Hypospadias - clinical approach

Clinical approach to a case of hypospadias

1. Approach to a case of Hypospadias Dr . Abhishek Pandey

2. Introduction Greek – “hypo” – under, “ spadon ” – rent / fissure Urethral opening proximal to the normal glanular location Occurs in 1 in 300 males (0.3%) Increase in incidence over last 25 yrs 90% are isolated penile defects Considered arrested development of prepuce & glans Correction is surgical

3. History Proximally located meatus Ventrally deflected or spraying urine stream Curved penis Split scrotum Urine trickling & ballooning of urethra – meatal stenosis Assessment of risk factors Presentation at circumcision – concealed variants

4. Risk Factors Genetic – Family history – positive in 7% families (RR-13 in 1° relatives) Endocrine disorders Placental – Birth weight – associated with low birth weight Environmental – Use of OCP after conception ( a/w middle & posterior forms) Endocrine disruptors - DES, PCBs, DDT

5. Examination Asymmetrical prepucial development – dorsal hood & ventral deficiency exposing glans & proximal meatus . Downward glans tilt Deviation of the median penile raphe Ventral curvature (VC) Scrotal encroachment onto penile shaft Midline scrotal cleft Peno -scrotal transposition – scrotum anterior & superior

6. Associated abnormalities Cryptorchidism – 10% case Open processus vaginalis or inguinal hernia – 9-15% case Meatal stenosis No relation between the severity of the hypospadias and associated anomalies of the upper- or lower urinary tract Isolated hypospadias regardless of severity – NOT an indication for Upper tract imaging

7. Isolated vs Syndromic Hypospadias Syndromic Hypospadias – WAGR syndrome – del 11p13 Smith- Lemli - Opitz syndrome Wolf- Hirschhorn syndrome Suspected in patients with – Developmental delay Dysmorphic facies Anorectal malformations

8. Isolated vs DSD with Hypospadias DSD suspected in Phenotypic boys with both – Hypospadias Undescended testis It is an indication for Karyotyping – DSD in 25% Most common associated DSDs Mixed Gonadal Dysgenesis Ovotesticular DSD More likely if – Proximal hypospadias + Nonpalpable testes

9. Concealed Hypospadias Normal prepuce concealing glanular to distal shaft meatus Detected at circumcision Circumcision should NOT be stopped Megameatus with intact prepuce – deeply grooved urethral plate extending laterally under skin edge

10. Chordee without Hypospadias Asymmetrical prepucial development with a normal glanular meatus Congenital Ventral Penile Curvature Classified as hypospadias if distal urethra is thin with deficient corpus spongiosum

11. Classification Distal Anterior Glanular , Subcoronal 50% Intermediate Middle Penile 30% Proximal Posterior Penoscrotal , Scrotal, Perineal 20%

12. Age for Surgery Healthy full term baby ≥ 3mon – daycare procedure Preterm baby >56 gestational weeks AAP – surgery to be done by 18mon – ↓ psychosexual stress Usual age at primary repair – 6-18mon Age at surgery in pre-pubertal 1° TIP repair is not a risk for complications [LE:2b] 1° TIP repair complication rate – 2.5 times higher in adults [LE:2a] Younger the child, lesser the discomfort following repair

13. Pre-operative Hormonal Treatment Androgens (testosterone / DHT / β - hCG ) increase penile length & glans circumference [LE:1b] Weak evidence for local / parenteral use in – proximal hypospadias small appearing penis reduced glans circumference reduced urethral plate s/e – genital pigmentation, pubic hairs, ↑ erections

14. Functional indications for surgery Proximally located (ectopic) meatus Ventrally deflected or spraying urinary stream Meatal stenosis Curved penis

15. Cosmetic indications for surgery Abnormally located meatus Cleft glans Rotated penis with abnormal cutaneous raphe Preputial hood Penoscrotal transposition Split scrotum

16. Ventral Curvature Distal hypospadias – VC in 10% – <30° after degloving Proximal hypospadias – 50% have no or <30° VC after degloving 50% have >30° after degloving VC corrected in 70% by degloving & excision of chordee Artificial erection – intra-operative corporeal hep -saline inj. VC correction – <30° - Dorsal plication >30° - Ventral corporotomies with or without grafting

17. The Thin Urethra

18. Assessment of Urethral Plate Mainstay of repair – preservation of vascularised urethral plate & its use for urethral reconstruction Wide urethral plate – Tubularized Narrow urethral plate – TIP Urethral plate incision deep – inlay graft If transected / excised for chordee correction – 2-stage repair Urethral plate elevation / mobilization not to be combined with TIP – focal devascularisation

19. Postoperative Management Anticholinergics for bladder spasms No difference in outcome with / without bandages No consensus on duration of dressing & stenting No data indicating benefit of SPC in addition / substitute for PUC No recommendation on medical prevention of NPTs – Ketoconazole , phenobarbitone , diazepam Post-op antibiotics - controversial

20. Risk factors for complications Proximal meatus Redo repair Glans width <14mm Complication rate – 10% in distal & 25% in proximal one-stage repairs Higher and variable rates (30-70%) in two-stage repairs. Tunica vaginalis barrier flap reduces risk of Fistulas

21. Follow-up Long term follow-up recommended till adolescence Urethral stricture & Meatal stenosis Voiding dysfunctions Recurrent penile curvature Diverticula Glanular dehiscence Ejaculatory disorder 50% complications requiring re-operation present >1yr after surgery

22. Outcome Assessment Validated objective scoring systems recommended to evaluate functional and cosmetic outcome Hypospadias Objective Scoring Evaluation – HOSE Hypospadias Objective Penile Evaluation (HOPE) Score Pediatric Penile Perception Score (PPPS)

23. Hypospadias Objective Scoring Evaluation – HOSE >= 14 Acceptable outcome May be used for initial severity assessment LE – 2b

24. Hypospadias Objective Penile Evaluation (HOPE) Score Meatus position Meatus shape Glans shape Penile skin shape Penile Axis Torsion Curvature on erection

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