Aphallia: its classification and management.

Aphallia is an extremely rare disorder with profound urological and psychological consequences. Approximately 60 patients have been reported on in the literature; we report our experience with 3 additional patients. Fifty patients had sufficient information to classify the condition according to the site of the urethral meatus. With the relative relationship of the urethral meatus to the anal sphincter several observations were noted. The more proximal the meatus the higher the incidence of other anomalies and the greater the number of neonatal deaths. Of the patients 30 (60 per cent) had a post-sphincteric meatus located on a peculiar appendage at the anal verge. These patients had the lowest incidence of other anomalies (1.2 per patient) and the highest survival rate (87 per cent). A total of 14 patients (28 per cent) had pre-sphincteric urethral communications (prostato-rectal fistula), of whom 36 per cent died in the neonatal period. Six patients (12 per cent) had urethral atresia. This group had no survivors and the highest incidence of other anomalies (4 per patient). Of our patients 2 had a post-sphincteric meatus and 1 had a pre-sphincteric meatus. In addition to correction of life-threatening anomalies the management of aphallia centers on establishing gender assignment. Bilateral orchiectomy, labial construction and urethral transposition should be done in the newborn period, if possible.