Esophageal strictures in children with recessive dystrophic epidermolysis bullosa: an 11-year experience with fluoroscopically guided balloon dilatation
Adolescent; Barium; Catheterization; Child; Preschool; Epidermolysis Bullosa; Dystrophica/co [Complications]; Esophageal Stenosis/et [Etiology]; Esophageal Stenosis/th [Therapy]; Female; Humans; Male; Retrospective Studies; Treatment Outcome; 24GP945V5T (Barium); feeding difficulties; surgical intervention; 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.
Azizkhan R G; Stehr W; Cohen A P; Wittkugel E; Farrell M K; Lucky A W; Hammelman B D; Johnson N D; Racadio J M
Journal of Pediatric Surgery
2006
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.jpedsurg.2005.10.007" target="_blank" rel="noreferrer noopener">10.1016/j.jpedsurg.2005.10.007</a>
Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa
adolescent; dysphagia; major clinical study; priority journal; follow up; scoring system; school child; human; article; female; male; adult; morbidity; treatment outcome; balloon dilatation; barium; body weight; contrast enhancement; endoscopic therapy; epidermolysis bullosa/th [Therapy]; epidermolysis bullosa; esophagus dilatation; esophagus stricture/th [Therapy]; nutritional status; propofol; feeding difficulties; surgical intervention; endoscopic balloon dilatation
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 S H C; Meenan J; Williams K N; Eady R A J; Prinja H; Chappiti U; Doig L; Thompson R P H
Gastrointestinal Endoscopy
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/S0016-5107%2803%2902379-4" target="_blank" rel="noreferrer noopener">10.1016/S0016-5107%2803%2902379-4</a>