title: Idiopathic female pseudohermaphroditism with urethral duplication and female hypospadias.
abstract: 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.
authors.affiliation: Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Aureen Ruby
authors.initials: AR
authors.lastname: D'Cunha
authors.affiliation: Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Jujju Jacob
authors.initials: JJ
authors.lastname: Kurian
authors.affiliation: Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
authors.forename: Tarun John K
authors.initials: TJ
authors.lastname: Jacob
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000000981:diagnostic imaging / Q000601:surgery; D000015:Abnormalities, Multiple / Q000601:surgery*; D003559:Cystostomy; D012734:Disorders of Sex Development; D005260:Female; D006801:Humans; D007021:Hypospadias; D008297:Male; D014463:Ultrasonography; D014521:Urethra / Q000002:abnormalities*; D001748:Urinary Bladder Neck Obstruction / Q000601:surgery; D014547:Urinary Diversion / Q000379:methods; D014621:Vagina / Q000002:abnormalities