[Esophageal strictures in children with epidermolysis bullosa].

UNLABELLED: Esophageal stricture is a rare but often severe complication of recessive dystrophic epidermolysis bullosa in children. The purpose of the study was to review this digestive complication with emphasis on diagnostic modalities and therapeutic management.

PATIENTS AND

METHODS

This was a retrospective study of two pediatric cases of esophageal stenosis that occurred during generalized recessive dystrophic epidermolysis bullosa of the Hallopeau-Siemens type.

RESULTS

The 2 patients were aged 8 years 8 months and 11 years 5 months, respectively. Dysphagia was of early onset, before the age of 10 years in both cases. Esophageal opacification led to the diagnosis of esophageal stenosis located in the upper 1/3 of the esophagus in 1 case and at the junction between the middle and the lower 1/3 of the esophagus in the other case. None of the 2 patients received medical treatment, and pneumatic dilation was the treatment method that was advocated. Esophageal endoscopy showed the stenosis and helped guide the positioning of the balloon catheter. These patients underwent 2 and 3 sessions of dilation, respectively, at intervals of 2 months and 1 year. Balloon dilation has allowed the patients to have a more comfortable life with decreased dysphagia and a substantial improvement in nutritional status. However, this improvement was transient (1 patient had symptomatic recurrence of stenosis after 3 years), which shows that monitoring of the patients and the resumption of dilatation sessions may be necessary.

CONCLUSION

Esophageal strictures in dystrophic epidermolysis bullosa of the Hallopeau-Simens type are severe and difficult to support. Pneumatic dilatation is the treatment of choice for the fragile esophagus. It gives satisfactory results and can be repeated without significant risk.