Isolated congenital esophageal stenosis

Abstract

The incidence of congenital esophageal stenosis (CES) is approximately 1 in 25,000 to 50,000 live births. There is associated esophageal atresia in one third of cases; the remainder are classified as isolated CES. Histologically, the anomaly may include tracheobronchial remnants, a membranous diaphragm, or diffuse fibrosis of the muscularis and submucosa. The authors report their experience with three patients who had isolated CES. The patients were free of symptoms for the first 6 months of life. All had difficulty with feeding from 6 to 12 months of age, which corresponded with the introduction of solids. Most solid feedings were regurgitated. Evaluation consisted of cine-esophagogram, pH monitoring, manometry, and endoscopy, with biopsies to exclude the diagnosis of gastroesophageal reflux. All patients underwent hydrostatic dilatation, but the benefit was only transient. The patients were referred for surgical correction when symptoms recurred. Limited resection of the esophageal stenosis with primary anastomosis was performed on all three patients via a left thoracotomy. The stenoses were located in the distal third of esophagus, near the junction with the middle third. Pathological examination showed tracheobronchial remnants in one patient and fibrotic muscle in the other two. The average age at the time of surgery was 19 months. The hospital stay averaged 8 days. The contrast study 1 week postthoracotomy showed esophageal patency in all patients, with no leakage. All were discharged from the hospital, tolerating solid food. CES should be sought for in patients who present with dysphagia to solid food that begins after the first 6 months of life. Its treatment is a limited esophageal resection and usually is associated with good results.