Urethral duplication in a child with VATER association

Abstract

Urethral duplication (UD) is a rare anomaly with about 300 cases reported to date, usually seen in males and often associated with a number of anomalies involving other organs.1 Effmann's classification2 has described three types of urethral duplication. One out of three patients has an associated vesicoureteric reflux (VUR).3 In type I which is also the most common type, the urethra is partially duplicated and is almost always asymptomatic, requiring no further treatment. In type II, complete duplication of the urethra is observed. Type II urethral duplication is further classified as type IIA1 if both urethras arise from the separate bladder necks, type IIA2 (Y-type duplication) if one channel arises from the other, and type IIB if duplication with one meatus is observed. Type III urethral duplication comprises complete duplication of the urethra and bladder.3 The exact embryogenesis of urethral duplication is not well understood. Symptoms vary depending on the type of duplication. Symptoms could include recurrent urinary tract infections (UTI), epididymitis, and urinary incontinence.4 Diagnosis is usually made on voiding cystourethrogram (VCUG) and/or retrograde urethrogram (RUG). We report a case of type IIA2 urethral duplication in a male child with multiple other anomalies.