Surgical management of esophageal stricture due to recessive dystrophic epidermolysis bullosa.
Esophageal stricture may occur as a complication of the dystrophic recessive form of epidermolysis bullosa. Mechanical dilatation by bougienage may cause more severe esophageal injury. The fourth reported case of esophageal replacement by colonic interposition for this condition is presented. Central venous total parenteral alimentation was a helpful adjunct in the surgical management of this patient. Colonic replacement is preferred over local resection of an esophageal stricture because in most instances there is extensive inflammatory involvement of the entire esophagus.
D000293:Adolescent; D002908:Chronic Disease; D003106:Colon / Q000637:transplantation; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000235:genetics*; D004940:Esophageal Stenosis / Q000601:surgery*; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D014182:Transplantation, Autologous