Epiphrenic Diverticulum in an Infant with Congenital Esophageal Stenosis Associated with Esophageal Atresia

Abstract

Congenital esophageal stenosis (CES) is a rare congenital disorder which may be isolated or associated with esophageal atresia (EA). It courses with esophageal outflow tract obstruction. Esophageal epiphrenic diverticulae are esophageal outpouchings above diaphragm which develop because of an underlying esophageal motor disorder. We present an infant who had CES associated with EA detected during the course of routine follow-up. She underwent several sessions of esophageal balloon dilatations for CES with some symptomatic improvement. However, an epiphrenic diverticulum (ED) developed during the course of treatment which was detected by further investigations. A surgical excision was performed with a successful outcome. An ED developed secondary to CES has not been previously reported. We have highlighted the diagnostic modalities employed, treatment options, and clinical follow-up for these two rare conditions in children.

Keywords: Esophageal atresia; children; congenital esophageal stenosis; epiphrenic diverticula; esophageal diverticula.

Figure 1

(a) Upper contrast study showing proximally dilated esophagus and a conical distal narrowing, (b) Initial balloon dilatation reaching to a size of 13.5 mm, (c) Upper contrast study showing epiphrenic diverticulum above diaphragm, (d) Mature chondroid tissue (arrows) in the wall of the resected diverticulum (H&EX100).

Figure 2

(a) Endoscopic view of proximal esophageal atresia repair site (arrows) which was widely patent, (b) Distal esophageal stenosis above gastroesophageal junction, (c,d) Endoscopic view of the epiphrenic diverticulum (ED) in relation to the esophagus (E) and the gastroesop-hageal junction (arrow).

Figure 3

T2W1 magnetic resonance images (a) Axial view of dilated mid-esophagus (arrow) with an intraluminal feeding tube, (b) Cystic structure (broken arrow) with hyperintense air-fluid level and probable connection with esophageal lumen, (c) Coronal view of proximal portion of the cystic structure (arrow-head) lying on the same plane with the dilated mid-esophagus (arrow) which contains an intraluminal feeding tube (d) Cystic structure (broken arrow) with hyperintense air-fluid level.