Lima Mario, Destro Francesca, Di Salvo Neil, Gargano Tommaso, Ruggeri Giovanni
Pediatric Surgery, Sant'Orsola Hospital, Bologna, Italy.
Pediatric Surgery, Sant'Orsola Hospital, Bologna, Italy.
J Pediatr Surg. 2017 Aug;52(8):1335-1339. doi: 10.1016/j.jpedsurg.2016.11.034. Epub 2016 Nov 17.
The spectrum of male urethral duplication is heterogeneous and it includes the Y-duplication. The malformation is rare and there is only a few case series reported in the literature. The management of Y-forms remains challenging for the surgeon and the long-term follow-up is still scarcely investigated. We report our 40-year experience in the management of patients with Y-duplication.
We conducted a restrospective analysis collecting information of patients with urethral Y-duplication treated at our department from April 1975 to April 2015. We investigated long-term effects of surgery by using a questionnaire.
Ten male patients with Y-duplication came to our attention. One was treated conservatively, seven underwent surgery and two were lost. Surgery consisted of removal of the ectopic branch (via perineal or ASTRA/anterior sagittal trans-rectal approach approach) and reconstruction of the orthotopic urethra. Post-operative complications included stenosis and infections. Long-term results are influenced by associated anomalies and significant problems (incontinence, urinary tract infections and orchiepididimitis) have been reported.
Y-duplication (or λ-duplication, as we prefer calling it) is a particular form of urethral duplication. The management of patients should be based upon the identification of the functional channel. The removal of the ectopic channel with ASTRA approach is safe and feasible. On the other hand, the reconstruction of the anterior urethra (when steno-atresic) is more challenging and justifies the need for many procedures. The P.A.D.U.A. (progressive augmentation by dilating the anterior urethra) technique was not effective. Skin tube grafts were responsible for infections ("hairy urethra"). BMFG (bladder mucosa free graft) urethroplasty is a good alternative, although associated with well-known complications. Associated anomalies influence long-term outcomes. Clinical study with type IV level of evidence.
男性尿道重复畸形的谱系具有异质性，包括Y型重复。这种畸形较为罕见，文献中仅报道了少数病例系列。Y型畸形的治疗对外科医生来说仍然具有挑战性，长期随访研究也很少。我们报告了我们在治疗Y型重复畸形患者方面40年的经验。
我们进行了一项回顾性分析，收集了1975年4月至2015年4月在我们科室接受治疗的尿道Y型重复畸形患者的信息。我们通过问卷调查来研究手术的长期效果。
10例男性Y型重复畸形患者引起了我们的关注。1例接受保守治疗，7例接受了手术，2例失访。手术包括切除异位分支（经会阴或经直肠前矢状入路）和重建原位尿道。术后并发症包括狭窄和感染。长期结果受到相关异常情况的影响，并且已经报道了一些严重问题（尿失禁、尿路感染和附睾炎）。
Y型重复畸形（或如我们更倾向称呼的λ型重复畸形）是尿道重复畸形的一种特殊形式。患者的治疗应基于对功能通道的识别。采用经直肠前矢状入路切除异位通道是安全可行的。另一方面，重建前尿道（当狭窄闭锁时）更具挑战性，这也说明了需要进行多次手术的必要性。P.A.D.U.A.（通过扩张前尿道进行渐进性扩大）技术效果不佳。皮管移植导致了感染（“多毛尿道”）。膀胱黏膜游离移植尿道成形术是一种很好的替代方法，尽管也伴有一些已知的并发症。相关异常情况会影响长期预后。证据等级为IV级的临床研究。
