (see Figure 2) by
Neurosurgery Service with final pathologic diagnosis
showing thyroid tissue compatible with metastatic
follicular thyroid carcinoma (see Figure 3). Subsequent neck ultrasound was performed showing a solid well-defined isoechoic thyroid nodule at the lower
right lobe measuring 1.4 cm in largest diameter; FNA
was done with a cytological diagnosis of Follicular
Neoplasm or Suspicious for a Follicular Neoplasm
(see Figure 4a). Patient was clinically and biochemically euthyroid, and underwent completion thyroidectomy on December 2013 with non-malignant results
(see Figure 4b).
*
Figure 2: Thoracic vertebra (T3) Corpectomy, T3 Titanium interbody cage and T2 to T4 posterior instrumentation.
Figure 3: Thoracic vertebra (T3) biopsy showing infiltrative follicular thyroid carcinoma between bony spicules (arrow), mostly a
microfollicular pattern (H&E, X40 magnification
*
Figure 4: Fine-needle aspiration of a right thyroid nodule was suspicious for a follicular neoplasm. Cells showed sheets of crowded
follicular cells, with slightly enlarged nuclei with granular chromatin (a) (Papanicolaou stain, x400 magnification). A
This case report illustrates a rare case of a minimally
invasive follicular carcinoma with well-differentiated
epithelium that showed an aggressive behavior. It
is important to consider metastatic thyroid carcinoma in the differential diagnosis of chronic back pain,
that may rarely, yet possibly progress to spinal cord
compression which carries severe morbidity. Clearly,
early detection of these signs and symptoms should
warn the clinician, calling for prompt diagnosis and
treatment.
A second review of the 2006 hemithyroidectomy was performed by Pathology Service at our Institution, with new
interpretation consistent with a well-differentiated follicular thyroid carcinoma
as it showed focal capsular invasion with
indeterminate lymphovascular invasion
(see Figure 5). After preparation with steroid therapy, patient underwent RAI therapy with 170 mCi of I-131 by the Nuclear
Medicine Service.
REFERENCES
DISCUSSION
Follicular thyroid carcinoma is defined
as a thyroid follicular epithelial cell neoplasm, with evidence of capsular and/or
vascular invasion (5). It has been traditionally classified as low- and high-risk
forms based on their overall survival
data (6). The histopathology varies from
a well-differentiated epithelium, in which
the microfollicular architecture is maintained, to poorly differentiated lesions,
which are invariably associated with a
worse prognosis (7). Moreover, two types
have been recognized in terms of their
degree of invasiveness, the minimally invasive follicular cancer (MIFC) and the
widely invasive follicular cancer (WIFC).
The MIFC pattern is defined as an encapsulated follicular neoplasm showing
definite capsular and/or vascular invasion, while the latter shows widespread
invasion into the thyroid parenchyma or
into the blood vessels. It has been described that the minimally invasive thyroid carcinoma is characterized by a
lower degree of aggressiveness with a
relative indolent course (6).
*
O
Follicular carcinoma is the second most
common type of thyroid cancer, typically
presenting as a thyroid nodule, but when
it spreads it usually does via hematogenous route, with distant metastases Figure 5: A review of previous left hemithyroidectomy resulted in the diagnosis of follicular carcinoma (a). Note the presence of tumor (asterisk) invading
occurring in about 10-15% of cases (2). outside
the tumor capsule (arrow) (b). (a: H&E, 40x magnification; b: H&E, 40x
Bone metastases are diagnosed clinical- magnification
ly in 2-13% of patients with differentiated
thyroid cancer; the great majority occurring in the ax- motor and sensory dysfunction also presenting in
ial skeleton where blood flow is high, forming mostly advanced disease (9). Magnetic resonance imaging
osteolytic lesions, as bone offers an advantageous (MRI) is now the gold standard in evaluating cases of
microenvironment for tumor growth (8). Spinal me- spinal cord compression (10). In such patients both
tastases typically affect the thoracic (60-80%), lum- surgical resection of symptomatic metastasis and rabar (15-30%) and cervical spine (<10%), with the dioactive iodine (RAI) therapy have been associated
posterior aspect of the vertebral body the most com- with improved survival. Moreover, in those lesions in
mon site of initial involvement. The spinal canal to which acute edema from metastatic lesions may prospinal cord ratio is smallest in the thoracic spine, duce complications, therapy with external radiation
making this area more prone to cord compression, and glucocorticoid must be considered to minimize
as was documented in our patient. Patients with potential tumor expansion (11). Most importantly,
spinal metastases tend to present with pain that be- each case needs to be individualized and a dedicatcomes progressive and unremitting over time, with ed multidisciplinary approach must ensue. 24 | BOLETIN Médico Científico de la Asociación Médica de Puerto Rico
1. Venkat R, Guerrero MA. “Recent Advances in the Surgical
Treatment of Differentiated Thyroid Cancer: A Comprehensive
Review.” The Scientific World Journal. 2013, Vol. 2013. 7 pages. Article ID 425136. Accessed on May 5, 2014. <http://dx.doi.
org/10.1155/2013/425136>.
2. Lee SL, Ananthakrishnan S. ”Overview of follicular thyroid
cancer.” In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.
Jan 17, 2014.
3. Carhill AA, Vassilopulou-Sellin R. “Durable effect of Radioactive Iodine in a Patient with Metastatic Follicular Thyroid Carcinoma”. Case Reports in Endocrinology. 2012. Vol. 2012, 5 pages. Article 231912. Accessed on January 8, 2014. <http://dx.doi.
org/10.1155/2012/231912>.
4. Quan GM, Pointillart V, Palussière J et al. “Multidisciplinary
Treatment and Survival of Patients with Vertebral Metastases
from Thyroid Carcinoma.” Thyroid. 2012, 22:125-130.
5. Gimm O, Dralle H. “Differentiated thyroid carcinoma”. In:
Holzheimer RG, Mannick JA, editors. Surgical Treatment: Evidence-Based and Problem-Oriented. Munich: Zuckschwerdt;
2001. Accessed on January 7, 2014. Available from: <http://
www.ncbi.nlm.nih.gov/books/NBK6979/>.
6. Collini P, Sampietro G, Rosai J et al. « Minimally invasive (encapsulated) thyroid carcinoma of the thyroid gland is the low risk
counterpart of widely invasive follicular carcinoma but not of insular carcinoma ». Virchows Arch. 2003, 442 :71-76.
7. Vishveshwaraiah PM, Mukunda A, Laxminarayana KK et al.
“Metastatic follicular thyroid carcinoma to the body of the mandible mimicking an odontogenic tumor”. J Can Res Ther 2013,
9:320-323.
8. Muresan MM, Olivier P, Leclère J et al. “ Bone metastases
from differentiated thryoid carcinoma.” Endocr Relat Cancer. 2008
,15:37-49.
9. Ramadan S, Ugas M, Berwick R et al. « Spinal Metastasis in
thyroid cancer ». Head & Neck Oncology. 2012, 4:39.
10. Sundaresan N, Sachdev VP, Steinberger A et al. "Decompression and Stabilization of Spinal Metastasis: The Mount Sinai
School of Medicine Experience" Advanced Techniques in Central
Nervous System Metastases. The American Association of Neurological Surgeons.1998, Chapter 15: 219-31.
11. Cooper DS, Doherty GM, Haugen BR et al. “Revised American Thyroid Association Management Guidelines for Patients with
Thyroid Nodules and Differentiated Thyroid Cancer”. American
Thyroid Association (ATA) guidelines taskforce on thyroid nodules
and differentiated thyroid cancer. Thyroid. 2009, 19:1167–1214.
Acknowledgement
Enrique Alvarado Burgos MD from the Diagnostic Radiology Department, UPR School of Medicine, who
help in the interpretation of the diagnostic image.
RESUMEN
El carcinoma folicular del tiroides es la segunda malignidad mas común de cáncer del tiroides y su incidencia ha aumentado dramáticamente en los últimos años. Aunque típicamente
se presenta como un nódulo en el tiroides, en
adición puede producir diseminación hematógena. Compresión del cordón espinal complicando
carcinoma del tiroides es raro con unos pocos
casos reportados en la literatura. Este caso
ilustra como un carcinoma folicular del tiroides
mínimamente invasivo desarrolló tal comportamiento agresivo y la importancia de considerar
carcinoma del tiroides metastásico en el diagnostico diferencial de dolor de espalda crónico
que pudiera progresar a compresión aguda del
cordón espinal con morbilidad severa.
ABSTRACT
Anal poorly differentiated neuroendocrine carcinomas are very rare aggressive neoplasm with
a poor prognosis. This high-grade malignancy
carcinoma can have small or non-small cells
composition. This case report illustrates a large
cell anal neuroendocrine carcinoma with tubulovillous component.
Index words: anal, large, neuroendocrine,
carcinoma, tubulovillous, adenoma
A
ANAL LARGE CELL
NEUROENDOCRINE
CARCINOMA WITH
TUBULOVILLOUS
ADENOMA
COEXISTENCE:
A Case Report
Iván E. Aponte Aponte MDa
William Cáceres MDb*
INTRODUCTION
Anal poorly differentiated neuroendocrine carcinomas (NEC) are very rare aggressive neoplasm with
a poor prognosis. This high-grade malignancy carcinoma can have small or non-small cells composition (1). This case report illustrates a large cell carcinoma with a tubulovillous component.
Case History
A 50-year-old female presented with stomach, neck
and back pain partly controlled with Skelaxin and
Celebrex. The history of systemic illness was positive for hyperlipidemia. Her past surgical history
included tubal ligation and hysterectomy. Family
history includes diabetes mellitus-unspecified type,
coronary artery disease and hypertension. Positive
aspirin allergy history. No toxics habits with ethanol or smoking reported. Her initial weight was 149
pounds, ECOG performance status II/IV and the
rectal digital examination did not report as free of
bright red blood on the glove or stool; nor masses reported. The initial PAP test result was within
normal limits. Severe constipation, ano-rectal pain
and anemia worsened in a two months period. Four
months after the initial visit, she had lost sixteen
pounds. Available laboratory data performed prior
to diagnostic colonoscopy reviewed. The results
showed a positive antiparietal cell antibody, above
normal folic acid, low iron levels, no macrocytosis,
normal B-12 and normal ferritin levels. Colonoscopy with biopsy was performed. The surgical pathology reported a final diagnosis compatible
with an anal mass neuroendocrine poorly differentiated (3/3), with adjacent villous adenoma. Immunohistochemistry analysis to the anal biopsy was positive for Chromogranin A and Syneptophysin. The
initial staging included chest X- Rays, Chest, Abdomen and Pelvic CT Scan disclosing stage IV disease. Three hypodense rounded lesions within the
liver, the largest measuring 6.6 cm, recorded along
with multiple pulmonary nodules, scattered throughout both lung fields, more prominent in the bases.
Medical Oncology and Hematology East Clinic, Humacao, Puerto Rico.
b
Hematology-Oncology Section, Department of Medicine, VA
Healthcare System, San Juan, Puerto Rico.. [email protected]
*Corrresponding author: William Cáceres MD - Hematology-Oncology Section, 10 Casia St., San Juan, Puerto Rico 00921.
E-mail: [email protected].
a
Chemotherapy was started nine months after the
initial visit. Her body weight had decreased to 121
pound and pain had continued. The agents infused
were Carboplatin and Camptosar. mediastinal, right
hilar lymphadenopathy and diffuse pulmonary nodules, too numerous to count, documented the continued progression of the disease. Also, marked interval progression of the hepatic metastatic disease with
increased size of previous lesions and the development of new ones were compatible with aggressive
behavior. A chemotherapy change with a combined
modality of radiotherapy to the ano-rectal lesions,
along with 5 F/U + LCV recommended as a palliative pain management intervention. The therapy did
control the pain, but an ulcerated area was a sign of
toxicity, having to stop such intervention. Fentanyl
transdermal patches controlled the pain and neutropenic fever managed. Other reported available laboratory results showed pancytopenia, elevated CEA
and elevated sedimentation rate. Weighing 110 pounds, not in chemotherapy infusions,
with hypoxemia, respiratory distress, deteriorated
performance status ECOG PS= IV/IV, and terminally
ill, was placed under the Hospice Service Care. The
patient died twelve months after diagnosis. DISCUSSION
Large cell poorly differentiated anal neuroendocrine
tumors are extremely rare (1). This non-small cell
type, high grade neuroendocrine carcinoma (NEC)
of the anal canal, is part of a subtype with aggressive distant malignant dissemination, a rapid distant
spread, large size of the primary tumor, the presence
AMPR App
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of metastasis and more aggressive behavior than
colorectal adenocarcinoma (2). Generally, these
NEC has a worse prognosis (2). To determine the
NEC, the immunohistochemical study by ChrA and
SNP were needed. CEA stain was not performed.
It is possible that an adenocarcinoma component
may have been present due to the presence of a
villous tubular adenoma in this poorly differentiated
NEC (3). A possible contribution of elevated folate
levels to the development, dissemination and tumor
growth, is uncertain. It is unknown the folate source
that contributed to higher than normal levels; if this
value relates to an external source replacement or
to a healthy dietary food intake. No anemia or macrocytosis was present, but there is positive immunological pernicious anemia pathology. Iron deficiency
was also reported, likely due to blood loss secondary to lower GI bleeding. Most NEC reported is compatible with a small cell type. Fewer large cells anal
NEC with a villous tubular adenoma component has
been reported. Benign tubulovillous adenoma of the
anal canal is known as an extremely rare diagnosis
(4) Surgery was not performed due to the initial advanced stage of the disease. Optimal chemotherapy
management has not yet been established (5).
REFERENCES
Links para la descarga gratuita de la aplicación
1. Bernick PE, Klimstra DS, Shia J, et al. Neuroendocrine carcinomas of the Colon and rectum. Dis Colon Rectum 2004;47:163169.
2. Miyamoto H, Nobuhiro K, Nishioka M, Ando T, Tashiro T, Hirokawa M, & Shimada M. (2006) Case Report: Poorly differentiated neuroendocrine cell carcinoma of the rectum: report of a
case and literature review. The Journal of Medical Investigation
Vol. 53 pp.317-320.
3. Colvin M, Delis A, Bracamonte E. Villar H & Leon LR. (2009).
Infiltrating arising adenocarcinoma arising in a villous adenoma
of the anal canal. World J Gastroenterol. Jul 28;15(28):3560-4.
4. Anand, B., Verstovsek, G., & Cole, G. (2006). Tubulovillous
adenoma of the anal canal: a case report. World J Gastroenterol.
Mar 21;12(11):1780-1.Medline, 12(11), 1780-1781.
5. Sorbye H1, Welin S, Langer SW, Vestermark LW, Holt N,
Osterlund P, Dueland S, Hofsli E, Guren MG, Ohrling K, Birkemeyer E, Thiis-Evensen E, Biagini M, Gronbaek H, Soveri LM,
Olsen IH, Federspiel B, Assmus J, Janson ET, Knigge U. (2006)
Predictive and prognostic factors for treatment and survival in
305 patients with advanced gastrointestinal neuroendocrine carcinoma (WHO G3): the NORDIC NEC study. Ann Oncol. 2013
Jan;24(1):152-60.
RESUMEN
Los carcinomas neuroendocrinos pobremente
diferenciados del ano son tumores bien agresivos asociados a una pobre prognosis. Este
tipo de malignidad de alto grado tiene una composición de células pequeñas y grandes. El
reporte de caso que sigue ilustra el comportamiento de un carcinoma de células grande con
un componente tubolovilloso.
PRIMARY IMMUNE REFRACTORY
THROMBOCYTOPENIA
IN ASSOCIATION WITH
SECONDARY
DISORDERS:
A Case Report
ABSTRACT
Immune thrombocytopenia is an autoimmune disorder characterized by
immunologic destruction of platelets.
It may occur in isolation or in association with other health disorders. We
describe a case were the pathology
involved an immune mediated mechanism initially thought to be isolated and
posteriorly linked to secondary causes.
Index words: primary, immune,
thrombocytopenia, secondary, disorders
Iván E. Aponte Aponte, MDa*
East Medical Hematology and Oncology Clinic, Humacao, Puerto Rico.
*Corresponding author: Iván E. Aponte Aponte MD – 260 Padua St., College
Park, San Juan, Puerto Rico 00921. E-mail: [email protected].
a
INTRODUCTION
Immune thrombocytopenia (ITP), defined as “immune thrombocytopenia purpura”, or “idiopathic
thrombocytopenia purpura”, is an autoimmune disorder characterized by immunologic destruction of otherwise normal platelets. It may occur in isolation (primary), or in association with other health disorders
(secondary). We describe a case were the pathology involved an immune mediated mechanism initially
thought to be isolated posteriorly linked to secondary
medical causes.
Case History
This is the case of a 66 year-old-female patient from
Puerto Rico who first came to Hospital de la Concepcion with chronic thrombocytopenia. Her history of
systemic illness was positive for hypertension. Allergy to penicillin was reported. She was found afebrile,
ambulating, with normal hemoglobin and white blood
cell counts, skin ecchymosis and abdominal wall
well-healed surgical scar. The platelets count was
21,000. The patient was diagnosed with a refractory
ITP. She had a prior history of low platelets for two
years. Intravenous Gamma Globulins (IVIG) was
infused in the past followed by intravenous steroids
and oral prednisone therapy dose of 60 mg daily for
three months. Transfusion of platelets and splenectomy, due to a persistent refractory thrombocytopenia,
were performed as therapeutic interventions. The
surgical spleen pathology report was consistent with
ITP, supporting the bone marrow biopsy result. Refractory ITP to splenectomy prompted the infusion of
Rituximab 700 mg IV infusion weekly for four doses. The patient persisted with lower than 30,000 platelets, thus, failing to the Rituximab therapy. She was
taking prednisone 40 mg to 60 mg per day, Amlodipine and Enalapril, afebrile and complaining about
dyspepsia. Initially, the challenge with IVIG in 2012,
was unsuccessful. A screening antibody test for H.
pylori, anti-platelets antibody panel, HCV infection,
HIV, Anti-phospholipid antibodies and lupus panel were among the secondary related pathologies
thought to be involved. Because of the dyspepsia
and no prior peptic therapy, empirical treatment for
H. pylori with Omeprazole, Metronidazole and Clarithromycin were started for fourteen days, considering a possible high background bacterial incidence
in our population, a favorable toxicity profile, the prescription availability and past penicillin allergy history. Simultaneously, Romoplastin, a thrombopoetin
receptor agonist was infused weekly for four consecutive doses. Weekly platelets counts were consistent
with higher and elevated persistent values. An initial
positive screening antibody test for H. pylori (1.8U/
ML) was followed, after the initial month, with a subsequent negative stool for fecal antigen. An interesting clinical finding was the conversion of an initially
positive Indirect HLA Class I antiplatelet antibody to
a negative result in an eight-week period. The recorded Platelet Glycoprotein antibody test was negative. The rest of the ordered laboratory studies as
well as the liver and spleen scan result failed to show
a related pathology. Counts above 50,000 platelets
were consistently achieved after Romoplastin was
discontinued for a total of nine weeks. The highest
platelet count was 300,000 at the fourth week interval, and the lowest count was 21,000 one day prior to
its initiation. An additional superimposed secondary
cause thought to be a medication-induced component. Amlodipine and Enalapril were recommended
to be discontinued, and a follow up given at one week
interval. Losartan was started as the alternative antihypertensive medication. The recorded platelet count
was 69,000, a considerable and significant increase
within one week of the antihypertensive medications
change. Careful ambulatory care planned follow-up
as secondary ITP has been reported in association
with the newer medication as well.
DISCUSSION
Severe refractory thrombocytopenia unresponsive
to IVIG, steroids, splenectomy and Rituximab, as in
this patient, may have a related secondary cause.
H. Pylori have been documented as a secondary
cause and its eradication has proven an effective
tool to improve the total platelet count (1). Past reviews have examined the diagnosis and the efficacy
of H. pylori eradication (2). Screening for H. Pylori
was performed in this patient with ITP and eradication therapy was used because of the positive testing
result. In patients found to have H. pylori infection,
based on urea breath test, endoscopic study or stool
antigen, therapeutic eradication should be administered (1). It is difficult to determine a minimum platelet count, its relative bleeding risk and the related
ages for the population ethnic group. This places
more importance to perform a complete work up to
exclude secondary ITP precipitants. These can include CMV, Varicella-Zoster, hepatitis C virus, vaccination against pneumococcus, Hemophilus influenza B, hepatitis B virus, post transplantation patients,
T-lymphocytic leukemia, Evans Syndrome, Chronic
Lymphocytic Leukemia and medication induced ITP
(3). It is necessary to treat the underlying pathology
and to consider thrombopoetin receptor agonist as
a therapeutic agent to increase the platelet count.
Thrombopoetin mainly considered in patients at risk
of bleeding who relapse after splenectomy or have
failed at least one other therapy. The need to perform a complete work-up to exclude a secondary related cause can help to identify a possible diagnosis
that could improve the outcome of this disease. Finally, this case raises the possibility of a relationship
between H. pylori and the illness severity of a refractory ITP after splenectomy and therapy failures in
Puerto Rico. Medications can also be playing a very
significant cause of this disease and frequently could
be underestimated as a participant pathology linked
to a primary ITP with secondary associated causes. Lastly, the case tends to support the benefits of Romoplastin in similar patients.
ACKNOWLEGEMENTS
Special thanks to Stephanie Gómez for her collaboration and support.
RESUMEN
La trombocitopenia inmune es un desorden autoinmune caracterizado por destrucción inmunológica de las plaquetas. Puede ocurrir de forma aislada o asociada con otros desordenes de
salud. Describimos el caso donde la patología
envuelta en el mecanismo inmune se estimaba
inicialmente aislada y posteriormente se encontró ligada a causas medicas secundarias.
AMPR App
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a la información que produce la Asociación Médica, además de proveerle
otras herramientas útiles.
REFERENCES
1. Neunert C, Lim W, Crowther M, Cohen A, Solberg, and Jr.
Crowther MA: American society of Clinical Oncology. The American Society of Hematology 2011 evidenced based practice
guidelines for immune thrombocytopenia. Blood 2011; 117 (16):
4190-4207
2. Stasi R, Sarpatuari A, Segal JB, Osborn J, Evangelista ML,
Cooper N, Provan D, Newland A., Amadori S, and Bussel JB: Effects of eradication Helicobacter pylori infection in patients with
immune thrombocytopenic purpura: a systematic review. Blood
2009; 113(16): 1231-1240
3. Douglas BC, James BB, Howard AL, and Lunning Prak ET:
The ITP syndrome: pathogenic and clinical diversity. Blood 2009;
83(26): 6511-6521
ABSTRACT
Wegener’s Granulomatosis or Granulomatosis with Polyangiitis (WG/GPA) is one of
the anti-neutrophil cytoplasmic antibody
associated vasculitis. Ocular manifestations the first presenting signs in patients
with WG/GPA. We report on two patients
diagnosed with WG/GPA and discuss
their pertinent clinical findings. Our case
report reviews the most common clinical
manifestations and ophthalmic associations to compare them with the physical
findings of two Hispanic patients. Further,
to our knowledge this is the first report of
ocular findings in patients with WG/GPA
in two Puerto Rican patients.
Index words: granulomatosis, polyangiitis, Puerto Rico
G
GRANULOMATOSIS
WITH POLYANGIITIS
IN PUERTO RICO:
Case Reports
Luis Aranguren MSa
Natalio J. Izquierdo MDb*
Armando Oliver MDc
University of Puerto Rico Medical Sciences Campus School of Medicine.
b
University of Puerto Rico Medical Sciences Campus, Department of
Surgery.
c
University of Puerto Rico Medical Sciences Campus, Department of
Ophthalmology.
*Corresponding author: Natalio J. Izquierdo MD - 369 De Diego
St., Torre San Francisco Suite 310, San Juan, Puerto Rico. 00923.
E-mail: [email protected]
INTRODUCTION
a
Granulomatosis with Polyangiitis also called Wegener’s Granulomatosis (WG/GPA) was named after
Friederich Wegener who first described the disease
(1-3). It is a multi-systemic granulomatous inflammatory disorder with vasculitis and necrotic manifestations, presumed to be of autoimmune origin (4). According to the American College of Rheumatology
(5), patients with WG/GPA have the following clinical finding: nasal or oral inflammation; pulmonary
findings; renal findings, and vascular-granulomatous
inflammation. For the purpose of classification, WG/
GPA was divided into limited and generalized subtypes (6). A very limited form of WG/GPA, with localized ophthalmic features only, has also been described (7). Ocular and orbital structure involvement
is common in patients with both limited and general forms of WG/GPA. Ocular findings occur in up to
60% of patients with WG/GPA and may be the initial
presentation of the disease.
Links para la descarga gratuita de la aplicación
Previous studies (8, 9), have reported that ocular
manifestations of WG/GPA may involve any ocular
structure including: scleritis (10-11), ulcerative and
necrotic conjunctivitis (12), peripheral ulcerative keratitis (PUK) (10), corneal stromal infiltration, clouding,
perforation, and sclerouveitis (8). Retinal manifestations of the disease include: retinitis, chorioretinitis,
macular edema, exudative retinal detachment, and
retinal necrosis (8). Common presentations of orbital WG/GPA include: proptosis, diplopia, periorbital swelling, reduced vision, eye redness, pain, and
epiphora (8,12).
Anti-neutrophil Cytoplasmic Antibody (ANCA) tests
have been demonstrated to have strong associations with WG/GPA, which is one of the ANCA-As
sociated Vasculitis (AAV) (13). Based on the pattern
of staining on ethanol-fixed neutrophils, ANCAs can
be divided into two main patterns. First, cytoplasmic-ANCA (c-ANCA) shows a diffusely granular, cytoplasmic staining pattern and is strongly associated
with WG/GPA. On the other hand, Perinuclear-ANCA
(p-ANCA), shows a perinuclear staining pattern, and
mainly associated with Polyarteritis nodosa (PAN). In
AAV, ANCA are shown to specifically target the antigen proteinase 3 (PR3), the predominant antigen
in WG/GPA, or myeloperoxidase, the predominant
antigen in Microscopic Polyangiitis (MPA), and these
can be detected by the enzyme-linked immunosorbet assay (ELISA) test. The exact mechanism by
which ANCA are generated is as yet undetermined,
but molecular mimicry by viruses or bacteria, drugs,
and genetic predisposition, such as the defective
allele PI*Z on chromosome 14q32.1(14) and HLADPB1,(15) have all been associated with WG/GPA
and may lead to the development of ANCA in patients with AAV (16-17).
We report on systemic and ocular findings of two
Puerto-Rican patients with WG/GPA.
Case History
Patient 1
This is the case of a 54 year-old male patient with
non-insulin dependent diabetes mellitus and hypertension who developed uveitic glaucoma, bilateral
scleritis, and bilateral diabetic retinopathy. He had a
positive rheumatoid factor, positive C-reactive protein (CRP), but a negative ANCA result. Upon urinalysis patient did not show proteinuria or hematuria.
A diagnostic nasal mucosa biopsy showed inflamed
respiratory mucosa. Patient’s medications included: oral Prednisone (20 mg.), Metformin, Pregabalin
(Lyrica), Rosuvastatin Calcium (Crestor), Liraglutide
(Victoza), Carvedilol, Losartan, and Insulin Lispro
(Humalog).
Patient’s best-corrected visual acuity was 20/30 and
20/80 in the right and left eye respectively. Refraction
was -2.25 + 1.00 x 170 Add 2.25; and -0.75 + 2.50
x 165 Add 2.25 in the right and left eye respectively.
Patient had traces of cells in the anterior chamber
of both eyes, and posterior vitreous detachment of
the left eye. He developed a posterior sub-capsular
cataract and underwent phacoemulsification with intraocular lens implantation in the left eye. Six months following surgery his Macular Optical Coherence Tomography (OCT) showed a Foveal Thickness of 163 +/- 7 microns and 208 +/- 29 microns
in the right and left eye, respectively. Total macular
volume was 6.31mm3 and 8.48mm3, in the right and
left eye, respectively (see Figure 1).
Patient 2
and left eye, respectively. She had bilateral nuclear
sclerosis, and a 0.8 Cup to disk ratio in both eyes
and a 30-2 Sita Fast Humphrey’s Visual Field test
compatible with glaucoma.
DISCUSSION
ANCA’s are a group of IgG antibodies directed
against the cytoplasm of neutrophil granulocytes
and monocytes and can be detected in the serum by
indirect immunofluorescence (13). ANCA tests have
been demonstrated to have strong associations with
WG/GPA as well as with the other ANCA associated
vasculitis (AAV). Previous studies have reported that
patients with WG/GPA have a positive C-ANCA (13).
In 1985, Van der Woude and co-worker first reported
that titers of ANCA often correlated well with the activity of the disease (18). In systemic WG/GPA, c-ANCA were found to be positive in 74% of patients, with
anti-PR3 detected in 87% (19-20). In current clinical
practice, ANCA detection is used for the diagnosis of
WG/GPA and as an indicators of the disease activity. However, the ANCA titer is not entirely reliable in
monitoring disease activity, and should not be solely
relied upon in the management of WG/GPA patients
(21). Findings in one of our patients correlate with
these studies. Patient 2 presented a positive C-ANCA result at 1:80 dilution of 16.2 which supported the
diagnosis of WG/GPA.
A 69 year-old female patient who presented with a
history of sinusitis, depression, lower gastrointestinal
bleeding, and uveitic glaucoma. She had a positive
C-ANCA. Upon urinalysis patient had a large amount
of blood and proteinuria of 100 mg/dl. A second urinalysis showed a hematuria and proteinuria over ≥ According to the American College of Rheumatolo300 mg/dl. This patient developed a superior periph- gy patients with WG/GPA have clinical findings ineral ulcerative keratitis of the right eye.
cluding: nasal or oral inflammation (oral ulcers or
bloody nasal discharge), pulmonary findings (nodPatient’s medications included: Brimonidine 0.2% ules, infiltrates or cavities), renal findings (hematuria
bid; Celluvisk drops; Prednisone 12.5 mg daily, and or proteinuria) and vascular granulomatous inflamMycophenolate Mofetil (Cellcept).
mation (within the arterial wall or perivascular area)
upon biopsy (5). Two or more positive of the above
Best-corrected visual acuity was 20/25 and 20/30 in mentioned findings have a sensitivity of 88.2% and a
right and left eye, respectively. Refraction was 1.00 specificity of 92.0% of describing WG/GPA (5).
+ .25 x 10 Add 2.00 and 1.25 + .25 x 170 in the right
Renal and lung disease in patients with WG/GPA are
often asymptomatic. Hematuria is usually microscopic, and a urinalysis is a sensitive and cost-effective
test to discover glomerulonephritis (12). Proteinuria
is defined as urinary protein excretion of greater
than 150 mg per day (23-24). Since results of urine
dipstick and Sulfosalicylic Acid tests are crude estimates of urine protein concentration and depend on
the amount of urine produced, they correlate poorly
with quantitative urine protein determinations (25).
For this reason, patients with persistent proteinuria
should undergo a quantitative measurement of protein excretion, which can be done with a 24-hour
urine specimen (23). In our study, Patient 1 had
inflamed respiratory mucosa with a biopsy consistent with granulomatous inflammation, but did not
demonstrate hematuria. Patient 2 had two urinalyses
showing hematuria and proteinuria, but did not have
nasal or oral inflammation. These findings are compatible with the American College of Rheumatology
description of patients with WG/GPA.
Orbital findings in patients with orbital WG/GPA include: proptosis due to an orbital mass, diplopia,
periorbital swelling, reduced vision, eye redness,
pain, and epiphora (8,12,26). The majority of patients with WG/GPA who have orbital involvement
show contiguous disease involving the nose and paranasal sinuses. However, orbital WG/GPA may also
occur de novo with orbital granuloma formation or orbital vasculitis. Even though Patient 1 had inflamed
respiratory mucosa on biopsy, there were no signs
of orbital involvement. Neither of our patients developed proptosis. Absence of these signs does not rule
out the diagnosis.
Eyelid involvement in WG/GPA is uncommon, but includes lid granulomas and ptosis. In severe cases,
lid destruction may also occur. The 'yellow-lid sign',
when associated with orbital inflammation, has been
suggested to point to the clinical diagnosis of WG/
GPA (27), and is described as resembling xanthelasma despite the patient having normal lipid metabolism (28). Neither of our patients had xanthelasma.
Dacroadenitis and lacrimal gland enlargements are
recognized features of adnexal involvement in WG/
GPA and may cause ocular sicca syndrome (29). Dacrocystitis and epiphora can occur as a consequence
of nasolacrimal disease, and nasolacrimal blockage
in WG/GPA may be a result of inflammatory spread
from adjacent paranasal inflammation, or a direct
consequence of focal WG/GPA inflammation (8,12).
Patient 2 used eye lubricants due to dry eye symptoms.
Figure 1. Macular Optical Coherence Tomography of Patient 1 representative of Macular Edema of the left
eye (right in figure).
Conjunctival involvement occurs in 16% of patients
with WG/GPA (30), manifesting as ulcerative and
necrotic conjunctivitis, which may result in marked
cicatricial changes of the conjunctiva (12). Both patients had been treated for red eyes. The differential
Figure 1-a: Hepatobiliary scan. Adequate hepatic tracer extraction; traumatic liver cyst (devoid of activity focus at hepatic
dome). Two-hour views show trail of activity from hepatic dome
into right hemithorax.
Figure 1-b: Thoracoabdominal diagram. Illustration of
the right pleurobiliary fistula.
8. Pakrou N, Selva D, Leibovitch I. Wegener's granulomatosis:
ophthalmic manifestations and management. Semin. Arthritis
Rheum. 35(5),284–292 (2006).
9. Joshi L, Hamour S, Salama AD, Pusey CD, Lightman S, Taylor
SRJ. Renal and ocular targets for therapy in Wegener's granulomatosis. Inflamm. Allergy Drug Targets 8(1),70–79 (2009).
(yellow
lids)
sis.
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Wegener's
granulomato79(5),453–456
(1995).
28. Dharmasena A, Tambe K, Shah N, Brown L, Vaidhyanath R, Sampath R. Yellow lid sign in Wegener's
granulomatosis.
Orbit
28(2–3),194–195
(2009).
10. Galor A, Thorne JE. Scleritis and peripheral ulcerative keratitis. Rheum. Dis. Clin. North Am. 33(4),835–854, vii (2007).
29. Khanna D, Shrivastava A. Suppurative dacroadenitis
causing ocular sicca syndrome in classic Wegener's granulomatosis. Indian J. Ophthalmol. 59(2),151–153 (2011).
11. Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and
scleritis: clinical features and treatment results. Am. J. Ophthalmol. 130(4),469–476 (2000).
30. Robinson MR, Lee SS, Sneller MC et al. Tarsal-conjunctival disease associated with Wegener's granulomatosis.
Ophthalmology
110(9),1770–1780
(2003).
12. Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegener's granulomatosis: clinical manifestations, differential diagnosis, and management of ocular and systemic disease. Surv.
Ophthalmol. 55(5),429–444 (2010).
31. García C, Voorduin S, Pedroza-Seres M. Diagnosis of
Wegener's granulomatosis in patients with ocular inflamatory disease. Gac. Med. Mex. 142(6),477–482 (2006).
13. Hazlita Isa, Susan Lightman, Charles D Pusey, Simon RJ
Taylor. Ocular Manifestations of Wegener’s Granulomatosis. In:
Expert Rev Ophthalmol. 2011;6(5):541-555.
14. Borgmann S, Endisch G, Urban S, Sitter T, Fricke H. A linkage disequilibrium between genes at the serine protease inhibitor gene cluster on chromosome 14q32.1 is associated with Wegener's granulomatosis. Clin. Immunol. 98(2),244–248 (2001).
15. Lamprecht P, Wieczorek S, Epplen JT, Ambrosch
P, Kallenberg CGM. Granuloma formation in ANCA-associated vasculitides. APMIS 127,32–36 (2009).
Index words: role, nuclear, imaging, traumatic,
hepatopleural fistula
33. Chan AYK, Li EK, Choi PCL, Liu DTL, Lam PTH,
Lam DSC. Unusual eye signs in Wegener's granulomatosis. Hong Kong Med. J. 13(3),241–242 (2007).
INTRODUCTION
16. Kallenberg CGM. Pathogenesis of ANCA-associated vasculitis, an update. Clin. Rev. Allergy Immunol. 41(2),224–231 (2011).
35. Wang M, Khurana RN, Sadda SR. Central retinal
vein occlusion in Wegener's granulomatosis without retinal vasculitis. Br. J. Ophthalmol. 90(11),1435–1436 (2006).
17. Flint J, Morgan MD, Savage COS. Pathogenesis of ANCA-associated vasculitis. Rheum. Dis. Clin. North Am. 36(3),463–477
(2010).
36. Morell-Dubois S, Quéméneur T, Bourdon F et al. [Central
retinal artery occlusion in Wegener'granulomatosis]. Rev. Med.
Interne. 28(1),33–37 (2007).
18. Pradhan VD, Badakere S S, Ghosh K, Almeida A. ANCA:
Serology in Wegener's granulomatosis. Indian J Med Sci 2005;
59:292-300
37. Costello F, Gilberg S, Karsh J, Burns B, Leonard B. Bilateral
simultaneous central retinal artery occlusions in wegener granulomatosis. J. Neuroophthalmol. 25(1),29–32 (2005).
19. Tsiveriotis K, Tsirogianni A, Pipi E, Soufleros K, Papasteriades
C. Antineutrophil cytoplasmic antibodies testing in a large cohort of
unselected Greek patients. Autoimmune Dis. 2011,626495 (2011).
38. Wong SC, Boyce RL, Dowd TC, Fordham JN. Bilateral central retinal artery occlusion in Wegener's granulomatosis and
α(1) antitrypsin deficiency. Br. J. Ophthalmol. 86(4),476 (2002).
20. Wiik A. Rational use of ANCA in the diagnosis of vasculitis. Rheumatology 41(5),481–483 (2002).
39. Janknecht P, Mittelviefhaus H, Löffler KU. Sclerochoroidal
granuloma in Wegener's granulomatosis simulating a uveal melanoma. Retina 15(2),150–153 (1995).
21. Finkielman JD, Merkel PA, Schroeder D et al. Antiproteinase 3
antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann. Intern. Med. 147(9),611–619 (2007).
22. Rao JK, Weinberger M, Oddone EZ, Allen NB, Landsman P, Feussner JR. The Role of Antineutrophil Cytoplasmic Antibody (c-ANCA) Testing in the Diagnosis of Wegener Granulomatosis: A Literature Review
and Meta-analysis. Ann Intern Med. 1995;123:925-932.
23. Carroll MF, Temte JL. Proteinuria in adults: a diagnostic
approach. Am Fam Physician. 2000 Sep 15;62(6):1333-40.
24. Laffeyette RA, Perrone RD, Levey AS. Laboratory evaluation of renal function. In: Schrier RW, Gottschalk CW, eds.
Diseases of the kidney. Boston, Mass: Little Brown, 1996:339.
25.
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26. Taylor SRJ, Salama AD, Pusey CD, Lightman
S. Ocular manifestations of Wegener's granulomatosis.
Expert
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Opthalmol.
2(1),91–103
(2007).
27. Tullo AB, Durrington P, Graham E et al. Florid xanthelasmata
Penetrating injuries are a major cause of mortality and morbidity. The most common diagnostic
tool used in this setting is CT scan. We present a
case of a traumatic hepatopleural fistula demonstrated by hepatobiliary scintigraphy. Traumatic
abdominal injuries to the liver and diaphragm
can result in complications, whose detection by
CT scan is not always straightforward. Hepatobiliary scintigraphy is very useful in the search
of bilious pleural effusions.
32. Biswas J, Babu K, Gopal L, Krishnakumar S, Suresh S, Ramakrishnan S. Ocular manifestations of Wegener's granulomatosis.
Analysis of nine cases. Indian J. Ophthalmol. 51(3),217–223 (2003).
34. Hijikata N, Takayanagi N, Yoneda K, Sugita Y, Kawabata Y. A case of scleritis as the initial clinical manifestation of limited Wegener's granulomatosis. Nihon Kokyuki Gakkai Zasshi 47(11),1025–1029 (2009).
RESUMEN
Granulomatosis de Wegener o Granulomatosis
con Polyangeitis (WG/GPA) es una de las vasculitis asociada a anticuerpos anticitoplasma
de neutrófilos. Las manifestaciones oculares
suelen ser los síntomas iniciales en pacientes
con GW/GPA. Reportamos sobre dos pacientes
diagnosticados con GW/GPA y discutimos sus
hallazgos pertinentes. Nuestro reporte recopila las manifestaciones clínicas más comunes y
las asociaciones oftálmicas para compararlas
a los hallazgos físicos de dos pacientes hispanos. Además, este es el primer reporte de los
hallazgos oculares en pacientes puertorriqueños
con WG/GPA.
THE ROLE OF
NUCLEAR
IMAGING IN
TRAUMATIC
HEPATOPLEURAL
FISTULA:
A Case Report
ABSTRACT
Trauma is the leading cause of death in the US among
ages 1-44 years old (1). An estimated 2.5 million people
are hospitalized in the US due to trauma each year (1).
To improve patient outcomes, we need adequate diagnostic tools to help emergency room physicians in medical decision-making. Evaluation of the trauma patient
can improve with the knowledge of the localization and
mechanism of injury; it is critical for choosing imaging
modality and treatment. Thoracoabdominal injuries due
to penetrating trauma can result in diaphragmatic and
liver injury. Injuries sustained by the diaphragm and liver could cause visceral herniation with or without strangulation or perforation, respiratory compromise due to
lung entrapment, bile leakage, hemothorax, empyema,
hematomas and internal hemorrhage. Penetrating traumas have been reported to produce approximately 65%
of diaphragmatic injuries (2). These injuries are small in
size (≤ 1-2 cm) making it difficult to identify by CT scan
(3). It can be difficult to establish the trajectory of injury
in right hemidiaphragmatic rupture since the diaphragm
and liver have similar radiodensity in CT scan (3). A diagnosis of bronchobiliary and pleurobiliary fistula is based
on radiologic imaging findings. CT scan is non-specific
to identify the biliopleural communication (4). In contrast,
hepatobiliary scintigraphy can provide information in cases of right hemidiaphragmatic trauma with suspected bile
leak by the antegrade visualization of the route of bile
flow and detection of areas of biliary disruption. This is
accomplished with the IV administration of a radiotracer
with physiological handling by the liver similar to that of
bilirubin. Brugge et al considered cholescintigraphy as
quite accurate (83%-87%) in predicting biliary leakage
(5). We present a case of traumatic hepatopleural fistula
demonstrated by scintigraphy.
Juan C Negrón MDa*
Ralph Martin MDa
Frieda Silva MDa
Gary Ehlert MDa
Nuclear Medicine Section, Radiological Sciences Department, UPR School of Medicine, Medical Sciences Campus,
San Juan, Puerto Rico.
*Corresponding author: Juan C Negrón MD - Nuclear Medicine Section, Radiological Sciences Department, UPR
School of Medicine, Medical Sciences Campus, University of Puerto Rico PO BOX 365067, San Juan, Puerto Rico
00936-5067. E-mail: [email protected].
a
a right white lung. At this time, the chest tube
was draining bilious fluid; a contrast enhanced
chest CT scan revealed a large right side pleural
effusion. The right liver lobe laceration was visualized extending into the right hemidiaphragm.
Two days after the CT scan, he underwent open
thoracotomy for decortication and drainage of
retained hemothorax and the bilious pleural effusion. In addition, Jackson-Pratt drainage was
placed at the gallbladder fossa. Six days later,
hepatobiliary scintigraphy was performed due
to continued drainage of bile and suspicion of a
hepatopleural fistula. The study was done following the intravenous administration of 6 mCi of
99mTc-mebrofenin. Serial images of the anterior
abdomen were acquired for 60 minutes. Delayed
anterior and right lateral views were obtained at
90-150 minutes.
Images of the hepatobiliary scintigraphy showed
prompt hepatic extraction of the radiotracer and a
photopenic (devoid of activity) defect in the right
hepatic dome that gradually filled-in with activity.
Anterior and right lateral views at two hours reCase History
vealed a trail of activity arising from the hepatic
dome into the right hemithorax; these findings
The patient had an exploratory laparotomy due to pen- were compatible with a communicating post trauetrating injury that revealed a diaphragmatic laceration, matic bile-containing cyst in the hepatic dome
along a grade II liver laceration and a moderate hemo- draining into the right pleural space (see Figures
peritoneum. Six days after admission chest film showed 1-a, 1-b). Nevertheless, the preferential route
of the bile flow was biliary-to-bowel transit. The relative amount of radiotracer at the bile extravasation
site is small compared to the amount of radiolabeled
bile excreted into the intestines.
DISCUSSION
Diaphragmatic and liver injuries have been reported
in 12-20% of cases of penetrating abdominal trauma
(3). These lesions occur more frequently in the left
side; right hemidiaphragmatic lesions are less frequent. This may be related to the stronger right diaphragm and to the protective effects of the liver (3). Thoracoabdominal injuries in the right side are related to a higher percentage of complications than
left-sided injuries, because of liver involvement on
this side (6). Liver injuries can result in free peritoneal
bile leak, which has been linked with increased morbidity, and longer hospital stays (7). Transdiaphragmatic biliary fistulas, with bile empyema, developed
in about 3 percent of the lacerations of the liver in
right-sided wounds (6); their presence is suspected
by the development of a right pleural effusion.
Current guidelines recommend the use of CT scan
as the initial imaging modality of choice in cases of
abdominal trauma. Several CT signs indicative of
diaphragmatic injury have been described in the literature; these include direct visualization of injury,
segmental diaphragm nonvisualization, intrathoracic
herniation of viscera, the dependent viscera sign,
diaphragm thickening, and peridiaphragmatic active
contrast extravasation (3). Larici and colleagues reported that the sensitivity and specificity of helical CT
in the diagnosis of penetrating diaphragmatic injury
were 86% and 79% respectively; sensitivity in patients with right side injuries was 79% (8). However,
it is well known that small diaphragmatic tears may
be missed by anatomic imaging modalities such as
CT. The initially missed diagnoses of diaphragmatic
injury by CT scan ranges form 12-63% (7).
Hepatobiliary scintigraphy is a relatively low radiation
dose nuclear medicine study that evaluates the bile
flow out of the liver into the small intestine, allowing evaluation of, among other things, hepatocellular
function, patency of the biliary system, and possible
bile extravasation. Following IV injection, the radiotracer is removed from the blood by a carrier-mediated anionic clearance into the hepatocytes and
secreted into the bile canaliculi, with eventual transit
into the small intestine. The study allows the evaluation of the principal and alternate excretory pathways
of the bile. Hepatobiliary scintigraphy also provides
useful prognostic information and is one of the most
sensitive tests to identify bile leaks (9).
In this case of right diaphragmatic injury, hepatobiliary
scintigraphy was able to confirm the active bile flow
across the diaphragm, crossing the border between
the abdominal to the pleural cavity. Furthermore,
it demonstrated that the preferential route of bile excretion was into the intestines and that the amount
of activity at the leakage site was small; this kind
of biliary leak usually seals spontaneously (9). Patients in whom the preferential route follows the biliary-to-bowel transit course usually respond to conservative therapy while those patients in whom bile
extravasation is the main route for biliary drainage
will probably require surgical intervention (9). The
nuclear study revealed, besides complementary diagnostic information to the CT scan findings, clinically relevant information regarding prognosis in this
patient. Fortunately the drainage of pleural effusion
decreased significantly and resolved spontaneously,
as anticipated given the scintigraphic results. No further intervention was required.
Prompt identification of the diaphragmatic lesions
decreases morbidity and mortality. Failure to make
the correct diagnosis and provide prompt treatment
may lead to delayed complications (10). Biliary scintigraphy is a useful diagnostic tool and
plays a complementary role with CT scan in complicated cases of liver and diaphragmatic injury where
thoracobiliary communications are suspected. Our case illustrates the extremely important diagnostic information that can be offered by hepatobiliary scintigraphy to clinicians in the clinical setting
of suspected bilious pleural effusion in patients with
right hemidiaphramatic trauma. Hepatobiliary scintigraphy can also provide information of prognostic significance by showing the preferential route of biliary
drainage: normal intestinal course pathway versus
biliary extravasation. In conclusion, when evaluating
thoracoabdominal trauma patients, combining biliary scintigraphy and other anatomic modalities will
allow a thorough assessment of possible complications, i.e. pleurobiliary fistula, associated with right
diaphragmatic injuries.
REFERENCES
1) Centers for Disease Control and Prevention, National Center
for Injury Prevention and Control. Web–based Injury Statistics
Query and Reporting System (WISQARS) [online]. Accessed
March 18, 2014.
2) Zarour AM, El-Menyar A, Al-Thani H, et al. Presentations and
outcomes in patients with traumatic diaphragmatic injury: a 15year experience. J Trauma Acute Care Surg. 2013; 74(6): 13921398
3) Sliker CW. Imaging of diaphragm injuries. Radiol Clin North
Am 2006; 44(2):199-211.
4) Delis, SG et al. Pleurobiliary fistula, a rare complication of hepatocellular carcinoma after locoregional chemotherapy: a case
report. Cases Journal 2009; 2:7992
5) Brugge WR, Rosenberg DJ, Alavi A. Diagnosis of postoperative bile leaks. Am J Gastroenterol. 1994; 89:2178-83.
6) Brewer III, LA, Burford TH: Thoracoabdominal Wounds: In:
Ahnfeldt AL, Berry FB, McFetridge E. Eds. Surgery in World War
II: Thoracic surgery, Washington, DC, US Army Medical Dept,
1965, Vol.II: 102-141
7) Fleming KW, Lucey BC, Soto JA, Oates ME. Posttraumatic
bile leaks: role of diagnostic imaging and impact on patient outcome. Emerg Radiol. 2006; 12: 103–7.
diagnosis of the causes leading to red eye in patients
with the WGA/GPA needs to be evaluated.
Previous studies (31,34), have reported that several
ocular findings in patients with WGA/GPA may lead
to blindness including: scleritis of the necrotizing type
(10,11,31), peripheral ulcerative keratitis (33), uveitis, ocular hypertension or glaucoma (32). Patient
1 had with bilateral scleritis, and uveitic glaucoma.
Patient 2 presented with PUK and a red eye. These
findings are compatible with the literature.
Retinal and choroid involvement in WG/GPA is rare
(8). Retinal vein occlusion (35), and central retinal
artery occlusion (36-38), may lead to blindness in
patients with WG/GPA. Patient 1 had diabetic retinopathy in both eyes and macular edema of the left
eye. These were associated to Diabetes.
Limitations of the study include a limited number of
patients; inability to ascertain an exact cause of the
disease, and poor specificity of available diagnostic
tests.
In conclusion, diagnosing patients with the WG/
GPA remains a challenge due to poor specificity of
current available screening tests, and non-specific
diagnostic tests. However, patients with multi-systemic disease and red eye, may benefit from screening with ANCA tests, to exclude the possibility of
WG/GPA, its morbidity and mortality. Further studies will continue to shed light on associated symptoms and efficacy of proposed treatment strategies.
8) Larici AR, Gotway MB, Litt HI, et al. Helical CT with saggital
and coronal reconstructions: accuracy for detection of diaphragmatic injury. AJR 2002; 179:451-457
9) Weissmann H, Byun KJC, Freeman LM. Role of 99mTc-IDA
scintigraphy in the evaluation of hepatobiliary trauma. Semin
Nucl Medicine 1983; 13: 199-222 10) Beigi AA, Masoudpour H, Sehhat S, Khademi EF. Prognostic
factors and outcome of traumatic diaphragmatic rupture. Turk J
Trauma Emerg Surg 2010; 16(3): 215-219
RESUMEN
Las heridas penetrantes son una causa importante de mortalidad y morbilidad. La
prueba diagnóstica más utilizada en estos
casos es la Tomografía Computarizada (TC).
Presentamos el caso de una fistula hepatopleural secundaria a trauma identificada
por escintigrafía hepatobiliar (EH). La lesiones traumáticas del hígado y el diafragma
pueden causar complicaciones, cuya detección por TC puede en ocasiones ser ambigua. La EH es muy útil en detectar derrame
pleural bilioso.
REFERENCES
1. F. Wegener: Über generalisierte, septische Gefässerkrankungen.
Verhandlungen der deutschen pathologischen Gesellschaft,
Stuttgart, 1936, 29: 202.
2. F Wegener, Über eine eigenartige rhinogene Granulomatose
mit besonderer Beteiligung des Arteriensystems und der Nieren.
Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1939, 102: 36-38.
3. G. C. Godman, Jacob Churg: Wegener's granulomatosis. Pathology and review of the literature. AMA. Archives of Pathology,
Chicago, 1954, 58: 533.
4. Holle JU, Laudien M, Gross WL. Clinical manifestations and
treatment of Wegener’s granulomatosis. Rheum. Dis. Clin. North
Am. 36(3),507–526 (2010).
5. Leavitt RY, Fauci AS, Bloch DA, et al. (August 1990). "The
American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis". Arthritis Rheum. 33 (8):
1101–7.
6. Carrington CB, Liebow AA. Limited forms of angiitis and granulomatosis of Wegener's type. Am. J. Med. 41(4),497–527 (1966).
7. Ahmad I, Lee WC, Nagendran V, Wilson F, Shortridge RTJ.
Localised Wegener's granulomatosis in otolaryngology: a review
of six cases. ORL J. Otorhinolaryngol. Relat. Spec. 62(3),149–
155 (2000)
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THE USE OF A FIBULAR
STRUT ALLOGRAFT
WITH DBM, CANCELLOUS
CHIPS AND BMP FOR A
10 CM HUMERAL SHAFT
INFECTED NON-UNION:
A Case Report
Pedro J. Reyes Martínez MDa
Pablo A. Marrero Barrera MDa*
Department of Orthopedic Surgery, UPR School of Medicine, Medical Sciences Campus.
*Corresponding author: Pablo A. Marrero Barrera, MD – University of Puerto Rico,
Medical Sciences Campus, Department of Surgery-Orthopedics PO Box 365067, San
Juan, Puerto Rico 00936-5067. E-mail:[email protected]
a
H
INTRODUCTION
Humerus fractures make up about 5% to 8% of all fractures and 10%
of all long bone fractures (1-4). Approximately 30% of all humeral fractures occur at the diaphysis. Most of these fractures can be treated
non-surgically with excellent outcomes; Sarmiento et al (5) reported
union rates of 94% for open and 98% for closed fractures of the humeral shaft after functional bracing. Nonunion of the humeral shaft occurs in 2% to 10% of non-surgically treated fractures and close to 15%
of fractures treated by open reduction and internal fixation (4-7). Increased incidence of nonunion is associated with open fractures, high
impact injuries, bone loss or fracture gapping, soft tissue interposition,
unstable fracture patterns, segmental fractures, impaired blood supply,
infection, and initial treatment with traction (4). A humeral fracture is
classified as nonunion if no progression towards healing is witnessed
in three months or there is evidence of a lack of union six months (7).
Union is expected to occur between 12 to 16 weeks. Risk factors for
developing nonunion include both biologic host factors (smoking history, disrupted blood supply, medical comorbidities) and mechanical
(fracture displacement and inadequate immobilization) factors. Patients who fail nonsurgical management for humerus fractures and develop a nonunion should preferably undergo surgery with the ultimate
goal of creating a biological environment that favors bone healing and
providing a stable mechanical construct that allows for early motion.
Currently, the standard of care for humeral nonunion is open reduction
and internal fixation of the defect with rigid compression plating and
autogenous bone grafting (8). Success has been reported with the use
of demineralized bone matrix (DBM) with or without bone morphogenic
protein (BMP) along with plating (9-12). Biologic augmentation with
BMP-2 and BMP-7 has been performed but to our knowledge, there
is no study that documents their efficacy in humeral non-union. There
are other techniques that have been recently described which include
intramedullary fibular strut autograft for atrophic proximal nonunions
and augmentation of compression plating in cases of previous surgical
treatment (13, 14), and dual compression plating for humeral shaft
nonunions with poor bone quality such as with patients who have osteoporosis or osteopenia (9, 15, 16).
ABSTRACT
Humeral shaft non-unions
occur in 2-10% of all fracture
cases. Increased incidence
of these non-unions can be
associated with ORIF, comminution, high impact injuries,
bone loss or fracture gaping. Treatment guidelines for
fracture non-union state that
fractures with gaps greater
than 4 cm should be treated
with vascularized fibular autografts or transportation with
an external fixator. Unfortunately these modalities carry
considerable donor site morbidity and patient will experience considerable discomfort, especially when dealing
with an external fixator. This
report demonstrates how the
use of a nonvascularized fibular strut can be effectively utilized as an alternate treatment
modality for large humeral
shaft non-union gaps. Further
studies should be conducted to support this method as
a viable treatment option for
non-union gaps greater than
4 cm.
Index words: fibular, strut,
allograft, cancellous, chip,
humeral, shaft, infected, nonunion
Bone loss can complicate fracture
healing and, as previously mentioned, is a risk factor for developing nonunion. Managing patients
with extensive bone loss can be a
challenge and advanced treatment
techniques are needed. A defect of
3 to 4 cm can undergo acute shortening and plate fixation, as functionality and motion of the arm is
not impaired. For defects greater
than 4 cm, the literature supports
the use of vascularized fibula transfer and bone transport (17-20);
however they offer significant donor site morbidity and the utilization
of an external device for extended
periods of time (respectively).
To our knowledge, the
use of fibular allograft
(nonvascularized) as a
strut graft for large gaps of
bone loss (> 4 cm) has not
been described. In this report, we present the case
of a diabetic woman who
presented with history of
humeral shaft infected
nonunion that failed two
prior attempts of surgical
treatment at another institution, and presented with
loose hardware and a 10
cm gap of bone loss with
positive CRP and ESR
parameters. We will describe the use of a fibular
strut allograft for augmentation of dual compression plating and the use
of both BMP and DBM
for biologic augmentation
of fracture healing, which
resulted in excellent bone
formation with a rigid fixation and functional arm.
Case History
This is the case of a 47
year-old-woman
with
past medical history of
hypertension and Diabetes Mellitus type II, taking
Avalide and Amaryl, who
presented to our Orthopedic clinics with instability
in her left arm associated
with decreased ROM of
elbow of three years duration. Patient refers that Figure 1: X-rays taken two years prior to visit, showed internal fixation with loose hardware and
she was in a car accident evidence of nonunion with poor bone formation and mild sclerosis around compression plating. A
three years ago, which compression non-locking plate with wire cerclage was used.
resulted in a humerus fracture, and required surgi- bone saucerization. A posterior approach to the hucal intervention for open reduction and internal fixa- meral shaft was taken. Areas of non-viable bone and
tion by a community orthopedic surgeon at the time. a large area of fibrotic nonunion were found with no
X-rays films taken two years prior to visit showed in- gross purulent discharge. The loose hardware was
ternal fixation with loose hardware and evidence of removed in its entirety as well as debridement of
nonunion with poor bone formation and mild sclero- large areas of bone fragments, which left a 10 cm
sis around compression plating (see Figure 1). Sub- bone loss gap in the humerus. Cultures were taken
sequent films three years after initial surgery showed and the area was irrigated with 3 liters of 0.9 normal
progressive osteolysis, bone resorbtion and hard- saline solution. The incision was closed using vycryl
ware loosening with continuing non-union with an 8 (for muscle tissue) and prolene (skin). Patient was
subsequently places in prophylactic IV vancomycin
cm gap in the humeral shaft (see Figure 2).
therapy for two weeks. During this time, pathology
Laboratory data revealed WBC count in 4.9, ESR report showed no abnormality in tissue specimen
level was reported at 96, CRP was found to be pos- except for necrotic fragments of bone, cartilage and
itive, glucose level was at 250, and HbA1c was at 8. connective tissue. Microbiology report from orthoDue to the high probability of chronic infection, the pedic device and tissues revealed no growth after 5
days.
patient was scheduled for removal of hardware and
On the second week after
the first surgery, patient
was taken again to the
operating room for open
reduction and internal fixation. Patient was placed
in right lateral decubitus
position. Skin incision was
done through previous
scar. After verifying correct
humeral length, non-union
was fixed utilizing dual
compression plates with
6 distal and 6 proximal
screws. A 10 cm non-vascularized fibular allograft
was then placed as a strut
to act as support for the
10 cm bone gap and was
fixed utilizing one screw
distally. Cancellous chips
were first placed around
fibular strut, followed by
demineralized bone matrix (DBM) paste around
the
cancellous
chips.
Bone Morphogenic Protein
(BMP) was then placed between the plates, posterior
to the DBM paste, cancellous chips and fibular strut
construct. The wound was
again closed using vycryl
and prolene sutures.
Patient was discharged
from orthopedic ward two
days postop with only
Figure 2: Subsequent X-rays 3 years after initial surgery showed progressive osteolysis,
bone resorbtion and hardware loosening with continuing non-union with an 8 cm gap in oral antibiotic prophylaxis
the humeral shaft
Figure 3: 2 month follow-up x-rays revealed callus formation over fibular
strut graft in both distal and proximal segments, with both graft and hardware in place
Figure 4: Two years after most recent surgery, graft remains in place and fracture non-union appears rigid with no instability.
with instructions for limited motion and no lifting of
objects. After two months of follow-up and physical
therapy, x-rays revealed callus formation over fibular strut graft in both distal and proximal segments,
with both graft and hardware in place (see Figure 3).
Elbow range of motion improved significantly from
30 degrees of extension to 20, and from 80 degrees
of flexion to 90 degrees. Laboratory results showed
CRP levels less than 6 and an ESR level of 20. The
patient was judged to have a fixed humeral shaft
non-union and was returned to full activity and asked
to return every four months for the next year to follow up fracture healing. After two year follow up, no
evidence of deterioration or infection was found and
patient reported return to full activity with no pain
(see Figure 4). Physical examination showed rigid
construct with no instability. Patient was discharged
from clinics and asked to return as needed.
DISCUSSION
Recommended treatment for humeral shaft nonunion with a gap greater than 4 cm includes the utilization of vascularized fibular autograft for restoration
of length while providing live bone tissue. Another
described alternative is the use of external hardware for bone transport. While effective, both these
techniques have serious downfalls: Autologous bone
transport has significant donor site morbidity, while
bone transport is a lengthy procedure that can take
months to complete, all with an uncomfortable external device.
This article describes a possible alternate treatment for large gap non-union humeral shaft fractures, specifically those greater than 4 inches with
great instability. The 47 year old woman described
most likely attained her non-union due to chronic infection, possible facilitated by her uncontrolled diabetes mellitus, as evidenced by her elevated blood
sugar and HbA1c levels. This patient with a 10 cm
humeral shaft non-union gap was adequately treated
using non-vascularized fibular allograft as a strut to
maintain gap length, while utilizing DBM, BMP and
cancellous chips to create a osteogenic and osteoinductive environment that eventually caused callous
formation that effectively closed the 10 cm gap and
completely healed the non-union in two months as
evidenced by serial x-rays films.
The use of a non-vascularized fibula along with BMP,
DBM and cancellous chips can be effectively utilized
as an alternate treatment modality for large humeral
shaft non-union gaps. Unlike the current treatment
recommendations, the use of non-vascularized fibula
as a strut has no donor site morbidity and contains
no external hardware, therefore providing the patient
with a much more comfortable recovery. Future studies can be directed towards determining the safety
and efficacy of this treatment option compared to fibular autograft and bone transportation.
REFERENCES
1. Volgas DA, Stannard JP, Alonso JE: Nonunions of the humerus. Clin Orthop Relat Res 2004;(419):46-50.
2. Green A, Norris TR: Proximal humeral fractures and glenohumeral dislocation, in Browner BD, Levine AM, Jupiter JB, Trafton
PG, Krettek C, eds: Skeletal Trauma: Basic Science, Management, and Reconstruction, ed 4. Philadelphia, PA, Saunders Elsevier, 2009, pp 1623-1754.
3. Schemitsch EH, Bhandar M, Talbot M:Fractures of the humeral shaft, in Browner BD, Levine AM, Jupiter JB, Trafton PG,
Krettek C, eds: Skeletal Trauma: Basic Science, Management,
and Reconstruction, ed 4. Philadelphia, PA, Saunders Elsevier,
2009, pp 1593-1622.
4. Ward EF, Savoie FH III, Hughes JL Jr: Fractures of the diaphyseal humerus, in Browner BD, Jupiter JB, Levine AM, Trafton PG
(eds): Skeletal Trauma: Fractures, Dislocations, Ligamentous
Injuries, ed 2. Philadelphia, PA: WB Saunders, 1998, vol 2, pp
1523-1547
5. Sarmiento A, Zagorski JB, Zych GA, Latta LL, Capps CA:
Functional bracing for the treatment of fractures of the humeral
diaphysis. J Bone Joint Surg Am 2000;82:478-486.
6. Rosen H: The treatment of nonunions and pseudarthroses of
the humeral shaft. Orthop Clin orth Am 1990;21:725-742.
7. Jupiter JB, von Deck M: Ununited humeral diaphyses. J Shoulder Elbow Surg 1998;7:644-653.
8. Healy WL, White GM, Mick CA, Brooker AF Jr, Weiland
Newsletter semanal por email.
AJ: Nonunion of the humeral shaft. Clin Orthop Relat Res
1987;(219):206-213.
9. Prasarn ML, Achor T, Paul O, Lorich DG, Helfet DL: Management of nonunions of the proximal humeral diaphysis. Injury
2010;41(12):1244-1248.
10. Hierholzer C, Sama D, Toro JB, Peterson M, Helfet DL: Plate
fixation of ununited humeral shaft fractures: Effect of type of bone
graft on healing. J Bone Joint Surg Am 2006;88(7):1442-1447.
11. Ring D, Kloen P, Kadzielski J, Helfet D, Jupiter JB: Locking
compression plates for osteoporotic nonunions of the diaphyseal
humerus. Clin Orthop Relat Res 2004;(425):50-54.
12. Taylor NL, Crow SA, Heyworth BE, Rosenwasser MP: Treatment of humeral nonunions with allograft, demineralized bone
matrix, and plate fixation. Osteosynthesis and Trauma Care 2005;
13(2):105-112.
13. Vidyadhara S, Vamsi K, Rao SK, Gnanadoss JJ, Pandian S:
Use of intramedullary fibular strut graft: A novel adjunct to plating
in the treatment of osteoporotic humeral shaft nonunion. Int Orthop 2009;33(4):1009-1014.
14. Willis MP, Brooks JP, Badman BL, Gaines RJ, Mighell MA,
Sanders RW: Treatment of atrophic diaphyseal humeral nonunions with compressive locked plating and augmented with an
intramedullary strut allograft. J Orthop Trauma 2013;27(2):77-81.
15. Badman BL, Mighell M, Kalandiak SP, Prasarn M: Proximal
humeral nonunions treated with fixed-angle locked plating and an
intramedullary strut allograft. J Orthop Trauma 2009;23(3):173179.
16. Cole PA: Endosteal allograft plating for the treatment of recalcitrant nonunions. Techniques in Orthopaedics 2004;18(4):344355.
17. Patel VR, Menon DK, Pool RD, Simonis RB: Nonunion of the
humerus after failure of surgical treatment: Management using the
Ilizarov circular fixator. J Bone Joint Surg Br 2000;82:977-983.
18. Ackerman G, Jupiter JB: Non-union of fractures of the distal
end of the humerus. J Bone Joint Surg Am 1988;70:75-83.
19. Jupiter JB: Complex non-union of the humeral diaphysis:
Treatment with a medial approach, an interior plate, and a vascularized fibular graft. J Bone Joint Surg Am 1990;72:701-707.
20. Wright TW, Miller GJ, Vander Griend RA, Wheeler D, Dell
PC: Reconstruction of the humerus with an intramedullary fibular
graft: A clinical and biomechanical study. J Bone Joint Surg Br
1993;75:804-807.
BOLETIN
RESUMEN
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Las seudoartrosis (no-unión) de diáfisis de humero ocurren en 2-10% de todos los casos de
fracturas. Entre los factores asociados a una
mayor incidencia de seudoartrosis, se encuentran las fracturas tratadas con reducción abierta y fijación interna, cominución, fracturas de
alto impacto, pérdida de hueso y distracción
de las fracturas. Las guías de tratamiento para
seudoartrosis indican que las fracturas con
una distracción mayor de 4 cm deben ser tratadas con injerto autólogo de fíbula vascularizada o transportación ósea con fijador externo.
Desafortunadamente estas modalidades traen
una gran morbilidad de la zona donante y los
pacientes pueden experimentar incomodidad
significativa, especialmente cuando se trata
de un fijador externo. Este reporte demuestra como el uso de injerto alogénico de fíbula no-vascularizada puede ser utilizado como
una modalidad de tratamiento alterna para las
seudoartrosis de diáfisis de húmero con una
distracción de más de 4 cm. Se deben realizar
más estudios para apoyar la viabilidad esta
modalidad.
A VERY RARE
NEOPLASM IN AN
IMMUNOCOMPETENT
PATIENT
ABSTRACT
Kaposi’s sarcoma is a rare malignancy requiring infection with human Herpes virus for development. We report
a case of a 76-year-old immunocompetent male with recurrent leg cellulitis. The cellulitis eventually developed
into a non-healing ulcer and a palpable
nodule consistent with nodular Kaposi’s sarcoma.
Emmanuel O. González-Irizarry MDa*
Luis A Figueroa-Jiménez MDb
Javier Ramos Rossy MDa
Rodolfo Estremera MDa
Javier Monserrate MDa
Amy Lee González-Márquez MDb
Luis Negrón-Garcίa MDc
Mόnica Santiago-Casiano MDc
William Cáceres-Perkins MDc
Index words: rare, neoplasm, immunocompetent, patient, Kaposi, sarcoma
K
INTRODUCTION
Kaposi’s sarcoma (KS) is a rare malignant,
slowly progressing, mesenchymal neoplasm
characterized by proliferation of connective
tissue and capillaries. Clinical presentation is
usually as nodules and red-purple plaques. It
occurs most often in older men of Mediterranean or Central/Eastern European ancestry,
in whom the lesions usually occur on the distal extremities, particularly the lower legs and
feet.
The exact nature of the disease is not clear. It
remains controversial whether the endothelial cells are of vascular origin, lymphatic origin
or both. Current data support the notion that
KS is a vascular hyperplasia with a tight link
to human Herpes virus 8 infection. The virus
was first identified in KS cells of a patient with
AIDS but later, it had been linked convincingly with all four types of KS, an association that
is necessary, but not sufficient to develop KS.
Other factors are also important in the etiology of Kaposi’s sarcoma, such as immunosuppression. We report the development of KS in
an immunocompetent male patient.
Internal Medicine Department, VA Caribbean Healthcare System, San
Juan, Puerto Rico.
b
Internal Medicine Department, San Juan City Hospital, San Juan, Puerto
Rico.
c
Hematology–Medical Oncology Section, VA Caribbean Healthcare System and San Juan City Hospital, San Juan, Puerto Rico.
*Corresponding author: Emmanuel O. González-Irizarry MD - Internal
Medicine Department, VA Caribbean Healthcare System, 10 Casia St.,
San Juan, Puerto Rico 00921. E-mail: [email protected]
a
Case History
This is a 76-year-old male patient with past
medical history of hypertension, unstable angina pectoris, chronic obstructive pulmonary
disease, gastroesophageal reflux, and colon
adenocarcinoma status post resection in year
2012 on complete remission. The patient presented to his primary care physician with the
chief complaint of a left heel lesion at the medial aspect of three months of evolution (see
Figure 1). This lesion was an open ulcer of
approximately one cm of diameter, elevated
approximately 3mm, with well-defined borders, draining serous as well as purulent material, and friable (see Figure 2). There was
absence of pain or tenderness upon palpation of the area. The patient received oral and
Figure 1: Leg nodules and erythematous/faintly purplish plaques, with associated grayish discoloration of the skin.
intravenous antibiotics but the lesion failed to improve. He was
referred to a dermatologist and pyoderma granuloma was the
first diagnostic impression. As the patient’s medical condition
did not improve, the decision to perform a skin biopsy was
made. A skin biopsy reported spindle cells, high vascularity and
intracellular hyaline globs and spaces, imparting a pattern that
is strikingly reminiscent of a cavernous hemangioma consistent with nodular Kaposi’s sarcoma (see Figure 3). No regional
lymphadenopathies were present. The patient had two previous
negatives HIV ELISA tests on 2010 and January 2012. Radiation
oncology service was consulted for further management.
The patient received three sessions of external bean radiotherapy initially, which were complicated with lymphedema and disease progression (3 new lesions: 6mm and 2mm on the left lateral foot and a 5mm left medial foot area). He also developed a
new lesion at dorsum of right foot. This area also received radiation therapy in two occasions. The clinical course complicated
with cellulitis at the left foot with purulent discharge for which
treatment with intravenous antibiotics was needed with subsequent clinical improvement. The patient remains on surveillance
and close follow up.
DISCUSSION
Figure 2: Left foot lesion at medial aspect and interdigits. Lesions are elevated approximately 3mm,
with well-defined borders
Kaposi sarcoma (KS) is a neoplasm with abnormal angiogenesis
that requires infection with a human herpes virus, HHV-8 (1).
This disease is most commonly in elderly men of Mediterranean
and Central European descent, but it can also occur in younger
individuals. It presents as purplish, reddish blue or dark brown/
black skin lesions (which can be macules, nodules, plaques) on
the lower extremities. It is a slow-growing tumor, localized and
indolent, but it can become disseminated and grow rapidly (2). The diagnosis is made through biopsy of the lesion(s). Involvement of mucous membranes, lymph nodes, and visceral organs
is rare. There are many treatments available but therapeutic
choices are often made based upon the experience and medical
discipline of the treating clinician and comorbid conditions (3). In our patient, diagnosis was made promptly due to the poor
response to antibiotics, and the timely biopsy performed by the
dermatology service. Observation rather than specific treatment for patients who have a limited number
of asymptomatic lesions that do not impair function is
the best approach in these cases (4).
Many treatments have been used to treat classic
KS, although no definitive cure is known. Surgery,
formerly recommended, is no longer indicated apart
from tissue analysis (5). Radiation therapy, chemotherapy or both, are preferred mode of treatment (5).
Radiation therapy is an important treatment, used for
many years in classic KS. Lesions of KS are highly
radiosensitive and the treatment is well tolerated and
temporarily controls large localized lesions (6).
For those with limited volume disease causing symptoms (for example, bleeding) or disfigurement; local
treatment is recommended. The choice of modality
(radiation therapy, excision, cryotherapy, laser ablation) depends on the site and extent of the disease
involvement as well as on patient preferences (7).
Systematic therapy should be considered in the following situations: diffuse symptomatic lesions on
multiple body parts that are not easily encompassed
in a limited number of radiation fields, diffuse involvement of a large portion of an extremity, bulky disease
in a localized area of one limb that cannot be encompassed within a single radiation therapy port, or
moderate associated lymphedema beyond what can
be controlled with elastic stockings (7).
A high index of suspicion should be present for those
lesions that do not respond to appropriate antibiotic therapy and further assessment is warranted in
these cases. Physicians should be aware of this rare
entity in immunocompetent patient.
1. Crosetti E, Succo G: Non-human immunodeficiency virus-related Kaposi’s sarcoma of the oropharynx: a case report and review of the literature, Journal of Medical Case Reports Dec 31,
2013;7:293.
2. Jan RA, Koul PA, Ahmed M, Shah S, Mufti SA, War FA: Kaposi
Sarcoma in a Non HIV Patient, International Journal of Health
Sciences Jul 2008; 2(2): 153–156.
3. Patel RM, Goldblum JR, Hsi ED: Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful in
the diagnosis of Kaposi sarcoma, Modern Pathology Apr 2004;
17(4): 456-460.
4. Moore PS, Chang Y: Kaposi’s sarcoma-associated herpes virus immune evasion and tumorigenesis: two sides of the same
coin?, Annu Rev Microbiol 2003; 57: 609-639.
7. Gottlieb JJ, Washenik K, Chachua A, Friedman-Kien A: Treatment of classic Kaposi’ sarcoma with liposomal encapsulated
doxorubicin, The Lancet Nov 8 1997;350(9088):1363-1364.
8. Phavixay L, Raynolds D, Simman R: Non-AIDS Kaposi’s Sarcoma Leading to Lower Extremity Wounds, Case Presentations
and Discussion, Journal of the American College of Clinical
Wound Specialists Jan 22 2013; 4(1):13–15.
RESUMEN
El sarcoma de Kaposi es un neoplasma maligno y raro que requiere infección con el virus del herpes humano para su desarrollo.
Presentamos el caso de un varón inmunocompetente evaluado debido a una celulitis
recurrente de la pierna. La celulitis eventualmente desarrollό una úlcera que no cicatrizό
y un nódulo palpable el cual requiriό una biopsia resultando en un diagnόstico de sarcoma de Kaposi tipo nodular.
Other local therapies that are useful for the management of symptomatic bulky KS lesions or for cosmetic reasons, but they do not prevent the development
of new lesions in untreated areas. These therapies
include: intralesional chemotherapy, topical alitretinoin, liposomal anthracyclines and taxanes (8).
REFERENCES
Figure 3: Surgical Pathology Diagnosis: spindle cells, high vascularity and intracellular hyaline globs and spaces, imparting a pattern that is strikingly reminiscent of a cavernous hemangioma consistent with nodular Kaposi’s sarcoma.
6. Marchell N, Alster TS: Successful treatment of cutaneous Kaposi’s sarcoma by the 585nm pulsed dye Laser, Dermatol Surg 1997;28(10):973-5.
5. Tur E, Brenner S, Landau M, Golan H: Treatment of Kaposi’s
sarcoma, Archives of Dermatology 1996;132:327-331.
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____de ____________________________de________
Giovanni Paraliticci, MDa
David Rodríguez-Quintana MDa
Ariel Dávila MDa
Antonio Otero-López MDa*
Department of Orthopedic Surgery, UPR School of Medicine, Medical Sciences Campus, San
Juan, Puerto Rico.
Corresponding author: Antonio Otero-Lopez MD - Department of Surgery-Orthopedics, UPR
Medical Sciences Campus, PO Box 365067, San Juan, PR 00936-5067. Email: [email protected]
a
B
INTRODUCTION
Bilateral femur neck fractures in premenopausal females or young adult males
occur in one of two scenarios, either high-energy trauma or secondary to metabolic disorders. Reports exist of two Japanese individuals with bilateral and
unilateral femoral neck fractures secondary to hypovitaminosis D (1,2). In
these cases both patients showed poor dietary habits and alcoholism. Most
reports in the literature of atraumatic bilateral femur fractures have documented causes of osteomalacia, including oncogenic osteomalacia, malabsorption, chronic steroid use, pregnancy, and alcoholism, among others (3-6).
Low levels of Vitamin D have been documented in normal healthy individuals
as well as in the orthopedic patient population seen on everyday orthopedic
practice (7). We present a young adult premenopausal female with atraumatic bilateral femoral neck fracture. This patient had all normal laboratory values
except for very low serum level of vitamin D3. Our patient was notified and
consented for presentation of her case in the literature.
Case History
Estudiante
Fecha de graduación
DIA
Bilateral atraumatic femur neck fractures in
young adult are very
rare. We report a young
premenopausal female
with osteomalacia secondary to vitamin D
deficiency and spontaneous bilateral femur
neck fractures. Patient
had no reported risk
factors for osteomalacia but hypovitaminosis
D was noted upon evaluation.
Osteomalacia
secondary to low serum levels of vitamin D
may lead to stress and
fragility fractures. Identification and treatment
of such patients may
decrease the incidence
of stress fractures and
possible complications.
Index words: atraumatic, bilateral, femoral,
neck, fracturas, premenopausal,
female,
hypovitaminosis
Email
Seguro Social # (opcional)
A TRAUMATIC
BILATERAL FEMORAL NECK
FRACTURES IN A HEALTHY
PREMENOPAUSAL FEMALE
WITH HYPOVITAMINOSIS D
ABSTRACT
FIRMA DEL SOLICITANTE
This is the case of a 46-year-old female patient with progressive bilateral hip
pain. Her hip symptoms began twenty-two days prior to presenting at our
clinic. She reported her bilateral hip pain as developing insidiously and associated with gradual bilateral knee pain. Symptoms did improve after a short
course of physical therapy and non-steroidal anti-inflammatory drugs. Given
some improvement in her pain she continued with her physical therapy regimen as directed by her primary physician who documented negative findings
on pelvis and bilateral knee radiographs. During this second stage of physical therapy she developed worsening bilateral thigh pain with radiation to both her gluteal and hip regions. At this time,
her hip pain was 7/10 on visual analogue scale with marked limitation of her
daily activities. At this time, her primary physician ordered further radiographic workup, which included radiographs, computerized tomography scan, and
pelvic magnetic resonance imaging. Imaging showed bilateral femoral neck
fractures and she was referred to our clinic for further evaluation and management.
The patient was 160 cm tall and her weight was 66
kg (body mass index; 25.9). She had a past medical history of hypothyroidism that was well controlled
with levothyroxine sodium 50 micrograms daily (TSH
– 2.04). She denied other medical conditions or medication treatments. She had no history of trauma, seizures, or bone metabolic diseases. Both her family
and dietary history were also unremarkable.
Upon arrival at our clinic patient had pain on inguinal
area upon ambulation and associated antalgic gait.
Both hips had decreased range of motion secondary to pain. Radiographs and CT-Scan showed possible stress fractures of bilateral femoral necks and
no looser’s zones on pubic rami or femoral cortex
(see Figures 1 & 2). Pelvic MRI did show evidence
of non-displaced acute bilateral femoral neck stress
fractures (see Figure 3). Dual-energy x-ray absorptiometry showed lumbar spine Z score -1.4, femoral
neck Z score 1.3, totals hip Z score -0.1. FRAX score
for major osteoporotic fracture and hip fracture were
2.6% and <0.1% respectively. Laboratory results displayed normal values of total
serum calcium (9.6 mg/dL; reference range, 9-10.5
mg/dL), serum phosphorus (3.7 mg/dL; reference
range, 3-4.5 mg/dL), and alkaline phosphatase (71
IU/L; reference range, 36-92 IU/L). Serum level of
1,25(OH)2vitamin D3 was decreased (18.3 pg/mL;
reference range, 15-80 pg/mL). The serum albumin
was normal (4.1 g/dL; reference range, 3.5-5.1 g/dL).
Her PTH level at most recent follow up was 23pq/ml
(reference range, 16-64pq/mL).
Given previous failure of conservative management
and symptoms of severe bilateral hip pain we recommended and internal fixation with two cannulated
hip screws for both hips (see Figures 4). Decision for
internal fixation versus arthroplasty was made given the non-displaced nature of the fractures. At the
time of surgery and given the possibility of osteomalacia a tricortical biopsy of the ilium was obtained.
It showed no histologic evidence of osteomalacia.
She was started on daily oral calcium and vitamin D
supplements (50,000 Units). Partial weight bearing
was allowed with assistive devices 60 days postoperatively. Complete weight bearing was not allowed
until 90 days postoperatively at which time patient
had complete radiographic healing apparent on radiographs. Follow up laboratories showed improvement in hypovitaminosis D (Vitamin D3 = 47.26 ng/
mL) following two months of oral supplements. Our
patient was able to return to her activities of daily living without assistance three months post operatively.
DISCUSSION
Vitamin D has received considerable attention in recent years due to studies demonstrating inadequate
levels in otherwise healthy populations (5,6). Recent
recommendations of the International Osteoporosis
Foundation and Osteoporosis Canada show that
optimum levels vitamin D should be around 30 ng/
ml (8,9). Vitamin D deficiency and osteomalacia are
reemerging as potential global conditions with the
prevalence of vitamin D deficiency being most severe in South Asia and the Middle East. In Puerto
Rico, a high prevalenc of hypovitaminosis D also exists (10).
Hypovitaminosis D may result in osteomalacia, a
metabolic bone condition resulting in weak demineralized bone. Long termed hypovitaminosis D may result in spontaneous fractures secondary to this poorly mineralized weakened bone. Multiple risk factors
for hypovitaminosis D exist such as: decrease sun
exposure, obesity, dietary deficiency, malabsorption,
medication-induced, breast-feeding, cholesterol-lowering agents, genetic disorder, anti-seizure medication, and hyperthyroidism. None of these risk factors
were identified in our patient.
Orthopedic surgeons and physicians in general
should be aware of these increasing trends in Vitamin D deficiency. Similar to the American Academy
of Orthopedic Surgeons program moving towards
better osteoporosis awareness and prevention of
fragility fracture, screening and early recognition of
high risk patients with vitamin D deficiency and osteomalacia can decrease the incidence of morbid hip
fractures.
Figure 1: Preoperative anteroposterior radiograph of the bilateral hip joints. There are no looser’s zones in the pubic rami or
stress fractures apparent in this examination.
In the present case, the patient had no history of major trauma or any known risk factors for osteomalacia. Her presenting symptoms of insidious atraumatic
bilateral thigh/inguinal pain imply a fragility fracture
secondary to possible bone mineral deficiency. Laboratory data did not demonstrate any abnormality in
the serum levels of calcium, phosphorus, or bone alkaline phosphatase, but she did have a low serum
level of 1,25 (OH)2vitamin D3. Moreover, radiographs
did not show typical features of osteomalacia such as
Looser's zone, but addition of CT-scanning did show
sclerotic vertical lines on both femoral necks implying
healing stress fractures. Given normal dietary and
other lifestyle habits the cause of the low serum level
of vitamin D in this case is still not clear.
To our knowledge, a previous report exists in the literature of bilateral femur fractures in a patient with
low levels of vitamin D. This report was in an Asian
patient with dietary deficiencies and alcoholism (1).
Our patient underwent bilateral internal fixation with
cannulated screws given the early recognition and
non-displaced fracture pattern. Further delay in treatment may have led to displacement of the fractures
and hip replacement surgery. With this, we think that
prevention and early diagnosis seem of paramount
importance to prevent occurrence and displacement
of hip stress fractures on patients with osteomalacia.
The increasing incidence of hypovitaminosis D in
the general population will lead to an increase in reported incidence of fragility fractures. Femoral neck
fractures, as in this case, require early diagnosis to
Figure 2: Preoperative coronal (a) and sagittal (b) tomographic images showing bilateral femoral necks with vertical sclerotic
lesions. Noted how lesions are on tension side of the bone.
prevent morbidities associated with fragility fractures. Given the previously reported elevated rate of undiagnosed hypovitaminosis D, preventive medicine with
screening and oral vitamin D supplementation should be implemented by primary
care physicians and orthopedic specialists.
REFERENCES
Figure 3: Preoperative T1 (a) and T2 Fat Suppression (b) magnetic resonance
imaging (MRI) showing bilateral femoral neck fractures.
1. Nagao S1, Ito K, Nakamura I. Spontaneous bilateral femoral neck fractures associated with a low
serum level of vitamin D in a young adult. J Arthroplasty. 2009 Feb; 24(2):322.e1-4.
2. Ohishi H1, Nakamura Y, Kishiya M, Toh S. Spontaneous femoral neck fracture associated with a
low serum level of vitamin D. J Orthop Sci. 2013
May; 18(3):496-9.
3. Rajeev A. Bilateral spontaneous inter-trochanteric fractures of proximal femurs. Int J Surg Case
Rep. 2014; 5(5):246-8.
4. Baki ME1, Uygun H, Arı B, Aydın H. Bilateral
femoral neck insufficiency fractures in pregnancy.
Eklem Hastalik Cerrahisi. 2014
5. Carter T1, Nutt J, Simons A. Bilateral femoral
neck insufficiency fractures secondary to vitamin
D deficiency and concurrent corticosteroid use--a
case report. Arch Osteoporos. 2014;9:172
6. Sivas F1, Günesen O, Ozoran K, Alemdaroğlu E.
Osteomalacia from Mg-containing antacid: a case
report of bilateral hip fracture. Rheumatol Int. 2007
May;27(7):679-81
7. Patton CM1, Powell AP, Patel AA. Vitamin D in
orthopedics. J Am Acad Orthop Surg. 2012 Mar;
20(3):123-9.
8. Hanley DA, Cranney A, Jones G, Whiting SJ, Leslie WD, Guidelines Committee of the
Scientific Advisory Council of Osteoporosis Canada: Vitamin D in adult health and disease: A review
and guideline statement from Osteoporosis Canada (summary). CMAJ 2010; 182():1315-1319.
9. Dawson-Hughes B, Mithal A, Bonjour J-P, et
al: IOF position statement: Vitamin D recommendations for older adults. Osteoporos Int 2010;
21():1151-1154. 10. Caro Y, Negron V, Palacios C. Association between Vitamin D Levels and Blood Pressure in a
Group of Puerto Ricans. PR Health Sci J.Sep 2012;
31(3):123-129.
RESUMEN
Las fracturas bilaterales del cuello femoral
en adultos jóvenes son raras bajo circunstancias no-traumáticas. En este reporte
presentamos un paciente con osteomalacia sin factores de riesgo por historial,
y fracturas bilaterales de cuello femoral.
En nuestra evaluación preoperatoria se
identificaron niveles bajos de vitamina D.
Osteomalacia secundaria a bajos niveles
de vitamina D puede llevar a fracturas de
estrés o fragilidad. El tratamiento de esta
condición debe ser preventivo. La identificación con tratamiento temprano de paciFigure 4: Postoperative radiograph showing bilateral cannulated screw fixation entes a riesgo puede prevenir complicaciones mayores asociadas con fracturas
of femoral neck fractures.
de estrés en la cadera.
RESUMEN
El síndrome de Lyell o necrólisis toxica
epidérmica es una entidad infrecuente
con fuerte resorte inmunológico. Es
una reacción de hipersensibilidad específica secundaria al uso de drogas
y otros factores desencadenantes.
Caracterizan el cuadro clínico las lesiones de todas las capas de la piel,
maculo-ampollares y descamativas de
amplia distribución y progresión. En
ocasiones genera gran compromiso
sistémico y disfunción orgánica múltiple potencialmente fatal. Su tratamiento se basa en tres pilares fundamentales: identificación y suspensión del o
los agentes desencadenantes, sostén
clínico general y curación de lesiones
dérmicas. La terapia inmunológica es
controversial y no sustentada en evidencia científica sólida. Presentamos
un caso clínico que resume todos los
aspectos de esta patología potencialmente letal.
SINDROME DE LYELL
POTENCIALMENTE
FATAL
Daniel Agustín Godoy MDab*
Erica Alvareza
Andrea Amigota
Mario Di Napoli MDcd
Luca Massotie
a
Unidad de Cuidados Neurointensivos, Sanatorio Pasteur, Catamarca,
Argentina.
b
Unidad de Terapia Intensiva, Hospital San Juan Bautista, Catamarca,
Argentina.
c
Servicio de Neurologia, Hospital General San Camillo de’ Lellis, Rieti,
Italia.
d
Seccion Neurologia, SMDN—Centro de Medicina Cardiovascular y Centro de Prevencion de Enfermedades Cerebrovasculares, Sulmona,
L’Aquila, Italia.
e
Medicina Interna, Hospital de Cecina, Italia.
*Correspondencia: Daniel Agustín Godoy MD - Unidad de Cuidados Neurointensivos, Sanatorio Pasteur, Chacabuco 675, 4700. Catamarca, Argentina. E-mail: [email protected]
Palabras indices: sindrome, Lyell, fatal
INTRODUCCION
El síndrome de Lyell o necrólisis epidérmica tóxica
(NET) es una reacción adversa grave a distintos
fármacos que afecta principalmente la piel y mucosas1-4. A pesar de ser una entidad poco frecuente,
(incidencia anual estimada en 1 o 2 casos por millón
de habitantes), es una verdadera emergencia médica, potencialmente fatal, ya que en ocasiones genera intensa respuesta inflamatoria sistémica con disfunción orgánica múltiple1-4. Caracterizan el cuadro
clínico las lesiones ampollares con marcado eritema
y erosiones hemorrágicas, conjuntamente con amplias zonas de piel desnudada por el desprendimiento epidérmico1-4. Si bien las drogas son identificadas como el principal factor desencadenante, puede
ocurrir como consecuencia de infecciones por VIH,
herpes simple o micoplasma pneumoniae1-4. El diagnóstico se basa en la clínica y se certifica por biopsia de piel la que evidencia necrosis epidérmica
completa1-4. El diagnóstico diferencial incluye entre
otros a la dermatosis por depósito lineal de IgA, pénfigo, penfigoide ampollar, y el síndrome de piel escaldada secundaria a infección por estafilococo1-4.
El pronóstico puede establecerse empleando la escala SCORTEN5 y su terapéutica radica en la identificación e interrupción del fármaco responsable,
soporte general en una unidad de cuidados intensivos y la utilización aunque controversial de agentes inmunomoduladores como las inmunoglobulinas
(Igb)1, 2, 6. La mortalidad oscila entre 25-35 %, pudiendo ser mayor en individuos añosos con áreas
extensas de desprendimiento epidérmico7. Más del
50 % de los sobrevivientes presentaran secuelas a
largo plazo8-11.
Caso clínico
Paciente del sexo femenino de 47 años de edad,
con antecedentes de hipotiroidismo por lo cual se
encontraba medicada con levotiroxina 125mcg/día
y vitíligo. Dos meses antes de su ingreso presenta
intensas cefaleas que no cedían con antiinflamatorios no esteroides y un episodio convulsivo tónico
clónico generalizado, razón por la cual se le efectuó
RMN con contraste, evidenciándose lesión expansiva en región temporoparietal derecha (ver Figura
1). Inicio tratamiento médico, con deltisona 30 mg/
día y lamotrigina 200 mg/día y se planifica cirugía.
Dos semanas más tarde nota la aparición de maculas eritematosas que no desaparecían con la digito presión, localizadas principalmente en el rostro,
cuello y miembros superiores, las que fueron progresando hacia la región anterior del tórax, espalda y
raíz de miembros inferiores, tornándose confluentes
y pruriginosas (ver Figura 2 A y B). Completan el
cuadro clínico odinofagia, conjuntivitis (C), síndrome
obstructivo laríngeo alto, candidiasis bucofaríngea
Figura 1. Cortes axial (A) y sagital (B) de la lesión ocupante de espacio que motivo el inicio de profilaxis
convulsiva con lamotrigina.
A
(D), síndrome de respuesta inflamatoria sistémica
(frecuencia cardiaca y respiratoria, 137 y 33 respectivamente, 38.3°C de temperatura rectal y 14700
blancos sin desviación a izquierda) shock (Tensión
arterial sistólica 76 mmHg, oliguria, mala perfusión
periférica, acidosis metabólica: pH 7.31; Bicarbonato
15 mEq/l) e injuria renal aguda (creatinina 2.9 mg/
dl, uremia 78 mg/dl). La analítica química evidencio
las anomalías mencionadas; los parámetros de coagulación, función hepática, electrolitos, glucemia y
niveles de hemoglobina estaban dentro de límites
normales. Destaca Proteína C reactiva ultrasensible
de 353. Perfil tiroideo: TSH 0,01 uUI/mL (0,3~3,0);
T4 libre 8,0 ug/dl (4,5- 12,5) y anticuerpos antitiroglobulina < 15,0 UI/ml (hasta 60.0). Dopaje de IgA
80 mg/dl (VN 70.0-400-0) y perfil Inmunológico negativo (anticuerpos antinucleares, anti DNA nativo,
anti SM, ANCA S Y P, anti RNP, anti RO y anti LA).
Complemento sérico C3: 121,6 mg/dl (84-193) y C4:
24,5 mg/dl (23-39). Proteinograma electroforético:
proteínas totales: 4,92 g/dl ( 6,40 - 8,40); albumina
: 2,83 g/dl (3,60-5,20); globulinas totales: 2,09 g/dl
( 2,2-4,0); alfa 1 globulinas : 0.18 g/dl ( 0,17-0,33);
alfa 2 globulinas : 0,54 g/dl ( 0,53-0,75); beta globulinas: 0,72 g/dl ( 0,51-0,91); gama globulinas : 0,64
g/dl (0,84-1-68): relación albumina/globulinas: 1,35 (
1,2-2,0)
Las lesiones dérmicas se tornaron dolorosas, impetiginizadas y descamativas en colgajos. Estaba
comprometido el 45% de la superficie corporal con
5 puntos en la escala SCORTEN. Hemocultivos y
cultivos de lesiones de piel no desarrollaron microorganismos de ninguna especie.
El diagnóstico del síndrome de Lyell fue confirmado
por biopsia de piel con inmunofluorescencia y serología para descartar lupus eritematoso en su variedad bullosa y dermatosis lineal por IgA.
El tratamiento consistió en apoyo general de todos
los parénquimas, fluidoterapia, vasopresores (noradrenalina 0.2 mcg/kg/minuto) durante 3 días, hidrocortisona para control de estridor laríngeo y como
coadyuvante de la terapéutica del shock, profilaxis
de sangrado gástrico y la trombosis venosa profunda
con ranitidina y enoxaparina respectivamente, suspensión de lamotrigina y rotación a levetiracetam, nistatina tópica bucal, alimentación enteral por sonda asoyeyunal por 3 semanas, inmunoglobulinas IV y
curación diaria oclusiva de lesiones dérmicas. Evolución favorable, con recuperación ad integrum a las
6 semanas de iniciado el cuadro.
DISCUSION
Figura 2. Lesiones características de necrosis epidérmica toxica y su localizacion (A): cara, cuello y región
anterior del tórax; (B): región dorsal; (C) conjuntivitis asociada; (D): compromiso bucofaríngeo. La paciente brindo su consentimiento informado por escrito, según las normativas vigentes (www.icmje.org y las
Instrucciones a los Autores), disponiéndose del mismo para quien lo requiera.
En 1956, Alan Lyell describió las lesiones dérmicas
muy similares a quemaduras profundas en jóvenes
que no habían padecido tal injuria12. Años más tarde se establecería la relación entre el síndrome descripto y la reacción adversa a drogas entre las que
se encuentran principalmente anticonvulsivantes
(carbamazepina, fenitoina, ácido valproico y lamotrigina como el caso que presentamos); antibióticos
(sulfonamidas, quinolonas), allopurinol y dipirona1-4,
7, 13.
La entidad que describimos es muy poco frecuente,
posiblemente asociada a cierta susceptibilidad
genética y determinados factores que pueden contribuir a su desarrollo, entre los que se destacan
además de las drogas, infecciones virales, cáncer y
radioterapia1-4,7.
En su patogenia se destaca un fuerte componente
inmunológico fundamentado en la recurrencia del
síndrome luego de nueva exposición al agente desencadenante1-4,7,13. La evidencia señala que es
una reacción de hipersensibilidad específica dirigida
contra los linfocitos B y keratinocitos del individuo
mediada por linfocitos T y moléculas citolíticas como
FaSL y granulolisina1-4, 14-16.
Lamotrigina es un nuevo fármaco utilizado para controlar episodios convulsivos parciales o generalizados17-19. De buena absorción oral, su metabolismo
es hepático. Efectos adversos comunes incluyen:
cefaleas, nauseas, vómitos, temblores y ansiedad e
insomnio. En 3-10% de los casos desencadena entre las dos y 8 semanas de su ingesta inicial, rash
cutáneo simple o exantemas maculopapulares que
desaparecen rápidamente al suspender la droga y
sin mayores consecuencias17-19.
Entre 1 y 10 por cada 10000 individuos que están
bajo tratamiento con lamotrigina se desencadenan
reacciones cutáneas severas entre las que se encuentran el síndrome de Lyell o necrosis epidérmica
toxica y el síndrome de Steven Johnson19. Entre los
factores detectados que contribuyen a la aparición
del síndrome se encuentran: altas dosis de inicio o
incremento rápido de las mismas como en nuestro
caso; historia previa de reacciones a otras drogas,
administración concomitante de otro anticonvulsivante y por razones probablemente hormonales el
sexo femenino4, 17-19.
Las guías internacionales de prescripción de anticonvulsivantes sugieren iniciar lamotrigina a 12.5
mg diarios durante 2 semanas, seguido por 25 mg
al día durante 2 semanas más, incrementando luego
25 mg semanales hasta lograr la dosis de mantenimiento diaria de entre 100-200 mg4.
Las lesiones dérmicas maculo eritematosas, ampollares y descamativas tienen morfología, localización,
distribución y forma de progresar características,
aparecen luego de un periodo de latencia variable
que promedia las 2 semanas luego de la exposición
al agente desencadenante y están precedidas por
un cuadro clínico similar al síndrome gripal1-4. En
ocasiones presentan una reacción inflamatoria
sistémica muy intensa con disfunción multiorganica
no asociada a sepsis y potencialmente letal como
16. Nassif A, Moslehi H, Le
Gouvello S, Bagot M, Lyonnet L,
Michel L, Boumsell L, Bensussan A, Roujeau JC: Evaluation
of the potential role of cytokines
in toxic epidermal necrolysis. J
Invest Dermatol 2004, 123:850855.
17. Schlienger RG, Shapiro LE,
Shear NH. Lamotrigine-induced
severe cutaneous adverse reactions. Epilepsia. 1998;39 Suppl
7: S22-6.
18. Mockenhaupt M, Messenheimer J, Tennis P, Schlingmann
J. Risk of Stevens-Johnson
syndrome and toxic epidermal
necrolysis in new users of antiepileptics. Neurology. 2005; 64:
1134-8.
19. Guberman AH, Besag
FM, Brodie MJ, Dooley JM,
Duchowny MS, Pellock JM, et
al. Lamotrigine-associated rash:
Risk/benefit considerations in
adults and children. Epilepsia.
1999; 40: 985–91.
20. Bastuji-Garin S, Rzany B,
Stern RS, Shear NH, Naldi L,
Roujeau JC: Clinical classification of cases of toxic epidermal
necrolysis,
Stevens-Johnson
syndrome, and erythema multiforme. Arch Dermatol 1993,
129:92-96.
la paciente que presentamos (compromiso hemodinámico, renal), la cual presento 5 puntos en la escala pronostica de SCORTEN5, con una mortalidad
predicha del 90%1-5 (ver Tabla 1).
En nuestra paciente con un fuerte precedente inmunológico (hipotiroidismo, vitíligo), el diagnóstico diferencial debe establecerse fundamentalmente con
el síndrome de Steven-Johnson20 (ver Tabla 2), y
otras entidades autoinmunes como el pénfigo en
todas sus variantes, lupus bulloso y dermatosis por
deposito lineal de IgA, las cuales fueron descartadas
por biopsia y serología1-4.
El tratamiento se basa en la identificación y suspensión del agente desencadenante, en este caso lamotrigina, sostén de órganos vitales, curación periódica de las lesiones dérmicas y si bien controvertido,
en nuestro casos dada la gravedad y alto riesgo de
mortalidad predicha por SCORTEN5, decidimos utilizar inmunoterapia con Igb IV a razón de 400 mg/
día durante 4 dias1-4, 6. La utilización de Igb se fundamenta en su capacidad de bloquear receptores
Fas en los keratinocitos previniendo apoptosis de
los mismos.
En conclusión, el caso clínico reportado cubre todos
los aspectos (diagnostico, pronostico y terapéutico)
de esta infrecuente pero posible letal complicación
secundaria al uso de un anticonvulsivante de incrementada utilización en los últimos años18.
REFERENCIAS
1. Harr T, French LE. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Chem Immunol Allergy. 2012; 97: 149–166.
2. Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet Journal of Rare Diseases
2010; 5: 39. doi:10.1186/1750-1172-5-39.
3. Rzany B, Correia O, Kelly JP, Naldi L, Auquier A, Stern R: Risk
of Stevens-Johnson syndrome and toxic epidermal necrolysis
during first weeks of antiepileptic therapy: a case-control study.
Study Group of the International Case Control Study on Severe
Cutaneous Adverse Reactions. Lancet 1999; 353: 2190-2194.
4. Kaur S, Dogra A. Toxic epidermal necrolysis due to concomitant use of lamotrigine and valproic acid. Indian J Dermatol.
2013; 58: 406. doi: 10.4103/0019-5154.117319.
5. Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz
J, Wolkenstein P: SCORTEN: a severity-of-illness score for toxic
epidermal necrolysis.
J Invest Dermatol 2000; 115:149-153.
6. Wootton CI, Patel AN, Williams HC. In a patient with toxic epidermal necrolysis, does intravenous immunoglobulin improve
survival compared with supportive care? Arch Dermatol 2011;
147: 1437-40.
7. Roujeau JC, Stern RS: Severe adverse cutaneous reactions
to drugs. N Engl J Med 1994; 331:1272-1285.
8. Sheridan RL, Schulz JT, Ryan CM, Schnitzer JJ, Lawlor D,
Driscoll DN, Donelan MB, Tompkins RG: Long-term consequences of toxic epidermal necrolysis in children. Pediatrics
2002; 109:74-78.
9. Herman TE, Kushner DC, Cleveland RH: Esophageal stricture
secondary to drug-induced toxic epidermal necrolysis. Pediatr
Radiol 1984; 14:439-440.
10. Barrera JE, Meyers AD, Hartford EC: Hypopharyngeal stenosis and dysphagia complicating toxic epidermal necrolysis. Arch
Otolaryngol Head Neck Surg 1998; 124:1375-1376.
11. Magina S, Lisboa C, Leal V, Palmares J, Mesquita-Guimaraes J: Dermatological and ophthalmological sequels in toxic
epidermal necrolysis. Dermatology 2003, 207:33-36.
12. Lyell A: Toxic epidermal necrolysis: an eruption resembling
scalding of the skin.
Br J Dermatol 1956, 68:355-361.
13. Mockenhaupt M, Viboud C, Dunant A, Naldi L, Halevy S,
Bouwes Bavinck JN, Sidoroff A, Schneck J, Roujeau JC, Flahault
A: Stevens-Johnson syndrome and toxic epidermal necrolysis: assessment of medication risks with emphasis on recently
marketed drugs. The EuroSCAR-study. J Invest Dermatol 2008,
128:35-44.
14. Chung WH, Hung SI, Yang JY, Su SC, Huang SP, Wei CY,
Chin SW, Chiou CC, Chu SC, Ho HC, et al.: Granulysin is a key
mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med 2008,
14:1343-1350.
15. Nassif A, Bensussan A, Boumsell L, Deniaud A, Moslehi H,
Wolkenstein P, Bagot M, Roujeau JC: Toxic epidermal necrolysis: effector cells are drug-specific cytotoxic T cells. J Allergy Clin
Immunol 2004, 114:1209-1215.
ABSTRACT
Toxic epidermal necrolysis or Lyell’s syndrome is a rare entity with strong compromise of immune system. It is a hypersensitivity reaction secondary to the use of specific
drugs and other triggers. Erythematous lesions of bullous aspect characterize the clinical picture affecting all layers of the skin,
scaly and widespread in distribution and
progression. Sometimes life threatening,
generate a severe inflammatory and systemic reaction with multiple organ dysfunction.
Treatment encompasses three objectives:
identification and suspension of triggers,
general clinical support and therapy to the
skin lesions. Immune therapy is controversial and not supported by solid scientific evidence. We report a case that summarizes all
aspects of this potentially lethal disease.
Review Articles/Artículos de Reseña
HEMATOMA SUBDURAL
CRÓNICO:
Fisiopatología y Manejo
Quirúrgico
Hernando Raphael Alvis-Mirandaa
Gabriel Alcalá-Cerraa
Andrés M. Rubianob
Luis Rafael Moscote-Salazara*
Departamento de Neurocirugía, Universidad de Cartagena, Colombia.
Departamento de Neurocirugía, Hospital Universitario de Neiva, Fundación
MEDITECH, Colombia.
*Correspondencia: Dr. Luis Rafael Moscote-Salazar - Universidad de Cartagena, Cartagena de Indias, Colombia. E-mail: [email protected]
a
b
INTRODUCCIÓN
El hematoma subdural crónico (HSC) fue descrito por primera
vez por J. J. Wepfer en 1658,(1) popularizándose tras tomar relevancia en la novela “Pierette” de Honore de Balzac en 1840,
quien describió su origen traumático y tratamiento quirúrgico,(2)
aun cuando se consideraba que era resultado de un proceso inflamatorio, que de hecho Virchow acuño con el nombre de “paquimeningitis hemorrágica interna” en 1857 (3,4). Totter en 1914 fue
quien estableció un vínculo pleno sobre el origen traumático de
los HSC.(1) El HSC es una colección hemática encapsulada,
situada por debajo de la duramadre, caracterizado fundamentalmente por la presencia de una membrana. Generalmente ocurre
en el adulto mayor (>65 años), y es tendencia su consideración
como un evento centinela (5).
El proceso de envejecimiento trae consigo una serie de repercusiones en todas las esferas del individuo (física, mental, social y familiar). A nivel del sistema nervioso se producen cambios
estructurales y neuroquímicos como disminución de circunvoluciones, disminución del tamaño de los ventrículos, disminución
de las neuronas y de neurotransmisores. Traduciéndose estos
cambios en modificaciones a nivel sensitivo, motores, perceptivos, memoria, sueño, que hacen al adulto mayor más propenso
a sufrir accidentes y manifestaciones de patologías diferente en
comparación con otras etapas de la vida.
Para el neurocirujano es un problema de manejo diario y de resolución quirúrgica relativamente sencilla en la mayoría de los
casos. Pero bajo su aparente “benignidad”, la mortalidad en pacientes con HSC es, en general, de entre 0,5% y 8%, con algunos
reportes que sobrepasan al 10% (6). La mejor comprensión de
la fisiopatología de esta entidad, la identificación de factores de
riesgo, el avance en los medios diagnósticos y el progreso en las
opciones terapéuticas han mejorado notablemente el pronóstico.
Ofrecer al lector una rápida revisión acerca de los nuevos pormenores del HSC es el objetivo de este trabajo.
RESUMEN
El hematoma subdural crónico es
una colección hemática encapsulada, situada por debajo de la duramadre, caracterizado fundamentalmente por la presencia de una
membrana. Generalmente ocurre
en el adulto mayor y es tendencia
su consideración como un evento centinela. La tomografía axial
computarizada simple de cerebro
es la ayuda imagenelógica actual
de elección para el diagnóstico de
esta entidad. Debe ser sospechada
especialmente en pacientes que
cursan con síndrome demencial
aunque sean de edad avanzada.
Aunque menos frecuente, debe
investigarse ante un paciente que
presente déficit neurológico transitorio. Aún no existe consenso con
respecto a la modalidad quirúrgica
ideal. El procedimiento que parece
más recomendado es el drenaje
aspirativo cerrado por orificios de
trépano. Se ha debatido si el lavado intraoperatorio de la colección
mejora los resultados.
Palabras índices: hematoma, subdural,
crónico,
fisiopatología,
manejo.
EPIDEMIOLOGÍA
La incidencia es de 1-3 casos por 100.000
habitantes por año, aumentando en los
mayores de 70 años a 7/100.000 habitantes (7–10). El 83% se presenta en pacientes mayores de 40 años. De acuerdo
con Karibe y cols (6), existe un incremento marcado en la incidencia del HSC, no
solo por la población en envejecimiento,
sino también por las tendencias médicas
(ejemplo, hemodiálisis, anticoagulación
y/o terapia antiplaquetaria).
La edad, acarrea factores de riesgo predisponentes, relacionados con la mayor
torpeza motriz, trastornos de la marcha,
extrapiramidales, aumento de la fragilidad capilar, sin olvidar, por supuesto la
importancia de la atrofia cerebral propia
del envejecimiento que permite un mayor
efecto de los traumas por aceleración y
desaceleración sobre las venas puentes
parasagitales que ocupan el espacio
subdural (8). Se ha señalado predominio
del sexo masculino, sin una explicación clara de esta
relación (11). A pesar de ser fácilmente tratable con
cirugía, el HSC tiene una alta tasa de recurrencia,
entre el 5% y el 30% (12,13).
ETIOLOGÍA
Diversos factores se han mencionado como causantes, siendo la causa más común el trauma craneoencefálico (14), alcoholismo crónico y la anticoagulación. En general, podemos establecer dos grupos
de pacientes en función de la edad, menores de 65
años, y mayores de esa edad, representando la distribución etaria en 20% y el 80%, respectivamente.
En los mayores de 65 años, el antecedente de TEC
es muy frecuente, cobran importancia otras comorbilidades como diabetes mellitus, enfermedad renal
en estadio final, hipertensión arterial, enfermedad
cerebrovascular, enfermedades neurodegenerativas, en terapia anticoagulante o antiplaquetaria; estas últimas son encontradas con mucha menor frecuencia en aquellos pacientes menores de 65 años,
sin embargo, se debe considerar que estos se ven
involucrados en eventos traumáticos violentos, más
que eventos triviales, por lo que es de esperar una
presentación clínica diferente en ambos grupos (15).
Se ha sugerido que el consumo de alcohol en forma
crónica a través de atrofia cortical, coagulopatía secundaria al daño hepático crónico y más probablemente por riesgo de trauma craneano favorecería la
lesión. En la literatura está presente entre 10 y 50%
de los casos. Otras causas descritas son los tumores
primarios, metástasis o malformaciones vasculares,
entre otras.
FISIOPATOLOGÍA
Para el desarrollo de los HSC se han propuesto dos
teorías, la teoría del gradiente osmótico, y la teoría
de la hemorragia recurrente proveniente de la cápsula del hematoma asociada con hiperfibrinólisis,
siendo esta última mayormente aceptada (16). Morfológicamente el HSC se caracteriza por presentar
una membrana interna avascular rica en colágeno
(17), y otra neomembrana externa con frágiles macrocapilares con un gran lumen, por lo que se les conoce como vasos sinusoidales, pericitos y músculo
liso distribuido irregularmente y células inflamatorias
(18,19), todo producto de una estrecha correlación
entre angiogénesis, quemotaxis inflamatoria y estimulación celular, en los que han sido vinculados múltiples factores como el Factor de crecimiento vascular
endotelial (VEGF, por sus siglas en inglés), y recientemente el Factor de Crecimiento Placentario (PlGF,
por sus siglas en inglés) (20).
Los vasos sinusoidales a menudo son fuente de sangrado multifocal, siendo este considerado como factor causal del aumento progresivo del hematoma. Se
considera que los HSC tienen incrementada su actividad fibrinolítica, los productos de la degradación
de la fibrina poseen propiedades anticoagulantes y
vasodilatadoras, llevando así a la construcción de la
teoría de Ito (21), en la que se sustenta hiperfibrinolisis local seguida de pérdida del poder coagulante
en el fluido del HSC. Además de la hiperfibrinolisis,
la cavidad del hematoma puede estar sometida a las
pulsaciones cerebrales continuas y cambios en la
posición de la cabeza (22), es así como esta transmisión de pulsaciones puede comprimir y descomprimir los vasos sinusoidales.
Los HSC tienen una ubicación relativamente local,
siendo su tamaño el principal factor en el desplazamiento cerebral. El flujo sanguíneo cerebral local
disminuye en la medida que incremente el grosor del
hematoma o con la severidad del desplazamiento cerebral, este último se correlaciona con alteraciones
del flujo sanguíneo local a estructuras profundas,
como el tálamo ipsilateral (23).
Se atribuye generalmente la presencia de hemiparesia a la reducción del flujo sanguíneo cerebral local
en la corteza rolándica (24), o a estructuras profundas (25), sin embargo se ha demostrado compromiso del flujo sanguíneo en la corteza y al tálamo
ipsilateral.
El desplazamiento de estructuras superficiales se
relaciona con el de estructuras profundas cuando
el hematoma supera la compensación espacial, por
lo que ciertos autores atribuyen la hemiparesia a la
reducción del flujo sanguíneo en el tracto piramidal
ipsilateral, incluyendo la corteza frontal y estructuras
subcorticales (26).
La presencia de cambios cognitivos, se asocia a
la reducción bilateral del flujo sanguíneo al tálamo,
este hecho guarda relación con el desplazamiento
y disfunción de la porción superior del tallo cerebral
(26). Sin embargo, Slotty y cols (27), evaluaron la
alteración de la perfusión cerebral en individuos con
HSC, mediante TAC de perfusión, encontraron que
la autorregulación de la perfusión cerebral local está
activa en el área cortical subyacente al HSC; tanto
el volumen como el flujo sanguíneo cerebral están
regulados al alta de manera significativa, concluyen
en su trabajo que la autorregulación cerebral está intacta en estos individuos, por lo que sugieren que los
déficits neurológicos obedecen muy probablemente
a una perfusión limítrofe.
La lateralidad izquierda de los HSC es un hecho reportado con frecuencia en la literatura médica, si corresponden a un proceso biológico, o a un infradiagnóstico de HSC derechos es poco claro, sin embargo
se debe considerar el hecho que HSC izquierdos se
reportan predominantemente con alteraciones de
la comunicación (afasia, disfasia, alexia, dislexia),
y/o pérdida de las funciones de la mano dominante,
que son percibidas por el paciente, su familia, y el
personal de salud. Las lesiones derechas, es decir,
no dominantes, muy infrecuentemente se asocian
con tales alteraciones, o lo hacen de forma sutil con
alteraciones en la atención, dispraxia geográfica o
del vestir, por lo que pueden pasarse por alto o subdiagnosticarse (28). Se ha observado que los HSC
bilaterales son más comunes en pacientes con cráneos asimétricos en comparación con individuos de
cráneo simétrico (41.7% vs 17.7%, p=0.01) (29).
Algunos autores manifiestan la importancia de evaluar la disfunción hipotálamo-hipofisaria en pacientes con HSC, dada la gran susceptibilidad al daño
del hipotálamo y de la hipófisis, especialmente por
las observaciones previamente realizadas en TEC y
Hemorragia subaracnoidea (HSA) (30).
PRESENTACIÓN CLÍNICA
El crecimiento progresivo del HSC se correlaciona
con una presentación clínica tardía y su coincidencia
en edades avanzadas, ya que existe una adaptación
encefálica, por una reducción en su peso de aproximadamente 200 g, entre los 40 y 60 años; de esta
manera aumenta el espacio extracerebral entre un
6 y un 11%, y deja así crecer a dicha colección subdural hasta crear problemas de espacio. El cuadro
clínico de presentación en el adulto mayor es variado. En 20 a 50% se ha descrito síndrome demencial,
que puede ser confundida con demencia propia del
paciente añoso. Por lo que se recomienda la investigación a través de tomografía axial computarizada
(TAC) cerebral a todo paciente con trastorno cualitativo de conciencia, no importando que sean de edad
avanzada. Puede presentarse también como un
déficit neurológico transitorio, cefalea, alteraciones
del lenguaje, hemiparesia, crisis convulsiva, etc. Típicamente, el HSC se presenta en individuos mayores de 60 años, hombres, alcohólicos o con discrasias sanguíneas y caídas frecuentes, que consultan
por un cuadro clínico progresivo caracterizado por
un síndrome motor, que agrega en la evolución signos y síntomas de hipertensión endocraneana.
Algunos ejemplos de manifestaciones inusuales incluye paraparesia intermitente (34), o cuadriparesia
(35).
DIAGNÓSTICO
Es extremadamente importante establecer un diagnóstico preciso, dado que puede interpretarse
como eventos isquémicos y tratarse como tal (antiagregantes plaquetarios o anticoagulación oral) lo
cual es absolutamente riesgoso. La tomografía axial
computarizada (TAC) simple de cerebro es la ayuda
imagenelógica actual de elección para el diagnóstico de esta entidad. El rendimiento de la resonancia
magnética no justifica su uso rutinario. Sin embargo
la IRM por su alto potencial para resolución, puede
ser ventajosa en el paciente joven en los que se halla
capas delgadas de vasos subdurales, no detectables
con TAC, especialmente en la fase isodensa. La IRM
ofrece una mejor visualización, localización y evaluación del efecto de masa que compromete estructuras adyacentes (31). Se ha demostrado que los
hallazgos por TAC o por IRM no datan con precisión
los HSC (37). La TAC puede mostrar una extensa
colección hipodensa extraaxial, con forma de media
luna, que puede borrar los surcos corticales y causar
desplazamiento de la línea media. Si hay sangrado
de repetición, puede haber densidad heterogénea,
indicativo de ambos componentes agudos y crónicos. Cuando la lesión es isodensa con el tejido cerebral adyacente, diagnóstico radiográfico puede ser
difícil. En las IRM, los HSC son hipointensos en T1
y 12 imágenes. En contraste, los hematomas hiperagudos son isodensos en T1 e hiperintensos en el
T2. Los hematomas agudos son isointensos en T1 e
hipointensos en T2. Los hematomas subagudos tardíos son hiperintensos en T1 e hiperintensa en T2.
TRATAMIENTO
hematoma probablemente conlleva un mejor pronóstico, corroborado por los hallazgos de la revisión de Liu
y cols (40), quienes demuestran el beneficio del drenaje postquirúrgico tras el tratamiento del HSC. Usar
irrigación parece ser benéfico, mientras que tanto el
“twist drill” como la opción “bur hole” son opciones
igualmente confiables. La inserción de un drenaje
subdural se asocia con una reducción significativa de
la recurrencia sintomática y en la necesidad de intervenciones quirúrgicas adicionales; además se asocia
a grandes mejoras en el pronóstico funcional, tanto
a corto como a largo plazo (41). Almenawer y cols
(42), compararon la eficacia y seguridad de múltiples
modalidades de tratamiento del HSC, no encontraron
diferencia significativa en términos de mortalidad,
cura, recurrencia entre el drenaje percutáneo y la
evacuación mediante “bur hole”. Encontraron mayor
mortalidad asociada al uso de corticosteroides, sin
impacto en las tasas de recurrencia o de cura. El uso
de dren tras haber drenado al hematoma se asoció a
una disminución significativa en recurrencia. La craniotomía se asoció a mayores tasas de complicaciones,
si se realizaba como primera opción de manejo, pero
fue superior en términos de manejo de recurrencia
con respecto a procedimientos mínimamente invasivos. El drenaje percutáneo con “twist drill” es una
opción de manejo de primera línea relativamente
segura y eficaz. Según los autores, los anteriores
hallazgos pueden resultar en ahorro en costos de salud, además de impactar disminuyendo riesgos potenciales del uso de anestesia general. Chiari y cols
(43), proponen una técnica novedosa que implica la
instalación de un tornillo hueco, enroscado mediante
“twist drill” que se conecta a un sistema de drenaje
cerrado, en términos generales parece ser una técnica segura y efectiva, sin embargo se requiere mejor
evidencia para evaluar el impacto de su empleo.
Hay controversia entre el empleo de uno o dos agujeros cuando se emplea craniectomía como técnica de
manejo quirúrgico, sin embargo Belkhair y cols (44),
a través de un meta análisis, no encuentra diferencias significativas entre la práctica de un solo o dos
“bur hole”, y no se asocian a una mayor tasa de revisiones.
Se debe considerar factores predisponentes que
alteren la coagulación (discrasias sanguíneas, anticoagulación, alcoholismo), factores de riesgo para
enfermedad degenerativa arterial (diabetes mellitus,
hipertensión arterial), y para el desarrollo de gradientes de presión (hidrocéfalo, epilepsia, punción lumbar, drenaje de LCR, atrofia cerebral). Es necesario
recordar que la frecuencia de focalización en HSC
bilaterales es menor, lo cual puede confundir su diagnóstico y retrasar el manejo (36).
Se han propuesto múltiples modalidades de tratamientos, desde la sola observación, el uso de medicamentos y diferentes tipos de cirugía. Dada la
fisiopatología, posiblemente la mejor opción es el
vaciamiento de la colección. El tratamiento conservador se basa en la teoría osmótica de formación
y crecimiento del HSC, mediante la administración
parenteral de sustancias hiperosmolares, que tienden a reducir, por absorción, el volumen del hematoma.
Sin importar el grupo etario, en todos hay preponderancia masculina. Diversas series han evaluado
grupos de pacientes con HSC en función de la edad,
y se ha observado que en menores de 50 años, el
HSC no difiere de aquello mayores de esa edad (31).
En menores de 70 años predomina la cefalea, entre
los 75 y 85 años predominan los déficits focales, especialmente la hemiparesia, con alteraciones de la
marcha. En mayores de 85 años también predominan los déficits focales (hemiparesia) pero con especial relación a los trastornos del habla (9,32,33).
Quirúrgico
A pesar de ser una de las entidades neuroquirúrgicas más comunes, no hay consenso respecto a la
técnica quirúrgica óptima para el HSC. Todas tienen
como meta la descompresión cerebral y prevenir
la recurrencia, manteniendo bajas tanto morbilidad
como mortalidad. Sin embargo las tasas de recurrencia pueden ser tan altas, como del 33% (38).
Anticoagulación
Hay escasa evidencia empírica sobre si reiniciar o no
anticoagulación en pacientes que presentan HSC y
que estaban en terapia anticoagulante, se necesita
mejor evidencia para definir si reiniciarla o no, y de
hacerlo con cual medicación, en qué momento y a
qué dosis y metas (45). De acuerdo con Yeon y cols
(46), la recurrencia de la hemorragia subdural se asocia a edades por encima de los 75 años, lo mismo
que a hematomas gruesos (≥25mm), pero no con el
estado de anticoagulación postquirúrgico; recomiendan que la warfarina no necesita estar suspendida
más allá del tercer día post drenaje del hematoma,
especialmente en aquellos pacientes con alto riesgo
tromboembólico.
Santarius y cols (39), han demostrado que el drenaje posterior a la evacuación quirúrgica del
Profilaxis anticonvulsivante
No se pueden realizar recomendaciones formales
sobre la profilaxis anticonvulsivante en estos pacientes, no hay evidencia en la literatura médica para justificar o no su empleo (47).
Recurrencia
Nakaguchi y cols (48), evaluaron ciertas características de los HSC buscando asociación con recurrencia. Clasificaron 126 HSC de acuerdo a la densidad,
arquitectura interna y extensión intracraneal. En la
característica densidad y arquitectura interna, clasificaron como HSC tipos homogéneo, laminar, separado
y trabecular. Consideraron que los HSC son inicialmente homogéneos, y pueden evolucionar hacia el
tipo laminar. Cundo el HSC es maduro está representado por el tipo separado, y cuando está en período
de reabsorción es de tipo trabecular. Encontraron que
en los HSC separados existe una alta tasa de recurrencia, siendo baja con el trabecular. De Acuerdo a la
extensión intracraneal encontraron que los HSC de la
base craneana recurren más, y los de la convexidad
lo hacen menos.
PRONOSTICO
Entre otros factores, además de la edad avanzada
de los pacientes o las frecuentes enfermedades intercurrentes que estos tienen, el pronóstico depende en
gran medida del estado clínico preoperatorio. El mismo está en relación con la demora en el diagnóstico y
la implementación del tratamiento. Los factores identificados como de buen pronóstico para la mejoría (luego del tratamiento quirúrgico) del deterioro cognitivo
asociado a HSC son: edad menor a 74 años, puntaje
menor a 5 en el score de actividades de la vida diaria
y puntaje mayor a 10 en el estudio mini mental.
Colecciones residuales post tratamiento son comunes, no se hace necesario la resolución tomográfica completa de la colección, tras 40 días, 15% de los
TAC control muestran persistencia de fluido.
Pueden ocurrir algunas complicaciones del manejo
de los HSC, entre las que se encuentran(36,49–51):
convulsiones (se incluye al status epilepticus), hemorragia intracerebral, falla en la reexpansión cerebral,
neumoencéfalo a tensión, y en el caso de no ser tratados, empiema subdural. La mortalidad derivada del
manejo quirúrgico está el rango de 0-8% (40,42,48).
CONCLUSIONES
El HSC representa una de las condiciones más frecuentes en la práctica neuroquirúrgica susceptible de
un tratamiento quirúrgico de relativa simpleza y efectividad. Constituye una patología relativamente frecuente en el adulto mayor, un población en aumento
a nivel mundial. Debe ser sospechada especialmente
en pacientes que cursan con síndrome demencial
aunque sean de edad avanzada. Aunque menos frecuente, debe investigarse ante un paciente que presente déficit neurológico transitorio. Su incidencia se
espera al alza, dada la mayor alerta en profesionales
de la salud y debido al acceso a los sistemas de salud, es por ello que se debe maximizar la eficiencia de
su manejo, al ser una prioridad.
El procedimiento que parece más recomendado es
el drenaje aspirativo cerrado por orificios de trépano.
Es un método sencillo, de corta duración, efectivo e
incluso puede realizarse con anestesia local. Se ha
debatido si el lavado intraoperatorio de la colección
mejora los resultados. El vaciamiento por un catéter
subdural fino con craneotomía mínima, realizada bajo
anestesia local junto a la cama del paciente tendría
igual o menor rendimiento que el drenaje por trépano convencional. La utilización de endoscopia, podría
aplicarse en HSC recurrentes, parcialmente tabicados.
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ABSTRACT
Chronic subdural hematoma is an encapsulated collection of blood underneath the Dura,
essentially characterized by the presence of
a membrane. It usually occurs in the elderly
considered as a sentinel event. Simple brain
computed tomography is the current imaging aid of choice for the diagnosis of this entity. It should be suspected especially in patients who present with dementia syndrome.
Although less frequent, it should be investigated in patients with transient neurologic
deficit. There is still no consensus regarding
the ideal surgical modality of treatment. The
procedure that seems most recommended is
closed suction drainage through burr holes.
It has been controversial whether intraoperative washing of such collection improves
outcomes.
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