ISOLATED CONGENITAL ESOPHAGEAL STENOSIS

The incidence of congenital esophageal stenosis (CES) is approximately 1 in 25,000 to 50,000 live births. There is associated esophageal atr esia in one third of cases; the remainder are classified as isolated C ES. Histologically, the anomaly may include tracheobronchial remnants, a membranous diaphragm, or diffuse fibrosis of the muscularis and sub mucosa. The authors report their experience with three patients who ha d isolated CES. The patients were free of symptoms for the first 6 mon ths of life. All had difficulty with feeding from 6 to 12 months of ag e, which corresponded with the introduction of solids. Most solid feed ings were regurgitated. Evaluation consisted of cine esophagogram, pH monitoring, manometry, and endoscopy, with biopsies to exclude the dia gnosis of gastroesophageal reflux. All patients underwent hydrostatic dilatation, but the benefit was only transient. The patients were refe rred 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 i n the distal third of esophagus, near the junction with the middle thi rd. Pathological examination showed tracheobronchial remnants in one p atient and fibrotic muscle in the other two. The average age at the ti me of surgery was 19 months. The hospital stay averaged 8 days. The co ntrast study 1 week postthoracotomy showed esophageal patency in all p atients, with no leakage. All were discharged from the hospital, toler ating solid food. CES should be sought for in patients who present wit h dysphagia to solid food that begins after the first 6 months of life . Its treatment is a limited esophageal resection and usually is assoc iated with good results. Copyright (C) 1995 by W.B. Saunders Company