Urology: Urethral Orifice Anomaly

Epispadias

Definition

  • Definition: Failure of the Urethra to Close with a Dorsal Urethral Orifice
  • Often Associated with Congenital Anomalies of Bladder Exstrophy-Epispadias Complex (BEEC)
    • Isolated Epispadias is Less Common (< 10%)

Diagnosis

  • Clinical Diagnosis
  • Additional Steps in Total Evaluation:
    • US to Evaluate for Proximal Urinary Tract Anomalies
    • Pelvic Radiograph to Evaluate for Pubic Diastasis
    • Cystourethrogram (MCUG) or Nuclear Scintigraphy to Evaluate for Vesicoureteral Reflux

Treatment

  • Surgical Repair
  • Postoperative Continence 50-90%

Epispadias 1

Hypospadias

Definition

  • Definition: Failure of the Urethra to Close with a Ventral Urethral Orifice
  • Most Often an Isolated Malformation but Can Be Associated with Multiple Syndromes

Classification

  • Distal (Anterior): Subcoronal or Glandular
    • Most Common (60-70%)
  • Midshaft (Penile): Distal, Midshaft or Proximal Penile
  • Posterior: Penoscrotal, Scrotal or Perineal

Diagnosis

  • Diagnosis: Clinical
  • US to Evaluate for Proximal Urinary Tract Anomalies

Treatment

  • Surgical Repair Between Ages 6-18 Months

Hypospadias Classification 2

References

  1. Ebert AK, Reutter H, Ludwig M, Rösch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis. 2009 Oct 30;4:23. (License: CC BY-2.0)
  2. Chen MJ, Macias CG, Gunn SK, Dietrich JE, Roth DR, Schlomer BJ, Karaviti LP. Intrauterine growth restriction and hypospadias: is there a connection? Int J Pediatr Endocrinol. 2014;2014(1):20. (License: CC BY-4.0)