Congenital Esophageal Stenosis

This is an uncommon anomaly, occurring in 1 in 25,000 to 1 in 50,000 live births (Murphy et al. 1995). Some consider esophageal stenosis to be a very mild variant of EA (Berrocal et al. 1999). The association with TEF in one-third of cases and other congenital anomalies in 17%-33% (Vasudevan et al. 2002) may support this theory. Most strictures are localized, 2-to 3-mm areas of narrowing approximately at the junction of the middle third and the distal one-third of the esophagus. Congenital esophageal stenosis may occur as an isolated narrowing due to ectopic cartilaginous tracheobronchial remnants, as an incomplete membranous diaphragm or web, or a localized segmental hypertrophy of the muscularis and submucosal layers with diffuse fibrosis (Murphy et al. 1995). There is a higher incidence of post-operative anastomotic leaks in children with TEF who have an associated congenital esophageal stenosis (Newman and Bender 1997). Esophageal stenosis usually presents when solid food is introduced or after impaction of a foreign body. In older children the differential diagnosis includes strictures due to reflux, caustic ingestion and sequelae from surgery. The diagnosis should be considered in an infant with dysphagia or with an impacted foreign body after EA repair. Esophageal stenosis must be considered in any child with acute dysphagia or foreign body impaction even without the antecedent history of atresia.

Treatment is resection with end-to-end esopha-geal anastomosis. Dilatation has a high incidence of esophageal perforation, especially in young children, and may be related to the length of the stricture and its transmural involvement (Newman and Bender 1997). The diameter of the lumen increases with age and growth of the patient, and with repeated dilatations, but will never be normal (Newman and Bender 1997). Some patients will benefit from initial dilatation, but most ultimately require surgery (Amae et al. 2003).

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