幽门闭锁合并大疱性表皮松解症、肠旋转不良及高位直肠肛门畸形伴直肠尿道瘘：成功治疗报告

Pyloric atresia associated with epidermolysis bullosa, malrotation, and high anorectal malformation with recto-urethral fistula: a report of successful management.

作者信息

Agarwala S, Goswami J K, Mitra D K

机构信息

Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Pediatr Surg Int. 1999;15(3-4):264-5. doi: 10.1007/s003830050572.

DOI:10.1007/s003830050572

PMID:10370040

Abstract

Pyloric atresia (PA) is an uncommon anomaly that may be associated with many other congenital anomalies, the commonest of which is junctional epidermolysis bullosa (JEB). Most of the cases of PA associated with JEB (Herlitz syndrome) reported have been fatal. A case of PA associated with JEB, malrotation, and a high anorectal malformation with a rectourethral fistula, which was hitherto undescribed, was successfully managed at our institution.

摘要

幽门闭锁（PA）是一种罕见的异常情况，可能与许多其他先天性异常相关，其中最常见的是交界性大疱性表皮松解症（JEB）。大多数报道的与JEB（赫利茨综合征）相关的PA病例都已死亡。我院成功处理了一例与JEB、肠旋转不良以及伴有直肠尿道瘘的高位肛门直肠畸形相关的PA病例，这种情况此前尚未见描述。