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Hypospadias

Hypospadias. Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section of Pediatric Urology. Hypospadias. Any condition in which the meatus occurs on the undersurface of the penis Usually 3 features ventral meatus

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Hypospadias

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  1. Hypospadias Stephen Confer, MD Ben O. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section of Pediatric Urology

  2. Hypospadias • Any condition in which the meatus occurs on the undersurface of the penis • Usually 3 features • ventral meatus • ventral curvature (chordee) • Dorsal "hood“; deficient foreskin ventrally

  3. Classification

  4. Embryology • Genital tubercle fuses in midline • Mesodermal folds create the urethral and genital folds • coalesce in midline as phallus elongates • Distal glans channel tunnels to proximal urethra as solid core then undergoes canalization

  5. Embryology • Prepuce forms as ridge of skin from corona • Hypospadias • Failure of ventral aspect to form • Dorsal hood • Chordee • Differential growth between normally developed dorsal tissue and underdeveloped ventral corporal tissue • Fibrous tissue distal to hypospadiac meatus

  6. Embryology • Prepuce forms as ridge of skin from corona • Hypospadias • Failure of ventral aspect to form • Dorsal hood • Chordee • Differential growth between normally developed dorsal tissue and underdeveloped ventral corporal tissue • Fibrous tissue distal to hypospadiac meatus

  7. Variations of Hypospadia

  8. Incidence • 1:300 live male births • 6000 boys each year in the US • Some genetic component • 8% of patients have father with hypospadias • 14% of patients have male siblings with hypospadias • If child with hypospadias, risk to next child • 12% risk with negative family history • 19% if cousin or uncle with hypospadias • 26% if father or sibling • More common in Caucasians (Jews and Italians) • Higher incidence in monozygotic twins (8.5x)

  9. Associated Anomalies • Undescended testes 9% and inguinal hernia 9% • Upper tract anomalies rare (1-3%) • Utriculus masculinus • 10 to 15% in perineal or penoscrotal hypospadias • Incomplete mullerian duct regression

  10. Associated Anomalies • Rule out intersex, especially with cryptorchidism • Adrenogenital syndrome • Mixed gonadal dysgenesis • Incomplete pseudohermaphroditism • True hermaphrotidism

  11. Associated Anomalies • hypospadias and cryptorchidism • high index of suspicion for an intersex state • Walsh reported the incidence of intersexuality in children with cryptorchidism, hypospadias, and otherwise nonambiguous genitalia to be 27% • nonpalpable testis were at least threefold more likely to have an intersex condition than those with a palpable undescended testis (50% versus 15% )

  12. Associated Anomalies • The idea that evaluation for an endocrine abnormality and/or intersex state should be undertaken in those with posterior hypospadias, regardless of gonadal position or palpability, is controversial but is supported in the literature, because significant, identifiable, and treatable abnormalities are common

  13. Further Evaluation • Only with severe hypospadias and sexual ambiguity • Includes testicular abnormalities • Up to 25% of these patients have enlarged utricles or other female structures • The incidence of abnormalities with other forms of hypospadias approximates that of the general population • Therefore no further evaluation is indicated

  14. History of Procedures • First in 100 to 200 A.D. • Heliodorus and Antyllus • Amputation distal to meatus • Dieffenbach, 1838 • Pierced glans to meatus and leave stent in place • Thiersch, 1869 • Local tissue flaps • Hook • Vascularized preputial flaps

  15. History of Procedures • Multistage repairs • Release chordee • Urethroplasty • One stage repairs • More feasible since the introduction of artificial erection, which has nearly eliminated inadequate chordee

  16. Treatment • Meatoplasty and glanuloplasty • Multiple techniques • Orthoplasty • Utilize artificial erection • Release urethra from fibrous tissue • Plicate dorsal tunica albuguinea • Ventral graft if needed

  17. Treatment • Urethroplasty • Onlay vascularized flap • Tubularized flap • Free graft • Skin cover • Mobilized dorsal prepuce and penile skin • Double faced island flap • Scrotoplasty

  18. Factors for Technical Success • Use of vascularized tissues • Careful tissue handling • Tension-free anastomosis • Non-overlapping suture lines • Meticulous hemostasis • Fine suture material • Adequate urinary diversion

  19. Technical Aspects • Instruments • Fine instruments for delicate tissue handling • Suture • Chromic- absorbs rapidly • 6-0 or 7-0 polyglycolic for buried sutures • Hemostasis • Tourniquet • Lidocaine with epinephrine • Low current Bovie, bipolar sticks to tissue

  20. Technical Aspects • Magnification • Dressing • Immobilzation and prevention of hematoma and edema • Diversions • Stent secured to glans with open drainage into a diaper

  21. Technical Aspects • Bladder spasms • Oxybutinin • Analgesia • Local penile block • Caudal block • Age at repair • 6 to 18 months

  22. Testosterone cream • May or may not be beneficial • considerable controversy surrounding the use of hormonal stimulation • whether to administer any adjunctive gonadotropins or hormones and, if so, which agent, route, dose, dosing schedule, and timing of treatment is to be employed • Gearhart and Jeffs (1987) administered testosterone enanthate intramuscularly (2 mg/kg body weight), 5 and 2 weeks before reconstructive penile surgery. They noted a 50% increase in penile size and an increase in available skin and local vascularity in all patients.

  23. Acute Complications • Wound infection • Poor wound healing 2 to ischemia of flaps • Edema • Drain tubes if free graft is used • Erections

  24. Chronic Complications • Urethrocutaneous fistula • Urethral diverticulum • Residual chordee • Persistent hypospadias • Urethral stricture • Hair bearing skin • Meatal stenosis • Excess skin • Balanitis xerotica obliterans

  25. Hypospadias Repair • Over 150 operations have be described • Distal hypospadias • Tubulization of the incised urethral plate (Snodgrass) • Meatal advancement (MAGPI) • Meatal-based flaps (Mathieu) • Proximal hypospadias • Onlay grafts • Vascularized inner preputial transfer flaps (Duckett) • Free grafts (skin, buccal mucosa)

  26. MAGPI

  27. Mathieu

  28. Redman and Barcat

  29. IslandOnlay

  30. Buccal Mucosal Graft

  31. Hypospadias - Conclusions • Common • Genetic component exists • Evaluation for associated anomolies with severe proximal hypospadias • Rule out intersex, especially with cryptorchidism • Multiple repairs exist, tailor to the patient, anatomy, and previous repairs

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