Idiopathic female pseudohermaphroditism with urethral duplication and female hypospadias

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...

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Bibliographic Details
Published in:BMJ case reports 2016-03, Vol.2016, p.bcr-2015-214172
Main Authors: D'Cunha, Aureen Ruby, Kurian, Jujju Jacob, Jacob, Tarun John K
Format: Article
Language:English
Subjects:
46, XX Disorders of Sex Development - diagnostic imaging
46, XX Disorders of Sex Development - surgery
Abnormalities, Multiple - surgery
Androgens
Asia
Bladder
Clitoris
Creatinine
Cystostomy
Disorders of Sex Development
Female
Females
Humans
Hypertension
Hypospadias
Indian Sub-Continent
Male
Ultrasonic imaging
Ultrasonography
Unusual Association of Diseases/Symptoms
Urethra - abnormalities
Urinary Bladder Neck Obstruction - surgery
Urinary Diversion - methods
Vagina
Vagina - abnormalities
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Summary: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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2015-214172