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女性假两性畸形、阴茎型尿道和后泄殖腔的罕见关联。

Rare association of female pseudohermaphroditism, phallic urethra, and posterior cloaca.

作者信息

Macarthur Mairi, Mahomed Anies

机构信息

Department of Paediatric Surgery, Royal Aberdeen Children's Hospital, Aberdeen AB25 2ZG, UK.

出版信息

J Pediatr Surg. 2006 Mar;41(3):576-9. doi: 10.1016/j.jpedsurg.2005.11.070.

Abstract

We describe a child with the rare clinical entity of female pseudohermaphroditism, accessory phallic urethra, and posterior cloaca who was successfully treated with posterior sagittal anorectovaginourethroplasty. Masculinization was limited to the external genitalia, and no chromosomal, metabolic, or adrenal abnormalities were detected. Associated pathology included bilateral vesicoureteric reflux, a non functioning dysplastic kidney, and bicornuate uterus. The investigation and surgical management of this particularly challenging combination of anomalies is detailed.

摘要

我们描述了一名患有女性假两性畸形、副阴茎尿道和后泄殖腔这一罕见临床病症的儿童,该患儿通过后矢状位肛门直肠阴道尿道成形术获得成功治疗。男性化仅限于外生殖器,未检测到染色体、代谢或肾上腺异常。相关病理包括双侧膀胱输尿管反流、一个无功能的发育异常肾脏和双角子宫。本文详细介绍了对这种特别具有挑战性的异常组合的检查和手术处理。

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