Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa.
BACKGROUND
Epidermolysis bullosa is a rare genetically determined disorder of the stratified squamous epithelium. Patients with the most severe forms develop scarring of the esophagus after ingestion of food. This results in dysphagia, which severely compromises the ability to eat. Maintenance of adequate nutritional intake is a central aim, but the most appropriate method is unknown.


METHODS
The results of endoscopic through-the-scope balloon dilation under propofol anesthesia in 53 patients with epidermolysis bullosa and esophageal strictures are reported.


RESULTS
Seventy-five percent of patients had a single stricture (range 1 to 6 strictures), most often in the proximal esophagus (median 20 cm from incisors). A total of 182 dilations were performed (median two per patient) over a median follow-up period of 3.5 years. For all but 3 patients, there was an improvement in the dysphagia score. There was a mean increase in weight after the procedure of 2.9 kg: 95% CI[2.0, 3.8]; p<0.001, over a median 29 days. There was no significant post-procedure morbidity.


CONCLUSIONS
Endoscopic balloon dilation is a safe and effective treatment for the esophageal strictures of epidermolysis bullosa. In the majority of patients, dilation relieves dysphagia and improves nutritional status.
Anderson
Simon H C
SH
Department of Gastroenterology, St. Thomas' Hospital, London, United Kingdom.
Meenan
John
J
Williams
Keith N
KN
Eady
Robin A J
RA
Prinja
Hasita
H
Chappiti
Usha
U
Doig
Laura
L
Thompson
Richard P H
RP
D000293:Adolescent; D000328:Adult; D002404:Catheterization / Q000379:methods*; D002648:Child; D003680:Deglutition Disorders / Q000209:etiology / Q000628:therapy; D004820:Epidermolysis Bullosa / Q000150:complications*; D004940:Esophageal Stenosis / Q000209:etiology / Q000628:therapy*; D004945:Esophagoscopy; D005260:Female; D006801:Humans; D008297:Male; D012720:Severity of Illness Index; D016896:Treatment Outcome