Case Report

Esophageal Atresia
Eric Tjahyadi*, Peby Maulina Lestari*
*Department of Obstetrics & Gynecology, Sriwijaya University, Palembang, Indonesia

ABSTRACT
Background: Esophageal atresia is a disruption of the esophagus, producing a blind-ending
esophageal pouch or tracheoesophageal fistula (or both), and resulting in a small or absent
stomach in bubble.
Objective: To present and discuss the diagnosis and management of esophageal atresia in
Fetomaternal Division of Dr. Mohammad Hoesin General Hospital Palembang.
Case: A 28-year-old multigravida pregnant woman was admitted to the Obstetrics and
Gynecology Department, Sriwijaya University Palembang with pregnancies with congenital
anomalies polihydrmanios. She has routinely controlled with OBGYN and referred to the
Moehammad Hospital because of the congenital anomalies. In ultrasound examination, there
were diminutive stomach bubble, polyhydramnios, “pouch sign”, and sandal gap.
Conclusion: Polyhydrmamnios is present in two thirds of cases of esophageal atresia.
Approximately 80% of patients with esophageal atresia have another associated anomalies
(Cardiac, Urogenital, Musculoskeletal, Craniofacial, and Neurologic). Esophageal atresia can
be part of a VATER syndrome (Vertebral defects, imperforate anus, trachea-esophageal
fistula, renal defects) or VACTERL association (Vertebral abnormality, Anal atresia, Cardiac
defect, Tracheoesophageal fistula, renal and radial limb abnormality)
Keywords: Esophageal atresia, tracheoesophageal fistula.

INTRODUCTION polyhydramnios, “pouch sign”, and sandal

Esophageal atresia is a disruption of the gap. After being scheduled for an elective
esophagus, producing a blind-ending cesarean section schedule, a female live
esophageal pouch or tracheoesophageal baby was born with body weight 2700
fistula (or both), and resulting in a small or gram, body length 48 cm, and normal
absent stomach in bubble.1 APGAR Score. She was diagnosed with
CASE REPORT esophageal atresia and tracheoesophageal
A 28-year-old multigravida came to the fistula and planned for thoracotomy. She
outpatient department with full term has been hospitalized in NICU. She had
pregnancy, weighing 62 kg and 155 cm been performed Tracheoesophageal
height. She has routinely controlled with ligation, anastomosis resection of esofago-
OBGYN and reffered to the Moehammad esofagostomy, and stam gastrostomy with
Hospital because of the congenital post operative diagnosis Type C esophageal
anomalies. In ultrasound examination, there atresia. After 28 days of observation in
were diminutive stomach bubble,
pediatric ward, she has been discharged and the third semester. A pouch sign may be
doing well. present, indicating a dilated upper
esophagus, posterior to a fluid-filled
DISCUSSION trachea. Tracheoesophageal fistula
Esophageal atresia is a disruption of the commonly accompanies esophageal atresia,
esophagus, producing a blind-ending with 86% of fetuses presenting with a
esophageal pouch or tracheoesophageal proximal esophageal atresia and a distal
fistula (or both), and resulting in a small or tracheoesophageal fistula. This allows fluid
absent stomach in bubble.1 to pass into the stomach, making prenatal
Incidence of esophageal atresia is diagnosis difficult.1
approximately 3/10.000 live births. Males Polyhydrmamnios is present in two thirds
2
slightly more common than females. of cases of esophageal atresia.
Esophageal atresia results from faulty Approximately 80% of patients with
embryogenesis. The pharyngeal foregut in esophageal atresia have another associated
the early embryo divides and elongates into anomalies (Cardiac, Urogenital,
trachea and esophagus. During this rapid Musculoskeletal, Craniofacial, and
elongation, the continuity of the esophageal Neurologic). Esophageal atresia can be part
tube is interrupted, resulting in a blind of a VATER syndrome (Vertebral defects,
upper esophageal pouch and a separate imperforate anus, trachea-esophageal
distal esophagus that communicates with fistula, renal defects) or VACTERL
the stomach. Esophageal atresia is a association (Vertebral abnormality, Anal
multifactorial, sporadic condition involving atresia, Cardiac defect, Tracheoesophageal
genetic predisposition and environmental fistula, renal and radial limb abnormality).
exposures (e.g., alcohol, smoking, Esophageal atresia also associates with
medications, infections, agriculture aneuploidy (trisomy 18,21).1
chemicals). 1 Threre are five types of esophageal atresia
Persistent lack of sonographic stomach namely proximal atresia with distal TEF
visualization after the 14th week of (82%), proximal and distal atresia, no
gestation should prompt a thorough fistula (9%), H-type fistula with no atresia
assessment. Esophageal atresia is suspected (6%), atresia with both proximal and distal
when there is a persistent absent or fistula (2%), and proximal TEF with distal
diminutive stomach bubble, despite atresia (1%).2
prolonged and repetitive imaging, which Management for esophageal atresia such as
concomitant polyhydramnios, particulary in antenatal monitoring for amniocentesis
should be considered for karyotype. Fetal tracheoesophageal fistula) in a term baby
echocardiogram is recommended to assess without congenital heart defects is
cardiac structure and function. Serial approximately 3%. Long term morbidity
ultrasound examinations to monitor fetal includes anastomotic complications,
growth, amniotic fluid, and worsening fetal reccurent fistula, esophageal reflux,
condition (e.g, hydrops fetalis). tracheomalacia, short-term growth
Amnioreduction may be indicated to reduce restriction, and potential for developing
risk of preterm delivery or relieve maternal gastroesophageal cancer.1-3
respiratory compromise. Fetal nonstress or CONCLUSION
biophysical profile testing (or both), twice Esophageal atresia is a disruption of the
weekl, beginning at 32-34 weeks. Prenatal esophagus, producing a blind-ending
neonatology and pediatric surgery esophageal pouch or tracheoesophageal
consultation to discuss postnatal fistula (or both), and resulting in a small or
mangement and prognosis. 3-4 absent stomach in bubble. Preparation for
Delivery in a tertiary care facility is delivery and team coordination is needed
recommended. Cesarean delivery is for reducing morbidity and mortality.
reserved for obstetric indications. Caution
is required because of risk for umbilical References
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Perinatal mortality related to isolated
esophageal atresia (with or without