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大疱性表皮松解症及其耳鼻喉科表现。两例病例报告。

Epidermolysis bullosa and its E.N.T. manifestations. Two case reports.

作者信息

Ramadass T, Thangavelu T A

出版信息

J Laryngol Otol. 1978 May;92(5):441-6. doi: 10.1017/s0022215100085583.

Abstract

The present paper describes two rare cases of epidermolysis bullosa dystrophica (recessive), one with oesophageal stricture and another with laryngeal stenosis, along with other usual features of the disease. Different views on the line of treatment of the oesophageal stricture as a complication of this dreadful disease are discussed. Replacement of the strictured oesophagus by coloplasty may be contemplated in the first case, if the patient develops increasing dysphagia. In the second case, with cicatrical stenosis of the larynx and hoarseness of voice which is a rare complication of the disease, not reported in the literature so far, a tracheostomy is contemplated when the child develops acute respiratory distress. These two cases are under observation.

摘要

本文描述了两例罕见的营养不良性大疱性表皮松解症(隐性)病例,一例伴有食管狭窄,另一例伴有喉狭窄,以及该疾病的其他常见特征。文中讨论了对于作为这种可怕疾病并发症的食管狭窄的治疗方案的不同观点。如果第一例患者出现吞咽困难加重的情况,可以考虑通过结肠成形术置换狭窄的食管。在第二例中,患者患有瘢痕性喉狭窄且声音嘶哑,这是该疾病一种罕见的并发症,目前文献中尚无报道,当患儿出现急性呼吸窘迫时,考虑进行气管切开术。这两例病例正在观察中。

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