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

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

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

17. 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

18. The Thin Urethra

19. 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

20. 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

21. 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

22. 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

23. 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)

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

25. Hypospadias Objective Penile Evaluation
(HOPE) Score
1. Meatus position
2. Meatus shape
3. Glans shape
4. Penile skin shape
5. Penile Axis
1. Torsion
2. Curvature on erection

27. THANK YOU