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Hypospadias

Hypospadias. Jillian E Peterson. Definition. Congenital anomaly of male urethra that results in abnormal ventral opening of urethra Hypo= below Spadon= opening. Anatomy Review. Classification. First degree (mild) Urethral opening within the glans or subcorona Second degree (moderate)

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Hypospadias

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  1. Hypospadias Jillian E Peterson

  2. Definition • Congenital anomaly of male urethra that results in abnormal ventral opening of urethra • Hypo= below Spadon= opening

  3. Anatomy Review

  4. Classification • First degree (mild) • Urethral opening within the glans or subcorona • Second degree (moderate) • Urethral opening on penile shaft • Third degree (severe) • Urethral opening within the scrotum or perineum

  5. Embryology • Male and female external genitalia develop from a common origin and are indistinguishable up to 7-8wks gestation • At apprx 9th week of gestation differentiation begins

  6. Embryology • In males, androgens stimulate growth of genital tubercule into phallic structure • Penile urethra formed from fusion of urethral folds • Hypospadias= failure of urethral folds to close

  7. Embryology • Penile curvature is normal in development • Resolves at end of urethral formation • Hypospadias associated w/ abnormal penile curvature • Referred to as chordee • Due to disruption in development of penile shaft

  8. Etiology • Cause largely unknown • Hypothesis include disruption of androgens • Associated factors  genetic & environmental • Advanced maternal age • Maternal DM • Pre-term birth • History of paternal hypospadias • Exposure to smoking/pesticides

  9. Epidemiology • Incidence • 0.3 to 0.7 % in live male birth • More common in Caucasians

  10. Evaluation • Family history • Genital exam • Glans • Urethral opening • Penile length • Term newborn male 2.5-3.5cm • Degree of penile curvature • Testicular descent

  11. Evaluation • Isolated hypospadias • Most common • No need for renal US because: • External genitalia forms after critical renal development • Arises from different embryological structures • Hypospadias + other organ system abnormalities • Renal US • Hypospadias + Cryptorchidism • Apprx 10% of patients w/ hypospadias • Increased risk of sexual developmental disorders • Can consider pelvic US, karyotyping, serum electrolytes

  12. Management • NO circumcision as foreskin can possibly be used in future surgical correction • Mild hypospadias • If without significant penile curvature can observe • Only issue is slight misdirection of urinary stream • Moderate/severe hypospadias • Surgical correction

  13. Surgery • Timing • 6 – 18 months • Goal • Urethral opening as close to ventral tip of penis as possible • Straightening of any chordee • Surgical technique • Primary tubularization • Onlay island flap • Two-stage repair

  14. Complications • Urethral fistula • Most common complication • Urocutaneous fistula • Two urinary streams • Requires surgical correction • Urethral stricture • Thin stream w/ straining on urination • Urethral diverticulum • Outpouching of reconstructed urethra w/ dribbling of retained urine after void

  15. Long term implications • Difficulty w/ urinary stream • Erectile Dysfunction • Infertility

  16. References • Baskin LS, Ebbers MB. Hypospadias: anatomy, etiology, and technique. J Pediatr Surg 2006; 41:463. • Brouwers MM, Feitz WF, Roelofs LA, et al. Risk factors for hypospadias. Eur J Pediatr 2007; 166:671. • Duckett JW. “Hypospadias. Campbell’s Urology”. Philadelphia, PA, USA: W.B. Saunders 1998: 2115 • Nelson’s Pediatrics • Shukla AR, Patel RP, Canning DA. Hypospadias. Urol Clin North Am 2004; 31:445. • UpToDate

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