Fate of males with urethral "Y-duplication": 40-year long follow-up in 8 patients

J Pediatr Surg. 2017 Aug;52(8):1335-1339. doi: 10.1016/j.jpedsurg.2016.11.034. Epub 2016 Nov 17.

Abstract

Purpose: 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.

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 surgery 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 anomalies 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 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.

Keywords: Astra; BMFG; Pediatric surgery; Pediatric urology; Urethral duplication.

MeSH terms

  • Follow-Up Studies
  • Forecasting*
  • Humans
  • Male
  • Plastic Surgery Procedures / methods*
  • Surveys and Questionnaires
  • Urethra / abnormalities*
  • Urethra / surgery
  • Urethral Diseases / congenital*
  • Urethral Diseases / diagnosis
  • Urethral Diseases / surgery
  • Urologic Surgical Procedures, Male / methods*