Hypospadias
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Hypospadias. Jillian E Peterson. Definition. Congenital anomaly of male urethra that results in abnormal ventral opening of urethra Hypo= belowSpadon= 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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Hypospadias

Hypospadias

Jillian E Peterson


Definition

Definition

  • Congenital anomaly of male urethra that results in abnormal ventral opening of urethra

  • Hypo= belowSpadon= opening


Anatomy review

Anatomy Review


Classification

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


Embryology

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


Embryology1

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


Embryology2

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


Etiology

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


Epidemiology

Epidemiology

  • Incidence

    • 0.3 to 0.7 % in live male birth

    • More common in Caucasians


Evaluation

Evaluation

  • Family history

  • Genital exam

    • Glans

    • Urethral opening

    • Penile length

      • Term newborn male 2.5-3.5cm

    • Degree of penile curvature

    • Testicular descent


Evaluation1

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


Management

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


Surgery

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


Complications

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


Long term implications

Long term implications

  • Difficulty w/ urinary stream

  • Erectile Dysfunction

  • Infertility


References

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.

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