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B LETN

Asociacin Mdica de Puerto Rico

CONTENIDO
3 Mensaje del Presidente de la AMPR
Rolance G. Chavier Roper

Original Article/Articulos originales


5 KNOWLEDGE

AND
MISCONCEPTIONS
ABOUT IMMUNIZATIONS AMONG MEDICAL
STUDENTS, PEDIATRIC AND FAMILY MEDICINE RESIDENT
Vilmarie Tan MS, Clarimar Borrero MD, Yasmn Pedrogo MD

10 EXCLUSIVE BREASTFEEDING REDUCES AS-

THMA IN A GROUP OF CHILDREN FROM THE


CAGUAS MUNICIPALITY OF PUERTO RICO
Jessica Gonzlez MD, Mariola Fernndez MD , Lourdes Garca Fragoso MD

13 EFFICACY, SAFETY AND COST-EFFICIENCY

OF USING AN ALTERNATIVE TECHNIQUE


FOR AUTOMATED EXCHANGE TRANSFUSION IN PEDIATRIC PATIENTS WITH SICKLE
CELL DISEASE
Mara B. Villar Prados MD, Ricardo Garca De Jess
MD, Alicia Fernndez Sein MD, Manuel Iglesias Garca MD

18 RESPIRATORY ILLNESS IN LATE PRETERM

INFANTS DURING THE FIRST SIX MONTHS


OF LIFE

Leilanie Prez MS, Zahira Corchado , Mariela Rodrguez , Dora Garca , Lizaida Medina MD, Arian Vicens
MD, Nerian Ortiz MD, Lourdes Garca MD, Yasmin
Pedrogo MD
21 PRENATAL BREASTFEEDING INTENTIONS

IN A GROUP OF WOMEN WITH HIGH RISK


PREGNANCIES
Hildamary Diaz Rozett MD, Lourdes Garcia Fragoso
MD

26 MINOR HEAD INJURY IN CHILDREN YOUN-

GER THAN TWO YEARS OF AGE: DESCRIPTION, PREVALENCE AND MANAGEMENT IN


THE EMERGENCY ROOM OF THE PEDIATRIC
UNIVERSITY HOSPITAL
Mara L. Fernndez MS, Linette Mejas MS, Nerian Ortiz MD, Lourdes Garca-Fragoso MD

30 ROOMING-IN IMPROVES BREASTFEEDING

INITIATION RATES IN A COMMUNITY HOSPITAL IN PUERTO RICO


Carmen W. Cotto MD, Lourdes Garcia Fragoso MD

Diseo Grfico e Ilustracin digital de cubierta realizados por


Juan Carlos Laborde
en el Departamento de Informtica de la AMPR
E-mail: webmaster@asociacionmedicapr.org

Review Articles / Articulos de Resea


33 ESOPHAGEAL ATRESIA: NEW GUIDELINES IN

MANAGEMENT

Jessica Gonzlez-Hernndez MS, Humberto Lugo-Vicente MD

39 CLINICAL VERSUS PATHOLOGIC DIAGNOSIS:


ACRODERMATITIS ENTEROPATHICA
Alicia Fernandez Sein MD

Case Reports / Reporte de Casos


45 CANDIDA ALBICANS MENINGITIS AND BRAIN
ABSCESSES IN A NEONATE: A Case Report
Ingrid M. Ancalle MD, Juan A. Rivera MD, Ins Garca
MD, Lourdes Garca MD, Marta Valcrcel MD

49 DENGUE VIRUS ASSOCIATED HEMOPHAGOCYTIC SYNDROME IN CHILDREN: A Case Report

Yadira Soler Rosario, MD, Ricardo Garcia MD, Alicia


Fernandez Sein MD

CIRCULAR-STAPLED
REA56 TRANSANAL
NASTOMOSIS AS A MANAGEMENT ALTERNATIVE FOR ANASTOMOTIC COLONIC STRICTURES: A NOVEL TECHNIQUE IN THE PEDIATRIC
PATIENT
Humberto Lugo-Vicente MD, Jorge J. Zequeira MD,
Joalex Antongiorgi MD

59 CME Questions
Catalogado en Cumulative Index e Index Medicus
Listed in Cumulative Index and Index Medicus No. ISSN-00044849
Registrado en Latindex -Sistema Regional de Informacin en
Lnea para Revistas Cientficas de Amrica Latina, el Caribe,
Espaa y Portugal
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Web site para el paciente: www.saludampr.org

JUNTA DE DIRECTORES
Dr. Rolance G. Chavier Roper
Presidente
Dra. Wanda G. Velez Andujar
Presidente Distrito Sur
Presidente Consejo de Educacin Mdica

Dr. Ral G. Castellanos Bran


Presidente Electo
Dr. Eduardo Rodrguez Vzquez
Presidente Saliente

Dr. Jos C. Romn de Jesus


Presidente Consejo tico Judicial

Dr. Pedro Zayas Santos


Secretario

Dra. Hilda Rivera Tubns


Presidente Consejo Relaciones y Servicios Pblicos

Dr. Jos R. Villamil Rodrguez


Tesorero

Dr. Salvador Torros Romeu


Presidente Consejo Servicios Mdicos

Dra. Hilda Ocasio Maldonado


Vicepresidente

Dr. Jaime M. Diaz Hernandez


Presidente Consejo Salud Pblica y Bienestar Social

Dr. Natalio Izquierdo Encarnacin


Vicepresidente
Dr. Ral A. Yordn Rivera
Vicepresidente
Dr. Arturo Arch Matta
Presidente Cmara Delegados

Dr.a. Ilsa Figueroa


Presidente Consejo Poltica Pblica y Legislacin
Dr. Eugenio R. Barbosa del Valle
Presidente Comit de Planes Prepagados y Seguros Mdicos
Dr. Hctor L. Cceres Delgado
Presidente Comit Afiliacin y Credenciales

Dr. Juan Rodrguez del Valle


Vicepresidente Cmara de Delegados

Dr. Ney Modesti Taon


Presidente Comit Ad-hoc de Compaerismo

Dr. Gonzalo Gonzlez Liboy


Delegado AMA

Dr. Jos A. Rodrguez Ruiz


Presidente Comit de Historia, Cultura y Religin

Dr. Rafael Fernndez Feliberti


Delegado Alterno AMA
Presidente del Comit Asesor del Presidente

Dr. Luis A. Romn Irizarry


Presidente Comit Mdico Impedido

Dr. Benigno Lpez Lpez


Presidente Distrito Este

Dra. Luisa Marrero Santiago


Presidente Comit de Seguros

Dr. ngel E. Michel Terrero


Presidente Distrito Sur

Dr. Jos I. Gerena Daz


Presidente Comit Ad-hoc Clnicas Multifsicas

Dra. Mildred R. Arch Matta


Presidente Distrito Central

Dr. Flix N. Cotto Gonzlez


Presidente Comit Ad-hoc de Reclutamiento y
Servicios al Mdico Joven

JUNTA EDITORA
Humberto Lugo Vicente, MD
Presidente
Luis Izquierdo Mora, MD

Juan Aranda Ramrez, MD

Melvin Bonilla Flix, MD

Francisco J. Muiz Vzquez, MD

Carlos Gonzlez Oppenheimer, MD

Walter Frontera, MD

Eduardo Santiago Delpin, MD

Mario. R. Garca Palmieri, MD

Francisco Joglar Pesquera, MD

Natalio Izquierdo Encarnacin, MD

Yocasta Brugal, MD

Jos Ginel Rodrguez, MD

2010

Mensaje del Presidente - President Message

Rolance G. Chavier Roper, MD

programas de residencias acreditados y crear nuevos


que produzcan pediatras que se queden y practiquen
en Puerto Rico

unque seguramente estaremos de acuerdo en que el valor de la vida humana es incalculable


y que la vida de un ser humano vale lo mismo no importa en que etapa de su existencia este, no hay duda
que, para la gran mayoria de nosotros, la vida y salud
de un nino es an ms relevante.


La Asociacin Mdica de Puerto Rico se complace en contribuir con estos propsitos a travs de la
publicacin de este nmero de nuestro boletn cientfico dedicado a la especialidad de pediatria.


Quizs sea por su inocencia y fragilidad, o por
todo lo que sabemos le espera en su desarrollo como
persona.


A travs de esta publicacin se abre un importante foro para la difusin de articulos de investigacin
realizados por residentes y fellows de pediatria.


Es por eso que, dentro de la profesin medica,
la especialidad de pediatria es tan respetada y querida.


Es tambin de nuestro agrado anunciar que
hemos reactivado la seccin de pediatria de la Asociacin Mdica y agradecemos al Dr. Angel Senquiz y a la
Dra. Hilda Rivera Tbens, entre otros, por su colaboracin en este propsito.


La Tranquilidad y felicidad de los padres y dems familiares cercanos de un paciente peditrico dependen de la salud de este y no hay tristeza mayor que
la de ver a un nio gravemente enfermo.


Invitamos a todos aquellos pediatras que an
no pertenecen activamente a la Asociacin Mdica a
ingresar nuevamente a esta, su casa.


Adems de la proficiencia en la profesin, una
de las caractersticas mas importantes en un mdico
y sobre todo en el pediatra, debe ser la empata. Hay
que saber sentir lo mismo que esos padres y tener
la paciencia y dedicacion para explicarles en detalle
aquella informacin que, aunque simple y rutinaria
para nosotros, es complicada e Intimidante para ellos
y ms an para los ninos.


Finalmente y como privilegio personal, deseo
reconocer a tres pediatras que fueron Importantes en
mi vida personal y profesional. En primer lugar, a la
Dra. Rosa Asmar de Deliz (qepd), quien fue mi pediatra
y primer contacto con la medicina. En segundo lugar, a
la Dra. Ana Navarro y por ltimo, a la Dra. Amalia Martnez Pico, cardiloga pediatrica y figura legendaria de
la medicina en Puerto Rico.


En Puerto Rico, nos honramos al tener una excelente facultad mdica y entre ellos, magnificos pediatras y subespecialistas en este rea.


Una vez ms, reiteramos el compromiso de la
Asociacvin Mdica de Puerto Rico con la educacin
medica en nuestro pas.


Para lograr mantener el numero adecuado de
profesionales en pediatria, necesitamos promover los

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

Original Articles - Artculos Originales


KNOWLEDGE AND
MISCONCEPTIONS ABOUT
IMMUNIZATIONS AMONG
MEDICAL STUDENTS,
PEDIATRIC, AND FAMILY
MEDICINE RESIDENT

ABSTRACT
Background: Previous research has indicated
that, despite being the most trusted source
of health information, medical students, residents and other health related professionals
lack accurate and current knowledge regarding immunization practices. Objective: To
evaluate medical students and primary care
resident knowledge about immunizations. Methods: Self-administered survey given to students from four medical schools, Pediatrics
residents (2 training programs) and Family
Medicine residents (2 programs). Data was
analyzed using Statistix 8.0. One-way ANOVA
test was used to compare means, and a p-value less than 0.05 was considered statistically
significant. Results: Participants (N=376) included 3rd (64%) and 4th (18%) year medical
students and a homogenous distribution of 1st,
2nd and 3rd year residents. The mean percent
of correct answers about immunizations was
61%. The participants showed poor knowledge about indications (62% correct answers),
contraindications (46% correct answers) and
myths (71% correct answers). Knowledge
about immunizations correlated with higher levels of education (p<0.01). Most participants
identified conferences (72%) as their primary
source to learn about immunizations followed
by books (48%) and the internet (36%). They
referred poor exposure to immunizations in
clinical settings. Conclusions: Most medical
students do not have the expected knowledge
about immunization indications and contraindications. Residents were not proficient in immunization contraindications. Both groups had
an adequate understanding about vaccination
myths. Efforts towards ensuring adequate exposure to immunizations education during training years are needed in order to eliminate
one of the barriers to adequate immunizations
in children.

Vilmarie Tan MS*


Clarimar Borrero MD**
Yasmn Pedrogo MD**
From the *School of Medicine and**General Pediatric Residency Program, Department of Pediatrics, UPR School of
Medicine.
Address reprints request to: Yasmn Pedrogo MD, UPR
School of Medicine, Department of Pediatrics, PO Box
365067, San Juan, PR 00936-5067. E-mail yasmin.pedrogo@upr.edu.

INTRODUCTION

TT


hroughout the years health maintenance
strategies such as childhood immunization have been
of uttermost importance in the eradication of disease
in populations and the prevention of disease in individuals. The responsibility to immunize children resides
in most pediatricians and family physicians; therefore it
is essential to evaluate their preparation and attitudes
towards vaccination (1). Healthcare personnel should
be familiar with the most recent immunization recommendations (2). Moreover, they should be aware of the
indications, contraindications and common myths that
may hold back vaccination. It has been observed that
children often lag behind in their immunization because their provider did not use the appropriate vaccination schedule or had misconceptions regarding their
contraindications (3-4). Furthermore, it is known that
some physicians are not familiar with the resources for
accurate immunization information (5). Therefore, it is
important to ensure proper education to physicians,
nurses and parents in order to achieve better vaccination outcomes in children.

It is currently required by medical schools and
licensing agencies that medical students, along with
pediatric and family medicine residents be competent
and proficient in their knowledge about indications and
contraindications regarding immunizations as part of
their clinical curriculum. At present, there is no publication that has evaluated the immunization knowledge in Puerto Ricos medical students, pediatric and
family medicine residents. Therefore, the primary objective of this study is to assess the knowledge about

Index words: immunization, medical, students,


pediatric, family, medicine, resident

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

with a P=0.0000 that a higher level of education provides a greater understanding about immunizations
as demonstrated by the increasing trend in average
scores at a higher level of education (Graph 2). This
was also true when evaluating participant performance exclusively for indications, contraindications and
misconceptions regarding immunizations (P < 0.01).
Moreover, we compared immunization knowledge
between specialties. This comparison revealed with a
P=0.0000 that pediatric residents were more familiar
with vaccine indications, contraindications and misconceptions (Graph 3).

immunizations (indications, important contraindications


and common misconceptions) among third (MSIII) and
fourth year (MSIV) medical students, pediatric and family medicine residents of Puerto Rico medical schools
and residency programs. As secondary objectives, we
evaluated medical students and residents exposure to
vaccines at immunization clinics and identify the primary source of information these health professionals
used to learn about immunizations.

MATERIALS AND METHODS



This was an observational, cross-sectional study with 376 subjects that included medical students,
pediatric residents and family medicine residents. A
self-administered survey was handed to medical students (MSIII & MSIV) of the four LCME credited medical schools in Puerto Rico (University of Puerto Rico
School of Medicine, Ponce School of Medicine, Universidad Central del Caribe School of Medicine and San
Juan Bautista School of Medicine), Pediatric Residents
and Family Medicine Residents at the UPR Medical
Science Campus, San Juan Municipal Hospital, Manat Medical Center and Hospital Federico Trilla (UPR
Carolina).

Graph 1: Distribution of participants per level


of education

6%

6%

5% 1%

MSIII
MSIV
PGYI
PGYII

18%

PGYIII

64%

PGYIV-V


The survey consisted of Student/Resident demographic data, multiple choice questions based on
the 2008 Immunization Schedule, and multiple choice
questions about common misconceptions and contraindications to immunizations. The surveys were handed
out and collected during the months of December 2008
to December 2009. Correct answers to the questions
were averaged and a score above 70% of correct answers was taken as satisfactory. The survey responses
were analyzed using Statistix 8.0. Descriptive statistics
(continuous variables and categorical variables) were
summarized using frequency, percentages and means.
One-way ANOVA test was utilized to compare means,
and a p-value less than 0.05 was considered statistically significant. This research project received approval
from the University of Puerto Rico Medical Sciences
Campus Institutional Review Board.

Graph 2: Knowledge about immunizations per


level of education

Mean

100
80
60
40
20
0
MSIII

90
80
70
60
50
40
30
20
10
0

RESULTS

PGYIII PGYIV-V
P=0.0000

Graph 3: Knowledge about immunizations


according to specialty

Mean


Medical students and residents completed a
total of 376 surveys. 52% of participants were female and 48% were male. Mean age for examinees was
26 years old. Most of the surveys were completed by
MSIII (64%), followed by MSIV (18%). A similar amount
of subjects (6%) of first and second year residents also
participated. 5% of participants were PGYIII and 1%
was PGYIV-V (Graph 1).

MSIV
PGYI
PGYII
Level of education

Medical Students

Pediatric Residents

Specialty


The survey evaluated immunization knowledge
in three areas: indications, contraindications and misconceptions. Questions tested facts according to the
2008 Immunization Scheme of the Advisory Committee on Immunization Practices ACIP CDC. Correct
responses were added for each individual in each of
the three areas evaluated and then an average score was assigned for the total obtained. We can then
compare the average total score obtained at each level
of education (MSIII-PGYIV). This comparison reveals

Family Medicine
Residents

P=0.0000


When assessing immunization indications we
found that in each of the seven questions we provided,
the majority of participants selected the correct answer.
Nevertheless, we found that two of the questions in this
are where answered correctly by less than the 50% of
the participants. These questions required knowledge
about immunization schedules and being familiar with
the MMR and Varicella vaccines. On the other hand,
in the evaluation of immunization contraindications we
found that 59% of the subjects knew that fever is not

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

a contraindication to immunization except in moderate


to severe diseases when vaccination must be postponed. 44% of participants did know that immunizing a
woman while breastfeeding is not contraindicated due
to risks of infecting the baby. Moreover, only 41% of the
subjects were aware that the MMR, MMRV, Measles or
Mumps vaccines can be administered to children allergic to eggs since there is no risk of anaphylaxis due
to egg protein allergy because these vaccines do not
contain derivatives of this protein (Graph 4).

Graph 5: Sources used for immunization


knowledge acquisition

Participants

300
200
100

Graph 4: Knowledge regarding


immunization contraindications

0
Books and
Journals

70

% Subjects

60
50
40
20

% of participants

10
0
Fever

Breastfeeding

Internet

Graph6: Sources used by residents to learn about


immunizations

Correct
Incorrect

30

Conferences Hands-on
and lectures experience
Sources

Egg Allergy

Contraindications


For the evaluation of the participants knowledge regarding vaccination misconceptions we included
10 true or false questions. Eight of these questions were
answered correctly by at least 70% of the subjects.
However, there were erroneous conceptions regarding
two of the statements presented in the survey. In the
first one only 45% of the participants knew that The Vaccine Adverse Event Reporting System (VAERS) has
not found various dangerous vaccine lots, which have
been removed by the FDA. Moreover, just 32% of the
subjects knew that the severity of chicken pox has not
decreased due to the increase amount of children vaccinated against it.

90
80
70
60
50
40
30
20
10
0

Books and Journals


Conferences and
Lectures
Hand-on Experience
Internet
PGYI

PGYII

PGYII

Level of Education

Graph 7: Exposure to immunizations


at clinics
Rarely

8%


In order to assess our secondary objectives for
this research we included some questions regarding the
sources utilized by the participants to get their immunization knowledge and how often they used them to get
vaccination information. Moreover we asked how much
they were exposed to immunization at clinics. We found
that 72% of the participants use conference and lectures as their primary source of immunization knowledge
followed by books and journals (Graph 5). In terms of
the use of these information resources there was an
even distribution in the answers: rarely and a few times
a year in which each category received 38%. Only 11%
of the participants referred using such resources on a
monthly basis, 7% reported using them a few times a
week and no more than 6% reported using them on a
daily basis. If we assess the use of these learning resources in the residents population we can appreciate
a tendency to use books and journals gain knowledge
about immunizations as the years of training increase
(Graph 6). When evaluating immunization exposure at
the clinics we found that 40% of the participants referred being rarely exposed to vaccination (Graph 7). If
we evaluate that exposure in residents only (Graph 8)

9%

A few times a year

5%
40%

Monthly
A few times a
week

38%

On a daily Baisis

% of participants

60

Graph 8: Residents Exposure to Immunizations


at Clinics

50

Rarely

40

Few times a year

30

Monthly

20

Few times a week


Daily

10
0
PGYI

PGYII

PGYIII-V

Level of Education

we observe that 50% of PGYII residents report being


rarely being exposed to immunizations, whereas the
33% PGYIII-V residents refer being exposed to vaccines a few times a year.

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

DISCUSSION

are asked by patients parents on a regular basis. Therefore, we believe they must be well known to medical
students and pediatric and family medicine residents.
Our results reveal that most medical students and residents reported being rarely exposed or exposed a few
times a year to immunizations. This finding could be
one of the factors that may have influenced the survey scores of the participants. Finally, our results suggest that medical students do not have the expected
knowledge about immunizations indications and contraindications; however, they had an adequate understanding about immunization misconceptions. On the
other hand, pediatric and family medicine residents do
not have the expected knowledge in immunization contraindications, but were knowledgeable about immunization indications and misconceptions. We believe
these results raise a red flag that should make medical
school and residencies reevaluate their strategies to
improve how they teach about immunizations.


Ever since the inoculation of cowpox lesions
from person to person, providing immunization to smallpox, vaccination has amazingly reduced the mortality from many infectious diseases (6). These outcomes have promoted the development of immunization
campaigns all over the world in an effort to eradicate a
variety of diseases. Nevertheless, despite the benefits
that immunization may provide to a population and to
an individual there are still children that do not receive
their vaccines on time or not at all. In some instances
this may be due to parental resistance to immunizations (5). In other cases it may be due to healthcare
personnel misconceptions about contraindications to
vaccinations (2). Whichever the reason that impedes
the proper administration of immunization to children,
it must be managed. Pediatricians and family medicine physicians are accountable for the immunization of
most children (1). Therefore, they have the responsibility to be knowledgeable about the indications, contraindications and misconceptions regarding immunizations. Moreover, they have to be able to transmit this
information to the patients parents in order to gain their
authorization instead of their refusal.


Limitations for this study include the fact that
this was a self-administered voluntary survey and we
can only assume that those that accepted to participate responded to the best of their knowledge. Moreover,
due to the voluntary aspect of the survey we could not
assess the entire population of Puerto Rico third and
fourth year medical students and pediatric and family
medicine residents.


It is a requirement by Medical Schools and
Licensing Agencies that Medical Students and Pediatric and Family Medicine Residents be competent
and proficient in their knowledge about indications and
contraindications regarding immunizations as part of
their Curriculum. Nevertheless, several studies have
revealed a lack of up to date immunization knowledge in medical students, residents and other healthcare
professionals.

REFERENCES
1.
Szilagyi P. G., Hager J., Roghmann K. J., et. al. Immunization Practices of Pediatricians and Family Physicians in the United
States, Pediatrics 1994; 94: 517-523.
2.
Ginder J. S., Cutts F. T., Barnett-Antinori M. E., et. al. Successes and Failures in Vaccine Delivery: Evaluation of the Immunization Delivery System in Puerto Rico, Pediatrics 1993; 91: 315320.
3.
Askew G. L., Finelli L., Lutz J, et. al., Beliefs and Practices
Regarding Childhood Vaccination Among Urban Pediatric Providers I New Jersey, Pediatrics 1995; 96: 889-892.
4.
Grabowsky M., Marcuse E. K., The Critical Role of Provider Practices in Undervaccination, Pediatrics 1996; 97:735-737.
5.
Levi B. H., Addressing ParentsConcerns About Childhood Immunizations: A Tutorial for Primary Care Providers, Pediatrics, 2007; 120: 18-26.
6.
Garber R. M., Mortimer E. A., Immunizations: Beyond the
Basics, Pediatrics in Review, 1992; 13: 98-106.


The majority of the participants in this study
were medical students, specifically MSIII. These subjects, although being on their clinical years of medical training, have several occasions in which they take
lectures and tests at their medical school campus as
compared to the MSIV students that have clerkships
in different clinical settings outside of their medical
school campus. Moreover, this distribution of participants goes in accordance with the fact that residency
programs accept a very limited amount of students per
year in comparison to a medical school.

Our results reflect that a higher level of education correlates with a greater understanding about
immunizations as demonstrated by the statistically significant increasing trend in average scores at a higher
level of education in the three areas evaluated. These
results may be explained by the acquisition of knowledge and experience throughout the years. Moreover, it
could be due to the use of books and journals by a high
percentage of residents to learn about immunizations.

dudas?


During the evaluation of the contraindications
area it was unexpected to find that more than half of
the participants did not know that immunizing a woman
while breastfeeding is not contraindicated due to risks
of infecting the baby or that the MMR, MMRV, Measles or Mumps vaccines can be administered to children
allergic to eggs. These facts are relevant, moreover,
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

RESUMEN
Estudios previos revelan que tanto los
estudiantes de medicina, residentes y otros
profesionales de la salud no poseen un conocimiento adecuado sobre vacunacin. Nuestro objetivo fue evaluar el conocimiento sobre
inmunizaciones de los estudiantes de medicina y residentes de pediatra y medicina de familia. Se administr una encuesta voluntaria
a los grupos antes mencionados y la informacin se analiz mediante el programa Statistix
8.0. Los participantes (N=376) incluan estudiantes de medicina de 3ro (64%), 4to (18%)
y una distribucin homognea de residentes
de 1ro, 2do y 3ro ao. En promedio, los participantes obtuvieron una calificacin de 61%
y se observ que el conocimiento sobre vacunas aumentaba con el nivel acadmico de
los participantes (P<0.01). La mayora de los
encuestados report el uso de conferencias
como fuente primaria para el aprendizaje de
inmunizaciones seguido de los libros. Sin
embargo, refirieron pobre exposicin clnica
a vacunas. En conclusin, la mayora de los
estudiantes de medicina no posee el conocimiento esperado en cuanto a indicaciones
y contraindicaciones de vacunas. Por otro
lado, los residentes no demostraron conocer
satisfactoriamente las contraindicaciones de
las vacunas. No obstante, ambos grupos demostraron un conocimiento adecuado sobre
mitos comunes de inmunizaciones. A tales
efectos, entendemos imperativo, que durante
los aos de entrenamiento clnico, se ofrezca
una mayor exposicin clnica a las vacunas y
as garantizar la inmunizacin adecuada de
la poblacin peditrica.

Confe en las soluciones informticas que le


ofrece la Asociacin Mdica de Puerto Rico:
108 aos de historia avalan nuestra permanencia y responsabilidad. Ms de 2 aos de investigacin en recursos de Recetario Electrnico y
Registros Electrnicos de Salud aseguran que
la solucin que proponemos es la ms adecuada a sus necesidades.

EXCLUSIVE
BREASTFEEDING
REDUCES ASTHMA IN
A GROUP OF CHILDREN
FROM THE CAGUAS
MUNICIPALITY
OF PUERTO RICO

ABSTRACT

Jessica Gonzlez MD *
Mariola Fernndez MD *
Lourdes Garca Fragoso MD **
From the *Department of General Pediatrics and the *Section of Neonatology, Department of Pediatrics, UPR School
of Medicine.
Address correspondence and reprints requests to: Lourdes
Garca Fragoso MD, UPR School of Medicine, Department
of Pediatrics, Neonatology Section, PO Box 365067, San
Juan, PR 00936-5067. E-mail: lourdes.garcia1@upr.edu.
Poster presentation at the Annual Puerto Rico Pediatrics Society meeting (February 2009), the annual Medical Sciences
Campus Research Forum (April 2009), and at the 2009 ALAPE meeting (November 2009).


Breast-feeding is the preferred method
of infant nutrition. Its role in preventing childhood asthma is controversial. Objective: Determine whether breastfeeding protects against the
development of bronchial asthma in children.
Methods: A survey was answered by parents
of children less than 18 years of age attending
a Pediatric clinic at Cidra, Puerto Rico from July
to December 2008. Results: A group of 175 mothers were included in the study. The mean age
was 28 years (range 14-50). The mean age of the
children was 5 years. There was family history of
asthma in 64% of the families. The prevalence
of asthma in these children was 50%. Sixty-six
percent of the mothers breastfed but only 27%
did it exclusively. Children who were exclusively
breastfed had a lower prevalence of asthma and
milk protein allergy. Conclusions: This study correlates with literature reports linking exclusive
breastfeeding to a reduction in asthma and other
allergic diseases.
Index words: exclusive, breastfeeding, asthma,
Caguas, Puerto Rico

INTRODUCTION


tudies have shown that Puerto Rican children have the highest prevalence of lifetime asthma
(26%) compared with non-Hispanic black children
(16%), non-Hispanic white children (13%), and Mexican children (10%). This appreciably higher asthma
morbidity rate experienced by Puerto Rican children
cannot be explained by socio-demographic and other
risk factors measured in the National Health Interview
Survey (1). Two separate community-based studies of
children in Puerto Rico found the lifetime prevalence
of asthma to be higher than 30%. Prez-Perdomo and
coworkers (2) found that parents of island Puerto Ricans who participated in the 2000 Behavioral Risk Factor Surveillance System reported a lifetime prevalence
of asthma in their children of 33.2%. While many studies have shown a high burden of asthma in mainland
US cities in which a large proportion of the Hispanic
population is of Puerto Rican background, fewer studies have focused on Puerto Ricans specifically (3). A
study with the objective to estimate the prevalence of
asthma in two municipalities of Northern Puerto Rico
showed that among 2,800 students the prevalence of
asthma was 46% in elementary schools and 24% in junior-high schools (4). The asthma prevalence in Puerto
Rican children being so high should prompt us to find
preventive measures. It is known that breastfeeding is
the preferred method of infant nutrition for multiple reasons. However, its role in the prevention of asthma and
other allergic conditions remains controversial. Multiple
studies have shown a preventive effect but whether the
relation is to exclusive breastfeeding or breastfeeding
for a specific amount of time remains to be determined.

The objective of our study was to determine whether


breastfeeding or its duration protects against the development of bronchial asthma in children.

MATERIAL AND METHODS



An anonymous survey, developed and validated for this study, was answered by mothers of children
less than eighteen years of age who attended a Pediatrics clinic in Cidra, Puerto Rico. The survey inquired
about breastfeeding duration, use of formula, diagnosis
of bronchial asthma, its severity, treatment received,
among other questions. The University of Puerto Rico,
Medical Sciences Campus, Institutional Review Board
approved the study. Statistix 8.0 was used to perform
the statistical analysis, which included frequency distribution, means and chi square for differences among
groups. A p value < 0.05 was considered statistically
significant.

RESULTS

A total of 175 mothers participated in the study.
The general characteristics of the mothers and children
are summarized in Table I. The mothers lived in four
municipalities from the Caguas Region in Puerto Rico,
most of them in Cidra (86%). These municipalities are
located in the central region of the island. There was
a positive family history of bronchial asthma in 64%
of patients. Fifty percent of the children had bronchial
asthma. Most of the children with asthma (68%) had

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10

one to 3 bronchial asthma exacerbations in a year;


18% had four to 6, 8% had seven to 10 and only 6%
had more than 10. Sixty three percent of children didnt
have a bronchial asthma related hospitalization in the
past year. Sixty six percent (66%) of children were
breastfed. Fifty-four percent (54%) were breastfeed for
at least three months, 29% for 6 months, and 20% for
more than 6 months. Only 27% of children were exclusively breastfeed, the others combined breast milk and
formula.
Table I: General characteristics of the participants
(N=175)
Characteristics

Participants

Age in years, mean

28 (14-50)

Age at child birth, mean

22 (13-43)

Children age, mean

5 (0-18)

Family history of bronchial asthma 64%



Exclusively breastfed children had a lower prevalence of bronchial asthma (p<0.01) and milk protein
intolerance (p<0.01) as seen in Table II. There was
no difference in the prevalence of bronchial asthma,
wheezing, milk protein intolerance, allergic rhinitis or
asthma related hospitalizations between breastfed and
formula fed patients when breastfeeding was not exclusive. No difference was found in the prevalence of
the above mentioned factors when compared to the
duration of breastfeeding (not breastfed, breastfed less
than six months, and breastfeed more than six months).
Table II: Exclusive breastfeeding, asthma and allergies
(N=128)
Characteristic

Bronchial asthma
Milk protein
intolerance
Allergic rhinitis
atopic dermatitis

Exclusive
Formula or p-value
Breastfeeding combined
(N=35)
(N=93)
35%

64%

<0.01

15%

41%

<0.01

20%

33%

NSS

NSS not statistically significant

DISCUSSION

Asthma is a chronic disease of the lung that has
been increasing at an alarming rate in industrialized
countries around the world over the last few decades.
Although considerable progress has been made in our
understanding of the underlying pathogenesis of the
disease, the exact causes of the increasing prevalence
are unknown. Studies suggest that most asthma develops in early childhood and that environmental factors
present early in life may be crucial in the development
of disease.


A systematic review of 12 prospective studies
concluded that exclusive breast-feeding during the first
months after birth is associated with lower asthma rates
during childhood (5). A study in which 331,100 mothers
of children between the ages of twelve and 24 months were questioned about breastfeeding duration and
physician-diagnosed asthma and wheeze in the previous year found that breastfeeding for nine months or
less was a risk factor for asthma in young children (6).
In 2002, Sears et al (7) published a study in which children were assessed every 2-5 years from ages nine to
26 years with questionnaires, and pulmonary function,
after a history of breastfeeding was previously recorded. He found that more children who were breastfed
reported current asthma than those who were not. Kull
and colleagues (8) studied the duration of breastfeeding for two years, finding that children exclusively
breastfed during four months or more exhibited less
asthma, less atopic dermatitis, and less allergic rhinitis
by two years of age. Bener and coworkers (9) surveyed
1,278 mothers in Qatar finding that asthma, wheezing,
allergic rhinitis, and eczema were less frequent in exclusive breastfed children, compared to infants with
partial breastfeeding and formula milk. A recent study
by Ogbuanu (10) assessing the association of breastfeeding and lung function in 10-year-old children
found that compared to those who were not breastfed,
forced vital capacity and peak expiratory flow were
increased in children who were breastfed for at least
four months. Also recently, Scholtens (11) showed that
breastfeeding was associated with lower asthma prevalence from three to 8 years of age in children of both
non-allergic and allergic mothers. On the other hand,
a study were data was collected about the duration of
breastfeeding and the prevalence of asthma fourteen
years later after birth, found that breastfeeding neither
increases nor decreases the prevalence of asthma in
children at 14 years (12). A population-based prospective cohort study that followed participants from the
age of seven to 44 years showed that at age 7 years,
exclusively breast-fed children with a maternal history
of atopy had a marginally lesser risk of current asthma
than those not exclusively breast-fed. However, after
age 7 years, the risk reversed, and exclusively breastfed children had an increased risk of current asthma at
14, and 44 years (13).

One potential limitation of our study is that the
prevalence of asthma is based on parental report but
this question has been validated and has been shown
in multiple studies to have high sensitivity and specificity in differentiating asthmatics from nonasthmatics
as compared to clinical diagnosis of asthma by a physician (3). Also all these children are followed by the
same primary pediatrician which allows for more uniformity in the diagnosis. Another potential limitation is
the sample size which may not have the power to find
some important associations. Our study did not find a
preventive effect for asthma when analyzing breastfeeding or not breastfeeding at all. We found a difference
when breastfeeding was exclusive which showed a
preventive effect for asthma and milk protein allergies.
The American Academy of Pediatrics recommends
exclusive breastfeeding for the first six months of life.
However, in this sample of mothers, only 27% were

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

11

exclusively breastfed and only 29% were breastfed for


six months. This is far from the goals set by Healthy
People for 2010 and shows that there is a long road
ahead of us if we want our children to have all the proven benefits that breastfeeding has.

REFERENCES

Kumdo
El Camino de la Espada
Una prctica milenaria que desarrolla
cuerpo y espritu.
Segn la tradicin Hwa Rang
Lunes a jueves de 5:30 a 7:00 pm
Puerto Rico Medical Association Do Jan
Ave. Fernndez Juncos 1305 (Pda 18)
Santurce - Puerto Rico
Para ms informacin llamar al
(787) 238-6722
o visite nuestro website
www.yuyonkumdo.webs.com

(1) Lara M, Akinbami L, Flores G, Morgenstern H. Heterogeneity of


childhood asthma among Hispanic children: Puerto Rican children
bear a disproportionate burden. Pediatrics 2006;117(1):43-53.
(2)Perez-Perdomo R, Perez-Cardona C, Disdier-Flores O, et al.
Prevalence and correlates of asthma in the Puerto Rican population: Behavioral Risk Factor Surveillance System, 2000. J Asthma
2003; 40:46574
(3) Cohen RT, Canino GJ, Bird HR, Shen S, Rosner BA, Celedn
JC. Area of residence, birthplace, and asthma in Puerto Rican children. Chest 2007 ;131(5):1331-8.
(4) Loyo-Berros NI, Orengo JC, Serrano-Rodrguez RA. Childhood
asthma prevalence in northern Puerto Rico, the Rio Grande, and
Loza experience. J Asthma 2006;43(8):619-24.
(5) Gdalevich M Mimouni D, Mimouni M. Breast-feeding and the
risk of bronchial asthma in childhood: a systematic review with meta-analysis of prospective studies. J Pediatr 2001;139(2):261-6.
(6) Dell S, To T. Breastfeeding and asthma in young children: findings from a population-based study. Arch Pediatr Adolesc Med
2001;155(11):1261-5.
(7) Sears MR, Greene JM, Willan AR, Taylor DR, Flannery EM, et
al. Long-term relation between breastfeeding and development of
atopy and asthma in children and young adults: a longitudinal study. Lancet 2002;360(9337):901-7.
(8) Kull I, Wickman M, Lilja G, Nordvall SL, Pershagen G. Breast
feeding and allergic diseases in infants-a prospective birth cohort
study. Arch Dis Child 2002;87(6):478-81.
(9) Bener A, Ehlayel MS, Alsowaidi S, Sabbah A. Role of breastfeeding in primary prevention of asthma and allegrgic diseases in a
traditional society. Eur Ann Allergy Clin Immunol 2007;39(10):33743.
(10) Ogbuanu IU, Karmaus W, Arshad SH, Kurukulaaratcgy RJ,
Ewart S. Effect of breastfeeding duration on lung function at age 10
years: a prospective birth cohort study. Thorax 2009;64(1):62-6.
(11) Scholtens S, Wijga AH, Brunekreef B, Kerkhof M, Hoekstra
MO, et al. Breastfeeding, parental allergy and asthma in children followed for 8 years. The PIAMA birth cohort study. Thorax
2009;64(7):604-9.
(12) Burgess SW, Dakin CJ, O'Callaghan MJ. Breastfeeding
does not increase the risk of asthma at 14 years. Pediatrics
2006;117(4):e787-92.
(13) Matheson MC, Erbas B, Balasuriya A, Jenkins MA, Wharton
CL, et al. Breast-feeding and atopic disease: a cohort study from
childhood to middle age. J Allergy Clin Immunol 2007;120(5):10517.

RESUMEN

La lactancia es el mtodo preferido de nutricin para infantes. Sin embargo, su rol en la prevencin del asma en nios es controversial. Objetivo:
Determinar si la lactancia es un factor protector para
el asma en nios. Mtodos: Una encuesta fue contestada por madres de nios menores de 18 aos
que visitaron una clnica peditrica en Cidra, Puerto
Rico de Julio a Diciembre de 2008. Resultados: Un
grupo de 175 madres fueron incluidas en el estudio.
La edad promedio fue 28 aos (14-50). La edad promedio de los nios fue 5 aos. Haba historial familiar de asma en 64% de las familias. La prevalencia
de asma en los nios fue 50%. Sesenta y seis por
ciento de las madres lactaron pero solo 27% lo hizo
exclusivamente. Los nios lactados exclusivamente
tuvieron una menor prevalencia de asma y de alergia a la protena de la leche. Conclusin: Este estudio correlaciona con reportes en la literatura que
asocian la lactancia exclusiva con una reduccin en
el asma y otras enfermedades alrgicas.

ABSTRACT
Background: Sickle cell disease (SCD) patients suffer complications requiring simple and/or exchange transfusion. In 1999 we developed an automated exchange technique using infusion pumps and
vascular catheters (IV Pump Method). Objective:
To prove that IV Pump Method is cost-efficient, and
as safe and effective as automated cell separators. Methods: Retrospective chart review of SCD
patients requiring exchange transfusion admitted
to PICU from 2003-2009. Evaluated method used,
complications, costs, and Hemoglobin S% (HgS%)
change, excluding patients not requiring exchange
transfusion. Results: Cost-reduction with IV Pump
Method is around $1000. Average HgS% reduction
using IV Pump Method was 30.3 vs. 28.8 in Blood
Cell Separator group (p = 0.84). We had no complications or mortalities, with the majority of patients
being male (p = 0.03) and on the oldest age group
(11-19 y/o) for both methods. Conclusion: The IV
Pump Method is a safe, effective, and cost-efficient
alternative to perform exchange transfusion.
Index words: automated, exchange, transfusion,
sickle, cell, disease

EFFICACY, SAFETY AND


COST-EFFICIENCY OF
USING AN ALTERNATIVE
TECHNIQUE FOR
AUTOMATED EXCHANGE
TRANSFUSION
IN PEDIATRIC PATIENTS
WITH SICKLE CELL
DISEASE
Mara B. Villar Prados MD*
Ricardo Garca De Jess MD*
Alicia Fernndez Sein MD*
Manuel Iglesias Garca MD**
From the *Pediatric Critical Care Section and **Pediatric Residency Program, Department of Pediatric, UPR School of
Medicine.
Address reprints request to: Maria Villar Prados, MD, UPR
School of Medicine, Department of Pediatrics, PO Box
365067, San Juan, PR 00936-5067. E-mail mvillarprados@
hotmail.com.

INTRODUCTION


ickle cell disease (SCD) is the most common congenital hemoglobinopathy, affecting 1 in 4000
Puerto Rican births (1, 2). Patients suffer from serious
complications, such as chronic anemia, infection, splenic sequestration, pain crisis, cholecystitis, mesenteric ischemia, cerebrovascular accidents, and acute
chest syndrome, among others (1). Treatment usually
includes simple and/or exchange transfusion, with the
goal of reducing hemoglobin S (HgS%) to less than
30%, particularly in critically ill patients (3). Exchange
transfusion quickly reduces HgS% without the risks of
iron overload or hyperviscosity, and the same rate for
alloimmunization, infection, hypocalcemia, and neurological deterioration as simple transfusions (4, 5).

Exchange transfusion can be performed using
automated blood cells separators (6, 7) but this technology is not available at all institutions. In 1999 we
developed an automated red cell exchange technique
using infusion pumps connected to vascular catheters
(IV Pump Method) (8), providing an effective procedure
for reducing HgS%. Vascular access is obtained by
cannulation of a central vein with a dual lumen catheter
(Quinton Permacath), or alternatively, using a central vein and an arterial catheter. The setup is prepared
as follows (See Fig.1):

Connect arterial port to a three-way, to allow
for heparin flushing if needed, then to an IV pump (e.g.
Abbott Plum A+) in the secondary line site, using IV
tubing and a male-male connection. A heparinized

Figure 1: Setup for performing exchange transfusion


using IV Pump Method.
Sickle cell
free PRBCs

Saline
solution

Heparinized
solution

Primary
site

IV
Pump

Secondary
site

Quinton
Catheter

Venous
side

Arterial
side

Primary
site

IV
Pump

Secondary
site

Bag Access Device

Three-way
Male-Male
connection

IntraVia
Container

solution with a final concentration of 2 units/ml will be


connected to the primary site. This pump empties to
an empty container (e.g. Baxter IntraVia Container)
via a Bag Access Device (ICU Medical, Inc.) to discard the extracted blood. Another IV pump is connected to the venous port through the secondary line
to transfuse the sickle cell free blood. A normal saline solution will be connected to the primary site. Total rate of infusion will be the same for both pumps.

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13

Patient must be heparinized, with an initial bolus of


50 units/kg, followed by a continuous infusion at 15
units/kg/hr for the length of the procedure. Total volume exchanged, as per Hematology Service recommendations, should be completed in no more than four
hours.

Our objective was to prove that the use of the
IV Pump Method is more cost-efficient, and as safe
and effective as automated blood cell separators performing exchange transfusion in SCD patients in order
to reduce HgS%.

MATERIALS AND METHODS



We conducted a retrospective chart review of
all SCD patients requiring exchange transfusion admitted to the PICU of a public, government subsidized,
tertiary Childrens Hospital in San Juan, Puerto Rico
from July 2003 to June 2009. Inclusion criteria were
all patients with SCD, males and females, ages 1 to
19 years of age, admitted to PICU for exchange transfusion per Hematology service recommendation. We
further divided patients by sex and age groups (1-4
years, 5-10 years, 11-19 years). We evaluated the method used for exchange transfusion, complications from
the procedure, and Pre- and Post- exchange transfusion Hg S%, obtained by hemoglobin electrophoresis
performed at our center. We also compared the costs
of performing the exchange transfusion using the IV
pump method vs. the automated blood cell separator,
a service provided by the local Red Cross Chapter. Exclusion criteria were patients admitted to the PICU with
SCD not requiring exchange transfusion. IRB approval
was obtained. Data was analyzed using InStat 3, Graph Pad Software. Statistical analysis was performed
using unpaired t-test. The significance level was p =
0.05. We made no assumptions about missing data.

RESULTS

We identified 39 admissions of SCD patients
admitted to PICU, for a total of 19 admissions and
20 exchange transfusions performed during the study period, since one patient received two exchange transfusions during her PICU stay (See Fig. 2).
SCD PICU
Admissions:
39 admissions

We excluded 6 patients due to incomplete data, and


used a total of 14 admissions for our study. We analyzed the sex and age of the patients, complications from
the procedure, and change in HgS% after the procedure (See Table I).

DISCUSSION

The cost of performing Therapeutic Hemapheresis Treatment provided by the American Red Cross
Blood Services of P.R. using the COBE Spectra Apheresis System (CaridianBCT) ranges from around $1000
to $1800, depending on procedure delays, procedures
performed on weekends and holidays, and extra procedures, to name a few examples. To this we must add
the cost of the catheter ($443), transfusion charges
($293), plus the cost of each unit of packed red blood
cells (PRBCs) ($142/unit) used for the procedure. The
cost for the IV Pump Method is around $43, plus the
cost of the catheter, transfusion charges, and each
unit of packed red blood cells and fresh frozen plasma
(FFP) ($113/unit). This represents a significant cost reduction of at least $1000 when compared to the cost of
the service provided by the American Red Cross. This
is particularly important in centers with scarce economic resources.

The average reduction in HgS% using the IV
Pump Method was 30.3, compared to 28.8 in the Blood
Cell Separator group, with a p = 0.84 ( 95% CI = -14.80
to 17.80 ). These results show that there is no statistically significant difference between the groups, and
that the IV Pump Method for exchange transfusion is
as effective as the Red Blood Cell Separator Method in
reducing HgS% (See Fig. 3). This provides a strong argument for the use of the IV Pump Method, particularly
in centers with limited resources that dont have access to an automated red cell separator and specially
trained personnel to operate the machine. Also, when
compared to manual techniques for exchange transfusion, the IV Pump Method requires less medical and
nursing interventions, decreasing the contamination
risk and medical and nursing care hours.

30.5
30

n = 14 admissions

IV Pump Method

29.5
29

Exchange transfusion:
20 admissions

30.3

Other
treatments:
19 admissions

28.8

Blood Cell
Separator Method

28.5
28

Reduction in HgS%

Figure 3: Average reduction in Hemoglobin S% after


Exchange Transfusion

Incomplete data:
6 admissions

Figure 2: PICU admissions of patients with SCD that required exchange transfusion.


One of the limitations of the IV Pump Method
is that on patients over 35 kg of weight, due to the large volume required to perform the exchange transfusion, including both sickle cell free PRBCs and FFP,
the length of the procedure extends to over four hours.

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14

Table I: Sex, age, method, complications, and Pre and Post Exchange Transfusion Hg S% of patients admitted to
PICU.

Sex

Age

Method

Complications

Pre HgS%

Post HgS%

Change

4 y/o IV Pump

None

20.9

8.1

12.8

6 y/o IV Pump

None

***

31.2

***

12 y/o IV Pump

None

***

24.6

***

4 y/o IV Pump

None

60.9

24.8

36.1

8 y/o IV Pump

None

67.8

34.5

33.3

13 y/o IV Pump

None

44.1

25.4

18.7

9 y/o IV Pump

None

66.4

28.5

37.9

14 y/o IV Pump

None

59.6

19.5

40.1

10 y/o IV Pump

None

69.7

28.7

41

12 y/o IV Pump

None

73.7

51.0

22.7

12 y/o Blood cell separator None

51.0

25.3

25.7

14 y/o Blood cell separator None

48.9

24.85

24.1

19 y/o Blood cell separator None

71.5

22.1

49.4

7 y/o Blood cell separator None

64.2

48.1

16.1

*** Data not available


M = Male; F = Female; HgS = Hemoglobin S
This is due to the rate limit on the IV pumps (999 ml/
hr). For this reason, when available, we use the Red
Cell Separator Method in most of our teenage patients.
It is important to mention that with the IV Pump Method
the patient will be exposed to both PRBCs and FFP, in
order to keep a physiologic blood composition, putting
the patient at risk of developing transfusion reactions
to both of these blood products. Another disadvantage
of the IV Pump Method is that it would not be practical
in an outpatient setting for chronic exchange transfusion therapy.

ge, there are no recent studies looking at mortality from


exchange transfusion in SCD patients in the literature.
We would need to further investigate other markers for
disease severity (e.g. PRISM scores, length of stay,
oxygen requirements, baseline Hct, home medications, previous events), and time-lines from hospital
admission to PICU admission to reach more concrete
conclusions about the low mortality in our study population. We also need to consider the socio-economic
background and access to specialized care of our population compared to patients in other countries.


We had no complications with any of our admissions in either group, including problems with clotting within the catheter. We had experienced complications in that regard in the past while using the IV Pump
Method, but we havent had any more cases since we
started systemic heparinization of all our patients. We
havent encountered any bleeding complications, hemodynamic instability, transfusion reactions, or acute
clinical deterioration. This demonstrates that the IV
Pump Method is as safe and as well tolerated as the
Red Cell Separator Method.


We had some unexpected findings. We
had three females and 11 males that underwent
exchange transfusion (p = 0.03) (See Fig. 4).


No mortality in either group was found for the
studied period, compared to an average mortality of
6.8% for all PICU admissions (p = 0.0001). All cause
mortality for pediatric SCD patients has been described in the literature ranging from as high as 12.5% in
some African countries (9, 10), to survival rates of 85%
at 18 years of age in the United States (11, 12), and
as high as 99% survival rate in London (13). Mortality
from exchange transfusion in neonates for hyperbilirubinemia has been reported as 0.3%. To our knowled-

14
12

10

Female

Male

4
2

Exchange
transfusion

IV Pump Method Blood Cell Separator

Figure 4: Sex Distribution of Patients Undergoing Exchange Transfusion

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

15

The fact that more males than females in our population ended up requiring exchange transfusion may be
due to males engaging in more extraneous physical
activity in our hot climate, becoming dehydrated and
more at risk of developing SCD complications treated
with exchange transfusion. This trend has been reported in some African countries (9, 10). We also noticed
that our patients ages ranged from 4 to 19 years of
age, with a mean age of 10.3 years. If we further divided patients by age groups (1-4 y/o, 5-10 y/o, and
11-19 y/o), we find that the majority of patients who
underwent exchange transfusion were on the oldest
age group for both methods (p < 0.05) (See Fig. 5).
This finding could be explained by the fact that SCD
patients suffer chronic pulmonary and vascular damage throughout the years, making older patients more at
risk of developing these complications.

We are the only center in the country that serves the SCD population and the only center where
exchange transfusion is performed as a treatment for
SCD complications. Our data spans through six years,
and is our plan to continue collecting and analyzing future data in hopes of using this information to better
guide patient care. The IV Pump Method represents
a realistic, safe, and effective alternative to perform
exchange transfusion. This is particularly important
in centers where due to geographic, socio-political, or
economic limitations it is not feasible to perform this
procedure using automated blood cell separators.

This study has several limitations. This is after
all, a retrospective study, in only one center in Puerto
Rico, looking at a procedure performed selectively in a
small patient population. Our hospital runs on a paperbased system, making it at times impossible to retrieve
all the necessary data for each admission. This fact further limited our n to 14 admissions, limiting as well our
statistical analysis. A prospective study design would
help us overcome this obstacle. It was also difficult to
pair patients in both method groups by age or sex, since we had a significant male predominance and older
patients using the Blood Cell Separator method.

CONCLUSION

1-4 y/o

5-10 y/o
3

11-15 y/o

Exchange transfusion

IV Pump Method

Blood Cell Separator

Figure 5: Age Distribution of Patients Undergoing Exchange Transfusion


3. Boga C, Kozanoglu I, Ozdogu H, et al. Plasma exchange in critically ill children with Sickle Cell Disease. Transfus Apher Sci. 2007
Aug;37(1):17-22. Epub 2007 Aug 17.
4. Melton C, Haynes J. Sickle Acute Lung Injury: Role of prevention and early aggressive intervention strategies on outcome. Clin
Chest Med 27 (2006) 487-502.
5. Swerdlow P. Red cell exchange in sickle cell disease. Hematology Am Soc Hematol Educ Program. Jan 2006: 48-53.
6. Lawson S, Oakley S, Smith N, et al. Red cell exchange in sickle
cell disease. Clin Lab Haematol. Apr 1999; 21(2): 99-102.
7. Janes S, Pocock M, Bishop E, et al. Automated Red Cell Exchange in Sickle Cell Disease. British Journal of Haematology.
97(2):256-258, May 1997.
8. Sotomayor F, Fernandez-Sein A, Gotay F. Technique for automated exchange transfusion in pediatric patients with Sickle Cell
Disease. Boletin Asoc. Med. de P.R. Vol.95:1.
9. Ye D, Kouta F, Dao L, et al. Pediatric Management of Sickle Cell
Disease: Experience at the Charles de Gaulle Childrens Hospital
in Ouagadougou. Sante. 2008 Apr-Jun;18(2):71-5.
10. Ikefuna A, Emodi I. Hospital admission of Patients with Sickle
Cell Anemia Pattern and outcome in Enugu area of Nigeria. Niger J
Clin Pract. 2007 Mar;10(1): 24-9.


The data suggests that the IV Pump Method
is a safe, effective, readily available, and cost-efficient
alternative to perform exchange transfusion in patients
suffering SCD related complications. Further data collection is warranted to increase our study population.
Further research is required for better understanding of
the disease process. The development of evidence based treatment guidelines will also result in the improvement of patient care and further reduction of morbidity.

11. Quinn C, Rogers Z, Buchanan G. Survival of Children with Sickle Cell Disease. Blood. 2004 Jun 1;103(11):4023-7.

REFERENCES

14. Hulbert ML. Exchange transfusion compared with single transfusion for first overt stroke is associated with a lower risk of subsequent stroke. J Pediatr. 2006 Jul; 149(5): 710-2.

1. Vichinsky F, Neumavr L, Earles A, et al. Causes and Outcomes of


the Acute Chest Syndrome in Sickle Cell Disease: National Acute
Chest Syndrome Group. N Engl J Med. 200 Jun 22;342(25):185565.
2. Informe Annual 2009 Vigilancia de Defectos Congenitos en
Puerto Rico. Departamento de Salud de Puerto Rico. August 2009.
http://www.salud.gov.pr/Programas/CampanaAcidoFolico/Estadisticas/InformeAnual2009.pdf

12. Yanni E, Grosse S, Yang Q, et al. Trends in Pediatric Sickle


Cell Disease-Related Mortality in the United States, 1983-2002. J
Pediatr. 2009 Apr;154(4): 541-5.
13. Telfer P, Coen P, Chakravorty S, et al. Clinical Outcomes in
Children with Sickle Cell Disease living in England: A Neonatal Cohort in East London. Haematologica. 2007 Jul;92(7):905-12.

15. Gladwin M, Vichinsky E. Pulmonary Complications of Sickle


Cell Disease. New Engl J Med. 2008 Nov 20; 359(21): 2254-65.
16. Arnaez-Solis J, Ortega-Molina M, Cervera-Bravo A, et al.
Evaluation of twenty-three episodes of acute thoracic syndro-

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

16

me in Patients with Sickle Cell Anemia. An Pediatr (Barc.). 2005


Mar;62(3):221-8.
17. Mallhou AA, Asha M. Beneficial effect of blood transfusion in
children with sickle cell chest syndrome. Am J Dis Child. 1988
Feb;142(2):178-82.
18. Van-Dumen JC, Alves JG, Bernardino L, et al. Factors associated with sickle cell disease mortality among hospitalized
Angolan children and adolescents. West Afr J Med. 2007 OctDec;26(4):269-73.
19. Loureiro MM, Rozenfeld S. Epidemiology of Sickle Cell Disease hospital admission in Brazil. Rev Saude Publica. 2005
Dec;39(6):943-9. Epub 2005 Dec 7.
20. Manci EA, Culberson DE, Yang YM, et al. Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 2003
Oct;123(2):359-65.
21. Adams D, et al. Erythropheresis can reduce iron overload and
prevent the need for chelation therapy in chronically transfused pediatric patients. J Pediatr Hemat/Onco. 18(1) Feb 1996:46-50
22. Masera N, Tavecchia L. Periodic erythroexchange is an effective strategy for high risk paediatric patients with sickle-cell disease.
Transf Apher Sci. Dec 2007, 37(3):241-7.
23. Graham JK, Mosunjac M, Hanzlick RL, et al. Sickle Cell Disease and sudden death: retrospective/prospective study of 21 autopsy cases and literature review. Am J Forensic Med Pathol. 2007
Jun;28(2):168-72.

RESUMEN
Trasfondo: Pacientes con Anemia Falciforme (AF)
sufren de complicaciones que requieren transfusin simple y/o de intercambio. En 1999 desarrollamos una tcnica de intercambio automatizado
usando bombas de infusin y catteres vasculares (Mtodo IV Pump). Objetivo: Probar que el
Mtodo IV Pump es costo-efectivo, y tan seguro
y eficiente como los separadores automatizados.
Mtodos: Estudio retrospectivo de expedientes de
pacientes de AF que requirieron transfusin de intercambio, admitidos a UCI entre 2003-2009. Evaluamos mtodo utilizado, complicaciones, costos y
cambio en Hemoglobina S% (HgS%), excluyendo
pacientes que no requirieron transfusin de intercambio. Resultados: La reduccin en costo con
el Mtodo IV Pump es de alrededor $1000. Reduccin de HgS% promedio con Mtodo IV Pump
fue 30.3 vs. 28.8 en el grupo de Separador Automatizado (p = 0.84). No tuvimos complicaciones o
mortalidad, con la mayora de los pacientes siendo
masculinos (p = 0.03) y en el grupo de mayor edad
(11-19 aos) para ambos mtodos. Conclusin: El
Mtodo IV Pump es una alternativa segura, costoefectiva y eficiente para realizar transfusiones de
intercambio.

Preprese a navegar por un sinfin de posibilidades


www.asociacionmedicapr.org

RESPIRATORY ILLNESS IN
LATE PRETERM INFANTS
DURING THE FIRST SIX
MONTHS OF LIFE

ABSTRACT

Leilanie Prez MS1


Zahira Corchado 1
Mariela Rodrguez 1
Dora Garca 1
Lizaida Medina MD2
Arian Vicens MD2
Nerian Ortiz MD2
Lourdes Garca MD3
Yasmin Pedrogo MD2
From the UPR Rio Piedras and Medical Science Campus1,
Department of General Pediatrics2 and Section of Neonatology3 of the UPR School of Medicine, Puerto Rico Health
Science Center.
Address reprints requests to: Yasmin Pedrogo MD, UPR
School of Medicine, Department of Pediatrics, PO Box
365067, San Juan, PR 00936-5067, e-mail address yasmin.
pedrogo@upr.edu.

INTRODUCTION


ince 1990 late preterm births has climbed
more than 25% (1). The term late preterm was adopted for infants born between 34 and 36 6/7 weeks gestations (GA) by the National Institute of Child Health
and Human Development (2). This group of infants is
vulnerable to a variety of illnesses and developmental
risk, primarily because they are deprived of the last few
weeks of development in the uterus. Compared to term
infants, they have higher rates of morbidity and mortality
at birth and are more frequently readmitted to the hospital during the neonatal period (3). Late preterm infants
have higher frequencies of hypoglycemia, kernicterus,
respiratory disease and seizures (4).

Late preterm infants are physiologically immature


and at risk for respiratory complications. The studys
objective was to determine the incidence of respiratory illnesses in a group of preterm infants (33-35
weeks) during the first six months of life. Methods:
Parents were contacted by phone in the six months
period after participating in an educational program
and a short survey was performed. Results: None
of the infants required admission to the intensive care unit in the newborn period. According to
parents, 71% of the babies had a common cold,
9% bronchiolitis, and 3% pneumonia. Fifty four
percent of the babies visited the emergency room
due to respiratory illnesses and (12%) required admission. Conclusion: Late preterm infants present
respiratory illnesses during the first months of life
which result in medical expenditures, emergency
room visits, and hospital admissions. Educational
interventions about preventive measures are needed to decrease the morbidity associated to these
illnesses.
Index words: prematurity, low birth weight, respiratory syncitial virus

childhood bronchiolitis and 50% of childhood pneumonia. It has been also found as a risk factor for wheezing
later in childhood.

There are several preventive strategies for
RSV, which involve palivizumab prophylaxis, limiting
contact with infected individuals, good hand washing
and avoidance of second hand smoking. Not every
preterm infant receives palivizumab prophylaxis, only
high risk ones are considered for it. According to the
AAP, infants born between 33-35 weeks GA can receive prophylaxis if they are less than 3 months of age at
the start of RSV season and if they have at least one
of the following risk factors: attending to child care or
a sibling younger than 5 years of age. In addition they
can receive it if they have cyanotic or complicated congenital heart disease, chronic lung disease, congenital
abnormalities of the airway or neuromuscular disease
(8). In Puerto Rico, RSV infection is frequent, but there
is no surveillance system to determine the incidence
or its epidemic levels. It is estimated that 20% of bronchiolitis cases seen on a monthly basis are caused by
RSV in Puerto Rico (9).


In the United States late preterm infants (34- 36
weeks gestation) account for nearly three quarters of
all preterm births, yet little is known about their morbidity risk (5). In Puerto Rico the preterm deliveries rate
increased 29.3% (11.6 % to 15.0 %) from 1990 to 2000.
That increase is mainly attributable to an increase in the
rate of preterm births between 32-36 weeks gestation

Despite preventive measures, respiratory in(6).
fections continue to be one of the leading causes of

In addition, those infants are physiologically im- infant hospitalizations. In Puerto Rico there is no data
mature and at risk for respiratory complications. The about respiratory associated morbidity in late preterm
increased risk of respiratory disease can be mainly infants. Therefore, the purpose of this study is to deterattributed to three factors: (a) underdeveloped lungs, mine the incidence of respiratory illnesses in a group of
(b) an immature immune system, and (c) incomplete late preterm infant, born at the University District Hostransfer of maternal antibodies (7). In late preterm in- pital (UDH) of Puerto Rico, during the first six months
fants immunity is further compromise posing them at of life. This data will provide background information
an increased risk for infections, including Respiratory for further studies about the impact that this population
Syncitial Virus (RSV) (7). RSV accounts for 80% of has on public health and health costs.
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18

MATERIALS AND METHODS



A pilot, observational longitudinal study of
preterm infants (33-35 weeks gestation) born in the
University District Hospital (UDH) from October 2007
through October 2008 was performed. Parents of those infants were contacted by phone in a six months
period after participating in an educational program
about prevention of second hand smoking. A telephone survey was performed, which included incidence of
infants respiratory illnesses, emergency room visits,
hospital admissions and RSV bronchiolitis. The study
was approved by the University of Puerto Rico, Medical Sciences Campus, Institutional Review Board. Percentiles and proportions were used to interpret data.

RESULTS

Subjects included 32 parents. The mean gestational age of the newborns was 34 weeks (33-35 weeks gestation). None of the infants required admission to
the intensive care unit in the newborn period. Parents
reported that after being home: 71% of the babies had
a common cold, 9% bronchiolitis and 3% pneumonia
(Table I). Forty one percent (41%) of the babies visited
the emergency room and twenty-one (21) proportion
of that percent were due to respiratory illnesses. Fifty four percent (54%) of the total ER visits were due
to respiratory illnesses compare to 46% that were related to other illnesses other than respiratory. Thirty
one percent of infants (31%) required admission to the
hospital and forty (40%) of those admissions were due
to respiratory illnesses. Seventy four percent (74%)
of the parents considered themselves to have lack of
knowledge about bronchiolitis due to RSV.


In our study, the most common respiratory illnesses were: common cold illness, bronchiolitis, asthma and pneumonia. Hospital admissions and emergency room visits were mostly associated to respiratory
illnesses. Other research studies have demonstrated
that respiratory and gastrointestinal disorders are the
most common diagnoses for readmission during the
first year of life of late preterm infants (10). Another
study done in California showed that respiratory disorders are the most common cause of readmission for
infants born at 35 weeks gestation (11).

It is evident that infants born between 33-35
weeks GA require greater attention in their management. Physically they look as term infants, but actually they are physiologically immature posing them to
an increase morbidity and mortality rate. This study
showed that most of the parents have lack knowledge about bronchiolitis due to RSV and its prevention.
Educational interventions for parents about preventive
measures, including proper hand washing, palivizumab
prophylaxis, and second hand smoke avoidance are
needed in order to decrease the morbidity associated
to these illnesses. Physicians and nurses also need to
be educated about careful evaluation, monitoring and
follow up of late preterm infants. In addition, further studies are required as the data group in this research
was too small to obtain epidemiologic values.

In summary, infants between 33- 35 weeks of
gestational age in Puerto Rico are at increased risk
of developing respiratory diseases, hospital readmissions, and emergency room visits during their first six
months of life. In consequence, parents and health care
workers should be educated about the management,

Table I: Incidence of Respiratory Illnesses of Preterm Infants Less than Six Month
of Age

Development of Disease

No Development of Disease

Upper Respiratory Infection

71%

29%

Bronchiolitis

9%

91%

Pneumonia

3%

97%

DISCUSSION

Late preterm infants present respiratory associated illnesses during the first months of life, which
result in medical expenditures, emergency room visits,
and hospital admissions. The main target group of this
study was the infants between 33 to 35 weeks of gestation due to the overlapping of late preterm (34- 36
weeks gestation) infants and those considered for the
administration palivizumab prophylaxis (33-35 weeks
gestation). Those infants of 33 to 35 weeks of gestation
are at increased risk of developing respiratory illnesses
and hospital admissions after being discharge home
at birth. Other study also reports that the late preterm
group is at a higher risk for re-hospitalization (15.2%)
compare to term infants (7.9%) (10).

evaluation, and monitoring of those infants. In Puerto


Rico further studies are required in order for therapies,
prophylaxis, and monitoring strategies to be formally
evaluated and modified in the required cases.

REFERENCES
1.
Hamilton B, Martin J, & Ventura S. Births: Preliminary data
for 2007. National Vital Statistics Reports. 2009. 57: 123.
2.
Engle A, Tomashek K, & Wallman C. "Late-preterm" infants: A population at risk. Pediatrics. 2007. 120: 13901401.
3.
Shapiro-Mendoza C, Tomashek K, Kotelchuck M, & et al.
Risk factors for neonatal morbidity and mortality among healthy
late preterm newborns. Seminars in Perinatology. 2006. 30: 54
60.

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

19

4.
Raju A. et al. Optimizing care and outcome for late preterm (near term) infants: a summary or the workshop sponsored by
the National Institute of Child and Human Development. Pediatrics.
2006. 118: 1207-1214.
5.
Shapiro-Mendoza C, Tomashek KM, Kotelchuck M, & et
al. Effect of late preterm birth and maternal medical condition on
newborn morbidity risk. . Declerq E SO Pediatrics. 2008. 121: 22332.
6.
Varela R. Perez R. Duerr A. et al. Infant Heath Among
Puerto Ricans- Puerto Rico and U.S. Mainland, 1998-2000. CDC
MMWR. 2003. 52: 1012-1016
7.
Coffman S. et al. Late Preterm infants and risk for RSV.
The American Journal of Maternal/ Child Nursing. 2009. 34: 378384.
8.
Pickering L, Baker C, Kimberlin D, et al. Red Book: 2009
Report of the Comitee on Infectious Disease. 28th ed. Elk Grove
Village, IL: American Academy of Pediatrics. 2009: 560-569
9.
Molinari M, Garcia I, Garcia L, et al. Respiratory Syncitial
Virus- related bronchiolitis in Puerto Rico.
10.
Mclaurin K. Hall C. Jackson Ea. Et al. Persistence of morbidity and cost difference between late preterm and term infants
during the first year of life. Pediatrics 2009. 123: 653
11.
Underwood M. Danielsen B. Gilbert M. Cost, causes and
rates of rehospitalization of preterm infants. J Perinatol. 2007. 27:
614

AGREGUE LA INFORMACION DE SU
OFICINA A UN WEBSITE VISITADO POR
LOS PACIENTES QUE NECESITAN DE
SUS SERVICIOS

RESUMEN
Los infantes pre-termino tardos son inmaduros fisiolgicamente y estn a riesgo de complicaciones
respiratorias. El objetivo del estudio fue determinar
la incidencia de enfermedad respiratoria en infantes
pre-termino (33-35 semanas) durante los primeros
seis meses de vida. Mtodos: Los padres fueron
contactados va telefnica en un periodo de seis
meses, y se hizo un cuestionario. Resultados: Al
nacer, ninguno de los recin nacidos fue admitido a
la unidad de cuidado intensivo. De acuerdo a los
padres, 71% de los bebs tuvo catarro, 9% bronquiolitis y 3% pulmona. Veinte y uno por ciento
de los bebes visit la sala de emergencia debido a
enfermedades respiratorias y 12 % requiri admisin. Conclusin: Los infantes pre-trmino tardos
presentan enfermedades respiratorias durante los
primeros meses de vida, lo cual resulta en gastos
mdicos, visitas a salas de emergencia y admisiones a hospitales. Se necesitan intervenciones
educativas acerca de las medidas preventivas para
poder disminuir la morbilidad asociada a estas enfermedades.

PRENATAL
BREASTFEEDING
INTENTIONS IN
A GROUP OF WOMEN
WITH HIGH RISK
PREGNANCIES

ABSTRACT
The American Academy of Pediatrics recommends exclusive breastfeeding for the first 6 months of life. In spite of a limited number of contraindications, there are mothers who decide not to
breastfeed their infants. Objective: To determine
intention to breastfeed in women with high-risk
pregnancies. Methods: Pregnant women who
attended the Obstetrics high-risk clinics at the
University District Hospital answered a survey.
Results: Participants included 186 women. Mean
maternal age was 27 years (15-47) and mean
gestational age 27 weeks (9-41). Ninety-four percent intended to breastfeed. The most common
reason for not planning to was the use of medications. Breastfeeding intentions were associated
to higher education (p<0.01) and to confidence in
their babies being able to be breastfed (p<0.01).
Conclusions: Women with high risk pregnancies
showed high interest in breastfeeding their babies but there are misconceptions that need to
be addressed so that more high risk babies can
benefit from breastfeeding.

Hildamary Diaz Rozett MD*


Lourdes Garcia Fragoso MD**
From the * Department of Pediatrics and ** Neonataology
Section Department of Pediatrics, UPR School of Medicine.
Address reprints requests to: Lourdes Garca MD, UPR
School of Medicine, Department of Pediatrics, Neonatology Section, PO Box 365067, San Juan, PR 00936-5067. Email: lourdes.garcia1@upr.edu.
Poster presentation at the Annual Puerto Rico Pediatrics Society meeting (February 2009), the annual Medical Sciences
Campus Research Forum (April 2009), and at the 2009 ALAPE meeting (November 2009).

Index words: prenatal, breastfeeding, high risk,


pregnancy

INTRODUCTION

MATERIALS AND METHODS


he American Academy of Pediatrics recommends exclusive breastfeeding for the first six months
of life. Exclusive breastfeeding is the reference against
which all alternative feeding methods must be measured with regard to growth, health, development, and
all other outcomes. Healthy People provides sciencebased, 10-year national objectives for promoting health
and preventing disease. Since 1979, Healthy People
has set and monitored national health objectives to
meet a broad range of health needs, encourage collaborations across sectors, guide individuals toward
making informed health decisions, and measure the
impact of our prevention activity. The breastfeeding
goals for 2010 were that 75% of the mothers initiate
breastfeeding in the hospital and 50% continue up to
6 months. (1). This includes premature and other highrisk infants either by direct breastfeeding and/or using
the mother's own expressed milk. There are only a few
conditions under which breastfeeding may not be in the
best interest of the infant. In spite of a limited number of
contraindications, there are still many mothers who decide not to breastfeed their infants. A number of factors
may influence whether women choose to breastfeed.
Studies suggest that many women believe that breastfeeding is inconvenient or inconsistent with their lifestyles or work commitments (2). But what if a pregnant
woman has a health condition or knows that her fetus
has a congenital anomaly? Would this affect their intention to breastfeed? The objective of our study was to
determine the knowledge and beliefs about breastfeeding of women with high-risk pregnancies and establish
the factors that affect their decision to breastfeed.


Participants included women who attended
the High-risk Clinics at the University District Hospital, Obstetrics-Gynecology Department at the Puerto
Rico Medical Center from March 2008 through March
2009 and who voluntarily agreed to participate. An
anonymous questionnaire developed and validated
for this study was administered at the mothers visit to
the clinic. The study was approved by the University of
Puerto Rico, Medical Sciences Campus, Institutional
Review Board.

Data Analysis

Categorical variables were evaluated using frequency and percentages. Continuous variables were
evaluated by mean and range. The differences among
groups were evaluated using Chi-square. Statistix 8.0
software was used for the analysis.

RESULTS



One hundred and eighty six (N=186) pregnant
women participated in the study. The mean maternal
age was 27 years (15-47). The mean gestational age
was 27 weeks (9-41). Most of them (77%) were referred to the high risk clinics due to a maternal condition,
being the most prevalent: high blood pressure, diabetes, and younger age. Ninety-four percent had the intention to breastfeed their babies. The most common
reason for not planning to breastfeed was the use of
medications (36%) followed by perceiving it as painful
(18%) and just not desiring to do so (18%).

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21


Breastfeeding intentions were associated to
higher education (p<0.01) and to feeling confident
in their babies being able to be fed with breast milk
(p<0.01). Breastfeeding intentions were not associated to maternal age, gestational age at the time of the
survey, having their first baby, or expecting the baby to
remain in the hospital after birth (Table I). The reason
for referral to the high risk clinic was only associated to
breastfeeding plans in women who were referred to the
clinic due to preterm labor who were less likely to plan
breastfeeding.

Eighty-seven percent had received information
about breastfeeding, mostly by the Women, Infant,
Children (WIC) program (48%), a nurse (26%) or the
media (25%). Fifteen percent thought that babies with
medical conditions cannot breastfeed and twenty-one
percent thought that women with chronic conditions do
not produce enough milk.

DISCUSSION

Breastfeeding is associated with nutritional,
immunological, emotional, economic and social benefits. Therefore, it is of paramount importance that the
factors that lead to early weaning be identified so as
to provide infants with as long a breastfeeding period
as possible. Several studies confirm that among the
factors that lead to early weaning are problems related
to the risk of infant exposure to maternal medications
(3). This was the more common reason for women
in our study not to have the intention to breastfeed.
Despite this concern, many women continue to use
medications while breastfeeding. A survey was given
to a cohort of women who delivered their babies at a
single center. The participants were asked to record
the medications they had taken during pregnancy and
subsequently were contacted each month during lactation to determine what medications they had taken.
Breastfeeding women took significantly more medications per month than pregnant women. Women who
were breastfeeding also took prescription medications
more frequently than women who were pregnant. The
medications most often used by breastfeeding women
were multivitamins, nonsteroidal anti-inflammatory
drugs, acetaminophen, progestins, antimicrobials, and
decongestants (4).

The most common reasons for women in our
study to be referred to the high risk clinic were high
blood pressure and diabetes. Are medications to
treat these two conditions safe while breastfeeding?
Although all medications transfer into human milk,
most do so in amounts that are subclinical. The clinician should evaluate each medication carefully, examine published data on the drug, and advise the mother
carefully about the use of medications while breastfeeding. In most cases, the amount of drug in milk is far
less than 4% of the maternal dose and often is extremely subclinical (5).

Antihypertensives frequently are used early
postpartum, and as a family, their use requires a high
degree of caution. Several beta blockers (atenolol,

acebutolol) have been reported to produce cyanosis,


bradycardia, and hypotension in some breastfed infants.

The safest beta blockers for breastfeeding mothers include propranolol and metoprolol. In older infants, the beta blockers apparently pose a reduced risk.
Angiotensin-converting enzyme inhibitors, due to their
potency, should be used somewhat cautiously early
postpartum when infants exhibit borderline hypotension. Captopril and enalapril are preferred due to lower
milk concentrations. Of the calcium channel blockers,
nifedipine or verapamil are preferred. Other antihypertensives, such as hydralazine and methyldopa, are
used commonly in pregnant patients. Studies suggest
that their concentrations in human milk are low and do
not produce clinical changes in breastfed infants (5).
The use of oral hypoglycemic agents in pregnant and
breastfeeding mothers is increasing because diabetes is one of the most common endocrine disorders
affecting women during pregnancy (5). The available
data suggest that the levels of glyburide and glipizide in milk are negligible and would not be expected
to cause adverse effects in breastfed infants; however,
monitoring of the breastfed infant for signs of hypoglycemia is advisable during maternal therapy with any of
these agents. Treatment with metformin during lactation is unlikely to lead to toxicity in the breastfed infant.
Given the safety profile of metformin, as compared
with sulfonylureas, it is advisable to consider metformin as first-line treatment during lactation if this drug
is appropriate for the particular patient. Nevertheless,
second-generation sulfonylureas are also likely to be
safe during lactation (6).

The other reason given by pregnant women in
our study was the perception of it being painful followed
by just not desiring to do so. Multiple factors may influence whether women choose to breastfeed. A general lack of social support, fear of pain or breast sagging,
and modesty/embarrassment may also contribute to the
decision to infant formula-feed rather than breastfeed
(2). This study shows that breastfeeding intentions correlated with higher education and to feeling confident
in their babies being able to be fed with breast milk.
It also showed that women had misconceptions about
breastfeeding. Ryser (7) reported improved rates of
breastfeeding intention and initiation among low income women following a program with targeted educational message addressing negative attitudes and social
norms. Breastfeeding knowledge has been reported to
have a strong correlation with breastfeeding confidence, breastfeeding duration, and intention to breastfeed
(8). A study in California showed that lower education
was negatively and independently associated with intention to breastfeed (9). Wen et al (10) showed that
mothers who had a tertiary education were 1.5 times
more likely to be aware of the recommendation for exclusive breastfeeding for the first 6 months of life than
those who had school certificate or less. Kaufman et
al (11) studied African American and Puerto Rican women in Brooklyn and reported that while women in their
study felt that breastfeeding was the best way to feed
their infants, their commitment turned into ambivalence
in the face of their perceptions about the dangers of

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22

breastmilk, the virtues of formula, and the practical and


sociocultural challenges of breastfeeding. Womens
ambivalence resulted in widespread complementary
feeding pattern that included breastmilk and formula,
and resulted in short breastfeeding durations.

This group of women with high-risk pregnancies showed high interest in breastfeeding their babies
but also showed misconceptions about the effects of
medications and lack of confidence in being able to
breastfeed their babies. These women were followed
up at a high risk clinic seen frequently by an obstetrician. Nevertheless, obstetricians were not identified as
sources of information about breastfeeding. Physicians
taking care of women with medical conditions affecting
the pregnancy or fetus should reinforce the desire to
breastfeed among their patients and identify misconceptions that may deter women from giving their newborns the best available nutrition.

(9) Lee HJ, Rubio MR, Elo IT, McCollum KF, Chung EK, Culhane JF.
Factors associated with intention to breastfeed among low-income,
inner-city pregnant women. Matern Child Health J 2005;9(3):25361.
(10) Wen LM, Baur LA, Rissel C, Alperstein G, Simpson JM. Intention to breastfeed and awareness of health recommendations:
findings from first-time mothers in southwest Sydney, Australia. Int
Breastfeed J 2009;16(4):9.
(11) Kaufman L, Deenadayalan S, Karpati A. Breastfeeding ambivalence among low-income African American and Puerto Rican
women in north and central Brooklyn. Matern Child Health J 2009
Jul 31 [Epub ahead of print].

Table I: Factors associated to the intention to breastfeed (N=186)


Characteristic

Breastfeeding No Breastfeeding

(N=175)

p-value

(N=11)

Maternal age (years)

26

26

NSS

Gestational age (weeks)

29

24

NSS

High school or beyond

87%

45%

p<0.01

First pregnancy

38%

36%

NSS

Baby will remain in hospital after birth

19%

30%

NSS

Confident that baby will be fed breastmilk

94%

42%

p<0.01

Previous information about breastfeeding

83%

70%

NSS

Maternal education

REFERENCES

RESUMEN

(1) Healthy People 2020: The road ahead. US Department of Health


and Human Services. http://www.healthypeople.gov/HP2020. Accessed 12/29/09.
(2) Hill GJ, Arnett DB, Mauk E. Breastfeeding intentions among
low-income pregnant and lactating women. Am J Health Behav
2008;32(2):125-36.
(3) Chaves RG, Lamounier JA. Breastfeeding and maternal medications J Pediatr 2004;80(5 Suppl):S189-98.
(4) Stultz EE, Stokes JL, Shaffer ML, Paul IM, Berlin CM. Extent
of medication use in breastfeeding women. Breastfeed Med. 2007
Sep;2(3):145-51.
(5) Hale TW. Drug Therapy and Breastfeeding: Antibiotics, Analgesics, and Other Medications. NeoReviews 2005;6(5)e233.
(6) Glatstein MM, Djokanovic N, Garcia-Bournissen F, Finkelstein
Y, Koren G. Use of hypoglycemic drugs during lactation. Can Fam
Physician 2009;55(4):3713.
(7) Ryser FG. Breastfeeding attitudes, intention and initiation in low
income women: the effect of the best start program. J Hum Lact
2004;20:300-5.
(8) Chezem J, Friesen C, Boettcher j. Breastfeeding knowledge,
breastfeeding confidence and infant feeding plans: effects on actual
feeding practices. J Obstet Gynecol Neonatal Nurs 2003;32:4047.

La Academia Americana de Pediatra recomienda


la lactancia exclusiva por los primeros 6 meses de
vida. A pesar de el nmero limitado de contraindicaciones, hay madres que deciden no amamantar
a sus infantes. Objetivo: Determinar las intenciones
de lactar en mujeres con embarazos de alto riesgo. Mtodo: Mujeres embarazadas que visitaron la
Clnica de Alto Riesgo Obsttrico del Hospital Universitario de Distrito contestaron una encuesta. Resultados: Las participantes incluyeron 166 mujeres.
La edad promedio fue 27 aos (15-47) y la edad
gestacional promedio 27 semanas (9-41). Noventa
y cuatro porciento planificaba lactar. La razn ms
comn para no planificarlo fue el uso de medicamentos. Las intenciones de lactar se asociaron a
mayor nivel de educacin (p<0.01) y a la confianza
en que sus bebs pudieran ser alimentados con leche materna (p<0.01). Conclusin: Las mujeres con
embarazos de alto riesgo demostraron inters en
lactar a sus bebs pero existen ideas errneas que
necesitan ser atendidas para que ms bebs de alto
riesgo puedan beneficiarse de la lactancia.

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

23

MINOR HEAD INJURY


IN CHILDREN YOUNGER
THAN TWO YEARS OF
AGE: DESCRIPTION,
PREVALENCE AND
MANAGEMENT IN THE
EMERGENCY ROOM OF
THE PEDIATRIC
UNIVERSITY HOSPITAL

ABSTRACT

Mara L. Fernndez MS*


Linette Mejas MS*
Nerian Ortiz MD**
Lourdes Garca-Fragoso MD**
From the *School of Medicine, **Department of General Pediatrics, UPR School of Medicine.
Address reprints request to: Nerian Ortiz MD, UPR School
of Medicine, Department of Pediatrics, PO Box 365067, San
Juan, PR 00936-5067. E-mail nerian.ortiz@upr.edu.

INTRODUCTION

Background: In children less than two years old,


minor head trauma can result in intracranial injury. No known studies exist that determine the
number of children younger than two years old
who visit the emergency room (ER) due to minor
head injury in Puerto Rico. Objective: To determine the prevalence of children with minor head
trauma and describe related issues. Methods:
Information was gathered from the medical records of children 0 to 2 years old who visited
the University Pediatric Hospital ER from 20042006. Several factors were analyzed. Results:
From our 136 subjects, there was a male prevalence of 59%. The predominant reason for head
injury was a fall (86%). There was abuse in 7%
of the subjects. Eighty-five percent (85%) of injuries occurred at home. Conclusions: The most
common etiology of head trauma was a fall at
home. The prevalence of abuse in 7% of these
children should alert physicians.
Index words: head trauma, infants, injury, falls

MATERIALS AND METHODS


n children less than two years old, minor head
trauma is a common injury that can result in skull fracture and intracranial injury (1). Head trauma is one of the
most common childhood injuries, annually accounting
for approximately 600,000 emergency department visits, 95,000 hospital admissions, and 550,000 hospital
days; hospital care costs alone exceed 1 billion dollars
per year (2-5). Most children receive a head injury as a
result of a fall (6). Mild head injury is usually considered
an insignificant event as any alteration of consciousness is transient and medical intervention is rare. Most
patients with mild head injury are currently discharged
from the emergency department without sequelae after
a brief observation period. Although the great majority
recovers, apparently uneventfully, the risk of life-threatening intracranial complication dominates attitudes to
management and requires a structured, logical approach. Despite the frequent occurrence of head injury in
children, diagnostic strategies differ among individuals
and institutions. To our knowledge, there are no previous studies that determine the mechanism of injury or
the number of children younger than two years old who
visit the emergency rooms due to minor head injury in
Puerto Rico. Also, no established protocol exists concerning the adequate management of these patients in
the hospitals in Puerto Rico. The purpose of this study
was to determine the prevalence of children with minor
head trauma who visit the emergency room of the University Pediatric Hospital and describe related issues
concerning minor head injury.


Information was gathered from the medical records of children 0-2 years old who visited the emergency room of the University Pediatric Hospital in San
Juan, Puerto Rico with a chief complaint of head trauma from January 2004 through December 2006.

Minor head injury has been described before by several authors (5) as patients with a Glasgow
Coma Scale score of > 13 and a history of loss of consciousness or amnesia for the event; loss of consciousness if evident of less than 20 minutes, hospitalization
of equal or less than 2 days, if this occurred and no
evidence of skull fracture; based on a Glasgow Coma
Scale score on arrival at the hospital of 15. For the
purpose of this study, minor head injury was defined
as a Glasgow score >13, loss of consciousness of less
than 1 minute, negative neurological examination, normal mental status, no skull fracture, vomiting less than
5 times without worsening, and normal head computed
tomography (CT) scan. Data gathered included gender and age group distribution, mechanism of injury,
anatomical location of the injury, location of the subject
at the time of injury, neurological findings on physical
examination, radiological tests performed, where the
patient was sent for observation, and consultations to
other specialists.

Statistical analysis of collected data was done
by using frequency, mean and range. Differences
among groups were evaluated using Chi-square and
Pearson t-test. A p-value less than 0.05 was considered

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

26

statistically significant. The study was approved by the


Medical Sciences Campus Institutional Review Board.

RESULTS

The study included 136 subjects between the
ages 0 to 2 years old. Table I shows the demographic
characteristics of the subjects. The predominant reasons for head injury were falls (86%) followed by suspicion of abuse (7%), and motor vehicle accidents (4%).
Eighty-five percent (85%) of these injuries occurred at
home and in all the cases there was an adult present at
the moment of the injury. The most common anatomical location for the lesion was the parietal region of the
skull (52%). Specific neurological findings analyzed
included crying (84%); hematoma (40%); scalp swelling (19%); vomiting (18%); and loss of consciousness
(2%). The mean Glasgow Coma Scale was 14 (range
13-15).

The radiological tests performed were CT
(58%); skull radiographs (34%); and C-spine (16%).
Thirty percent of the infants who had a skull radiograph
performed had a fracture. A CT scan was performed
more commonly in infants with a skull x-ray showing a
fracture (86% of patients with fracture vs. 27% of patients without fracture [OR 16, 95% CI 2.98-86, p<0.01].
It was also performed more commonly in infants less
than one year old (74% of infants less than 1 year old
vs. 41% of those 1-2 years of age [OR 3.97, 95% CI
1.9-8.2, p<0.01]. Sixty-seven percent (67%) of the CT
scans had positive findings related to injury. The most
common CT scan finding was fractures (51%) followed
by bleeding (44%), and edema (9%). The finding of a
fracture in the CT scan was associated to the presence of a hematoma [OR 3.9, 95% CI 1.5-10.1, p<0.01],
or bleeding [OR 6.7, 95% CI 2.4-18.5, p<0.01] in the
physical exam. There was no association between
fractures and loss of consciousness, persistent crying,
vomiting, swelling, or seizures. The finding of bleeding
in the CT scan was also associated to the presence of
a hematoma [OR 24.7, 95% CI 7.4-81.6, p<0.01] in the
physical exam.

Ninety-six percent (96%) of the patients remained at the emergency room for observation and 4%
were sent home. The median length of observation
was 9 hours (range (0.5 - 71 hours). Eleven percent
(11%) of the children was admitted to the hospital. The
median length of stay for hospitalized patients was 4
days (range 1-27 days). From the subjects that were
considered for consultation, 66% were evaluated by a
neurosurgeon, 18% by a neurologist, and 51% by a
social worker.

DISCUSSION

Anatomically, the head of the child represents a
relatively greater proportion of mass and body surface
area. Furthermore, stability largely depends on ligamentous rather than bony integrity. The pediatric brain
has higher water content and is thus softer and more
susceptible to acceleration-deceleration injuries. The
unmyelinated brain is thus more susceptible to shear
injuries than surgically treatable lesions. Infants and

young children tolerate increases in intracranial pressure better than adults because the cranial sutures are
not fused and the thin skull is more compliant than in
adults.

The mechanism of head injury in pediatric populations differs by age. In infants and children less
than 2 years of age, head injury usually results from
falls from furniture or a caregivers arms (1). This study
is consistent with that but the finding of suspicion of
abuse in 7% of the subjects was alarming. It represents
that seven out of 100 children who visited the emergency room due to head injury were potential victims
of abuse. Child abuse has to be suspected in order for
it to be identified. A childs immature judgment, vulnerability to child abuse, dependency on adult supervision, and exposure to inadequate safety measures in
play areas enhance the risk of injury (2). Clinicians in
charge of initial evaluations of infants presenting to the
emergency room with head injury need to be aware of
this possibility. The fact that 51% of the subjects in this
study were evaluated by a social worker could mean
that child abuse or neglect was a concern that needed
to be ruled out.

Our study showed a male predominance as
previously documented in the literature (3) with 47%
of them between 12 to 24 months old which coincides with the time when children are learning to walk
and are active exploring their surroundings. The mean
Glasgow Coma Scale for the patients was documented
as 14 and the specific neurological findings included
hematoma, scalp swelling, vomiting, and loss of consciousness. It is important to take into consideration that
84% of the children cried after sustaining the head injury. The significance of these signs is unclear since
these findings can occur frequently in those children
who have intracranial injuries but also in those who do
not (4). The anatomical area most commonly affected
was the parietal region of the skull. Minor head injury
occurred mainly at home and in all the cases there was
an adult present at the moment of the injury. Once the
children were taken to the emergency room, a CT was
performed to 58% of the children and skull radiograph
was performed to 34% of the children. The literature
has not been clear about which of the two, CT or skull
radiograph, is the most appropriate modality to use.
Some authors suggest that CT must be done to children younger that two years old always. Because significant intracranial injuries can occur in the absence
of skull fractures, skull radiographs are not generally
recommended for screening when a CT is readily available (5). The fact that neurological symptoms (loss of
consciousness, crying, vomiting, seizures) in our study
did not correlate with intracranial injury suggests that a
more careful approach should be taken with these children. Findings such as a hematoma and bleeding in the
physical exam should prompt the physician to strongly
consider a CT scan for head injury evaluation.

Most children were observed in the emergency
room and 66% of them were consulted to the neurosurgery service. These children were not followed up
after they left the ER, and information regarding neurological outcomes after sustaining a head injury was
beyond the scope of the study.

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

27

A comprehensive approach to the head injured child


begins in the pre-hospital area and continues through
acute hospital management and ultimate rehabilitation. Predetermined protocols facilitate accurate assessment and treatment during the initial moments of
care (6). It is important for every institution evaluating
young children with minor head trauma to develop protocols for the uniform evaluation of these children.
Table I: General characteristics of the participants
(N=136)
Characteristics

3.
Homer CJ, Lawrence K. Technical report: head injury in
children. Pediatrics 1999;104:78-84.
4.
Roddy SP, Cohn SM, Moller BA, Duncan CC, Gosche JR,
Seashore JH, Touloukian RJ. Minimal head trauma in children revisited: is routine hospitalization required? Pediatrics 1998;101:575577.
5.
Quayle KS, Jaffe DM, Kuppermann N, Kaufman BA, Lee
BCP, Park TS, McAlister WH. Diagnostic testing for acute head
injury in children: when are head computed tomography and skull
radiographs indicated? Pediatrics 1997;99:11-18.
6.
McKinlay A, Dalrymple-Alford JC, Horwood LJ, Fergusson DM. Long term psychosocial outcomes after mild head injury
in early childhood. J Neurol Neurosurg Psychiatry 2002;73:281288.

Participants

Age

0 3 months old

21%

4 6 months old

14%

7 9 months old

13%

10 12 months old

5%

1 - 2 years old

47%

RESUMEN
Introduccin: En nios menores de dos aos, trauma menor a la cabeza puede resultar en dao intracraneal. No se conocen estudios que determinen la prevalencia de nios menores de dos aos
que visitan la sala de emergencia debido a trauma
menor de cabeza. Objetivo: Determinar la prevalencia de nios menores de dos aos que visitan
la Sala de Emergencia del Hospital Peditrico Universitario debido a trauma menor de cabeza. Mtodos: Se obtuvo informacin de los expedientes
mdicos de pacientes de 0-2 aos que visitaron
la sala de emergencia del Hospital Peditrico Universitario en los aos 2004-2006. Se analizaron
varios factores. Resultados: De una muestra de
136, se observ una prevalencia masculina de
59%. La razn principal del trauma fue una cada
(86%). Se observ abuso en 7% de los sujetos.
Conclusiones: La etiologa ms comn fue una
cada en la casa. La prevalencia de abuso debe
alertar a los mdicos.

Gender

Males

59%

Females

41%

REFERENCES
1.
Schutzman SA, Barnes P, Duhaime AC, Greenes D, Homer C, Jaffe D, Lewis RJ, Luerssen TG, Schunk J. Evaluation
and management of children younger than two years old with
apparently minor head trauma: proposed guidelines. Pediatrics
2001;107:983-993.
2.
Committee on Quality Improvement, American Academy
of Pediatrics and Commission on Clinical Policies and Research,
American Academy of Family Physicians. The management of
minor closed head injury in children. Pediatrics. 1999;104:14071415.

Cada da es ms importante la utilizacin de la tecnologa informtica en todas las


profesiones.
La Asociacin Mdica de Puerto Rico est colaborando con la clase mdica para
que se incorpore adecuadamente a la misma.
Mantngase informado envindonos su direccion email a travs de nuestro registro en www.asociacionmedicapr.org
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

28

B LETN

ASOCIACIN MDICA DE PUERTO RICO

Instrucciones para los Autores

Slo sern considerados los artculos que cumplan estas instrucciones.

Instructions to Authors

We will take only articles that follow this instructions.

ACEPTAMOS SOLO DOCUMENTOS DIGITALES

WE ACCEPT DIGITAL DOCUMENTS, ONLY

El Boletn acepta para publicacin artculos relativos a medicina,


ciruga y las ciencias afines. Igualmente acepta artculos especiales
y correspondencia que pudiera ser de inters general para la profesin
mdica. Se requiere que los autores se esfuercen en perseguir claridad,
brevedad, e ir a lo pertinente en sus escritos, no importa el tema o formato
del manuscrito. El artculo, si se aceptara, ser con la condicin de que se
publicara nicamente en la revista.

The Boletn will accept for publication contributions relating to the various
areas of medicine, surgery and allied medical sciences. Special articles
and correspondence on subjects of general interest to physicians will also
be accepted. All material is accepted with the understanding that is to be
published solely in this journal. All authors are urged to seek clarity, brevity,
and pertinence in the manuscripts regardless of subject or format.

FORMATO:

FORMAT:

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texto justificado, espacio simple, doble espacio entre parrafos. Titulos en
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justified, single space, double space between paragraphs. Tittles in bold
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El articulo debe comenzar con una breve introduccin en la cual se
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Las referencias deben seguir el patrn que se describe a continuacin.
1. Para artculos de revistas: Apellido(s) e iniciales del nombre del autor(es),
ttulo del artculo, nombre de la revista, ao, volumen, pginas. Por ejemplo:
Villavicencio R: Soplos inocentes en pediatra, Bol Asoc Md P Rico 198 1;
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nmero de edicin, ciudad, casa editora, ao y pgina. Por ejemplo: Keith
JD, Rowe RD, Vlad P: Heart disease in infancy and childhood, 3d. Ed., New
York, MacMillan, 1978: 789
3. Para citacin de libros donde el editor(es) no es el autor(es) del captulo
citado se aade el autor(es) del captulo y el ttulo del mismo. Por ejemplo:
Olley PM: Cardiac arrythmias; In: Keith ID, Rowe RD, Vlad P Eds. Heart
disease in infancy and childhood, 3d Ed., New York, MacMillan, 1978: 275301

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which should state its purpose. The main sections (for example, Materials
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Generic names of drugs should be used; trade names my also be given
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Abstract
An abstract not longer than 250 words for clinical studies and no longer than
150 words for case reports and reviews. It must include the main points that
present the core of the article and the exposition of the problem, method,
results, and conclusions. The Abstract should be written both in Spanish
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should be enclosed in parentheses on the line or writing and not as
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should be listed in the numerical order in which they are first cited in the
text.The titles of journals should be abbreviated according to the style used
in the "Cumulative Index Medicus" published by the American Medical
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1. For periodicals: Surname and initials of author(s), title of article, name
of journal, year, volume, pages. For example: Villavicencio R.: Soplos
inocentes en pediatra. Bol Asoc Med P Rico 198 1; 73: 479 87. If there are
more than 7 authors list only 3 and add et al.
2. For books when the authors of the cited chapter is at the same time the
editor: Surname and initials of author(s), title, edition, city, publishing house,
~ear and page. For example: Keith JD, Rowe RD, Vlad P: Heart disease in
infancy and childhood, 3d Ed., New York, MacMillan, 1978: 789
3. For chapter in book when the author of the chapter is not one of the Olley
PM: Cardiac arrythmias: In: Keith JD, Rowe RD, Vlad P. Eds. Heart disease
in infancy and childhood, 3d Ed. New York, MacMillan, 1978, 275-301

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ROOMING-IN IMPROVES
BREASTFEEDING
INITIATION RATES IN A
COMMUNITY HOSPITAL
IN PUERTO RICO

ABSTRACT
Several studies have shown that rooming-in can
have a positive impact on lactation success. The
objective of this study was to assess if the establishment of rooming-in in a community hospital has
an impact on breastfeeding success. Methods: Participants included volunteer women who delivered
babies at a community hospital. The participants
answered a survey. Results: Sixty females were
included. The mean age was 23 years (18-38) and
the mean gestational age 38 weeks (34-41). Breastfeeding in the hospital was reported by 54% with
6% doing it exclusively. Rooming-in was practiced
by 70%. Rooming-in was associated to breastfeeding (OR 5.0, 95% CI 1.5-16.9, p=0.0069). Thirtypercent of the babies in rooming-in started breastfeeding during the first 6 hours after delivery as
compared to none of those not roomed-in. Conclusion: The advantages offered by the rooming-in
practice in the maternity ward of this hospital resulted in successful breastfeeding in this group of
mothers.

Carmen W. Cotto MD*


Lourdes Garcia Fragoso MD
From the *Department of General Pediatrics and the **Section of Neonatology, UPR School of Medicine.
Address reprints requests to: Lourdes Garca MD, UPR
School of Medicine, Department of Pediatrics, Neonatology
Section, PO Box 365067, San Juan, PR 00936-5067Email:
lourdes.garcia1@upr.edu.

INTRODUCTION


ooming-in is the practice where the baby
is placed on the mothers bed and is cared by the mother while she is in the hospital. Several studies have
shown that rooming-in can have a positive impact on
lactation success. Rooming-in enables the mother to
respond whenever their infants show signs of readiness to feed, and this helps to establish a good milk
flow. The World Health Organization has developed
the Baby Friendly Hospital Initiative with 10 steps to
successful breastfeeding (1). One of these steps is the
practice of rooming-in where mothers with healthy babies should stay with them in the same room day and
night, except for periods up to an hour for hospital procedures. At present, there are no Baby Friendly Hospitals in Puerto Rico and most do not practice rooming-in
in the maternity wards. This study intended to assess if
the establishment of rooming-in in a community hospital has an impact on breastfeeding success.

MATERIAL AND METHODS



Participants included volunteer adult women
who delivered babies at the Carolina University Hospital in Puerto Rico. This is a community hospital giving
obstetric services mostly to a low socio-economical
status population from the east of Puerto Rico. The
hospital has a partial rooming-in policy where babies
stay with their mothers for 12 hours during the day if
they choose to do so. The nursery has a breastfeeding
room available day and night. The participants answered an eighteen items anonymous survey on the day
of the newborns discharge from the hospital. The recruitment of participants occurred during the months of
February and March of 2006. The University of Puerto
Rico, medical Sciences Campus, Institutional Review
Board and the Privacy Board approved the study.


In this study, the breastfeeding terms and definitions of the Interagency Group Action on Breastfeeding

Index words: rooming, breastfeeding, community


hospital, Puerto Rico
were used (2). Breastfeeding was defined as either fully or partially breastfeeding the newborn. Fully breastfed infants received breast milk as their only source of
milk. Partially breastfed infants received breast milk in
combination with infant formula. Formulafed infants
received infant formula as their sole source of nutrition.

Statistical analysis



Frequency distributions and percents were used
to describe the categorical variables. Means, median,
and ranges were used to describe the continuous variables. The presence of statistical associations was
evaluated using Pearsons chi-square or Fishers exact
test, when appropriate, The Student t-test, when appropriate, compared quantitative variables between two
groups. The level of significance was p<0.05. Data
entry and statistical analysis was performed using Statistix 8.0.

RESULTS



Sixty females were included in the study. Table
I summarizes the general characteristics of the participants. The mean age was 23 years and the mean
gestational age was 38 weeks. Fifty-four percent of
the mothers reported breastfeeding in the hospital. Six
percent of the mothers practiced exclusive breastfeeding. Table II shows the analysis of factors associated
to the practice of rooming-in. The rooming-in practice
was significantly associated to breastfeeding (OR 5.0,
95% CI 1.5-16.9). Women who used the rooming-in
were more likely to breastfeed their babies (66% vs
28%, p=0.0069). Thirty-percent of the women practicing rooming-in started breastfeeding during the first

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

30

Table III: Factors associated to breastfeeding


(N=60)

6 hours after delivery as compared to none of those


whose babies were at the nursery.

Other factors evaluated that resulted in no association to breastfeeding included: maternal age and
education, type of delivery, birth weight, and gestational age at birth (Table III). Seventy-four percent of
the participants stated that they would combine breast
milk and formula after getting home with the baby. Only
10% had plans to breastfeed exclusively.
Table I: General characteristics of the participants
(N=60)
Characteristics

Participants

Age in years, mean

23(18-38)

Education

Less than high school

23%

High school

72%

University

5%

Characteristics

(1)

(2)

p value

Maternal age (years)

23

23

NSS

Gestational age (weeks)

38

38

NSS

Cesarean section

50% 26% NSS

Birth weight (pounds)

7.0

Rooming-in

84% 52% 0.0069

Used breastfeeding room

63% 22% 0.0019

Mother hospital stay (mean) 3 days 3 days NSS

Delivery

Gestational age at delivery, weeks 38(34-41)

DISCUSSION

Cesarean section delivery

Mothers hospital stay, mean 3 days (1-21)


Babies hospital stay, mean 3 days (2-7)

Rooming-in

70%

Breastfed in hospital

54%

6%

Exclusive breastfeeding

Table II: Factors associated to the practice of rooming-in (N=60)


Characteristics

(1)

(2)

p value

23

23

NSS

Gestational age (weeks)

38

38

NSS

Cesarean section

43% 17% NSS

Birth weight (pounds)

7.0

Breastfed in hospital

66% 28% 0.0069

Used breastfeeding room

40% 50% NSS

Maternal age (years)

7.0

NSS

Babies hospital stay (mean) 3 days 4 days NSS


Mother hospital stay (mean) 3 days 4 days NSS
(1) Rooming-in
(2) No rooming-in
NSS not statistically significant

NSS

Babies hospital stay (mean) 3 days 3 days NSS


(1) Breastfeeding
(2) No breastfeeding
NSS not statistically significant

40%

6.8



Rooming-in has been shown to have a relation
to breastfeeding initiation and success worldwide. In
1983, Procianoy and colleagues (3) reported an association between rooming-in and the plans to continue
breastfeeding after discharge. In Nicaragua, StrachanLindenberg and coworkers showed that at one week
postpartum the percentage of mothers fully breastfeeding was higher in the group who were roomed-in. At 4
months, the rate of breastfeeding was also higher (4).
A study in Japan showed that breastfeeding frequency
was significantly higher in infants rooming-in (5). Nevertheless, the benefits of rooming- in may have only
short-term beneficial effects requiring breastfeeding
guidance so that the effect is sustained (6).

Hospital practices can affect breastfeeding rates. A study in Colorado showed that breastfeeding
duration improved significantly when mothers experienced five specific hospital practices: breastfeeding
within the first hour, breastmilk only, infant rooming-in,
no pacifier use, and receipt of a telephone number for
use after discharge (7). In another study (8), the practices most consistently associated with breastfeeding
beyond 6 weeks were initiation within 1 hour of birth,
giving only breast milk, and not using pacifiers. Bringing the infant to the room for feeding at night if not
rooming in and not giving pain medications to the mother during delivery were also protective against early
breastfeeding termination. Compared with the mothers
who experienced "Baby-Friendly" practices, mothers
who experienced none were approximately 13 times
more likely to stop breastfeeding early. More recently,
(9) a study from Italy found that cesarean section, lack
of information about the advantages of breastfeeding,
and absence or partial absence of rooming-in increase
the risk of complementary breastfeeding during hospital stay. In turn, complementary breastfeeding during

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31

hospital stay was the major factor associated with not


breastfeeding at 1 month of age.

There are other benefits of rooming-in in addition
to breastfeeding. The mother-infant relationship may
be affected by rooming-in (1). OConnor and coworkers
found more cases of substantial parental abuse or neglect in a group not roomed-in (10). More recently, a
study found that mothers not rooming-in scored closeness to their babies as less important than those who
roomed-in with their babies (11). An interesting finding
in that study was that mothers who left their babies in
the nursery at night more often perceived that the staff
believed their babies should stay at the nursery, rating
closeness between mother and infant lower. This states the importance of not only having the practice of
rooming-in established in the hospital but educating
the staff about its benefits.


Breastfeeding rates in Puerto Rico are below
the 2010 Healthy People Goals. In Puerto Rico many
hospitals do not practice rooming-in. Understaffing and
safety concerns have prevented its establishment in
some hospitals. Others, as this community hospital,
have establish a partial rooming-in policy. Although
this is not the ideal situation, it allows the mother more
one on one contact with her baby during their hospital
stay. In this group of mothers, this made a difference
in the establishment of breastfeeding, showing higher
rates among those women who practiced the roomingin. The number of women breastfeeding exclusively
was very low. Culture may play a role as Puerto Rican
women seem to combine breast milk and formula quite frequently. Kaufman (12) reported that low-income
Puerto Rican women living in New York felt that breastfeeding was the best way to feed their infants but their
commitment turned into ambivalence in the face of
their perceptions about the dangers of breast milk, the
virtues of formula, and the practical and sociocultural
challenges of breastfeeding. Womens ambivalence resulted in a widespread complementary feeding pattern
that included breast milk and formula, and resulted in
short breastfeeding durations.


The number of mothers who were able to breastfeed their baby during the first 6 hours after delivery
increased with the rooming-in practice. This is still far
from the Baby Friendly Initiative goal to help mothers
initiate breastfeeding within 0.5 hours of birth. One aspect not assessed in this study was the reasons for
not practicing the rooming-in. In this hospital, this is a
voluntary practice since the hospital has a well baby
nursery with staff to care for the babies 24 hours a day.
Studying this further may help us eliminate other barriers to breastfeeding success.

4.
Strachan-Lindenberg C, Cabrera-Artola R, Jimenez V. The
effect of early post-partum mother-infant contact and breastfeeding
promotion on the incidence and continuation of breast-feeding. International Journal of Nursing Studies, 1990;27(3):179-86.
5.
Yamauchi Y, Yamanouchi I. The relationship between rooming in/not rooming-in and breastfeeding variables. Acta Paediatr
Scand 1990;79(11):1017-22.
6.
Perez-Escamilla R, Segura- Millan S, Pollit E, Dewey KG.
Effect of the maternity ward system on the lactation success of lowincome urban Mexican women. Early Human Dev 1992;31(1):2540.
7.
Murray EK, Ricketts S, Dellaport J. Hospital practices that
increase breastfeeding duration: results from a population-based
study. Birth. 2007;34(3):202-11.
8.
DiGirolamo AM, Grummer-Strawn LM, Fein SB Effect of
maternity-care practices on breastfeeding. Pediatrics. 2008;122
Suppl 2:S43-9.
9.
Asole S, Spinelli A, Antinucci LE, Di Lallo D. Effect of hospital practices on breastfeeding: a survey in the Italian Region of
Lazio. J Hum Lact 2009;25(3):333-40. Epub 2009 Apr 7.
10.
O'Connor S, Vietze PM, Sherrod KB, Sandler HM, Altemeier III WA. Reduced incidence of parenting inadequacy following
rooming-in. Pediatrics 1980;66(2):176-82.
11.
Svensson K, Matthiesen AS, Widstrom AM. Night roomingin: who decides? An example of staff influence on mothers attitude.
Birth 2005;32(2):99-106.
12.
Kaufman L, Deenadayalan S, Karpati A. Breastfeeding
ambivalence among low-income African American and Puerto Rican women in north and central Brooklyn. Matern Child Health J
2009 Jul 31. [Epub ahead of print].

RESUMEN
Varios estudios han demostrado que el alojamiento en conjunto tiene un impacto positivo en el xito de la lactancia. El objetivo
de este estudio fue determinar si el establecimiento del alojamiento en conjunto en un
hospital de la comunidad tiene impacto en
el xito de la lactancia. Mtodos: Los participantes incluyeron mujeres voluntarias que
dieron a luz en un hospital de la comunidad.
Las participantes contestaron una encuesta.
Resultados: Se incluyeron 60 mujeres. La
edad promedio fue 23 aos (18-38) y la edad
gestacional promedio fue 38 semanas (3441). Cincuenta y cuatro por ciento lactaron
en el hospital y 6% lo hicieron de manera
exclusiva. Setenta por ciento escogieron el
alojamiento en conjunto el cual se asoci a
un mayor nmero de madres que lactaron
(OR 5.0, 95% CI 1.5-16.9, p=0.0069). Conclusin: Las ventajas ofrecidas por el alojamiento en conjunto en la sala de maternidad
de este hospital resultaron en una lactancia
exitosa para este grupo de madres.

REFERENCES
1.
World Health Organization, Division of Child Health and
Development. Evidence for the ten steps to successful breastfeeding. 1998. Geneva. Pages 62-7.
2.
Labbok M, Krasovec K. Toward consistency in breastfeeding definitions. Stud Fam Plann 1990;21(4):226-30.
3.
Procianoy RS; Fernandes-Filho PH, et al. The Influence of
rooming in on breastfeeding. J Trop Pediatr 1983;29(2):112-14.

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32

Review Articles - Artculos de Resea


ABSTRACT
Esophageal atresia is the most common congenital anomaly of the esophagus in newborns. This review article
discusses the incidence, embryological classification, diagnosis and associated anomalies of esophageal atresia. Emphasis is placed in the current
guidelines of standard surgical management of this congenital condition.
Index words: esophagus, atresia, guidelines, management

ESOPHAGEAL ATRESIA:
NEW GUIDELINES
IN MANAGEMENT
Jessica Gonzlez-Hernndez MS*
Humberto Lugo-Vicente MD**

From the * UPR School of Medicine, and ** Section of Pediatric Surgery, Department of Surgery, UPR School of Medicine, Puerto Rico Health Science Center, San Juan, PR.
Address reprints requests to: Humberto Lugo-Vicente MD
PO Box 10426, San Juan, PR 00922. E-mail: titolugo@
coqui.net.

INTRODUCTION

EMBRYOLOGY/CLASSIFICATION


Esophageal Atresia (EA) is the most common
congenital anomaly of the esophagus. Most cases
of EA are associated with tracheo-esophageal fistula
(TEF). More than three centuries has elapsed since the
first reported case. Following Leven, Ladd and Haight
adversities to accomplish successful repair of this defect, the morbidity and mortality of EA has improved
in response to the development of neonatal intensive
care units (NICU), use of parenteral nutrition, better
surgical techniques, improved antibiotics and prenatal
diagnosis.1-4


The esophagus and trachea develop from the
foregut bud. During the fourth week of fetal development the trachea forms as a ventral diverticulum of this
bud. Abnormal embryogenesis of the laryngeo-tracheoesophageal groove and subsequent septum formation
allows persistent communication between the trachea
and esophagus. The distal esophagus elongation occurs with the heart and lung development reaching a
maximum length by the seventh week. The proximalthird striated muscle develops from the pharyngeal
pouches and its blood supply comes from the thyrocervical trunk. Aggressive surgical dissection of this portion is permitted due to the submucous blood supply.
The middle and distal esophagus has smooth muscle
derived from dorsal mesenchymal tissue and its blood
supply arise from segmental roots of the aorta.15 Aggressive surgical dissection of this segment leads to
ischemia.

INCIDENCE

EA occurs in one of every 2500 to 5000 live births with a slight male predominance.5-7 Risk of having
a baby with EA increases with the first pregnancy, older
mother and twin pregnancy. Familial cases have been
reported.1, 6, 8, 9 Teratogenic influence has been identified after prolonged use of estrogen, progesterone or
thalidomide during pregnancy, and infants born to diabetic mothers. An experimental model of EA was developed in 1996 by exposing fetal rats to Adriamycin.
In 2004, the Adriamycin rat model was used to make
a correlation between EA, amniotic fluid volume variations and other visceral malformations.10

Chromosomal abnormalities are frequent in
children with EA. They include Trisomy 13, 18 (Edward)
and 21 (Down) along with the VACTERL association.7,
11, 12
A deletion of band 11.2 of the long arm of chromosome 22 has also been associated with EA.13 Recently,
a deletion in chromosome band 17q22-q23.2 was associated with esophageal atresia, tracheoesophageal
fistula and conductive hearing loss.14


Published evidence using the Adriamycin rat
model has revealed that EA forms as a blind-ending
pouch and the distal TEF develops as the middle branch
of a tracheal trifurcation. The distal foregut anlage is
switched toward a pulmonary phenotype, it trifurcates
and its middle branch grows caudally to fistulized into
the stomach.16 This theory encompassing a respiratory
origin to the distal esophagus explains the poor motility
observed, the presence of airway cartilage in the histology of the lesion and presence of ectopic esophageal
lungs.

Several variations of EA have been described
(see Figure 1). Most common variant anomaly (87%) is
proximal EA associated with distal TEF. The proximal
esophagus is hypertrophied and dilated from amniotic

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liquid pressure. Impingement on the trachea by the


dilated pouch is the cause of faulty cartilaginous ring
development also known as tracheomalacia. Second
most common anomaly is pure EA (8%) associated
with a poorly developed distal esophagus. Following in
frequency is isolated TEF. The other variants are insignificant and usually diagnosed intraoperatively.6

DIAGNOSIS

Prenatal diagnosis of EA using ultrasound relies
on the finding of a small or absent fetal stomach bubble
associated with maternal polyhydramnios. Using these
criterias, the positive predictive value is 56% and the
sensitivity of establishing the diagnosis 42%.17-18 Polyhydramnios is most commonly seen in pure EA. Visualizations of the blind-ending esophagus during fetal
swallowing, known as the pouch sign, can also lead to
prenatal diagnosis.17, 19-21 Maternal alpha fetoprotein levels has brought inconclusive evidence in the prenatal
diagnosis of EA.22

Most babies born with EA have symptoms since
the first hours of birth. They consist of excessive salivation (mucosity), first feeding causing reflux, asphyxia
and cough followed by cyanosis, respiratory distress
and inability to pass an orogastric tube. The abdomen
could be either scaphoid or distended depending on
the presence of a distal TEF. The diagnosis of EA is
confirmed after watching a coiled orogastric tube in the
proximal esophagus in simple chest x-ray films. Contrast studies are rarely needed and of potential disaster
(aspiration). If in doubt a very small quantity (0.5 to 1
cc) of diluted barium can be instilled through the tube
to delineate the proximal esophageal pouch. A constant distance of 10-11 cm between the nares and proximal esophageal pouch is most often found. Abdominal
films should be obtained to rule out the occurrence of
associated gastrointestinal anomalies. Other diagnostic modalities include esophagoscopy, fluoroscopy and
bronchoscopy. The presence of air in the gastrointestinal tract suggests that a distal TEF is present, whereas
absent air makes the diagnosis of pure EA.1 Due to the
high incidence of associate malformations in children
born with EA, an initial evaluation should screen for
other defects using echocardiogram to exclude ductaldependent lesions, preoperative renal ultrasound if the
child has not voided and chromosomal analysis.

Congenital isolated TEF brings problems during early diagnosis and management. More than Htype is N-type, due to the obliquity of the fistula from
the trachea (carina or main bronchi) to esophageal
side (see Figure 2) anatomically at the level of the neck
root (C7-T1). Pressure changes between both structures can cause entrance of air into the esophagus, or
esophageal content into the trachea. Thus, the clinical
manifestation that we must be aware for early diagnoses are cyanosis, coughing and choking with feedings,
recurrent chest infections, persistent gastrointestinal
distension with air, and hypersalivation.17 Diagnosis is
confirmed with an esophagogram, or video-esophagogram (high success rates, establish level of the
TEF). Barium in the trachea could be caused by aspiration during the procedure. Upon radiologic doubt,

bronchoscopy should be the next diagnostic step. Delay in surgery is generally due to delay in diagnosis
rather than delay in presentation.23

ASSOCIATED ANOMALIES

The pathogenetic mechanisms that produce
EA also affect other systems. Between 50 and 70%
of children born with EA have associated congenital
anomalies that affect prognosis and survival. Cardiovascular anomalies are the most commonly found
(29%), followed by genitourinary (14%), gastrointestinal (13%), skeletal (10%) and chromosomal defects
(4%).24 Neurologic anomalies associated include neural tube defects, hydrocephaly, holoprosencephaly and
macrophthalmia. Other less common anomalies are
choanal atresia, cleft lip, abdominal wall defects and
diaphragmatic hernias. The association of two or more
system anomalies and the severity of associated anomalies influence mortality in esophageal atresia.3, 6, 24,

25


In 1973, the VACTERL association was described. VACTERL is the most common association (1436%) seen in EA and includes:

Vertebral anomalies

Anal malformations

Cardiac malformations

Tracheo-Esophageal fistula (must be one of the associated conditions)

Renal deformities

Limb radial defects.


Other associations included within EA include
CHARGE (coloboma, heart disease, retarded development/growth or central nervous system abnormalities,
genital hypoplasia or hypogonadism and ear abnormalities or deafness), Downs, Potter, Fryns, Fanconi syndrome and others.3, 24, 26

Cardiac defects determine mortality. Risk of
death in an infant born with EA and a cardiac defect is
30%. The most common cardiac anomaly is ventricular
septal defect. Other cardiac defects include tetralogy
of Fallot, patent ductus arteriosus and anomalies of
the aortic arch.8, 27 In infants with non-duct-dependent
cardiac anomalies, repair of the esophagus takes precedence whereas in ductal-dependent lesions, temporary control can usually be achieved with prostaglandin E.6 Genitourinary anomalies include hypospadia,
undescended testis, renal agenesis, hydronephrosis,
vesico-ureteral reflux and ambiguous genitalia. The
most common gastrointestinal anomalies include imperforate anus, duodenal atresia, malrotation, annular
pancreas and pyloric stenosis.

Several deformities of the respiratory system
associated with EA can result in a fatal outcome. They
include an ectopic superior right bronchus associated
with a trifurcated trachea or tracheal bronchi, congenital

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34

bronchial stenosis, tracheal, laryngeal and pulmonary


agenesis.23 Because these abnormalities may be related to the occurrence of respiratory disorders such
as tracheomalacia and atelectasis, early bronchoscopic examination may be useful in patient with predominantly respiratory symptoms.

Chromosomal anomalies such as trisomy 18
and 21 are rarely seen. The correction of EA in children
with Trisomy 18 brings challenging ethical questions
that should be discussed with the parents.

SURGICAL MANAGEMENT

Figure 1. Types and Frequency of Esophageal Malformations

Preoperative Management

Repair of EA is not an emergency. Time should
be taken to optimize the physiologic state of the baby
before urgent repair. This includes secure the airway,
avoid further aspiration and manage the associated
pneumonitis (chest physiotherapy). An orogastric tube
(Replogle) set to low intermittent suction can remove
secretions from the upper esophageal pouch. The child
should be placed in a semi-sitting position to avoid gastric content travel from the stomach to the lungs through
the TEF. Next, intravenous fluids and broad-spectrum
antibiotics should be administered. Lab work-up should
include hemogram, serum electrolytes, arterial blood
gases, and type and cross match for 20 cc/kg of weight
of packed red blood cells. Informed consent should be
obtained before surgery.

All patients with esophageal atresia should
have an echocardiogram prior to surgery to assess the
position of the aortic arch. Operative repair should be
done through the contralateral side of the aortic arch.6

Figure 2. Isolated TEF


Premature babies with EA and respiratory distress from hyaline membrane disease in need of mechanical ventilation are a special group. Inefficient
ventilation due to preferential escape of air through the
TEF in the presence of stiff lungs exposes the child to
gastric perforation, abdominal compartment syndrome
and hypoxia. Temporary balloon occlusion of the distal esophageal segment has brought conflicting results
while emergent ligation of the TEF is the best management alternative for the affected child (see Figure 3).6,
28

Intraoperative Management

As management for infants with esophageal
atresia has markedly improved, new surgical techniques have been developed.29 In addition to the conventional open repair, a minimally invasive thoracoscopic
repair was first successfully done by Lobe and Rothenberg in 2002.30-31 Many studies have been conducted
to establish the thoracoscopic repair in the new standard of care.32-34 Studies have concluded that thoracoscopic repair is safe and comparable with conventional
open repair of esophageal atresia in terms of short-term
outcomes such as operative time, postoperative leak
and stricture rates.32 Long-term benefits of the thoracoscopic repair needs to be further assessed since a published study has failed to reveal the advantage in terms
of postoperative esophageal motor function.33

Figure 3. Prematurity, Hyaline Membrane Disease and


Inefficient ventilation in a child with EA and TEF

Intraoperative management depends on type
of defect and associated malformations. The final objective is to establish bowel continuity while preserving
the native esophagus of the child.

The standard of care remains the open thoracotomy repair and at the operating room, the management of EA with distal TEF entails the following guidelines:

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1. Thoracic approach should be contralateral to the


aortic arch, most often this means a right thoracotomy. A high axillary skin crease incision allows
unrestricted access to the posterior mediastinum,
carries minimal morbidity and provides excellent
scar cosmesis.35-36
2. Whenever possible preservation of the latissimus
dorsi and serratus anterior muscles should be done
(muscle-sparring thoracotomy) to avoid shoulder
deformity, scoliosis, and winged scapula.
3. Extrapleural approach to the posterior mediastinum is preferred since a rib does not have to be
removed. Anastomotic leaks using the extrapleural
approach are better tolerated.
4. Division of the azygous vein near the superior vena
cava entrance to help dissect the TEF is operator
dependant since many surgeons prefer not to ligate the azygous vein. The TEF is watertight ligated
with small absorbable sutures (6-0 PDS or Vicryl).
5. Anesthesiologist will help identify the proximal
esophageal stump while exerting pressure with
an oro-esophageal tube. The proximal esophageal stump can be aggressively dissected up the
neck, but avoid using the cautery since recurrent
laryngeal nerve damage is a possibility. The distal
esophageal stump is dissected carefully since its
blood supply is more precarious.
6. A primary esophageal anastomosis between both
stumps can be accomplished most often using one
layer full thickness interrupted absorbable sutures.
Around eight stitches are needed. The surgeon
must develop judgement on the tension exerted
when performing the anastomosis. A small 5 Fr.
trans-anastomotic tube is placed in the stomach for
drainage and early gavage feeding. Once finished
a chest drain is left near the anastomosis and placed to underwater sealing suctioning (see Figure
4).
7. Intercostal closure and muscle apposition follows
with transfer of the baby to the NICU.

Management of the baby born with pure esophageal atresia remains a challenge since it depends on
the distance (gap) between the esophageal stumps.
There are different alternatives of treatment but an individually decision should be made for each patient.
Initially, a gastrostomy is done. This will help start feedings and permit measurement of the gap distance.
The proximal esophageal stump can be serially dilated
using Bakes metal dilators, while bolus gastrostomy
feeding will help the distal esophageal stump to grow
(see Figure 5). Meticulous nursing care is needed to
avoid respiratory problems from chronic saliva aspiration. Once a gap of one centimeter is obtained between
the esophageal stumps, an anastomosis can be done.

Several techniques are described to gain length and accomplish a tension free anastomosis during
repair, such as circular or spiral myotomy, flap esophagoplasty or extrathoracic extension, all performed on
the proximal esophageal pouch.6, 8 Esophageal replacement can be accomplished using preferably colon,
stomach or jejunum discussion of which is beyond the
scope of this review. It has been proved that patient undergoing primary repair had generally better outcomes

Figure 4. Surgical Repair of EA with TEF

Figure 5. Long gap EA, suction catheter in upper pouch


and gastrostomy
than those who underwent an esophageal replacement.37 Primary repairs in long gap esophageal atresia
would cause severe anastomotic tension and would
increase the risk of leakage and stricture formation. A
new approach has surged for these patients, growth
induction of the esophageal stumps by internal or external traction. A staged esophageal lengthening has
been achieved requiring three operations but resulting
in an esophagus-only repair with the gastroesophageal
junction below the diaphragm.38 Further studies have
demonstrated the efficacy of the growth procedure to
treat the full spectrum of EA including those with most
rudimentary lower segments and the longest gaps. The
long gap-EA patients treated by the growth procedure
and an early active course of dilations and treatment of
GE reflux seem indistinguishable from the much more
favorable short gap EA/TEF patients.39

Isolated TEF management consists of surgical closure through a right cervical approach. A small
guide-wire threaded through the fistula during bronchoscopy helps identified the fistulous tract. Working in
the tracheo-esophageal groove can cause injury to the
recurrent laryngeal nerve with subsequent vocal cord
paralysis. Recurrence after closure is rare.40

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Postoperative Care

With moderate or severe tension on the repair,
the child benefits from postoperative prophylactic paralysis and mechanical ventilation to avoid neck movement, further tension, breakage or leak of the anastomosis.6 The chest tube is left until it clogs or patency
of the esophagus is documented. The presence of
pneumothorax or saliva in the chest tube suggests an
anastomotic leak. Gavage feedings through the transanastomotic tube can be started after the third postoperative day. A barium swallow is performed seven to
ten days after the repair to document the presence of
leak, stricture, dysmotility and gastroesophageal reflux
(GER).

Since esophageal stricture is the one most feared complication after esophageal repair, published
studies have tried to determine whether routine balloon dilatation of the anastomosis after repair of an
esophageal atresia with distal fistula is superior to wait
and see policy with dilatation only when symptoms arise. Studies have reported that dilatation on demand
results in fewer procedures, fewer hospitals days and
likely lower cost.41-42

COMPLICATIONS

Repair of EA and TEF can be plagued with early
and late morbidity. The three most common anastomotic complications are in order of frequency: stricture,
leakage and recurrent TEF. Late complications include
gastroesophageal reflux (GER), tracheomalacia and
dysmotility.43

Anastomotic strictures (or stenosis) are a
common finding after repair of EA. Incidence can be
as high as 50%. Predisposing factors include the use
of breaded silk, thepresence of GER and leakage of
the anastomosis. Strictures are associated with choking, frequent respiratory infections and foreign body
impaction. Most children respond to dilatation. Those associated to GER should be fundoplicated.4, 17, 44

Esophageal leaks occur in 10-15% of all cases. Leaks that occur during the first 72 hours after
repair present as tension pneumothorax. An immediate
esophagogram should be done with water-soluble materials to exclude a complete esophageal breakdown
that might need repair, esophageal exclusion and/or
gastrostomy. Late leaks are usually small, present with
saliva in the chest drain and close spontaneously with
proper drainage and nutrition.17

Recurrent TEF after surgical repair for EA occurs in approximately 3-15% of cases. Tension on the
anastomosis followed by leakage may lead to local inflammation with breakage of both suture lines enhancing the chance of recurrent TEF. Once established, the
fistula allows saliva and food into the trachea, therefore
clinical suspicion of this diagnosis arises with recurrent
respiratory symptoms associated with feedings after
repair of EA. Diagnosis is confirmed with cineradiography of the esophagus or bronchoscopy. A second
thoracotomy is very hazardous, but is the most effective method to close the recurrent TEF. Either a pleural

or pericardial flap will effectively isolate the suture line.


A pericardial flap is easier to mobilize, provides sufficient tissue to use and serves as template for ingrowth
of new mucosa should leakage occurs. Other alternatives are endoscopic diathermy obliteration, endoscopic
chemocautherization, laser coagulation, or fibrin glue
deposition.40, 45-47

GER is commonly identified after successful repair of EA. Constant gastric bathing of an esophageal
anastomosis causes inflammation, edema and stricture formation. GER has been implicated in development
of esophageal leaks in the immediate postop period.
The presence of GER in EA is associated to shortening
of the esophagus and vagal nerve denervation during
repair. Stricture development, recurrent pneumonia,
failure to thrive, Barrett epithelium and severe esophagitis are indications for fundoplication.4, 17, 48-51

Tracheomalacia refers to a structural/functional generalized or localized weakness of the tracheal
rings' support resulting in partial respiratory obstruction.
Most cases are associated with EA and as such flaccid
tracheal development after external pressure from the
dilated proximal blind esophageal segment has been
proposed as pathogenetic mechanism. Most cases
develop expiratory obstruction since only the intrathoracic trachea if affected. The harsh barking cough is
the most characteristic initial symptom. Nutritional problems are the result of difficulty breathing as cyanotic
attacks might occur during feeding. Other incitatory
elements are intercurrent respiratory infections and aspiration. Severe forms are characterized by life-threatening apneic spells, inability to extubate the airways,
and episodic pneumonia. A cough and wheeze may
progress to complete airway obstruction and cyanosis.
Diagnosis is obtained with simple lateral thoracic films
(narrow slit-like appearance), bronchoscopy during
spontaneous breathing (antero-posterior narrowing in
expiration), cinetracheobronchography (allows extent
of tracheal collapse) or cine CT studies. Reflux must
be rule out and manage aggressively. For mild to moderate symptoms no management is necessary as the
child will improve with time. For severe life threatening
tracheomalacia aortopexy must be undertaken. Failed
aortopexy may need tracheal reinforcement with autologous cartilaginous grafts.6, 8, 52

RESULTS

Survival of EA has improved dramatically during the past fifty years. A general survival rate of 80 to
95% can be accomplished when children with severe
associated malformations are excluded. The original
Waterstons risk classification has yielded to several
modifications. High-risk patients are those with very
low birth weight (less than 1500 grams), associated
cardiac anomalies, chromosomal defects, ventilator
dependant and long gap.

Although the long-term outcome of EA patients
seems favorable, respiratory and gastrointestinal symptoms plus functional abnormalities remain frequent. One
third of long-term patients report having impaired quality of life because of respiratory infections, dyspnea,

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difficulties in swallowing and coughing at night.53 Almost 20% of patients have GER symptoms. The rate of
symptoms decreases with age. Gastric metaplasia and
esophagitis and the high rate of tracheal, esophageal,
and gastric inflammation suggest a need for long-term
follow-up. Gastric metaplasia is a premalignant lesion
of the esophagus.51, 54 Results can be classified as excellent if the child is totally asymptomatic, good if he
has occasional difficulty eating some foods. Children
with frequent deglutition or respiratory problems are
rated as regular results.

Growth and development after successful EA
repair remains within the 50th percentile. Children with
EA have more learning, emotional, and behavioral problems than children in the general population. A highrisk group of children with major associated congenital
anomalies, who had been ventilated as a newborn,
were at special risk for cognitive problems.55 Children
with esophageal replacement procedures fare worst
that primarily repair cases. These children develop
anastomotic strictures, ulceration, bleeding, redundancy and bezoar formation more commonly.

REFERENCES
1-
O'Neill JA, Rowe MJ, Grosfeld JL, et al: Pediatric Surgery. St. Louis, MI:
Mosby Inc. 1998, pp 941-963
2-
Konkin DE, O'hali WA, Webber EM, Blair GK: Outcomes in esophageal
atresia and tracheoesophageal fistula. J Pediatr Surg 38 (12): 1726-9, 2003
3-
Yang CF, Soong WJ, Jeng MJ, Chen SJ, et al: Esophageal atresia with
tracheoesophageal fistula: ten years of experience in an institute. J Chin Med Assoc
69 (7): 317-21, 2006
4-
Rintala RJ, Sistonen S, Pakarinen MP: Outcome of esophageal atresia
beyond childhood. Semin Pediatr Surg 18 (1): 50-6, 2009
5-
Adonis S. Ioannides, Andrew J. Copp: Embryology of oesophageal atresia.
Semin Pediatr Surg 18: 2-11, 2009
6-
Spitz L: Oesophageal atresia. Orphanet J Rare Dis 11 (2): 24, 2007
7-
Keckler SJ, St. Peter SD, Valusek PA, et al: VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of
the literature. Pediatr Surg Int 23 (4): 309313, 2007
8-
Spitz L: Esophageal atresia. Lessons I have learned in a 40-year experience. J Pediatr Surg 41 (10): 1635-40, 2006
9-
Celli J, van Beusekom E, Hennekam RC, Gallardo ME, Smeets DF, et al:
Familial syndromic esophageal atresia maps to 2p23-p24. Am J Hum Genet 66 (2):
436-44, 2000
10-
Frana WM, Gonalves A, Moraes SG, Pereira LA, Sbragia L: Esophageal
atresia and other visceral anomalies in a modified Adriamycin rat model and their correlations with amniotic fluid volume variations. Pediatr Surg Int 20 (8): 602-8, 2004
11-
Shaw-Smith C: Genetic factors in esophageal atresia, tracheo-esophageal
fistula and the VACTERL association: roles for FOXF1 and the 16q24.1 FOX transcription factor gene cluster, and review of the literature. Eur J Med Genet 2010 53 (1):
6-13, 2010
12-
Goyal A, Jones MO, Couriel JM, Losty PD: Oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child Fetal Neonatal Ed 91 (5): F381-4, 2006
13-
Digilio MC, Marino B, Bagolan P, et al: Microdeletion 22q11 and oesophageal atresia. J Med Genet 36: 137-139, 1999
14-
Puusepp H, Zilina O, Teek R, Mnnik K, Parkel S, et al: 5.9 Mb microdeletion in chromosome band 17q22-q23.2 associated with tracheo-esophageal fistula and
conductive hearing loss. Eur J Med Genet 52 (1): 71-4, 2009
15-
Moore KL, Persaud TVN: The Developing Human. Pennsylvania, WB
Saunders pp 212-213, 2008
16-
Crisera CA, Connelly PR, Marmureanu AR, et al: Esophageal atresia with
tracheoesophageal fistula: suggested mechanism in faulty organogenesis. J Pediatr
Surg 34 (1): 204-8, 1999
17-
Holland AJ, Fitzgerald DA: Oesophageal atresia and tracheo-oesophageal
fistula: current management strategies and complications. Paediatr Respir Rev 11 (2):
100-6, 2010
18-
Houben CH, Curry JI: Current status of prenatal diagnosis, operative
management and outcome of esophageal atresia/tracheo-esophageal fistula. Prenat
Diagn 28 (7): 667-75, 2008
19-
Kalache KD, Wauer R, Mau H, et al: Prognostic significance of the pouch
sign in fetuses with prenatally diagnosed esophageal atresia. Am J Obstet Gynecol
182 (4): 978-81, 2000
20-
Kalache KD, Wauer R, Mau H, Chaoui R, Bollmann R: Prognostic
significance of the pouch sign in fetuses with prenatally diagnosed esophageal atresia.
Am J Obstet Gynecol 182 (4): 978-81, 2000
21-
Has R, Gnay S: Upper neck pouch sign in prenatal diagnosis of esophageal atresia. Arch Gynecol Obstet 270 (1): 56-8, 2004
22-
Van Rijn M, Christaens GCML, Hagenaars AM, et al: Maternal serum
alpha-fetoprotein in fetal anal atresia and other gastro-intestinal obstructions. Prenat
Diagn 18: 914-921, 1998
23-
Ng J, Antao B, Bartram J, Raghavan A, Shawis R. Diagnostic difficulties
in the management of H-type tracheoesophageal fistula. Acta Radiol 47 (8): 801-5,
2006
24-
Stoll C, Alembik Y, Dott B, Roth MP: Associated malformations in patients
with esophageal atresia. Eur J Med Genet 52 (5): 287-90, 2009
25-
Saing H, Mya GH, Cheng W: The involvement of two or more systems and
the severity of associated anomalies significantly influence mortality in esophageal

atresia. J Pediatr Surg 33 (11): 1596-8, 1998


26-
Arzu Pampal: CHARGE: An association or a syndrome? Int. J. Pediatr.
Otorhinolaryngol, 2010
27-
Allen SR, Ignacio R, Falcone RA, Alonso MH, et al: The effect of a rightsided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 41 (3): 479-83, 2006
28-
Maoate K, Myers NA, Beasley SW: Gastric perforation in infants with oesophageal atresia and distal tracheo-oesophageal fistula. Pediatr Surg Int 15 (1): 24-7,
1999
29-
Orford J, Cass DT, Glasson MJ. Advances in the treatment of oesophageal
atresia over three decades: the 1970s and the 1990s. Pediatr Surg Int 20 (6): 402-7,
2004
30-
Rothenberg SS: Thoracoscopic repair of tracheoesophageal fistula in newborns. J Pediatr Surg 37 (6): 869-72, 2002
31-
Lobe TE, Rothenberg SS, Waldschmidt J, et al: Thoracoscopic repair of
esophageal atresia in an infant: a surgical first. Pediatr Endosurg Innovat Tech 3 ():
141-8, 1999
32-
Al Tokhais T, Zamakhshary M, Aldekhayel S, et al: Thoracoscopic repair
of tracheoesophageal fistulas: a case-control matched study. J Pediatr Surg 43 (5):
805-9, 2008
33-
Kawahara H, Okuyama H, Mitani Y, Nomura M, Nose K, et al: Influence of
thoracoscopic esophageal atresia repair on esophageal motor function and gastroesophageal reflux. J Pediatr Surg 44 (12): 2282-6, 2009
34-
MacKinlay GA: Esophageal atresia surgery in the 21st century. Semin Pediatr Surg 18 (1): 20-2, 2009
35-
Klmn A, Verebly T. The use of axillary skin crease incision for thoracotomies of neonates and children. Eur J Pediatr Surg 12(4): 226-9, 2002
36-
Bianchi A, Sowande O, Alizai NK, Rampersad B: Aesthetics and lateral
thoracotomy in the neonate. J Pediatr Surg 33 (12): 1798-800, 1998
37-
Holland AJ, Ron O, Pierro A, Drake D, Curry JI, Kiely EM, Spitz L: Surgical
outcomes of esophageal atresia without fistula for 24 years at a single institution. J
Pediatr Surg 44 (10): 1928-32, 2009
38-
Till H, Muensterer OJ, Rolle U, Foker J: Staged esophageal lengthening
with internal and subsequent external traction sutures leads to primary repair of an
ultralong gap esophageal atresia with upper pouch tracheoesophagel fistula. J Pediatr
Surg 43 (6): E33-5, 2008
39-
Foker JE, Kendall TC, Catton K, Munro F, et al: Long-gap esophageal
atresia treated by growth induction: the biological potential and early follow-up results.
Semin Pediatr Surg 18 (1): 23-9, 2009
40-
Richter GT, Ryckman F, Brown RL, Rutter MJ: Endoscopic management of
recurrent tracheoesophageal fistula. J Pediatr Surg 43 (1): 238-45, 2008
41-
Koivusalo A, Turunen P, Rintala RJ, van der Zee DC, Lindahl H, Bax NM: Is
routine dilatation after repair of esophageal atresia with distal fistula better than dilatation when symptoms arise? Comparison of results of two European pediatric surgical
centers. J Pediatr Surg 39 (11): 1643-7, 2004
42-
Koivusalo A, Pakarinen MP, Rintala RJ: Anastomotic dilatation after repair
of esophageal atresia with distal fistula. Comparison of results after routine versus
selective dilatation. Dis Esophagus 22 (2): 190-4, 2009
43-
Mortell AE, Azizkhan RG: Esophageal atresia repair with thoracotomy: the
Cincinnati contemporary experience. Semin Pediatr Surg 18 (1): 12-9, 2009
44-
Ko HK, Shin JH, Song HY, Kim YJ, Ko GY, Yoon HK, Sung KB. Balloon
dilation of anastomotic strictures secondary to surgical repair of esophageal atresia in
a pediatric population: long-term results. J Vasc Interv Radiol 17 (8): 1327-33, 2006
45-
Bruch SW, Hirschl RB, Coran AG. The diagnosis and management of recurrent tracheoesophageal fistulas. J Pediatr Surg 45 (2): 337-40, 2010
46-
Farra J, Zhuge Y, Neville HL, Thompson WR, Sola JE. Submucosal fibrin
glue injection for closure of recurrent tracheoesophageal fistula. Pediatr Surg Int 26
(2): 237-40, 2010
47-
Sung MW, Chang H, Hah JH, Kim KH. Endoscopic management of recurrent tracheoesophageal fistula with trichloroacetic acid chemocauterization: a preliminary report. J Pediatr Surg 43 (11): 2124-7, 2008
48-
Koivusalo A, Pakarinen MP, Rintala RJ: The cumulative incidence of significant gastrooesophageal reflux in patients with oesophageal atresia with a distal
fistula--a systematic clinical, pH-metric, and endoscopic follow-up study. J Pediatr
Surg 42 (2): 370-4, 2007
49-
Castilloux J, Noble AJ, Faure C: Risk factors for short- and long-term morbidity in children with esophageal atresia. J Pediatr 156 (5): 755-60, 2010
50-
Bergmeijer JHLJ, Tibboel D, Hazebroek FWJ: Nissen fundoplication in the
management of gastroesophageal reflux occurring after repair of esophageal atresia.
J Pediatr Surg 35 (4): 573-576, 2000
51-
Deurloo JA, Ekkelkamp S, Taminiau JA, Kneepkens CM, et al: Esophagitis
and Barrett esophagus after correction of esophageal atresia. J Pediatr Surg 40 (8):
1227-31, 2005
52-
Van der Zee DC, Bax NM. Thoracoscopic tracheoaortopexia for the
treatment of life-threatening events in tracheomalacia. Surg Endosc 21 (11): 2024-5,
2007
53-
D.C. Little, F.J. Rescorla, J.L. Grosfeld, K.W. West, L.R. Scherer, and S.A.
Engum: Long-Term Analysis of Children With Esophageal Atresia and Tracheoesophageal Fistula. J Pediatr Surg 38 (6): 852-6, 2003
54-
Schalamon J, Lindahl H, Saarikoski H, Rintala RJ: Endoscopic follow-up in
esophageal atresia-for how long is it necessary? J Pediatr Surg 38 (5): 702-4, 2003
55-
Bouman NH, Koot HM, Hazebroek FW: Long-term physical, psychological,
and social functioning of children with esophageal atresia. J Pediatr Surg 34 (3): 399404, 1999

RESUMEN
Atresia esofgica es la condicin congnita ms comn del esfago en recin nacidos. Este articulo de
resea discute la incidencia, clasificacin embriolgica, diagnostico y anomalas asociadas en atresia
del esfago. Se establece nfasis en los principios
actualizados del manejo quirrgico de esta condicin congnita.

CLINICAL VERSUS
PATHOLOGIC DIAGNOSIS:
ACRODERMATITIS
ENTEROPATHICA

ABSTRACT
Malnutrition is a well-known cause of infant
morbidity and mortality. In developed societies
malnutrition still exists, especially in patients
fed insufficient diets during chronic and critical
illnesses. Malnutrition impacts all organs. Skin
manifestations are common, and its diagnosis is
mostly visual. We present four cases of malnutrition with associated zinc deficiency were the
clinical diagnosis proved correct. Acrodermatitis
Enteropathica cannot be distinguished histologically form other forms of deficiency dermatitis
and psoriasis. A thorough history and physical
examination plus the clinical response to therapy
are sufficient to make the correct diagnosis.

Alicia Fernandez Sein MD *

* From the Section of Pediatric Critical Care, Department of


Pediatrics, UPR School of Medicine.
Address reprints requests to: Alicia Fernandez-Sein, MD Pediatric Critical Care Section, Department of Pediatrics,
UPR School of Medicine, Puerto Rico Health Science Center, PO BOX 365067, San Juan, PR 00936-5067. E-mail:
alicia.fernandez@upr.edu

Index words: clinical, pathological, diagnosis,


acrodermatitis, enterohepathica

WW

First Case

INTRODUCTION

orldwide, malnutrition is one of the leading causes of infant morbidity and mortality. In third
world countries up to 25% of children or 90 million
of them are severely food deprived. The decrease in
breast feeding is probably the most important factor in
infant malnutrition.

Contrary to general believe, malnutrition is also
present in developed societies. In our society, malnutrition most commonly presents as Kwashiorkor or
protein deprivation relative to caloric intake. Malabsorption, inappropriate or insufficient diet, chronic and
critical illnesses cause malnutrition more frequently
than we suspect (1).

The duration and severity of the malnutrition
are determinant in the degree of the disturbances it
causes. Life phases of rapid growth and development
are more susceptible to inadequate dietary intake.
Therefore, malnutrition in infancy occurs rapidly and its
effects may be recognized relatively early (1).

As a systemic disorder, malnutrition impacts
every organ and system. Skin manifestations may be
suggestive or not of a specific nutrient deficiency. After detailed history is taken, diagnosis is mostly visual.
Pathologic findings are confirmatory of our clinical suspicion. Clinical response to therapy is another tool for
final diagnosis. We present four cases in which clinical
diagnosis proved to be correct.


Nine month-old-boy who developed erythematous rash over the legs and was treated with topical
steroids without improvement. The rash progressed to
arms, back, chest and face. Skin biopsy was compatible with Psoriasis and the patient was sent to the University Pediatric Hospital. He had been born at term,
without complications, and had been breastfed until
2 months of age. From then on diarrheas developed
and milk formula was changed several times without
success. Finally, he was kept in vegetables, fruits and
juices. No milk, cereals or meats were given from then
on. The mother also reported that during the last month the baby had lost previously gained milestones. He
had also became irritable and hypoactive. Upon admission, weight and height was less than 5th percentile.
He was pale and edematous; the hair brittle and thin;
crusted lips, reddened periorbital areas; generalized
erythematous rash, scaly and crusted at some areas,
reticular pattern in legs; denuded hands and feet (see
Pictures 1 and 2). The patient was admitted to the intensive care unit due to hypovolemic shock, anasarca and malnutrition. Serum electrolytes were within
normal range except for serum albumin 1.8 g/dl with
normal cholesterol values. During the following days,
the patient received antibiotics, blood transfusions and
total parenteral nutrition with added zinc. He required mechanical ventilation and inotropic support due
to sepsis and Acute Respiratory Distress Syndrome.
During this hospitalization the patient developed several sepsis episodes with various organisms: Klebsiella
Pneumonia, Serratia, Staphylococcus epidermidis and
Candida. IV immuno globulins were given to improve

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Picture 1: Pale, edematousGeneralized erythematous


rash, reticular pattern in legs, scaly and crusted in
some areas. Denuded hands and feet.

Picture 2:
Thin brittle hair, erythematous, crusted scaly skin,
reddened periorbital areas.

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immunological status. He also developed abdominal


compartment syndrome, secondary to tense ascites.
Paracentesis was performed to improve blood pressure, renal perfusion and urinary output. Protein loss was
replaced with 25% albumin. Slowly after three weeks
he started to improve and we were able to increase
successfully post-pyloric feedings. By the fourth week
of hospitalization he was taken off the ventilator. The
skin had become completely normal, and the baby
started to attain previously lost milestones (see Picture
3).
Picture 3:
After 4 weeks of hospitalization, patient tolerated enteral nutrition, skin became normal and started to attain
previously lost milestones

Picture 4:
2 m/o girl with frequent formula changes due to diarrhea.
Became irritable and with poor sucking.

Admission

Admission

Second Case

Two month-old-girl with frequent formula changes due to diarrhea. Became irritable and with poor
sucking. On admission the patient was irritable, and
had an erythematous rash around the mouth and genitalia, edematous extremities, with reticulated erythematous rash on both legs. Intravenous zinc was given.
Three days on zinc and the rash disappeared. By the
tenth day of hospitalization the baby was smiling and
sucking well (see Picture 4).

3 days in zinc supplementation

Third Case

Six year-old-girl whose unstable mother fed her
only with orange juice and crackers. She developed
diarrheas and skin rash. On admission was pale and
apathic and somnolent. She had scanty, thin, blond
hair. There was erythematous rash around the mouth,
including lips, and in genital area. The tongue was
deeply red and no papillae were noted. Oral zinc supplementation was started. After one week the erythematous lesions disappeared and the girl became more
talkative, sleeping less. She still had to be fed through
an NG tube since she refused to change her diet (see
Picture 5).

10 days in therapy

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Fourth Case

Seven month-old-boy with Phenylketonuria
who developed vomiting and diarrheas. He was kept
off his special diet with Lo-Phenalac for one week. Mother noted severe erythematous rash around mouth,
genital and anal regions. Intravenous zinc supplementation was started and skin changes disappeared in
four days. His special formula was re-started and tolerated (see Picture 6).

Picture 5:
6 y/o girl whose mother only fed her orange juice and
crackers.
Fotos taken 2 weeks after total enteral
nutrition and zinc supplementation.

DISCUSSION

Acrodermatitis enteropathica (AE) was first described in 1936 by Brandt but it was not until 1973 that
the link between zinc deficiency and AE was formally
hypothesized by Moynahan (2, 3). Primary or congenital AE is a rare autosomal recessive disorder caused by the inability to absorb sufficient zinc from diet in
the duodenum and jejunum. Secondary AE caused by
dietary zinc deficiency is very common in developing
countries, especially in non-breast fed infants. In our
society groups at high risk of developing zinc deficiency include: vegetarians, alcoholics, malabsorption syndromes and premature infants (4)

Clinical presentation is similar for both congenital and acquired AE: irritability, growth retardation, diarrhea, diffuse non-scarring alopecia, skin lesions and
delayed wound healing. The hair often has a reddish
tint, is brittle and thin and may exhibit alternating dark
and bright bands. Cutaneous eruption consists of eczematous pink scaly plaques which can become vesicular, pustular or desquamative. The psoriasiform lesions
develop over the extremities, perioral and anogenital
regions (5). Skin lesions will become secondarily infected with bacteria and Candida albicans. Angular
cheilitis or perleche and paronychia are early common
manifestations of zinc deficiency.


Milk intolerance and gastroenteritis are very
common during infancy. Usually these episodes are
short and self-limited. Regretfully, some physicians attempt to control symptoms by changing formulas. Such
is the case in two of our patients. Such quick changes
predispose to intolerance and finally to inappropriate
dietary intake (6). Breastmilk provides less amounts
of zinc than cows milk but it is more readily absorbed
in human milk. Breastmilk remains the best source of
zinc for babies younger than 6 months. Since maternal-fetal zinc transfer occurs during the last ten weeks
of gestation, premature babies are at increased risk of
developing zinc deficiency. Other sources of zinc include whole grain cereal, legumes, meat, chicken and
fish. The zinc content of a particular food correlates
with the protein content, but the bioavailability of zinc in
protein-rich plant foods is much less than that of animal
foods.

Insufficient protein intake causes zinc
deficiency and secondary Acrodermatitis Enteropathica (AE). Zinc deficiency decreases
cellular immunity. It deprives the patient from the beneficial effects of scavenging oxidative free radicals and

nitric oxide in the gut making the patient more susceptible to toxin-producing bacteria or enteroviral pathogens. Zinc deficiency causes an increase in adenylyl
cyclase activitiy in the gut producing augmentation of
intestinal chloride secretion. This all lead to chronic
diarrhea and decreased nutrient absorption with which
zinc deficiency becomes more severe and malnutrition
worsens (7).

Zinc metabolism is highly regulated and accurately measuring the bodys zinc status is difficult. Zinc
levels are reduced during acute inflammatory processes. Therefore, depressed serum zinc values do not
always indicate zinc deficiency. On the other hand, zinc
levels may be normal in a deficiency dermatitis that will
improve with zinc supplementation. Also, in hypoalbuminemic states zinc levels may be diminished since

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42

zinc is albumin bound. We routinely do not measure


zinc levels, but observe dermatological improvement
with zinc supplementation. This was observed in the
four patients reported.

Picture 6:
7 m/o boy with Phenylketonuria not tolerating his diet.
Erythematous rash around mouth, genital and anal regions.


Zinc deficiency should be corrected initially
intravenously 3-5mg/day. When the gastrointestinal
tract is functional again, oral therapy may be employed
using 0.5-1 mg/kg/day of elemental zinc. Skin lesions
improve rapidly upon correction of zinc deficiency. Ma
labsorption takes longer to improve and nutrition has to
be supplemented parenterally for a while. Secondary
immunodeficiency will disappear as nutrition and protein synthesis improves.

AE can not be distinguished histologically from
other forms of deficiency dermatitis and psoriasis. Fortunately, each of these conditions can be diagnosed by
clinical history, physical examination and response to
therapy. Our first patient had a biopsy compatible with
psoriasis but all his symptoms improved with zinc supplementation. Complete resolution of their problems
was observed in the other three patients.

The cases which we have presented provide
different scenarios where insufficient diets lead to malnutrition and zinc deficiency. They are also examples
that a complete history and physical examination can
not be substituted and are of outmost importance in
diagnosing Acrodermatitis Enteropathica.

REFERENCES

1. Schwartz D. Failure to thrive: an old nemesis in the new millennium. Ped in Review. 2000;21(8)
2. Moynahan EJ, Barnes PM. Zinc deficiency and a synthetic diet
for lactose intolerance. Lancet 1973;1:676-7.
3. Moynahan EJ. Acrodermatitis enteropathica: a lethal inherited
human zinc deficiency disorder. Lancet 1974;2:399-400.
4. Maverakis E, Fung M et al. Acrodermatitis enteropathica and an
overview of zinc metabolism. J Am Acad Dermatol 2007;56(1):116124.
5. Hambidge M. Human zinc deficiency. J Nutrition. 2000 130;(5S
Suppl):1344-9S.
6. Unicef. The State of the Worlds Children 1998. Oxford University Press.
7. Wapnir RA. Zinc deficiency, malnutrition and the gastrointestinal
tract. J Nutrition. 2000;130(5SSuppl):1388-92S.

RESUMEN
Malnutricin es una de las causas ms frecuentes
de morbilidad y mortalidad infantil. La malnutricin
existe tambin en sociedades desarrolladas, usualmente asociada a nutricin insuficiente durante enfermedades crticas crnicas debilitantes. La malnutricin impacta todos los rganos, siendo comn
las manifestaciones cutneas. Presentamos cuatro casos de malnutricin con deficiencia de zinc
asociada. En los cuatro casos el diagnstico clnico
fue correcto y determin la rpida mejora de los
pacientes. La Acrodermatitis Enteroptica no se
distingue histolgicamente de otras dermatitis o de
la Psoriasis. Un historial y examen fsico completo,
ms una rpida respuesta a la administracin de
zinc son suficientes para hacer el diagnstico.
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A
M
P
R

Muros antiguos...
corazn modelo del ao

ABSTRACT
Background: Invasive Candida infection is an increasingly important cause of morbidity and mortality in the neonatal intensive care unit. Neonatal candidemia occurs in 4-15% of extremely low
birth weight infants. Meningitis occurs in 5-9% of
patients with candidemia. A few infants with Candida meningitis (4%) present ventriculitis or brain
abscess. Observations: We report a neonate born
at 34 weeks gestational age, who at 12 days old
presented apnea and seizures. Cerebrospinal fluid
(CSF) grew Candida Albicans, and blood cultures
were negative. A head sonogram and head computed tomography scan were negative. Brain magnetic resonance imaging (MRI) detected multiple
brain parenchyma micro abscesses. Conclusions:
This case confirms the need of obtaining adequate cultures including blood, urine, and CSF when
sepsis is suspected. Neuroimaging studies should
be included in diagnostic workup of patients with
systemic fungal infection. Improved neuroimaging
techniques such as MRI may lead to earlier diagnosis of cerebral abscesses.
Index words: candida, albicans, meningitis, brain,
abscess, neonate

Case Reports / Reporte de Casos


CANDIDA ALBICANS
MENINGITIS AND BRAIN
ABSCESSES IN A
NEONATE: A Case Report
Ingrid M. Ancalle MD
Juan A. Rivera MD
Ins Garca MD
Lourdes Garca MD
Marta Valcrcel MD
From the University of Puerto Rico School of Medicine,
Department of Pediatrics, Neonatology Section, San Juan,
Puerto Rico.
Address reprints requests to: Juan A. Rivera MD, UPR
School of Medicine, Department of Pediatrics, Neonatology
Section, PO BOX 365067, San Juan, PR 00936-5067. Email juan.rivera49@upr.edu
Presented at the XXVIII Medical Science Campus Research
and Education Forum (Poster presentation) March 2008,
and the XV Pediatrics Latin-American Association Annual
Meeting (Poster presentation) November 2009.

INTRODUCTION


nvasive candida infection is an increasingly important cause of morbidity and mortality in the
neonatal intensive care unit. Any Candida species
may cause disease in neonates. C albicans remains
the most frequently isolated yeast species in infected
neonates (1, 2), followed by C parapsilosis infections,
which have exponentially increased during the past
decade (2, 3). Neonatal candidemia occurs in 4-15%
of extremely low birth weight infants (4, 5). Meningitis
occurs in 5-9% of patients with candidemia (6). A few
infants with Candida meningitis (4%) present ventriculitis or brain abscess (4). Candida meningitis and brain
abscess are associated with significant mortality and
severe neurological impairment (5, 6). We report a low
birth weight premature neonate with Candida meningitis and brain abscesses.

Case Report

A baby girl preterm adequate for gestational
age was born to a 24 year old mother G3, P2, A0 with
history of bronchial asthma. Prenatal tests (HIV, Hep B
and VDRL) were negative and maternal blood type and
group was O negative. Pregnancy was complicated by
premature rupture of membranes at 33 weeks gestational age (6 days prior to delivery) and the mother was
treated with intravenous (IV) antibiotics and antenatal steroids delivery) and the mother was treated with

intravenous (IV) antibiotics and antenatal steroids. The


patient was born by non sterile, spontaneous vaginal
delivery at 34 1/7 weeks gestational age. APGAR score was 8 at 1 minute and 9 at 5 minutes. Birth weight
was 2040 grams. On the first day of life the patient
presented respiratory distress due to pneumonia. She
required oxygen supplementation and was treated with
IV ampicillin and gentamycin. On day #3 she presented abdominal distention and diagnosis of suspected
necrotizing enterocolitis was made. She completed 10
days of IV antibiotics. Feedings were restarted on day
#8. On day # 12 the patient developed apneic episodes followed by seizures on the next day. Workup for
sepsis was performed and the patient was started on
IV amikin, vancomycin and imipenem. A spinal culture
was performed, the cerebrospinal fluid (CSF) had pleocytosis and the gram stain was negative. Viral infection was suspected and she was started on acyclovir.
On day #17 of life (#5 of antibiotics) CSF culture grew
yeast, later identified as Candida albicans. She was
started on amphotericin B. Blood cultures were negative for fungi. The CSF culture was sent for sensitivity
and Candida was sensitive to amphotericin B, voriconazole and fluconazole. A head sonogram on day # 14
and a head computed tomography on day #15 did not
report any abnormality. A head magnetic resonance on
day # 18 reported multiple small cystic lesions in the
brain parenchyma, consistent with micro abscesses

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45

(See Figure 1). On the fifth day of treatment with amphotericin B, flucytosine was added to the treatment.
Amikin, vancomycin and imipenem were discontinued
after 14 days of treatment, and acyclovir discontinued
on day #12 of treatment. All cultures were negative for
bacteria and workup for viral infections was also negative. Studies for immunologic diseases were negative.
The patients clinical course was complicated by retinitis of the right eye (See Figure 2) and by hydrocephaly
requiring a ventriculoperitoneal shunt. The patient was
treated with antifungal therapy until eye lesions resolved and brain lesions were inactive by neuroimaging.
She received a total cumulative dose of 70 mg/kg of
amphotericin B therapy and 65 days of flucytosine at
100 mg/kg/day. The patient did not have a central catheter and was on parenteral nutrition for 10 days prior
to the diagnosis of systemic fungal infection.

DISCUSSION

Neonatal candidemia occurs in 4-15% of extremely low birth weight infants (4, 5). In recent multicenter
studies, 5-9% of patients with candidemia had evidence of Candida meningitis (6). Identified risk factors for
invasive candida infection include birth weight less than
1000 g (especially <750 g), exposure to more than two
antibiotics, prolonged third generation cephalosporin
exposure, parenteral nutrition including lipid emulsion,
central venous catheter, preceding fungal colonization,
intrauterine growth restriction, pulmonary hemorrhage,
and abdominal surgery (7, 8, 9). Intrauterine infection
with Candida is a rare complication of pregnancy. It is
associated with foreign body during pregnancy (intrauterine device). Antibiotic administration before delivery
is effective to prevent GBS infection and standard of

Figure 1 Brain MRI: Multiple small cystic lesions


in the brain parenchyma.

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Figure 2 Retina Picture: Retinal lesion on right eye


care in preterm PROM, but it may increase babys exposure to Candida by overgrowth in the vagina and
potential spread to uterus. Vaginal candidiasis occurs
frequently among pregnant women, especially in the
last trimester. Initial clinical signs of Candida meningitis are indistinguishable from those of other causes
of systemic infection (10) and most of the infants with
Candida meningitis have negative blood cultures (5,
6, 11). Normal cerebrospinal fluid parameters do not
exclude Candida meningitis (6, 10). A small group of
infants with Candida meningitis (4%) present ventriculitis or brain abscess (prevalence in 21 articles ranges from 0%-69%) (4). Candida meningitis and brain
abscess are associated with significant mortality and
severe neurological impairment (5, 6). In this case,
the patient presented nonspecific symptoms of sepsis
such as apnea, bradycardia and abdominal distention;
later she presented seizures. High index of suspicion
is necessary to identify this kind of infection. Whenever
sepsis is suspected in a neonate, adequate cultures,
including cerebrospinal fluid culture should be obtained
for fungi since up to 9% of patients may have Candida
meningitis. In this case the blood cultures were negative and only cerebrospinal fluid cultures grew Candida,
showing that if CSF culture is not performed the diagnosis may be missed.


Since Candida meningitis may be complicated by multiple small brain abscesses, neuroimaging
should be included in the diagnostic workup of patients
with systemic fungal infection. In the case reported,
head sonogram and head CT failed to give an early
diagnosis of brain abscesses; showing that improved
neuroimaging techniques such as MRI lead to earlier
diagnosis of cerebral abscesses.

Of the previously mentioned risk factors for
Candida infections in the neonate, the patient was on
parenteral nutrition but she did not have a central catheter. She had premature rupture of membranes, and
although vaginal fungal infection was not reported by
the obstetrician, the possibility of vertical transmission
exists. The cause of the pneumonia was not identified,
and candida can not be excluded. Patients born to mothers with vaginal candidiasis near the time of delivery
or with premature or prolonged rupture of membranes
should be identified, and fungal infection should be
considered in these neonates when they present signs
of sepsis.

The associated morbidity and mortality due to
fungal infections in the neonate is high. The patient
we report had hydrocephaly and retinitis secondary to

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47

Candida infection, these complications may affect her


developmental outcome.

In conclusion, fungal infections should be considered in neonates with signs and symptoms of sepsis.
A complete sepsis workup should be done including a
CSF culture, and in patients with systemic fungal infection, neuroimaging should be included as part of the
workup to identify disseminated disease.

9.
Benjamin DK Jr, Ross K, McKinney RE Jr, Benjamin DK,
Auten R, Fisher RG. When to suspect fungal infection in neonates:
A clinical comparison of Candida albicans and Candida parapsilosis fungemia with coagulase-negative staphylococcal bacteremia.
Pediatrics. 2000 Oct; 106(4):712-8.
10.
Fernndez M, Moylett EH, Noyola DE, Baker CJ. Candidal meningitis: a 10-year review. Clinical Infectious Diseases. 2000
Aug; 31(2):458-63.
11.
Stronati M, Decembrino L. Neonatal invasive candidiasis.
Minerva Pediatr. 2006 Dec; 58(6):537-49.

RESUMEN
REFERENCES
1.
Rao S, Ali U. Systemic fungal infections in neonates. J
Postgrad Med 2005;51:27-9
2.
Kaufman D. Fungal infections in preterm infants. Available at: www.emedicine.com/ped/TOPIC3085.HTM accessed on
3/24/2008.
3.
Leibovitz E. Neonatal candidiasis: Epidemiologic, clinical
and therapeutic aspects. Infect Med 20(10):494-498, 2003
4.
Benjamin DK Jr, Poole C, Steinbach WJ, Rowen JL,
Wlash TJ. Neonatal Candidemia and End-Organ Damage: A Critical Appraisal of the Literature Using Meta-analytic Techniques.
Pediatrics. 2003 Sept; 112(3):634-640.
5.
Benjamin DK Jr, Stoll BJ, Fanaroff AA, McDonald SA, Oh
W, Higgins RD, Duara S, Poole K, Laptook A, Goldberg R; National
Institute of Child Health and Human Development Neonatal Research Network. Neonatal candidiasis among extremely low birth
weight infants: risk factors, mortality rates, and neurodevelopmental outcomes at 18 to 22 months. Pediatrics. 2006 Jan; 117(1):8492.
6.
Cohen-Wolkowiez M, Smith PB, Mangum B, Steinbach
WJ, Alexander BD, Cotton CM, Clark RH, Walsh TJ, Benjamin DK
Jr. Neonatal Candida meningitis: significance of cerebrospinal fluid
parameters and blood cultures. Journal of Perinatology. 2007;
27:97-100.
7.
El- Masry FA, Neal TJ, Subhedar NV. Risk factors for
invasive fungal infection in neonates. Acta Paediatrica. 2002;
91(2):198-202.
8.
Chapman RL. Prevention and treatment of Candida infections in neonates. Seminars of Perinatology. 2007 Feb; 31(1):3946.

Trasfondo: Las infecciones sistmicas por Cndida


Albicans son una causa importante de morbilidad y
mortalidad en la unidad de cuidado intensivo neonatal. La incidencia de fungemia por Cndida en los
infantes de extremo bajo peso es de 4-15%. Cinco
a nueve por ciento de los pacientes con fungemia
por Cndida tienen meningitis; de los cuales 4%
presentan ventriculitis o absceso cerebral. Observaciones: Reportamos el caso de un infante nacido
a las 34 semanas de gestacin, quien a los doce
das de nacido present apnea y convulsiones. El
cultivo de lquido cefalorraqudeo creci Cndida
Albicans y los cultivos de sangre fueron negativos.
El sonograma de cabeza y la tomografa computarizada de cabeza fueron normales. La resonancia
magntica detect mltiples micro-abscesos en
el parnquima cerebral. Conclusiones: Este caso
confirma la necesidad de obtener cultivos adecuados; incluyendo cultivos de sangre, orina y lquido
cefalorraqudeo, cuando se sospecha sepsis en un
neonato. Al estudiar al paciente con infecciones
sistmicas por hongo, se debe incluir estudios de
neuroimagenes. Las tcnicas de neuroimagen ms
avanzadas, como la resonancia magntica, diagnostican abscesos cerebrales ms temprano.

AMPR2010

Clnicas Multifsicas de Salud para la Comunidad

ABSTRACT
Hemophagocytic Syndrome (HS) is a clinico-pathologic entity characterized by activation of T lymphocytes and macrophages. It may be diagnosed in
association with malignant, genetic, or autoimmune
diseases, but is most linked with Epstein-Barr virus.
There are few reports of association between HS
and Dengue in pediatrics. Dengue fever, caused by
a flavivirus, is an important mosquito-transmitted disease. It can cause increased vascular permeability
that leads to a bleeding diathesis or disseminated
intravascular coagulation known as dengue hemorrhagic fever (DHF). We present the case of a 10
month-old-female who developed DHF and dengue
shock syndrome, requiring admission to intensive
care unit. She developed hemophagocytosis diagnosed by bone marrow aspiration and atypical skin
changes that have not been previously described
in association with dengue fever. This is an unusual
case of dengue related hemophagocytic syndrome
that adds to the limited pediatric cases reported in
literature.
Index words: dengue, virus, hemophagocytic, syndrome

DENGUE VIRUS
ASSOCIATED
HEMOPHAGOCYTIC
SYNDROME IN CHILDREN:
A Case Report

Yadira Soler Rosario, MD*


Ricardo Garcia MD**
Alicia Fernandez Sein MD**

From the *Department of Pediatrics and **Critical Care Pediatric Section, UPR School of Medicine.
Correspondence and reprints request should be sent to Yadira Soler Rosario, MD - UPR School of Medicine, Department
of Pediatrics, PO Box 365067, San Juan, PR 00936-5067.
E-mail: ysoler@hotmail.com.

INTRODUCTION


emophagocytic Syndrome (HS) is a clinico-pathologic entity characterized by activation and
uncontrolled proliferation of T lymphocytes and macrophages, leading to cytokine overproduction. Patients usually present with an acute febrile illness,
hepatosplenomegaly, and pancytopenia [1]. Characteristic biochemical markers include elevated tryglicerides, ferritin, and low fibrinogen [2]. HS in children
has been linked to viral, bacterial, fungal, and parasitic
infections, and to a broad spectrum of malignancies
and genetic disorders, such as Chediak-Higashi disease, Griscelli Syndrome, and Familial Erythrophagocytic Lymphohistiocytosis (FEL) [1]. Leading triggering
agents in infection associated hemophagocytosis are
viruses of the herpes group, especially Epstein-Barr virus and Cytomegalovirus. Dengue virus is considered
as an uncommon etiologic agent for infection associated hemophagocytosis [3].

Dengue fever, caused by dengue virus, is an
important mosquito-transmitted disease due to its increased worldwide incidence and its related complications. It is caused by mosquito-borne arboviruses of
the Flavivirus family. The most common manifestations
of dengue virus infection are high fever, rash, and musculoskeletal pain [4]. Common hematologic presentations of dengue infection in early stage of this disease
are thrombocytopenia, hemoconcentration, and leukopenia [3]. Dengue hemorrhagic fever (DHF) and its
most severe form, dengue shock syndrome (DSS), are
potentially fatal dengue complications. The underlying
pathogenesis of dengue hemorrhagic fever includes a

rapidly developing capillary leakage syndrome with hemoconcentration and venous pooling. If uncorrected, it
may quickly lead to hypotension and shock [5].

Several observations have been made on changes in the bone marrow of patients with dengue fever
and Dengue hemorrhagic fever. There are few reports
with detailed studies of marrow aspirates throughout
the course of the disease [6]. In the early stage, a markedly hypocellular marrow with abnormal megakaryocytopoiesis is common [7]. Hemophagocytosis is an
uncommon presentation of dengue infection. Dengue-related hemophagocytosis has been described in
the literature [3-4,6,8-14]. However, a minority of these reported cases are in pediatric patients [3,9-10,13],
and even less in infants. In an analysis of etiology and
outcome of secondary hemophagocytic lymphohistiocytosis in children, a statistically significant association
between poorer prognosis and patients age less than
three years was identified [15].

We describe a 10-month-old-infant with DHF
and DSS who developed hemophagocytosis. The patient developed septic shock and multi organ failure
requiring admission to PICU, support with mechanical
ventilation, and inotropics. During the course of her
illness, she presented atypical skin changes in distal
upper extremities that have never been reported in pediatric patients with dengue and HLH. There are some
reports in literature on the cutaneous manifestations of
dengue viral infection. Cutaneous findings that have
been described include: morbilliform eruption,

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49

petechiae, ecchymoses, mucosal involvement such as


crusting of lips and tongue, soft palate vesicles, and
macular blanchable erythema of trunk and limbs [1621].

Case Report

This is the case of a 10-month-old-female patient with past history of prematurity 33 weeks gestational age, twin pregnancy, history of bronchial asthma,
no allergies, taking no medications at home, with immunizations up to date. Patient had been doing well
until one week prior to admission, when she developed
fever of 38.6oC, associated to green nasal secretions,
cough, and erythematous non-pruritic rash over her entire body. Patient was admitted to the Community Pediatric Hospital general ward with an initial diagnosis
of viral syndrome. During admission, patient persisted
with fever, and also developed irritability and decreased
oral intake, and was started on IV antibiotic Ceftriaxone
and antiviral Oseltamivir. Three days later, patient developed episodes of coffee-ground vomiting, and mother started noticing generalized swelling. Chest X-rays
done at that time showed bilateral pleural effusions.
With these symptoms and findings, a suspicion of Dengue Hemorrhagic Fever was established, and the patient was admitted to the Pediatric Intensive Care Unit
(PICU). Upon admission, patient developed respiratory
difficulty requiring oxygen at 50 %, and was found to
have severe anemia (hgb 3.2/ hct 8.6), thrombocytopenia of 27,000 (previous platelet count of 475,000),
prolongued PT and PTT, and hypofibrinogenemia. On
physical exam, she had generalized anasarca, and hepatosplenomegaly. She required support with packer
red blood cells (PRBC), platelets, fresh frozen plasma
(FFP), and IV Antibiotics. Vancomycin, Cefotaxime,
Gentamycin, and antiviral Oseltamivir were continued
for a total of five days. She also had elevated liver enzymes (AST 2219/ ALT 835), hypoalbuminemia (2.3),
elevated CPK (4588), LDH (3454), and ferritin levels
(16,500). Due to intravascular fluid depletion and generalized edema, patient was started on bolus of albumin
25%. A central femoral vein was cannulated. Patient
then started presenting fluid filled vesicles in right upper
extremity, initially in puncture sites, which spread and
worsened rapidly, forming multiple bullaes with purpuric patches and macules (see Figure 1). Bilateral upper
extremity arterial Doppler showed no evidence of deep
venous thrombosis. Due to the rapid progression and
worsening of skin changes, patient was transferrred to
our PICU, for pediatric surgery evaluation.

Upon arrival to our institution, patient was in
critically ill condition, hypoactive, with anasarca, abdominal distention that was compromising breathing,
hepatosplenomegaly, tense edema in all extremities,
multiple ecchymoses in all puncture sites, faint distal
pulses in all extremities, right arm and hand purpuric
patches and macules with overimposed bullae, and left
hand clear fluid bullae. Skin changes in left hand had
not been present in prior institution, and these worsened rapidly in the initial hours of her admission to our
unit. Due to patients presentation, poor respiratory
effort, and vital signs showing tachycardia and wide
pulse pressure, patient was intubated and placed on

mechanical ventilator- pressure SIMV, with parameters:


TV 90, Rate 25, PEEP 8, PS 12, FIO2 50%, IT 0.8. She
was started on inotropic support with Dopamine at 12.5
mcg/kg/min and Dobutamine at 10 mcg/kg/min. Due to
anasarca, patient also started on Furosemide drip at
0.5 mg/kg/hr, and albumin 25% at a dose of 3 grams
per day. Due to severe abdominal distention, she was
placed NPO receiving caloric requirements through
parenteral nutrition. Due to skin changes that were
evolving so rapidly and high possibility of bacterial infection, Infectious disease service was consulted, and
patient was started on IV antibiotics Cefepime, Clindamycin, Gentamycin, Zyvox, and Penicillin G. Titers for
Epstein-Barr virus, Cytomegalovirus, Dengue, Herpes,
Parvovirus B19, Hepatitis A & B, HIV were done. A
dose of IVIG at 1 gram/ kg was given. Due to high suspicion of adrenal insufficiency, patient received stress
dose of Solucortef and continued receiving IV steroids.
Laboratories at the time showed WBC 8,000; hgb 6.6/
hct 18.6, platelets 112,000, prolonged PT 16.7 and PTT
64, fibrinogen 167, and D-dimers 2.49. Metabolic panel
showed hypokalemia (2.6), hypomagnesemia (1.6), albumin 3.3, increased liver enzymes (AST 1435, ALT
546), increased LDH (2290), increased CPK (5205),
lactate elevated (47), and elevated ferritin (8250). Urinalysis showed hematuria. Patient received transfusion
with PRBCs, FFP, and Vitamin K. Due to the findings
in laboratory studies of hypofibrinogenemia, increased ferritin, cytopenia affecting more than two cellular lines, Hemophagocytic Syndrome was suspected,
and patient was evaluated by pediatric hematology/
oncology service. Bone marrow aspiration and biopsy
were done, and results were positive for hemophagocytosis. Chemotherapy was reccommended, but, due
to patients hemodynamic and respiratory instability, it
was not ordered initially. Patient received sedation and
analgesia with Midazolam and Fentanyl by continuous
infusion, and muscle relaxation with Vecuronium. Her
skin condition was evaluated by dermatology service,
and skin biopsy was done, with results of epidermal
necrosis. Pediatric Surgery service evaluated patient,
and reccommendations were to provide daily local care
with silver antimicrobial wound gel, covered with kerlix
gauze. Daily surgical reevaluation was done, and patient did not require surgical debridement.

During the day of admission, patient presented
clinical deterioration, manifested as decreased peripheral saturation, worsening hypoxia in ABGs, requiring
increased PEEP in mechanical ventilator (up to 14).
Chest X-ray showed worsening of right pleural effusion,
so chest tube placement was done. Initial extraction of
yellow fluid- 65 mL, from which samples were sent to
the laboratory for: culture, cellularity, chemistry, and flow
cytometry. Patient also presented hypotension despite
inotropic support, so an assessment of acute adrenal
insufficiency caused by septic shock was made, and
patient received Solucortef stress dose. Cortisol levels
were done, with results of 17.22. Cardiology evaluation at the time showed a minimal anterior pericardial
effusion, an ejection fraction of 67 % (with inotropic support), mild tricuspid and pulmonary regurgitation, and
no vegetations. Patient also required muscle relaxation
with Vecuronium by continuous infusion. After these interventions, patients respiratory state started showing

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50

slow but steady improvement.

Ca 10.6, Albumin 4.5, AST 59, ALT 42, triglycerides


151, ferritin 447. Patient was found in a good state of
health, still with mild muscular weakness. IgM titers
were positive. Patients skin continued receiving daily
local care. Extremities were never again punctured in
our institution, blood samples were taken from central
vein catheter. Patients skin started improving slowly
but steadily, and she continued receiving follow-up
by dermatology and pediatric surgery service. Patient
remained in mechanical ventilation for a total of eight
days, after which she was extubated successfully, and
tolerated therapy with nasal cannula at 1 Liter/minute.
After extubation, patient also started on diet by sucking, and she tolerated it very well. She received a total
of seven days of IV antibiotics, and 10 days of IV Fluconazole. Diuretic therapy was continued, but doses


After aggressive initial interventions, patients
respiratory state started improving, and mechanical
ventilator parameters were weaned slowly as tolerated.
Blood pressures started improving, and inotropic support with dopamine drip was discontinued after three
days of therapy. Dobutamine drip was discontinued after four days of therapy. Patient persisted with edema,
and therapy with diuretics was continued, but patient
with persisitent hypokalemia that required oral and IV
potassium supplementation. Hypoalbuminemia improved after three days of IV albumin infusion. Patient
continued receiving TPN, which was combined with enteral nutrition with hydrolyzed formula. Enteral nutrition
was increased until achieving patients nutritional goal
of 100 kcal/kg/day.
Patient did present
h y p e r g l y c e m i a Figure 1
that required the- Laboratory Values
rapy with insulin,
with improvement. LAB Day 1(1) Day 5 (2) Day 6 Day 7 Day 8
Patient persisted WBC 11.7
13.5
13
13.9 13.1
with liver enzyme Hgb 12.5
12.5
3.2
7.3
9.7
elevation, but the35.5
8.6
20.9 27.5
se started decrea- Hct 36.5
475
27
154 83
133
sing. LDH, ferritin Plt
levels also persis- Neu 59.7
41.6
49 51
57
ted elevated. Due
Lym
20.3
50.9
39
38
35
to the findings of
7
10.9 11
9
hemophagocytic Mono 18.6
syndrome,
the- Gluc 112
118
74
83
101
rapy with dexa- BUN 7
19
18
10
7
methasone was
Creat
0.2
0.4
0.43
0.4
0.28
started for a total
136
133
141 140 136
of 28 days, rec- Na
commended
by K
4.7
4.4
5.7
3
2.9
ped hematology/ Cl
106
106
108 100
99
oncology
serviCO2
22
20
14
27
31
ce. Chemotherapy with etoposide Ca
9.2
7.4
8.4
8.7
8.3
was started when Mg
2.3
2
1.9
patient achieved
3.7
5.2
5.3 4.5
a more stable res- T.Prot
Alb
2.3
3.7
4.2
3.1
piratory and cardiovascular state. AST
2219
2108 2025 1901
Patient continued ALT
835
658 753 360
receiving IV anti4588
biotics. Blood, uri- CPK
T.bili
0.4
2.2
1.6 0.6
ne, skin, pleural,
and stool cultures Chol
48
were negative, but Trig
73
trachea
culture
16500
was positive for Ferritin
yeast, so patient LDH
3454
also received the- Pt
22.1
15.8 13.4
rapy with FluconaPtt
144
64.8 57.6
zole. IgM titers for
116 148
EBV, CMV, Parvo- Fib
virus, and Herpes D-dimer
were
negative.
HIV 1 & 2 was
negative. Influen- (1) Initial Symptoms
za A & B test was (2) Admission to PICU of initial institution
negative. Dengue (3) Admission to PICU of our institution

Day 9(3) Day 10 Day 11 Day 12


8
5.8
9.3
9
6.6
11.5
10.5
7.7
18.6
32.9
31
23
112
72
103
92
58
65
54
45
38
23
31
45
2
7
10
10
104
98
136
168
2
6
24
21
0.16
0.17
0.26
0.19
137
137
140
144
2.6
2.4
2.8
2.8
92
86
84
95
32
29
37
34
8.8
8.1
9.7
10.1
1.6
2
2.3
2.2
5.5
6.7
6.8
6.8
3.9
3.8
4.1
4.4
1435
1066
880 758
546
385
349 364
5205
2876
1.2
2.68
2.2
1.17
54
80
69
166
8250
8250 8250
2290
2290 2290
16.7
16.3
14.7 14.2
64
45.5
35.2 34.2
167
221
2.49
2.37 3.4

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51

Day 13
15
11.3
33
60
55
37
8
296
13
0.17
145
6
105
27
11
2.2
4.1

were decreased and these were eventually discontinued. Analgesia and sedation were progressively weaned. Patient was transferred to Hematology/Oncology
ward after 11 days in the Pediatric Intensive Care Unit,
where she continued receiving chemotherapy and IV
steroids as reccommmended by Pediatric Hematology/
Oncology Service. At Oncology ward, patient remained
admitted for 9 more days. She persisted with marked
muscular weakness, for which brain MRI was done,
with normal findings. Physical Medicine and Rehabilitation service evaluated patient, and she showed marked improvement with physical therapies. Patient was
discharged home with follow-up by Pediatric Hematology-Oncology service, to continue chemotherapy as
scheduled as outpatient.

Follow- up examination in oncology clinics
showed: WBC 12.8, hgb 11, hct 32, plt 528, Na 140,
K 6 (hemolyzed), Cl 108, CO2 22, BUN 11, creat 0.4,
Ca 10.6, Albumin 4.5, AST 59, ALT 42, triglycerides
151, ferritin 447. Patient was found in a good state of
health, still with mild muscular weakness.

DISCUSSION

Hemophagocytic Syndrome is an uncommon
but severe illness associated with a variety of infectious agents, as well as genetic, neoplastic, and autoimmune diseases. Hemophagocytic Syndrome in
the context of infection is best described as part of a
spectrum of EBV- associated illness resulting in clonal
proliferation of T-lymphocytes, with excessive activation of macrophages. This syndrome may be difficult to
distinguish from T-cell lymphoma and should be treated
aggresively with etoposide- based chemotherapeutic
regimens. Hemophagocytic Syndromes associated
with other infectious illnesses, including EBV, CMV,
tuberculosis, typhoid fever, and leishmaniasis, may
resolve with treatment of the underlying infection, and
their recognition is important as they mimic malignant
disease. Epstein-Barr virus-associated Hemophagocytic Syndrome is almost universally fatal if untreated,
with death usually resulting from hemorrhage, infection, or multiorgan failure [22].

Dengue fever, a syndrome caused by several
arthropod-borne viruses, is characterized by biphasic fever, myalgia or arthralgia, rash, leukopenia, and
lymphadenopathy. Dengue hemorrhagic fever is a
severe, often fatal, febrile disease caused by dengue
viruses. It is characterized by capillary permeability,
abnormalities of hemostasis, and, in severe cases, a
protein-losing shock syndrome (dengue shock syndrome), which is thought to have an immunopathologic
basis. In dengue, neutropenia and thrombocytopenia
are common, and bone marrow changes have been
described: Hypocellular marrow, abnormal megakaryocytopoiesis [23], bone marrow suppression affecting all marrow elements [7,24-25]. Dengue infection
of hematopoietic cells is not cytotoxic but slows cell
proliferation [23]. Dengue virus is considered as an
uncommon etiologic agent for infection associated hemophagocytosis. A literature search shows fewer than
20 case reports of dengue related hemophagocytosis
[3]. Nelson et al [6] initially reported several postmortem

cases in children with dengue infection who presented hemophagocytosis. Aside from these postmortem
cases, there have been less than 10 reports of dengue related hemophagocytosis in live children [3,910,13,15,26], and even less in infants. Veerakul et al
[15] found a statistically significant association between poorer prognosis in secondary hemophagocytic
syndrome and patients age less than 3 years old. Our
case report features a 10 month old female patient
with an acute febrile illness, who developed pancytopenia, and progressing to dengue hemorrhagic fever
and dengue shock syndrome. Most of the previously
reported cases of hemophagocytic syndrome are dengue hemorrhagic fever. A causal relationship between
hemophagocytosis and the severity of dengue infection has been suggested but not fully established [6].
Cutaneous manifestations in dengue infection have
been described. The most common skin findings reported in dengue infection are: morbilliform eruption,
petechiae, ecchymoses [16,18-19,21], mucosal manifestations such as crusting of the lips, vesicles in
soft palate, erythema of tongue [16,21], and macular
blanchable erythema [17,20]. Our patient had very distinct skin findings in distal upper extremities: fluid filled
vesicles, initially in puncture sites, which spread and
worsened rapidly, forming multiple bullaes with purpuric patches and macules. These findings created an
initial differential diagnosis of purpura fulminans, toxic
epidermal necrolysis, or infectious skin process such
as necrotizing fascitiis. The patient had sepsis, which
could be the cause of purpura fulminans, had been
treated with several medications prior to arrival to our
institution (Oseltamivir, Ceftriaxone), which could have
caused TEN, and she could have had a secondary infectious skin process. All these etiologies were considered, so dermatology intervention was required, and
skin biopsy revealed epidermal necrosis and thrombosis. Patient improved progressively with IV antibiotics,
supportive therapy for shock and DIC. This type of skin
manifestation had not been associated to dengue infection before, and this case raises the possibility of a
relationship between skin findings in dengue fever and
severity of illness.

REFERENCES
1.
Gagnaire MH, Galambrun C, Stephan JL, et al. Hemophagocytic Syndrome : A Misleading Complication of Visceral
Leishmaniasis in Children- A Series of 12 Cases. Pediatrics. 2000;
2000; Vol.106 No.4.
2.
Janka, Gritta E. Familial and acquired hemophagocytic
lymphohistiocytosis. Eur J Pediatr. 2007; 166: 95-109.
3.
Jain D, Singh T. Dengue virus related hemophagocytosis: a rare case report. Hematology. 2008. Vol 13, No.5.
4.
Lu P, Hsiao H, Tsai J, Chen T, Feng M, Chen T, Lin
S. Dengue Virus- Associated Hemophagocytic Syndrome and Dyserythropoiesis: A Case Report. Kaiohsiung J Med Sci. January
2005; Vol 21. No 1.
5.
Morens, David M. Dengue Fever and Dengue Hemorrhagic Fever. The Pediatric Infectious Disease Journal. Volume
28(7), July 2009, pp 635-636.
6.
Nelson ER, Bierman HR, Chulajata R. Hematologic
phagocytosis in postmortem bone marrows of dengue hemorrhagic
fever. American Journal of Medical Sciences. 1966; 252: 68-74.
7.
Bierman H, Nelson ER. Hematodepressive Virus Diseases of Thailand. Annals of Internal Medicine. 1965; Vol 62;
Number 5: pp 867-883.
8.
Wong KF. Dengue virus infection-associated hemophagocytic syndrome. American Journal of Hematology. 1991; 38(4):
339-40.

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52

Figure 2: Skin Findings

9.
Rueda E, Mendez A, Gonzalez G. Hemophagocytic
Syndrome associated with dengue hemorrhagic fever. Biomedica.
2002; 22(2): 160-166.
10.
Ramanathan M, Duraisamy G. Hemophagocytosis in
dengue hemorrhagic fever: a case report. Annals of Academic Medicine Singapore. 1991; 20 (6): 803-804.
11.
Nakamura I, Nakamura-Uchiyama F, Komiya N, Ohnishi
K. A case of dengue fever with viral-associated hemophagocytic
syndrome. Kansenshogaku Zasshi. 2009; 83(1): 60-63.
12.
Srichaikul T, Punyagupta S, Kanchanapoom T, Chanokovat C, Likittanasombat K, Leelasiri A. Hemophagocytic Syndrome
in Dengue hemorrhagic fever with severe multiorgan complications.
Journal of Medical Association of Thailand. 2008; 91(1): 104-109.
13.
Vijayalakshmi AM, Ram Ganesh VR. Hemophagocytic
Syndrome associated with Dengue Hemorrhagic Fever. Indian Pediatrics. 2009; Vol 46, 545.

14.
Gajinov Z, Vuckovic N, Duran V, Matic M, Ivkov-Simic M,
Rajic N. Viral form of hemophagocytic syndrome with erythrodermal clinical picture- case report. Med Pregl. 2008; 61(7-8):405408.
15.
Veerakul G, Sanpakit K, Tanphaichitr VS, Mahasandana
C, Jirarattanasopa N. Secondary hemophagocytic lymphohistiocytosis in children: an analysis of etiology and outcome. Journal of
Medical Association of Thailand. 2002; 85 Suppl 2: S530-541.
16.
Thomas EA, John M, Bhatia A. Cutaneous manifestations
of dengue viral infection in Punjab. International Journal of Dermatology. 2007, 46, 715-719.
17.
Saleem K, Shaikh I. Skin lesions in hospitalized cases of
dengue Fever. Journal Coll Physicians Surg Pak. 2008; 18(10):
608-11.
18.
Mahe A, Lamaury I, Strobel M. Mucocutaneous manifestations of dengue. Presse Med. 1998; 27(37):1909-13.

BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

53

19.
Lupi O, Tyring S. Tropical Dermatology: Viral Tropical diseases. Journal of the American Academy of Dermatology. Volume 10, Issue 5.
20.
Caumes F, Santi C, Felix H, Bricaire F, Danis M, Gentillini
M. Cutaneous signs of dengue, apropos of 3 cases. Bull Soc Pathol Exot. 1993; 86(1):7-11.
21.
Desruelles F, Lamaury I, Roudier M, Goursaud R, Mahe
A, Castanet J, Strobel M. Cutaneo-mucus manifestations of dengue. Annals Dermatologica Veneorol. 1997; 124(3): 237-241.
22.
Fisman DN. Hemophagocytic Syndromes and Infection.
Emerging Infectious Diseases. 2000;6(6); Centers for Disease
Control and Prevention (CDC).
23.
Nakao S, Lai CJ, Young NS. Dengue Virus, a Flavivirus,

propagates in human bone marrow progenitors and hematopoietic


cell lines. Blood. Vol 74, No 4, 1989: pp 1235-1240.
24.
Gawoski JM, Ooi W. Dengue Fever mimicking plasma
cell leukemia. Archives of Pathology in Lab Medicine. Vol 127,
August 2003.
25.
La Russa VF, Innis BL. Mechanisms of dengue virus-induced bone marrow suppression. Baillieres Clinical Haematology.
1995 Mar; 8(1):249-270.
26.
Pongtanakul B, Narkbunnam N, Veerakul G, Sanpakit K,
Viprakasit V, Tanphaichitr VT, Suvatte V. Dengue hemorrhagic fever in patients with thalassemia. Journal of Medical Association of
Thailand. 2005 Nov; 88 Suppl 8: S80-5.

Figure 3: Bone Marrow Images of Hemophagocytosis

RESUMEN
Figure 4: Patients skin one month after discharge from
hospital

El Sindrome hemofagoctico es una entidad caracterizada


por activacin descontrolada de macrfagos y linfocitos
T. Se ha diagnosticado en asociacin a malignidad, enfermedades genticas o autoimmunes, pero primordialmente a infecciones con Epstein-Barr. Hay pocos reportes de
asociacin entre el sndrome hemofagoctico y el virus del
dengue en pediatra. La fiebre de dengue, causada por un
flavivirus y transmitida por un mosquito, causa un aumento
en la permeabilidad vascular. Esta puede provocar sangrado o coagulacin intravascular diseminada conocida como
dengue hemorrgico. Presentaremos el caso de una nia
de 10 meses con dengue hemorrgico y sndrome de shock
por dengue, requiriendo admisin a la Unidad de Intensivo
Peditrico. Desarroll sndrome hemofagoctico, diagnosticado con aspirado y biopsia de mdula sea. La paciente
present unos cambios dermatolgicos no antes descritos
en asociacin a dengue. Este es un caso atpico de sndrome hemofagoctico asociado a dengue, que se aade a los
pocos casos peditricos reportados.

eHr?
eRx?
dudas?

SOLUCIN

www.asociacionmedicapr.org

TRANSANAL
CIRCULAR-STAPLED
REANASTOMOSIS AS A
MANAGEMENT
ALTERNATIVE
FOR ANASTOMOTIC
COLONIC STRICTURES:
A NOVEL TECHNIQUE IN
THE PEDIATRIC PATIENT
Humberto Lugo-Vicente MD*
Jorge J. Zequeira MD**
Joalex Antongiorgi MD
From the *Section of Pediatric Surgery, ** Department of General Surgery, and U.P.R. School of Medicine.
Address reprints requests: Humberto Lugo-Vicente MD
PO Box 10426, San Juan, PR 00922. E-mail <titolugo@
coqui.net>

INTRODUCTION



ectal strictures in children can occur after
inflammatory bowel disease, trauma, or most commonly following a colorectal anastomosis (1). Anastomotic colorectal strictures are usually defined as being
less than 10 to 12mm in diameter and are usually short
(<1 cm) in length. Colonic and rectal anastomotic strictures occur with an incidence ranging from 3% to 30%
(2-5). Most respond to sequential dilatations, while
others are more recalcitrant will need resection (6). In
this report and review of literature, we propose a safe
and effective approach to anastomotic rectal strictures
in children by resecting and anastomosing the strictured segment of bowel with the use of a transanal circular stapling device.

Case History

This is the case of a 12 year-old-male patient
who first came to the Puerto Rico Medical Centers
University Pediatric Hospital on November 2008 with
history of severe constipation since birth and a rectosigmoid transitional zone in a barium enema. After performing a rectal biopsy, the patient was diagnosed with
Hirschsprungs disease. The patient has a history of
prior right-sided colectomy for perforated necrotizing
enterocolitis during the neonatal period. In preparation for definitive therapy, the patient was taken to the
operating room on January 2009 where a sigmoid loop
colostomy was performed in order to decompress his
megacolon. Three months later a modified pull-through
procedure was planned. During mobilization of the left
colon, the bowel circulation of the pull-through segment was felt to be precarious and an original Soave
procedure was performed, leaving the bowel past the

ABSTRACT

A 12 year-old-male patient with a recalcitrant rectal anastomotic stricture following two
failed endorectal pull-through (Soave) procedures for Hirschsprungs disease was satisfactorily
managed with transanal resection using a circular
stapling device. This is the first reported case of a
benign colonic anastomotic stricture treated transanally with a circular stapling device in a pediatric patient.
INDEX WORDS: transanal, stapled, reanastomosis, anastomotic, colonic, stricture
anus for several centimeters. Continuous distal bowel
ischemia prompted further proximal mobilization, pullthrough and anastomosis with the creation of a proximal loop ileostomy. The child developed a symptomatic
rectal stricture causing tenesmus and abdominal pain
that required management with daily Hegar dilatations.
Dilatations could not progress beyond the 18 mm size
through the persistent stricture. A distal ileostogram
was performed showing a short (< 1 cm) stricture.
Then, a short colonoscopy revealed viable mucosa
before and after the rectal stricture. Due to the recalcitrant and symptomatic nature of the rectal stricture a
resection and re-anastomosis by means of a circular
mechanical stapler was planned.

During surgery the patient was placed in lithotomy position, anal retractors were introduced and the
stricture was re-evaluated, admitting barely a single digit (see Figure 1). The Tyco CEEA 28mm instrument
was forced through the stricture with the anvil still connected to the device although partially unscrewed. The
CEEA 28mm was fired according to Tyco instructions
with the anvil distal to the stricture and its stapling portion in a proximal location. After removing the circular
stapling device, a complete ring of tissue was obtained. A new circumferential-stapled anastomosis was
observed inside the intestinal lumen and it admitted
two digits comfortably (see Figure 2). The child was
discharged home the same day of the procedure. The
patient was re-evaluated 2 weeks after the procedure
and the anastomosis admitted a 20mm Hegar dilator
comfortably. Six months after the ileostomy closure the
child is asymptomatic and continent.

DISCUSSION

Anastomotic strictures are a severe complication of colorectal anastomoses (7). Colonic and rectal
anastomotic strictures occur with an incidence ranging
from 3% to 30% (2-5). Some of the factors that may
contribute to the formation of an anastomotic rectal
stricture include tissue ischemia, infection following
anastomotic leakage, inflammatory response to anastomotic sutures or staples, defunctionalization, circular
stapler size, or fecal contact within the anastomosis (16). Patients with rectal strictures can present with intestinal obstruction, frequent bowel movements, lower
abdominal fullness, or tenesmus followed by anal pain.

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56

Figure 1: Pinpoint rectal stricture.

therapy, transanal incision, or


excision and reanastomosis by
means of a circular stapler (4).
Postoperative rectal strictures
usually respond well to direct
dilation without the need for
endoscopic balloons (5). Nevertheless, these techniques
may have drawbacks such as
recurrence, psychological implications of repeated anal manipulation, rectal perforation,
and need for special and costly
equipment (1).

Circular-stapled
anastomosis of the rectum is a
safe and quick technique that
is utilized frequently for benign
and malignant colonic diseases
(4). Adult patients with rectal
anastomotic strictures who fail
manual dilations have satisfactorily undergone reanastomosis using a circular stapler to
create a new circumferentialstapled anastomosis of bigger
size while resecting the scar
tissue (1,4,5,7). In the case we
report, the patient complained
of tenesmus and abdominal
pain due to his rectal stricture,
which was partially controlled
with daily manual dilatations.
The manual dilatations produced a sufficiently pliable stricture to allow forced passage
of the anvil. In other reports,
water-soluble contrast material
has been needed to couple the
anvil with the firing instrument
under fluoroscopic guidance.
In cases where the lumen is
completely obliterated, the anvil can be introduced through
a proximal stoma or colotomy.
The anvil can then be drawn
through the stricture via colonoscopy utilizing a snare and
coupling it to the body of a
circular stapling gun permiting
excision of the stricture (5,8).
Circular-stapled reanastomosis
is a simple, effective, and costefficient technique that can be
safely used to manage recalcitrant rectal strictures in children.

Figure 2: After re-anastomosis with circular stapler

A rectal examination, endoscopic evaluation and radiographic contrast study are often necessary to determine the length and configuration of the stricture,
prior to definitive therapy. Treatment is generally based
on their cause, location and appearance (5). Various
methods have been described, including endoscopic
techniques like balloon dilation and/or stents, transanal manual or bougie dilations, microwave coagulation

REFERENCES
1) Shimada S, Matsuda M, Uno K, Matsuzaki H, Murakami S, Ogawa
M: A New Device for the Treatment of Coloproctostomic Stricture
After Double Stapling Anastomoses. Ann Surg 1996;224:603-608.
2) Suchan KL, Muldner A, Manegold BC: Endoscopic treatment
of postoperative colorectal anastomotic strictures. Surg Endosc
2003;17:1110-1113.

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57

3) Garcea G, Sutton CD, Lloyd TD, Jameson J, Scott A, Kelly MJ:


Management of benign rectal strictures: a review of present therapeutic procedures. Dis Colon Rectum 2003;46(11):1451-60.
4) Pabst M, Giger U, Senn M, Gauer JM, Boldog B, Scweizer W:
Transanal treatment of strictured rectal anastomosis with circular
stapler device: Simple and safe. Dig Surg 2007;24:12-14.
5) McKee R, Pricolo V: Stapled revision of complete colorectal
anastomotic obstruction. Am Journal Surg 2008;195:526-527.
6) Lillehei CW, Leichtner A, Bousvaros A, Shamberger RC: Restorative proctocolectomy and ileal pouch-anal anastomosis in children. Dis Colon Rectum 2009;52(9):1645-1649.
7) Nissotakis C, Sakorafas GH, Vugiouklakis D, Kostopoulos P, Peros G: Transanal circular stapler technique: a simple and highly
effective method for the management of high-grade stenosis of low
colorectal anastomoses. Surg Laparosc Endosc Percutan Tech
2008;18(4):375-378.
8) Rees JR, Carney L, Gill TS, Dixon AR: Management of recurrent
anastomotic stricture and iatrogenic stenosis by circular stapler.
Dis Colon Rectum 2004;47(6):944-947.

OFICINAS ADMINISTRATIVAS
SUBSCRIPCIONES Y ANUNCIOS
Asociacin Mdica de Puerto Rico

PO Box 9387 SANTURCE, Puerto Rico 00908-9387


Tel 787-721-6969 Fax: 787- 724-5208
Email: mlaureano@asociacionmedicapr.org

RESUMEN

ANUNCIOS EN BOLETIN Y WEB SITE

Se presenta un nio de doce aos que desarrollo una estrechez en su anastomosis rectal despus de dos intentos fallidos de llevar a cabo un
procedimiento de descenso colnico por la enfermedad de Hirshcsprungs. Esta estrechez fue
manejada de forma satisfactoria re-haciendo la
anastomosis usando un aparato mecnico circular de anastomosis. Este caso representa el primer reporte de una estrechez benigna colnica
manejada con un aparato de grapas mecnicas
en un paciente peditrico.

ralicea@asociacionmedicapr.org
Web Site: www.asociacionmedicapr.org

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Mdica de Puerto Rico y la Asociacin Mdica
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La Asociacin Mdica de Puerto Rico no se hace
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Todo anuncio para ser publicado debe reunir las
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La AMPR no se har responsable por material y/o
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Todo artculo recibido y/o publicado est sujeto a
las normas y reglamentos de la Asociacin Mdica
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58

CME Credits Boletin Vol 102 No 01, 2010


Questions from article entitled: KNOWLEDGE AND
MISCONCEPTIONS
ABOUT
IMMUNIZATIONS
AMONG MEDICAL STUDENTS, PEDIATRIC AND
FAMILY MEDICINE RESIDENTS, by Taon V et al.
1.
According to literature, which of the following is
a common cause that impedes a child from receiving
an immunization on the proper schedule?
a.
Shortage of immunizations
b.
Missed appointments
c.
Patient is receiving antibiotics
d.
Physician misconceptions regarding immunization contraindications
e.
Patient has diarrheal illness
2.
Why it is currently required by the Medical
Schools and Licensing Agencies that Pediatric and Family Medicine residents be competent in their knowledge about immunizations?
a.
Because the responsibility to immunize children resides mostly in pediatricians and family medicine physicians.
b.
Because several studies have revealed a lack
of up to date immunization knowledge in residents and
other healthcare professionals.
c.
Because physicians need to be able to clearly
transmit immunization information to the patients parents in order to gain their authorization instead of their
resistance against vaccines.
d.
Because proper immunization education to
physicians can ensure better immunization outcomes
in children.
e.
All of the above.

5.
Which of the following statements is True?
a.
The Vaccine Adverse Event Report System
(VAERS) has found various dangerous vaccine lots
which have been removed by the FDA.
b.
The severity of chicken pox has decreased due
to the increasing amount of children immunized against
it.
c.
Fever is not a contraindication to immunization,
except in a moderate to severe acute illness when immunization should be deferred for later.
d.
Immunizing a woman while breastfeeding is
contraindicated due to the risk of infecting her baby
through passage of virus in breast milk.
e.
Children with egg allergy should not receive
MMR, MMRV, measles, or mumps vaccine without having a previous skin testing due to risk of anaphylaxis.
Questions from article: EXCLUSIVE BREASTFEEDING REDUCES ASTHMA IN A GROUP OF CHILDREN FROM THE CAGUAS MUNICIPALITY OF
PUERTO RICO, by Gonzlez J et al.
6. Children of which ethnic group have the highest prevalence of lifetime asthma?
a.
Non-Hispanic Black
b.
Mexican
c.
Non-Hispanic white
d.
Puerto Rican
e.
Chinese
7. Which of the following has not been shown in studies
to be decreased in infants exclusively breastfed?
a.
Allergic rhinitis
b.
Asthma
c.
Atopic dermatitis
d.
Eczema
e.
Poison ivy

3.
Can the MMR, MMRV, Measles or Mumps be
administered to children allergic to eggs?
a.
No, because it contains derivatives of egg protein.
b.
Yes, because it does not contains derivatives of
egg protein.
c.
No, because they first need to have a skin testing due to risk of anaphylaxis.
d.
Yes, because the amount of cross reactivity
with egg protein is small.
e.
No, because although it does not contain egg
protein derivatives its anaphylactic potential is uncertain.

Questions from article: RESPIRATORY ILLNESS IN


LATE PRETERM INFANT DURING THE FIRST SIX
MONTHS OF LIFE, by Prez L et al.

4.
When can fever be considered a contraindication for immunization?
a.
Always
b.
If the patient has mild fever and is receiving antibiotics
c.
If the patient has a mild acute illness with low
grade fever
d.
When a patient has moderate to severe acute
illness
e.
Fever is never a contraindication to immunization.

9. Late preterm infants are defined as:


a.
Infants born between 32 and 34 6/7 weeks
gestations
b.
Infants born between 34 and 36 6/7 weeks
gestations
c.
Infants born between 35 1/7 and 36 6/7 weeks
gestations
d.
Infants born between 33 and 36 6/7 weeks
gestations
e.
Infants born between 33 and 35 6/7 weeks
gestations

8. Ogbuanu and coworkers showed that at 10 years of


age children who were breastfed had:
a. Decreased forced vital capacity
b. Increased peak expiratory flow
c. Decreased tidal volume
d. Increased total lung capacity
e. Decreased functional residual capacity

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10. Late preterm infants:


a. Are not physiologically immature and at risk for respiratory complications
b. Have not higher rates of morbidity and mortality at
birth compare to term infants
c. Are more frequently readmitted to the hospital during
the neonatal period compare to term infants.
d. Are all considered for palivizumab immunoprophylaxis
e. Do not account for most of the premature births
11. The increased respiratory diseases risk of late preterm infants can be mainly attributed to:
a.
Immature immune system
b.
Underdeveloped lungs
c.
Incomplete transfer of maternal antibodies
d.
alternatives A and B
e.
All of the above
12. The most common diagnoses for readmission during the first year of life of late preterm infants are related to the following systems:
a. Neurological and Respiratory
b. Cardiovascular and Respiratory
c. Cardiovascular and Neurological
d. Gastrointestinal and Respiratory
e. Gastrointestinal and Cardiovascular
Questions from article: PRENATAL BREASTFEEDING INTENTIONS IN A GROUP OF WOMEN WITH
HIGH RISK PREGNANCIES, by Diaz Rozett H et al.
13. The Healthy People 2010 goal regarding breastfeeding is that:
a.
90% of the mothers initiate breastfeeding
b.
25% of the mothers breastfeed their infants up
to 2 years old
c.
75% of the mothers continue to breastfeed after getting back to work
d.
100% of the mothers give colostrum to their babies
e.
50% of the mothers continue breastfeeding up
to 6 months
14. Which of the following antihypertensive medications have been associated to bradycardia and hypotension in some breastfed infants?
a. Angiotensin converting enzyme inhibitors
b. Beta-blockers
c. Calcium channel blockers
d. Hydralazine
e. Methyldopa
15. Which of the following factors improve the rates of
breastfeeding?
a. feelings of embarrassment
b. fear of pain
c. higher education
d. lack of social support
e. use of medications
Questions from article: MINOR HEAD INJURY IN
CHILDREN YOUNGER THAN TWO YEARS OF AGE:
DESCRIPTION, PREVALENCE AND MANAGEMENT
IN THE EMERGENCY ROOM OF THE PEDIATRIC
UNIVERSITY HOSPITAL by Fernandez ML et al.

16. Children less than two years are prone to suffer


head trauma mainly due to:
a.
Immature visual function
b.
Inability to walk appropriately
c.
Lack of adequate care
d.
The greater proportion of mass and body surface area represented by their heads
17. In children, minor head trauma:
a. Commonly occurs as a results of falls
b. Commonly requires surgical interventions
c. Is a very common cause of fractures and intracranial
injuries
d. Loss of consciousness is a common sign
18. Upon evaluation of minor head trauma in a child
less than two years old:
a. Diagnostic strategies differ among individuals and
institutions
b. Gold standards protocols exist to determine plan of
action
c. Skull radiographs are helpful to establish management
d. Vomiting correlates clearly with severity of trauma
Questions from article: ROOMING-IN IMPROVES
BREASTFEEDING INITIATION RATES IN A COMMUNITY HOSPITAL IN PUERTO RICO, by Cotto CW
et al.
19. Which hospital practice is not associated to increase duration of breastfeeding?
a.
breastfeeding within the first hour
b.
giving breastmilk only
c.
allowing fathers in the delivery room
d.
availability of infant rooming-in
e.
no pacifier use
20. Which of the following factors increases the risk of
complementary breastfeeding?
a. At home delivery
b. Having a support group in the hospital
c. Full time rooming-in
d. Cesarean section
e. Maternal knowledge about breastfeeding advantages
21. The Baby Friendly Initiative helps mothers initiate
breastfeeding:
a. after the baby is observed at the nursery
b. within 1 hour after delivery
c. when the babys glucose level is normal
d. as soon as the mother produces enough milk
e. during the first 24 hours
Questions from article: ESOPHAGEAL ATRESIA:
NEW GUIDELINES IN MANAGEMENT by Lugo-Vicente HL.
22. What is the most common congenital anomaly of
the esophagus?
a. proximal esophageal atresia with distal and proximal
tracheoesophageal fistula
b. pure tracheoesophageal fistula
c. proximal esophageal atresia with proximal

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60

tracheoesophageal fistula
d. proximal esophageal atresia with distal tracheoesophageal fistula
e. pure esophageal atresia
23. Maternal polyhydramnios is most commonly associated with:

CME Credits Boletin Vol 102 No 01, 2010


Check correct answers.

ANSWERS

a. proximal esophageal atresia with distal and proximal


tracheoesophageal fistula
b. pure tracheoesophageal fistula
c. proximal esophageal atresia with proximal tracheoesophageal fistula
d. proximal esophageal atresia with distal tracheoesophageal fistula
e. pure esophageal atresia

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24. Effective management of uncomplicated proximal


esophageal atresia with distal tracheoesophageal fistula consist of:
a. gastrostomy
b. fundoplication
c. closure of tracheoesophageal fistula with esophageal anastomosis
d. closure of tracheoesophageal fistula
e. esophageal anastomosis

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BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010

61

(In vitro data; clinical significance unknown.


Full course of therapy is complete in 7 days.)1,2
n

ZYMAR ophthalmic solution rapidly eradicates


key pathogens in vitro, including:
S aureus: eradicated in 15 minutes1,*
S epidermidis: eradicated in 30 minutes1,*
S pneumoniae: eradicated in 10 minutes2,*
H influenzae: eradicated in 5 minutes2,*

* Time to reach kill threshold. 10 CFU/mL is the lower limit of detection and is
indistinguishable from complete kill.

ZYMAR ophthalmic solution is indicated for the treatment of bacterial conjunctivitis caused by susceptible strains
of the following organisms: Corynebacterium propinquum, Staphylococcus aureus, Staphylococcus epidermidis,
Streptococcus mitis, Streptococcus pneumoniae, and Haemophilus influenzae. (Efficacy for this organism was studied
in fewer than 10 infections.)
Important Safety Information: NOT FOR INJECTION. ZYMAR ophthalmic solution should not be injected
subconjunctivally, nor should it be introduced directly into the anterior chamber of the eye. As with other antiinfectives, prolonged use may result in overgrowth of nonsusceptible organisms, including fungi. If superinfection
occurs, discontinue use and institute alternative therapy. Patients should
be advised not to wear contact lenses if they have signs and symptoms of
bacterial conjunctivitis.

The most frequently reported adverse events occurring in approximately 5%


to 10% of the overall study population were conjunctival irritation, increased
lacrimation, keratitis, and papillary conjunctivitis.
Please see brief prescribing information on adjacent page.
1. OBrien TP. Antimicrobial efficacy of ZYMAR and Vigamox against Staphylococcus species. Refract Eyecare Ophthalmol. 2003;7(12):15-18. 2. Novosad BD, Callegan MC.
Killing of Streptococcus pneumoniae, methicillin-resistant Staphylococcus aureus (MRSA), and Haemophilus influenzae ocular isolates by fourth-generation fluoroquinolones.
Poster presented at: 78th Annual Meeting of the Association for Research in Vision and Ophthalmology; April 30-May 4, 2006; Fort Lauderdale, FL.
2009 Allergan, Inc., Irvine, CA 92612 www.allergan.com marks owned by Allergan, Inc.
ZYMAR is licensed from Kyorin Pharmaceutical Co., Ltd., Tokyo, Japan. APC50TC09 803807

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