II. Epidemiology

  1. Incidence: 1 in 150 per male births

III. Predisposing factors: Family History

  1. Father with Hypospadias: 8% risk
  2. Brother with Hypospadias: 14% risk

IV. Pathophysiology

  1. Congenital defect of Urethral meatus development
  2. Urethral folds incompletely fuse
  3. Ectopic Urethral meatus between glans and perineum

V. Associated Conditions

  1. Undescended Testicle (10% association)
  2. Inguinal Hernia
  3. Hydrocele
  4. Chordee (ventral penile curvature)

VI. Classification

  1. Anterior (Distal penis and glans): 50% of cases
  2. Middle (Midshaft of penis): 30% of cases
  3. Posterior (Scrotum, Perineum): 20% of cases

VII. Management

  1. Avoid Circumcision (foreskin needed for repair)
  2. Pediatric urology referral at age 3 to 4 months
  3. Surgical repair at 6 to 12 months of age
    1. Distal Hypospadias (most common)
      1. Typically repaired in a single surgery (curvature repaired at same time)
      2. Urethral catheter is typically in place for 1 week following surgery
      3. Few complications
    2. Proximal Hypospadias
      1. Evaluate for disorder of Sexual Development if Undescended Testicle also present
      2. Often requires 2 procedures for repair (esp. if severe curvature)
      3. Second surgery if needed is performed at 6 months
      4. Uses foreskin for repair graft (if second procedure needed)

VIII. Complications: Surgical Repair

  1. Complications 5-15% (highest for posterior Hypospadias)
  2. Urethrocutaneous fistula or Urethral Diverticulum
  3. Meatal stenosis
  4. Wound Infection, Hematoma or dehiscence

IX. Complications: Utreated

  1. Urine stream deformity
  2. Sexual Dysfunction associated with curvature
  3. Infertility associated with proximal Urethral meatus

X. References

  1. Behrman (2000) Nelson Pediatrics, Saunders, p. 1645-7
  2. Granberg (2024) Mayo Clinic Pediatric Days, lecture attended 1/18/2024
  3. Borer (1999) Urol Clin North Am 26(1): 15-37 [PubMed]

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