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

J Pediatr Urol. 2017 Feb;13(1):42.e1-42.e7. doi: 10.1016/j.jpurol.2016.10.008. Epub 2016 Nov 3.

Abstract

Introduction: Urethral 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.

Objective: To report experience with the management of 20 urethral duplication patients and propose a classification of this anomaly.

Materials and methods: A 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.

Results: Immediate 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.

Discussion: Urethral 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.

Conclusions: The 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.

Keywords: Paediatric surgery; Paediatric urology; Urethral duplication.

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Male
  • Retrospective Studies
  • Surveys and Questionnaires
  • Urethra / abnormalities*
  • Urethral Diseases / classification*
  • Urethral Diseases / congenital
  • Urethral Diseases / physiopathology
  • Urination / physiology*