Practical and functional classification of the double urethra: A variable, complex and fascinating malformation observed in 20 patients

SummaryIntroductionUrethral duplication is a rare and variable malformation of the urinary tract, with non-univocal and complex management. In addition, different classification schemes have been proposed, but none have considered all the possible variants.ObjectiveTo report experience with the management of 20 urethral duplication patients and propose a classification of this anomaly.Materials and methodsA retrospective analysis collecting information regarding 20 patients (Table) with urethral duplication treated at a single institution over the past 40 years. Three females and 17 males were treated: two had vesico-urethral duplication, eight had urethral duplication with a single bladder, and eight had 'λ' duplication.ResultsImmediate postoperative complications included urethral dehiescence (n = 1) and urethral stenosis (n = 2). The progressive augmentation by dilating the urethra (PADUA) technique was ineffective. During follow-up, the following were recorded: urinary incontinence (n = 2), urinary tract infection (n = 3), hypertension (n = 3) and erectile dysfunction (n = 1). All patients were satisfied with the aesthetic result.DiscussionUrethral duplication is a rare anomaly with male preponderance. Four types of duplication were described, on the basis of anatomy and management: vesical and urethral duplication (type 1), urethral duplication with single bladder (type 2), 'λ-type' duplication (type 3) and 'miscellanea' (multiple urethral channels, spindle urethra, other female forms, type 4). A full description of the malformation and surgical approach was given for each type. The advantages of the classification were compared with the literature.ConclusionsThe proposed classification should be a useful tool, based on the required surgical approach, even if surgery should be tailored to the patient. It is important to restore the anatomy and achieve urinary continence. Surgery can be challenging and a multi-step process, especially in cases of 'λ' duplications.Table. TypeSex (n)SymptomsSurgeryImmediate postoperative complications1F (n = 1), M (n = 1)Incontinence, asymptomaticBladder unification + ectopic urethra removal + urethroplasty (n = 1); epispadic urethra and abortive bladder removal (n = 1)Urethral dehiscence (n = 1)2AM (n = 4)Asymptomatic (n = 3), mucous discharge (n = 1)Conservative (n = 2); accessory urethra removal (n = 2)-2DF (n = 1)Urge incontinenceVulvar urethrostomy and posterior channel endoscopic closure2BM (n = 2)Mucous dischargeTermo-ablation (n = 1), accessory channel removal (n = 1)None2CM (n = 2)Incontinence, UTIUrethroplasy, ectopic urethra removal (n = 2)Urethral stenosis (n = 1)3M (n = 8)Anal voiding (n = 6), incontinence (n = 1), UTI (n = 1), anuria (n = 1)ASTRA + urethroplasty with BMFG (n = 3); ASTRA + urethroplasty (n = 2); perineal resection of the ectopic channel + urethroplasty (n = 1); vesicostomy (n = 1); perineal urethrostomy and PADUA (n = 2)Urethral stenosis (n = 3); terminal renal failure (n = 1)2BF (n = 1)Double urinary streamVulvar and clitoridoplasty, ectopic urethra removalNoneASTRA, anterior sagittal transrectal approach; BMFG, bladder mucosa free graft; PADUA, progressive augmentation by dilating the urethra anterior; UTI, urinary tract infections.