退行性营养不良性大疱性表皮松解症患儿合并食管狭窄:荧光镜下球囊扩张术的11年经验

来源 :世界核心医学期刊文摘(儿科学分册) | 被引量 : 0次 | 上传用户:liuleizishen
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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. 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. The use of an endoscope carries the risk of 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 pat ient 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 be considered as a first line of treatment.
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