Esophageal strictures in children with recessive dystrophic epidermolysis bullosa: an 11-year experience with fluoroscopically guided balloon dilatation.

BACKGROUND

Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder that is associated with significant esophageal strictures, resulting in dysphagia and nutritional failure. Although endoscopically guided balloon dilatation is a widely used treatment, the use of an endoscope carries the risk of oropharyngeal trauma. To minimize this risk, we have eliminated its use.

METHOD

We reviewed the charts of all RDEB patients who underwent balloon dilatation for esophageal strictures between August 1993 and March 2005. Balloon dilatation procedures were performed under anesthesia and with fluoroscopic control.

RESULTS

We performed 92 dilatations on 25 RDEB patients. Most patients reported immediate relief of symptoms, rapid recovery, and resumption of adequate food intake within 1 day. The mean interval between dilatations was 1 year. Six patients (24%) have required only 1 dilatation, and 1 of these 6 has had a dilatation-free interval of 25 months. One patient with a history of multiple dilatations has remained dilatation-free for 5 years. No procedure-related complications have occurred.

CONCLUSIONS

Fluoroscopically guided balloon dilatation is a gentle, safe, effective, and repeatable technique that should be considered as a first line of treatment.