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

7. 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

8. Variations of Hypospadia

10. 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)

11. 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

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

13. 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% )

14. 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

15. 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

16. History of Procedures First in 100 to 200 A.D. Dieffenbach, 1838
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

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

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

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

20. 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

21. Technical Aspects Instruments Suture Hemostasis
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

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

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

24. 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.

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

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

27. 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)

28. MAGPI

29. Mathieu

30. Redman and Barcat

31. Island Onlay

32. Buccal Mucosal Graft

33. 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