Exstrophy variant in a female child

Description

Bladder exstrophy–epispadias complex is a spectrum of rare congenital malformations involving the urinary, genital and musculoskeletal systems. The condition can be subdivided into classic/typical forms, comprising epispadias, classical bladder and cloacal exstrophy, and variant/atypical forms, including duplicated, covered and pseudoexstrophy. Diagnosis can be made clinically. Incidence varies. However, the occurrence is higher in males. Genetic and environmental components likely contribute to the aetiopathogenesis, which remains unknown.1 2 There is a hypothesis that cloacal exstrophy variant could result from an ischiopagus conjoined twinning with blighting of one twin.3

We present a 9-year-old girl with urinary incontinence. She was initially referred at 2-1/2 years for ambiguous genitalia but did not continue follow-up. The mother denied receiving prenatal care or completing postnatal studies. On examination, she has a bifid clitoris, symphysis pubis widely separated and low-placed umbilicus with midline muscle defect where the bladder seemed to herniate...

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