Cloacal Exstrophy and Gender Management

Cloacal exstrophy is a rare condition, in the spectrum of the exstrophy–epispadias complex. There is an exposed bladder and cecal plate with prolapsed ileocecal junction and an omphalocele. The pubic bone is widely separated, which leads to the clitoris and phallus being spaced apart, creating challenges in its reconstruction. It is known to be associated with several other systemic anomalies including spinal and skeletal defects, which add to the morbidity of the condition. With improved early survival beyond the neonatal age group and success in the early surgical management separating bladder and bowel segments, gender management and genital reconstruction is a matter of dilemma and deliberation, especially for those with the male genotype. Female genital reconstruction is relatively straightforward, however, whether a child born with the XY genotype should be reared as female or should an attempt be made to reconstruct the phallus from diminished and limited phallic structures is still concerning. Despite evidence now suggesting a male gender of rearing for these patients, it remains a matter of debate and needs to be decided after discussing with the parents regarding outcomes.