What is the initial management for a newborn with bifid scrotum and hypospadias?

Initial Management of Bifid Scrotum with Hypospadias in a Newborn

A newborn with bifid scrotum and hypospadias requires immediate evaluation to rule out a disorder of sex development (DSD), particularly if the testes are nonpalpable, and should NOT be circumcised until this evaluation is complete. 1

Immediate Assessment (First 24-48 Hours)

Rule Out Disorder of Sex Development

• Examine for testicular position bilaterally - palpable versus nonpalpable testes is the critical first determination 1
• A phenotypic male newborn with bilateral nonpalpable testes and hypospadias represents a potential DSD emergency, as this could be a 46,XX individual with congenital adrenal hyperplasia presenting with virilization 1
• Immediate specialist consultation (pediatric endocrinology and urology) is mandatory if bilateral nonpalpable testes are present 1
• Failure to diagnose congenital adrenal hyperplasia can result in life-threatening electrolyte disturbances (hyponatremia, hyperkalemia) and shock 1

Initial Physical Examination Details

• Document the exact location of the urethral meatus (penoscrotal, scrotal, or perineal) 2, 3
• Assess for presence and degree of chordee (penile curvature) 4, 2
• Evaluate the severity of scrotal bifidity and any penoscrotal transposition 3, 5
• Do NOT circumcise - the foreskin tissue is essential for future surgical reconstruction 2

Diagnostic Workup

If Bilateral Nonpalpable Testes Present

• Measure müllerian inhibiting substance (anti-müllerian hormone) to evaluate for anorchia 1
• Consider additional hormone testing including 17-hydroxyprogesterone, testosterone, and karyotype 1
• Pelvic ultrasound to assess for müllerian structures 1

If Testes Are Palpable (Isolated Hypospadias with Bifid Scrotum)

• Baseline renal and bladder ultrasound to assess for associated genitourinary anomalies 6
• No urgent urodynamic testing is required in the newborn period for isolated hypospadias 1

Counseling and Planning

Parent Education

• Explain that surgical reconstruction is typically performed between 6-12 months of age for hypospadias 4
• For severe penoscrotal hypospadias with bifid scrotum, discuss that repair may be single-stage or two-stage depending on severity 3, 7
• Emphasize that the foreskin must be preserved for reconstruction 2

Surgical Planning Considerations

• Single-stage repairs using techniques like Hodgson XX or Koyanagi can achieve 80% success rates even in severe penoscrotal hypospadias with bifid scrotum 3
• Z-plasty techniques for scrotal reconstruction provide superior aesthetic outcomes compared to simple midline closure, avoiding dimpling and contracture 5
• Two-stage repairs may be preferred by some surgeons for the most severe cases, with first stage addressing chordee and scrotoplasty, followed by urethroplasty 7

Referral Timeline

Refer to pediatric urology within the first 1-2 months of life to allow adequate time for surgical planning and family preparation before the optimal surgical window at 6-12 months 4, 2

Critical Pitfalls to Avoid

• Never circumcise a newborn with hypospadias - this eliminates essential tissue for reconstruction 2
• Do not delay DSD evaluation if bilateral nonpalpable testes are present - this is a medical emergency 1
• Avoid reassuring parents that "mild" bifid scrotum doesn't need treatment - even moderate bifidity benefits from Z-plasty reconstruction to prevent long-term aesthetic concerns 5
• Do not assume isolated hypospadias means no other anomalies - obtain baseline renal ultrasound as genitourinary anomalies can coexist 6