A complex case of megalourethra and its repair

Abstract

Megalourethra is a rare malformation of the urethra caused by a lack of corpus sponigosum and in some cases corpora cavernosa in the region of the distal urethra. The absence of these structures causes a ballooning of the urethra despite there being no mechanical obstruction. A male child presented with so-called fusiform megalourethra, with absence of the corpora cavernosa and urethral duplication. A voiding cystourethrogram was used to diagnose a fusiform megalourethra with pronounced meatal stenosis and extreme stenosis of pendulous urethra. In addition, there was urethral duplication in the form of an accessory urethra stretching from the urethral colliculus to the perineum. Absence of the corpora cavernosa was also suspected in the distal urethra. The surgical procedure involved pendulous urethroplasty with an onlay technique using urethral duplication and penile reduction. This method of treating megalourethra has not been previously reported. The operative technique for fusiform megalourethra with genital malformation has to be tailored to each individual case, depending on the intraoperative and endoscopic findings.