Why Gender Reassignment in Congenital Aphallia?

Congenital aphallia or penile agenesis is a complex urogenital malformation and an extremely rare condition in the medical literature with reported incidence of 1 in 30 million live births. The condition is apparent at birth and is almost never missed. Objectives behind the study were to present the management and solve the dilemma with congenital aphallia. We present a retrospective study of three cases with congenital aphallia. The patients presented between 2008 and 2014. Our initial infant had female sex reassignment, which was lost to follow-up. Learning from the previous experiences, we decided to rear the next two infants as male. Obstructive uropathy was managed with early urethral transposition in the second and third case, while vesicostomy was performed in the first case. Both second and third patients underwent urethroplasty, they are passing urine from the urethral meatus situated at the pubis. All procedures were undertaken only after thorough counselling and discussion with the parents. Congenital aphallia requires multistage management. Obstructive uropathy should be managed with early urethral transposition or urethral advancement techniques. In the second stage, the meatal opening may be advanced near the pubic region by creating Byer’s flap urethroplasty. It is very important to propose early sex reassignment. Gender reassignment must be individualized according to age at presentation, rearing sex of child and psychological evaluation of child and acceptability of the family. Present trend is towards male sex reassignment.