Congenital esophageal stenosis (CES) is a rare cause of esophageal narrowing due to intrinsic esophageal wall abnormalities such as tracheobronchial remnants (TBR), web, and fibromuscular dysplasia (FMD). It is sometimes associated with esophageal artesia (EA).

The medical records of children, who underwent operation due to CES at the Asan Medical Center from Jan 1990 to Dec 2007, were retrospectively reviewed.

Among 12 patients (M : F=6 : 6), 9 patients had TBR and 3 had FMD. The median age of operation was 25 months (7 mo~6 years). Four patients underwent operation for esophageal atresia at neonate, one underwent operation for duodenal atresia and one for ventricular septal defect. Recurrent vomiting or dysphagia was developed at weaning period or introduction of solid foods. The lesions of stenosis were low-esophagus in 11 and mid-esophagus in 1. Segmental resection of lesion and end-to-end anastomosis of esophagus were completed successfully by means of laparotomy in 9 and thoracotomy in 3 (left in 1, right in 2). One patient treated with ballooning at first, suffered from mediastinitis due to esophageal perforation. Postoperative complications were anastomosis leakage in 2 and lung abscess in 1. Esophageal ballooning for anastomosis stricture was performed in 2 patients and an esophageal stent was needed in 1 patient. All patients are relieved from dysphagia and able to eat solid food.

CES is rare but should be considered for recurrent vomiting and dysphagia, especially for those who underwent operation for EA. Differential diagnosis from other causes such as gastro-esophageal reflux is essential and surgical treatment is treatment of choice.