D'Cunha Aureen Ruby, Kurian Jujju Jacob, Jacob Tarun John K
Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
BMJ Case Rep. 2016 Mar 10;2016:bcr-2015-214172. doi: 10.1136/bcr-2015-214172.
Female hypospadias is a rare anomaly of the female urethra where it opens on the anterior vaginal wall anywhere between the introitus and the fornix. It is often associated with other genitourinary anomalies such as Cloacal malformation, female pseudohermaphroditism, nonneurogenic neurogenic bladder and urethral duplication. Idiopathic female pseudohermaphroditism is extremely rare, and most cases occur secondary to adrenogenital syndrome or maternal androgen exposure. We report a unique case of a 1-year and 4-month-old girl who presented with ambiguous genitalia and renal failure secondary to a non-neurogenic neurogenic bladder. On further evaluation, she was found to have urethral duplication with a hypospadiac female urethra. She initially underwent a vesicostomy and was further planned to undergo an appendicular Mitrofanoff at an older age. The mainstay of treatment in these cases includes relief of bladder outlet obstruction and recovery of renal function by adequate urinary drainage. Clitoral reduction, if cosmetically warranted, may be planned at puberty.
女性尿道下裂是一种罕见的女性尿道异常，尿道开口于阴道前壁，位于阴道口和穹窿之间的任何位置。它常与其他泌尿生殖系统异常相关，如泄殖腔畸形、女性假两性畸形、非神经源性神经源性膀胱和尿道重复畸形。特发性女性假两性畸形极为罕见，大多数病例继发于肾上腺生殖器综合征或母体雄激素暴露。我们报告了一例独特的病例，一名1岁4个月大的女孩，因非神经源性神经源性膀胱出现生殖器模糊和肾衰竭。进一步评估发现，她患有尿道重复畸形，伴有尿道下裂的女性尿道。她最初接受了膀胱造瘘术，并计划在年龄稍大时进一步接受阑尾代输尿管腹壁造口术。这些病例的主要治疗方法包括缓解膀胱出口梗阻，并通过充分的尿液引流恢复肾功能。如果出于美容需要，可在青春期计划进行阴蒂缩小术。
