Congenital Esophageal Stenosis

Abstract

Congenital esophageal stenosis (CES) is a rare condition. There are three pathological types of CES: with tracheobronchial remnants in the esophageal wall, fibromuscular thickening of the esophageal wall, and membranous mucosal diaphragm or web. Congenital esophageal atresia is the most common associated anomaly. Symptoms of CES include vomiting or regurgitation, dysphagia, recurrent respiratory tract infections, and growth retardation. Esophagograms of stenosis exhibit tapered or abrupt narrowing of the esophagus with various degrees of dilatation of its suprastenotic portion. Esophagoscopy, manometric study, and pH monitoring are helpful tools for differential diagnoses in distinguishing CES from achalasia and secondary esophageal stricture due to gastroesophageal reflux (GER). Balloon dilatation is the first choice of treatment in CES. When patients fail to respond to repeated attempts of bougienage, surgical intervention should be considered. Resection of the stenosis followed by end-to-end esophageal anastomosis is a general surgical treatment. Good outcome of circular myectomy has been also reported. Complications, including an iatrogenic esophageal perforation following bougienage and leakage after segmental resection and reconstruction of the esophagus, have been reported. While good prognosis has been reported following dilatation and/or surgery when needed, it was also reported that dysphagia occurred frequently regardless of the therapeutic option at follow-up. Close, long-term follow-up is highly recommended.