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 ventral curvature (chordee) Dorsal "hood“; deficient foreskin ventrally

Classification

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

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

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

Variations of Hypospadia

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)

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

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

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% )

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

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

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

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

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

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

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

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

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

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

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.

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

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

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)

MAGPI

Mathieu

Redman and Barcat

Island Onlay

Buccal Mucosal Graft

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