Esophageal strictures in children with recessive dystrophic epidermolysis bullosa: experience of balloon dilatation in nine cases.

Abstract

Recessive dystrophic epidermolysis bullosa is a rare disorder characterized by extreme vulnerability of the squamous epithelium and mucous membranes. Minor trauma such as is caused by swallowing solid food is followed by blistering and scarring. Stricture formation at the pharyngoesophageal junction (C6 or C7) is the severest complication of this disease. We evaluate the effectiveness of Microvasive Rigiflex balloon dilatation and extensive nutritional support as a primary treatment for this condition. Nine of 21 recessive dystrophic epidermolysis bullosa patients developed esophageal strictures at the level of the pharyngo-esophageal junction (C6). We treated them with intensive nutritional therapy followed by balloon dilatation, which produces longitudinal pressure and provides prompt relief from esophageal stricture. Eleven balloon dilatations have been performed in 9 patients. All patients had poor physical development and were severely malnourished; extensive nutritional support was required before treatment could begin. Balloon dilatation was performed once in seven patients and twice in two patients. No recurrent stricture formation was observed after balloon dilatation. Intensive nutritional support followed by balloon dilatation is the first choice of treatment for esophageal strictures complicating recessive epidermolysis bullosa. By following this regime, invasive surgery can be avoided.