HYPOSPADIA

dr. Moch. Syahroni Far, SpU, M.Kes

INTRODUCTION
Congenital Abnormality 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

INCIDENCE
1:300 live male births 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)

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

CLASSIFICATION

VARIATIONS OF HYPOSPADIA

HISTORY

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

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

INDICATION FOR SURGERY

According to Cecil (1932), the only reason for operating on any hypospadiac patient is to correct deformities that interfere with the function of urination and procreation.

PRE OPERATION EVALUATION

1. 2. 3. 4. 5. Associated Anomalies Age for undergoing surgery Endocrine abnormality Hormonal stimulation, is it need? Chosen technique, Over 150 operations have be described 6. Any Contraindication for surgery?

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.

ASSOCIATED ANOMALIES
Undescended testes 9% and inguinal hernia 9% Upper tract anomalies rare (13%) 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

GENERAL PRINCIPLE FOR HYPOSPADIA REPAIR

1. Orthoplasty
Utilize artificial erection Release urethra from fibrous tissue, Chordeectomi Plicate dorsal tunica albuguinea Ventral graft if needed Neourethral formation --- first layer Neourethral coverage with Subcutaneus dartos flap, tunica albuginea --- second layer

2. Urethroplasty

3. Meatoplasty And Glanuloplasty 4. Skin Coverage

ARTIFICIAL ERECTION

PLICATE TUNICA ALBUGENIA

VENTRAL GRAFT

SUBCUTANEOUS DARTOS FLAP

TUNICA ALBUGINEA

ALGORITM

MEATAL ADVANCEMENT

ADVANCEMENT TECNIQUE (DUCKETT)

TUBULARIZATION TECHNIQUE (THIERSCH)

MEATAL-BASED FLAPS (MATHIEU)

REDMAN AND BARCAT

ONLAY TECHNIQUE

ONE STAGE (DUCKETT)

Vascularized inner preputial transfer flaps

TWO STAGE
1. Chordectomy 2. Urethroplasty

TWO STAGE
1. Chordectomy 2. Urethroplasty

FREE GRAFT (MUCOSAL BUCCAL GRAFT)

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
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
Immobilzation and prevention of hematoma and edema

Diversions
Stent secured to glans with open drainage into a diaper

TECHNICAL ASPECTS
Bladder spasms
Oxybutinin

Analgesia
Local penile block Caudal block

Age at repair
6 to 18 months

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 - 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

Thank you so much for learning