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.

Disorder of sexual development, urinary bladder duplication, pancake kidney and neural tube defect

“…One such case of female pseudohermaphroditism with urethral duplication was reported by D'Cunha et al. [13] However, in their case, one urethra was opening normally, and the second one was draining into the vagina. In addition, their case did not include duplication of the urinary bladder.…”

Female hypospadias presenting with urinary retention and renal failure in an adolescent: uncommon and late presentation with significant hidden morbidity

“…Diagnosis could be established only after antegrade cystoscopy. Anecdotal case reports are available for this uncommon entity 4 5. The cause appears to be voiding with stenosed meatus causing bladder outlet obstruction leading to obstructive uropathy.…”