Fluoroscopically Guided Dilation of Esophageal Strictures in Patients With Dystrophic Epidermolysis Bullosa: Long-Term Results
children; experience; management; balloon dilatation; benign esophageal stricture; dystrophic epidermolysis bullosa; eb; fluoroscopically guided balloon dilation; Radiology Nuclear Medicine & Medical Imaging; stenosis; feeding difficulties; epidermolysis bullosa; surgical intervention; fluoroscopically guided dilation of esophageal strictures; dysphagia; nutritional failure; gastronomy tube placement
OBJECTIVE. The purpose of this study was to investigate the immediate and long-term outcomes after fluoroscopically guided balloon dilation of esophageal strictures in a series of patients with dystrophic epidermolysis bullosa (DEB). MATERIALS AND METHODS. Between 2005 and 2011, the medical records of all patients with DEB treated with fluoroscopically guided balloon dilation of esophageal strictures were included in the study and retrospectively analyzed. The indication for treatment was dysphagia attributed to at least one radiologically verified esophageal stricture. The primary endpoints of the study included procedural technical success, clinical improvement assessed with a 0-4 dysphagia score, and major complication rate. Secondary endpoints were patient survival and reintervention rates. RESULTS. Nineteen consecutively registered patients with DEB (age range, 10-51 years; mean, 30 +/- 12.2 years) and dysphagia due to esophageal strictures were treated with fluoroscopically guided balloon dilation. In total, 90 procedures and 121 dilations were performed to manage 28 lesions. Balloon diameter ranged from 8 to 18 mm. The mean follow-up time was 47.51 +/- 16.64 months (range, 17-73 months). The technical success rate was 96.7% (87/90). There were no major complications. The mean reintervention rate was 1.19 dilations per patient per year, and the postprocedural dysphagia score (0.72 [95% CI, 0.56-0.87]) was significantly lower than baseline (2.50 [95% CI 2.35-2.65]) (p < 0.001). CONCLUSION. Repeated fluoroscopically guided balloon dilation is safe and effective for the management of dysphagia caused by esophageal strictures in DEB. Use of this technique was associated with marked clinical improvement in dysphagia and satisfactory long-term reintervention rates with no major complications.
Spiliopoulos S; Sabharwal T; Krokidis M; Gkoutzios P; Mellerio J; Dourado R; Adam A
American Journal of Roentgenology
2012
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/10.2214/ajr.11.8159" target="_blank" rel="noreferrer noopener">10.2214/ajr.11.8159</a>
Balloon dilatation in esophageal strictures in epidermolysis bullosa and the role of anesthesia
adolescent; dysphagia; retrospective study; priority journal; school child; preschool child; clinical effectiveness; patient safety; human; article; child; female; male; adult; clinical article; young adult; colon interposition; gastrostomy; anesthesia; balloon dilatation; epidermolysis bullosa; esophagus stenosis/co [Complication]; esophagus stenosis/th [Therapy]; digestive endoscope; esophagus balloon; esophagus perforation; feeding difficulties; surgical intervention; endoscopic balloon dilatation
Esophageal involvement, which causes stricture, is a complication in epidermolysis bullosa. This causes dysphagia and malnutrition and leads to deterioration of skin lesions in these patients. The charts of 11 patients with epidermolysis bullosa and esophageal stricture who were included into dilatation program between 2003 and 2015 were retrospectively reviewed. Seven of the patients were female and four were male. The median age was 14 (2-32) years. The mean body weight of patients was 27.8 (9-51) kg. The location and number of strictured parts of the esophagus were previously evaluated with upper gastrointestinal contrast study and after that flexible endoscopy was used for dilatation. Eight patients had middle esophageal, three patients had proximal esophageal and one of them had both proximal and middle esophageal strictures. The strictures were dilated 56 times in total (mean 5 times). One patient underwent gastrostomy and was medically followed-up after a perforation occurrence during the dilatation procedure. In a 32-year-old female patient, colon interposition was performed after four dilatations since optimal nutritional and developmental status could not be achieved. The dilatation program of nine patients is still in progress. Seven of them can easily swallow solid food but two of them have some difficulties in swallowing between dilatations. One patient rejected the program and quitted, while one patient refused colon interposition and died because of complications related to amyloidosis during the dilatation program. After resolution of the swallowing problem, skin lesions were observed to heal quickly. Epidermolysis bullosa is a rare cause of dysphagia. Esophageal balloon dilatation with flexible endoscopy is a safe and efficient method in patients with this condition.
Gollu G; Ergun E; Ates U; Can O S; Dindar H
Diseases of the Esophagus
2017
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/10.1111/dote.12503" target="_blank" rel="noreferrer noopener">10.1111/dote.12503</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>
Endoscopic balloon dilatation of esophageal strictures in children
Children; Pediatrics; experience; management; Dysphagia; Otorhinolaryngology; atresia; Balloon dilatation; conservative treatment; dilation; epidermolysis bullosa; Esophageal strictures; ingestion; repair; feeding difficulties; surgical intervention; endoscopic balloon dilatation
Aim of the study: To assess the efficacy and safety of endoscopic balloon dilatation of esophageal strictures in children. Material and methods: Design: retrospective case series; population: 49 patients under 18 years of age referred to our center for esophageal strictures; treatment: endoscopic balloon dilatation: outcome parameters: residual dysphagia, weight gain, iatrogenic esophageal perforation, assessment of the esophageal lumen by endoscopy or esophagogram. Results: The three main etiologies were esophageal atresia (49%; n = 24), corrosive injury (25%; n = 12), and epidermolysis bullosa (14%; n = 7), followed by a heterogeneous group of rarer causes (12%; n = 6). The number of dilatations ranged from 1 to 8 sessions per patient (median +/- SEM: 2 +/- 0.3). The length of the follow-up period ranged from 20 to 109 months (median +/- SEM: 40 +/- 4 months). Treatment was successful in 86% of cases (n = 42). Twelve percent of patients (n = 6) had a residual stenosis requiring surgery, and a further one still experienced swallowing difficulties requiring enteral nutrition via gastrostomy in spite of the absence of significant residual stricture. Results were less satisfactory in cases of corrosive injury than with other etiologies. Three esophageal perforations were observed (6% of patients; 2% of procedures). All were medically treated. Conclusions: Endoscopic balloon dilatation is a simple, safe and efficacious treatment of esophageal strictures in children. (C) 2011 Elsevier Ireland Ltd. All rights reserved.
Alshammari J; Quesnel S; Pierrot S; Couloigner V
International Journal of Pediatric Otorhinolaryngology
2011
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.ijporl.2011.07.031" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2011.07.031</a>