1304 MEGALOURETHRA-A MINI EPIDEMIC?

Abstract

Megalourethera is a congenital abnormality secondary to abnormal formation of erectile tissue of the penis, not primarily a problem with the urethra. In the complete (fusiform) type all erectile tissue is involved leading to massive dilatation of the penis. In the incomplete scaphoid form the deficiency is limited to the corpus spongiosum associated with ballooning of the terminal portion of the urethra. In a short period of time, four infants were referred to the service with a fusiform lesion, in three leading to a massively dilated phallus. Three of the four infants had other associated abnormalities reminiscent of the VATER group. The abnormalities included TE fistula, vertebral anomalies, radial hypoplasia, and absence of thumb. Other than season of year, there were no significant features of family history or pregnancy history suggesting a particular teratogen. Although thirty cases described in the literature had megalophallus, only six were secondary to a fusiform lesion. Four of the six had a constellation of abnormalities similar to our infants. It is therefore important to differentiate the causes of megalourethra as the fusiform type is often associated with other abnormalities, and may well be a lethal condition.