Fate of males with urethral “Y-duplication”: 40-year long follow-up in 8 patients Mario Lima, Francesca Destro ⁎, Neil Di Salvo, Tommaso Gargano, Giovanni Ruggeri Pediatric Surgery, Sant'Orsola Hospital, Bologna, Italy abstract article info Article history: Received 30 July 2016 Received in revised form 17 October 2016 Accepted 5 November 2016 Available online xxxx Key words: Urethral duplication Pediatric urology Pediatric surgery Astra BMFG Purpose: The spectrum of male urethral duplication is heterogeneous and it includes the Y-duplication. The mal- formation is rare and there is only a few case series reported in the literature. The management of Y-forms re- mains 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. Materials and methods: 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 sur- gery by using a questionnaire. Results: 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 anom- alies and significant problems (incontinence, urinary tract infections and orchiepididimitis) have been reported. Conclusions: 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 ante- rior 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. © 2016 Elsevier Inc. All rights reserved. Urethral duplications are rare congenital malformations, most com- monly encountered in males [1,2]. They represent a heterogeneous spectrum of anomalies including the so-called “Y-type” duplication [3]. The peculiar characteristic of Y-duplication is that there are two ure- thral channels, one penile (usually stenotic) and one posterior that ends in the anus, rectum or perineum [4]. This form has been described in about 50 cases in literature [3]. In addition to the on-going discussion related to the search for a suitable terminology, there is still concern for the treatment of affected patients [5,6]. Indeed, the urethral recon- struction in these cases is considered one of the most difficult urological reconstructive procedures [7,8]. In this article we report our experience with seven patients with Y-duplication and we discuss the manage- ment, complications and long-term follow-up. 1. Materials and methods We reviewed all medical charts of patients with urethral duplication that came to our attention from April 1975 to April 2015. We selected only patients with Y-duplication (that we prefer calling λ-duplication). We further grouped our patients according to the features of the orthotopic urethral branch (Fig. 1): pure form (A: both patent orthotopic and ectopic urethra); steno-atresic form (B: with stenotic orthotopic urethra and a patent ectopic urethra) and abortive form (C: with stenotic orthotopic urethra and a just hinted posterior ectopic branch). We analyzed the patients' demographics, associated anomalies, type of management (conservative or surgical), post-operative complica- tions and data related to long-term follow-up. Follow-up data were collected through telephone contact and by visiting patients at our outpatient clinic. We elaborated a questionnaire aimed at assessing both urinary and sexual functions. Journal of Pediatric Surgery xxx (2016) xxx–xxx ⁎ Corresponding author at: Via Massarenti 11, Bologna, Italy. Tel.: +39 3477989391. E-mail address: Francesca_destro@hotmail.com (F. Destro). http://dx.doi.org/10.1016/j.jpedsurg.2016.11.034 0022-3468/© 2016 Elsevier Inc. All rights reserved. Contents lists available at ScienceDirect Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg Please cite this article as: Lima M, et al, Fate of males with urethral “Y-duplication”: 40-year long follow-up in 8 patients, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.11.034