J Korean Assoc Pediatr Surg.  2018 Jun;24(1):1-4. 10.13029/jkaps.2018.24.1.1.

Congenital Esophageal Stenosis in Children: From Etiology to Prognosis

Affiliations
  • 1Department of Pediatric Surgery, Pusan National University Children's Hospital, Yangsan, Korea.
  • 2Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea. spkhy02@snu.ac.kr

Abstract

Congenital esophageal stenosis (CES) is a rare disease that has been reported to occur once in every 25,000 to 50,000 births. According to its etiology, CES is divided into 3 subtypes, tracheobronchial remnants (TBR), fibromuscular hypertrophy (FMH) and membranous diaphragm (MD). Symptoms begin at the weaning period and the introduction of solid food around 6 months with dysphagia and vomiting. Esophagography is first screening test and endoscopic ultrasonography plays important roles to diagnose subtypes deciding therapeutic plan. TBRs were generally treated with surgical resection and end-to-end anasotomosis, whereas FMH and MD had good response rate to endoscopic or radiologic guided dilatation. This article reviews the literature on the etiology, clinical course, diagnosis and management of CES including recent opinion.

Keyword

Congenital esophageal stenosis; Esophagus; Tracheobronchial remnants; Child

MeSH Terms

Child*
Deglutition Disorders
Diagnosis
Diaphragm
Dilatation
Endosonography
Esophageal Stenosis*
Esophagus
Humans
Hypertrophy
Mass Screening
Parturition
Prognosis*
Rare Diseases
Vomiting
Weaning
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