Hypospadias

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1. Overview

Hypospadias is a common congenital anomaly of the male urethra where the urethral meatus (opening) is located on the ventral aspect (underside) of the penis, rather than at the tip of the glans. It is one of the most frequent congenital anomalies affecting the male genitourinary system. [1,2]

The Diagnostic Triad

1. Ventral Urethral Meatus: Ectopic opening anywhere from glans to perineum.
2. Dorsal Hood: The prepuce (foreskin) is deficient ventrally and hooded dorsally.
3. Chordee: Ventral curvature of the penis, especially during erection, resulting from dysplastic tissue or fibrotic bands.

[!IMPORTANT] Circumcision is contra-indicated. The foreskin is precious vascularised tissue used for the surgical reconstruction (urethroplasty). Parents must be counselled against neonatal circumcision.

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2. Epidemiology

Incidence

Hypospadias affects approximately 1 in 200 to 1 in 300 live male births globally, making it one of the most common congenital anomalies. [1,2] The incidence has shown a rising trend over the past several decades, particularly in Western countries. This increase is thought to be multifactorial, involving improved ascertainment, environmental factors, and potential endocrine disruption. [2,3]

Geographic Variation

• Europe and North America: Incidence ranges from 4 to 8 per 1,000 male births.
• Asia: Lower reported rates (2-4 per 1,000), though reporting practices may vary.
• Temporal trends: Studies from the 1970s-2000s showed doubling of incidence in some regions. [2]

Risk Factors

Maternal Factors:

• Advanced maternal age (> 35 years). [3]
• Primiparity (first pregnancy).
• Pre-gestational diabetes mellitus.
• Obesity (BMI > 30 kg/m²).
• Use of assisted reproductive technology (IVF/ICSI). [4]

Paternal Factors:

• Paternal hypospadias (genetic component).
• Advanced paternal age.

Environmental Exposures:

• Endocrine-disrupting chemicals (EDCs): pesticides, phthalates, polychlorinated biphenyls (PCBs). [5]
• Occupational exposures in agriculture or industrial settings.

Genetic Factors:

• Family history: 7-10% risk for subsequent male siblings if one brother affected. [2]
• Paternal history increases risk 2-3 fold.
• Associated with chromosomal anomalies in 5-10% of cases (especially severe hypospadias).

Prematurity and Low Birth Weight:

• Preterm birth (less than 37 weeks) and low birth weight (less than 2500g) are independent risk factors. [3]

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3. Pathophysiology

Embryological Development

Normal penile and urethral development occurs between weeks 8 and 14 of gestation. The urethral folds on the ventral aspect of the genital tubercle fuse in a proximal-to-distal direction to form a tubularized urethra. [6]

Critical Steps:

1. Week 8: Genital tubercle elongates under androgenic influence.
2. Weeks 8-12: Urethral folds begin to fuse ventrally, forming the urethral groove.
3. Weeks 12-14: Complete fusion reaches the glans, creating the glanular urethra.
4. Week 14: Formation of the prepuce begins, growing circumferentially from the corona.

Hypospadias results from:

• Incomplete fusion of the urethral folds.
• Failure of urethral plate canalization at the glans.
• Deficiency of mesenchymal tissue on the ventral aspect.

Hormonal Regulation

Testosterone and its metabolite, dihydrotestosterone (DHT), are critical for masculinization of the external genitalia. [7]

Key Androgens:

• Testosterone: Produced by fetal Leydig cells under luteinizing hormone (LH) stimulation.
• DHT: Converted from testosterone by 5α-reductase (type 2) enzyme; has higher affinity for androgen receptors.

Mechanisms of Androgen Insufficiency:

1. 5α-reductase deficiency: Autosomal recessive; causes ambiguous genitalia or severe hypospadias.
2. Androgen insensitivity syndrome (AIS): Partial AIS may present with hypospadias.
3. Leydig cell hypoplasia: Reduced testosterone synthesis.
4. Anti-androgen exposure: Maternal exposure to environmental EDCs (phthalates, pesticides). [5]

Chordee Formation

Chordee (ventral penile curvature) is present in 10-30% of hypospadias cases. [8]

Causes:

1. Skin tethering: Deficient ventral skin and dartos fascia.
2. Dartos and Buck's fascia dysplasia: Fibrous tissue replaces normal tissue. [9]
3. Corporal disproportion: Asymmetric development of corpora cavernosa.
4. Urethral plate tethering: Short, inelastic urethral plate.

Recent collagen studies show altered COL2A1:COL1A1 ratios in hypospadiac dartos tissue, suggesting connective tissue dysgenesis. [9]

Associated Anomalies

Genital:

• Cryptorchidism: 8-10% of cases. [2]
• Inguinal hernia/patent processus vaginalis: 9-15%.
• Penile torsion: Rotation of penile shaft (usually counterclockwise).

Renal:

• Renal anomalies are rare in isolated hypospadias (1-3%).
• Routine renal ultrasound is NOT indicated unless:
  • Severe/proximal hypospadias.
  • Associated cryptorchidism.
  • Other congenital anomalies present.
  • Suspected syndromic diagnosis.

Syndromes Associated with Hypospadias:

• Opitz G/BBB syndrome (hypertelorism, dysphagia).
• Smith-Lemli-Opitz syndrome (microcephaly, syndactyly).
• Hand-foot-genital syndrome.
• WAGR syndrome (Wilms tumour, Aniridia, Genitourinary anomalies, mental Retardation).

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4. Classification

Hypospadias is classified anatomically based on the location of the urethral meatus after correction of chordee (if present).

Anatomical Classification

Anterior (Distal) Hypospadias (50-70% of cases): [10]

1. Glanular: Meatus on the ventral glans (most common subtype).
2. Coronal: Meatus at the coronal sulcus.
3. Subcoronal: Meatus just proximal to corona.

Middle Hypospadias (20-30%): 4. Distal penile: Meatus on distal penile shaft. 5. Midshaft: Meatus at mid-penile shaft.

Posterior (Proximal) Hypospadias (20-30%): 6. Proximal penile: Meatus at proximal shaft. 7. Penoscrotal: Meatus at the junction of penis and scrotum. 8. Scrotal: Meatus on the scrotum. 9. Perineal: Meatus posterior to scrotum; often associated with bifid scrotum.

[!NOTE] Proximal hypospadias (penoscrotal, scrotal, perineal) requires investigation for Disorders of Sex Development (DSD), especially if associated with cryptorchidism or micropenis. [11]

Severity Grading

Mild: Glanular, coronal (requires single-stage repair; good prognosis).

Moderate: Penile shaft (may require single or two-stage repair).

Severe: Penoscrotal, scrotal, perineal (often requires two-stage repair; higher complication rate; DSD workup mandatory).

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5. Clinical Presentation

Neonatal Presentation

Hypospadias is usually identified during the Newborn and Infant Physical Examination (NIPE) in the first 72 hours of life.

Classic Findings:

1. Ectopic meatus: Visualized on the ventral surface (not at the tip).
2. Dorsal hood: Foreskin forms a hood dorsally; deficient ventrally.
3. Hooded prepuce: Absent ventral foreskin; "circumcised" appearance ventrally.

Associated Features:

• Glanular cleft: Incomplete formation of the ventral glans.
• Chordee: Ventral curvature (best assessed during erection or artificial erection test intraoperatively).
• Penoscrotal transposition: Scrotum wraps around the base of the penis.
• Bifid scrotum: Scrotum split in midline (more common with severe forms).

Presentation in Older Children

If diagnosis is missed at birth (rare in developed countries), hypospadias may present with:

• Spraying of urine: Downward deflection of urinary stream.
• Difficulty with aiming: Cannot stand to urinate effectively.
• Cosmetic concerns: Parents note abnormal appearance of penis.
• Chordee: Curvature noted during erections in adolescents.

Symptoms in Adolescents/Adults (Uncorrected)

• Psychosocial distress regarding appearance.
• Difficulty with sexual intercourse (if chordee present).
• Infertility (if severe proximal hypospadias with abnormal semen delivery).

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6. Clinical Examination

Systematic Examination of the Penis

Inspection:

1. Meatus location: Identify exact position (glanular, coronal, shaft, penoscrotal, etc.).
2. Glans morphology: Look for glanular cleft or splayed glans.
3. Prepuce: Note dorsal hooded prepuce; absent ventral foreskin.
4. Penile shaft: Assess for torsion (rotation of shaft relative to glans).
5. Scrotum: Check for bifid scrotum, penoscrotal transposition.

Palpation:

1. Testes: Palpate both testes in scrotum. If absent, palpate inguinal canal for cryptorchidism.
2. Penile shaft: Feel for fibrous bands or tissue dysplasia along ventral surface.
3. Urethral plate: Palpate the urethral groove distal to meatus.

Assessment of Chordee:

• Difficult to assess in the flaccid state.
• Artificial erection test: Performed intraoperatively by injecting saline into corpora cavernosa to assess true degree of curvature.

Red Flags Requiring Urgent Investigation:

• Bilateral cryptorchidism + hypospadias → DSD workup.
• Micropenis (less than 2.5 cm stretched length in term neonate) + hypospadias → Endocrine evaluation. [12]
• Bifid scrotum + perineal hypospadias → Karyotype, pelvic ultrasound.

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7. Investigations

Distal/Mild Hypospadias (Isolated)

No investigations required.

• Routine renal ultrasound is NOT indicated.
• Proceed to surgical planning if repair indicated.

Proximal/Severe Hypospadias

Investigations are indicated if ANY of the following features are present: [11]

• Penoscrotal, scrotal, or perineal hypospadias.
• Bilateral cryptorchidism.
• Unilateral cryptorchidism with severe hypospadias.
• Micropenis.
• Bifid scrotum.
• Palpable gonad in labioscrotal fold.

Investigations:

1. Karyotype:

• To exclude disorders of sex development (46,XX virilized female or 46,XY undervirilized male).

2. Endocrine Panel:

• 17-hydroxyprogesterone (17-OHP): Screen for congenital adrenal hyperplasia (CAH).
• Testosterone: Assess Leydig cell function.
• Luteinizing hormone (LH) and follicle-stimulating hormone (FSH).
• Dihydrotestosterone (DHT): If 5α-reductase deficiency suspected.
• Anti-Müllerian hormone (AMH): Marker of Sertoli cell function.

3. Pelvic/Abdominal Ultrasound:

• Assess for Müllerian structures (uterus, vagina) suggesting DSD.
• Identify undescended testes.
• Rarely, MRI pelvis if ultrasound inconclusive.

4. Genitography/Contrast Studies:

• Not routinely performed; may be used in complex DSD cases to delineate urogenital anatomy.

5. Renal Ultrasound:

• Only if:
  • Other congenital anomalies.
  • Syndromic features.
  • Family history of renal disease.

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8. Management

General Principles

Goals of Hypospadias Repair: [13]

1. Functional: Permit normal urination while standing (straightforward stream).
2. Cosmetic: Create a normal-appearing penis (glans-tip meatus, straight shaft).
3. Sexual: Ensure straight erections for future sexual function.

Non-Surgical Management:

• Observation only for very mild glanular hypospadias with meatus near tip and no chordee.
• Parental counselling and reassurance.

Indications for Surgery

Surgery is indicated for: [13]

• Proximal meatus requiring standing urination.
• Chordee causing significant curvature (> 20-30 degrees).
• Cosmetic concerns (abnormal appearance).
• Psychosocial distress (older children/adolescents).

Pre-Operative Considerations

Timing of Surgery:

• Optimal age: 6-18 months. [14]
  • "Benefits: Amnesia of event; easier catheter management in nappies; reduced psychological impact; larger penile size than neonatal period but before toilet training."
• Delayed surgery: May be necessary if child presents late or has complex anatomy.

Pre-Operative Testosterone Stimulation:

• Used in cases of micropenis or small penile size to increase tissue bulk.
• Protocol: Topical DHT gel or intramuscular testosterone (1-2 mg/kg monthly for 3 months). [12]
• Evidence: Increases penile length and girth; may improve surgical outcomes.

Pre-Operative Counselling:

• Surgical technique options.
• Risks of complications (fistula, stenosis, stricture).
• Need for potential re-operation (10-20% overall). [15]
• Post-operative catheter care ("double nappy" technique).

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9. Surgical Techniques

Distal Hypospadias Repair

1. TIP (Tubularized Incised Plate) Repair / Snodgrass Technique [16]

• Most common technique for distal hypospadias (glanular, coronal, distal shaft).
• Principle: Longitudinal incision of urethral plate to widen it; then tubularize over catheter to create neourethra.

Steps:

1. Stay sutures in glans for traction.
2. Degloving of penis to assess chordee.
3. Artificial erection test to confirm straightness.
4. Midline incision of urethral plate (Snodgrass incision).
5. Tubularization of incised plate over 6-8 Fr catheter using fine absorbable sutures (6-0 or 7-0 PDS/Vicryl).
6. Coverage with dartos flap or tunica vaginalis flap (waterproofing layer).
7. Glansplasty (reconstruction of glans wings over neourethra).
8. Skin closure.

Advantages:

• Excellent cosmetic results (slit-like meatus at glans tip).
• Low fistula rate (5-10%). [16]
• Preserves urethral plate (maintains vascularity).

Disadvantages:

• Risk of meatal stenosis (5-10%) due to incision scarring. [16]
• Not suitable for severe hypospadias.

2. MAGPI (Meatal Advancement and Glanuloplasty Incorporated):

• For very distal glanular hypospadias with minimal chordee.
• Advances meatus to glans tip; reconstructs glans.

3. Mathieu (Flip-Flap) Repair:

• Uses ventral skin flap to create neourethra.
• Good for coronal/subcoronal hypospadias.

4. Onlay Island Flap (Duckett):

• Preputial flap onlayed onto urethral plate.
• For distal-mid shaft hypospadias.

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Proximal Hypospadias Repair

Principles:

• More complex anatomy; often significant chordee.
• May require two-stage approach if urethral plate is poor quality or heavily scarred.

1. Single-Stage Repair:

Tubularized Incised Plate (TIP):

• May be used for proximal shaft if good-quality urethral plate after chordee release.

Onlay Island Flap (Duckett):

• Preserves urethral plate; onlays preputial skin flap.

Transverse Preputial Island Flap (Duckett):

• Harvests inner preputial flap; tubularizes to extend urethra.

2. Two-Stage Repair (Bracka): [17]

• Stage 1 (Initial surgery):

  • Release chordee (excise fibrous tissue, divide urethral plate if tethering).
  • Confirm straight penis with artificial erection.
  • Lay graft (buccal mucosa or preputial skin) onto ventral surface to create new urethral plate.
  • Allow 6-12 months for graft maturation and vascularization.

• Stage 2 (6-12 months later):

  • Tubularize the matured graft to form neourethra.
  • Glansplasty and skin closure.

Indications for Two-Stage:

• Severe proximal hypospadias (penoscrotal, scrotal, perineal).
• Severe chordee requiring extensive release.
• Redo surgery after failed prior repair.
• Poor-quality or absent urethral plate.

Graft Options:

• Buccal mucosa (inner cheek): Most popular; robust, hairless, good "take."
• Preputial skin: If sufficient available.
• Bladder mucosa: Rarely used.

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Chordee Correction

Chordee must be corrected before urethroplasty to ensure straight erections.

Techniques:

1. Degloving and Dartos Excision: Removes tethering ventral skin/dartos.
2. Urethral Plate Division: If urethral plate is causing tethering.
3. Corporal Plication (Nesbit): Dorsal plication sutures to shorten dorsal corpora (if ventral lengthening insufficient).
4. Ventral Lengthening: Rarely, grafts (tunica albuginea or dermis) to lengthen ventral corpora.

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10. Post-Operative Care

Catheterization

Urethral Stent/Catheter:

• Transurethral silicone stent (6-8 Fr) left in situ for 5-10 days. [18]
• Diverts urine away from repair to allow healing.
• Suprapubic catheter rarely used (increases morbidity).

"Double Nappy" Technique: [18]

• Inner nappy: Worn normally for stool containment; hole cut in front for catheter to pass through.
• Outer nappy: Collects urine draining from catheter.
• Allows easy catheter management at home without complex devices.

Analgesia

Peri-operative Analgesia:

• Caudal block: Commonly used; provides excellent immediate post-op pain relief. [19]
  • "Controversy: Some studies suggested caudal blocks increase fistula risk (theoretical pelvic vasodilation/bleeding), but recent meta-analyses show no significant association. [19]"
  • Remains widely used and recommended by most centers.
• Pudendal/dorsal penile nerve block: Alternative regional technique.
• Systemic analgesia: Paracetamol, ibuprofen, opioids as needed.

Post-Discharge Analgesia:

• Regular paracetamol and ibuprofen for 5-7 days.
• Opioids (e.g., morphine sulfate oral solution) if significant pain.

Antibiotics

Prophylactic Antibiotics:

• Perioperative single-dose intravenous antibiotics (e.g., co-amoxiclav, cefuroxime).
• Catheter in situ: Oral prophylaxis (e.g., trimethoprim, cephalexin) until catheter removed (controversial; not universally used).

Catheter Removal

• Timing: Day 5-10 post-operatively (varies by surgeon preference and technique).
• Outpatient removal or day-case admission.
• Monitor first void after removal (ensure patent urethra, no significant obstruction).

Wound Care

• Keep area clean and dry.
• Nappy changes: Gentle cleansing; avoid vigorous rubbing.
• Dressings (if used): Usually absorbable, fall off by day 3-5.

Follow-Up

Early Follow-Up (2-4 weeks):

• Wound check: Assess for infection, fistula, meatal stenosis.
• Urinary stream assessment.

Long-Term Follow-Up:

• 6 months, 1 year, then annually until puberty.
• Monitor for late complications: stricture, fistula, meatal stenosis, recurrent chordee.
• Puberty follow-up: Assess penile growth, erections, curvature.
• Discharge to adult urology if ongoing issues.

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11. Complications

Hypospadias surgery has a complication rate of 10-30% depending on severity and technique. [15,20]

Early Complications (0-6 weeks)

1. Bleeding/Hematoma:

• Incidence: 1-3%.
• Management: Usually conservative; rarely requires evacuation.

2. Wound Infection:

• Incidence: 2-5%.
• Management: Antibiotics; ensure catheter patent.

3. Catheter Issues:

• Blockage (kinking, clot).
• Premature dislodgement.
• Bladder spasm causing bypassing (urine leaking around catheter).

4. Wound Dehiscence:

• Partial breakdown of glansplasty or skin closure.
• Incidence: 2-5%.
• Management: May heal by secondary intention vs. require revision.

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Late Complications (> 6 weeks)

1. Urethrocutaneous Fistula: [15,20]

• Definition: Abnormal connection between neourethra and skin; urine leaks from ventral surface during voiding.
• Incidence: 5-15% (higher in proximal repairs).
• Presentation: Parents report urine spraying from side of penis.
• Management:
  • "Small fistulas (less than 3 mm): May close spontaneously (10-20% chance)."
  • "Persistent fistulas: Surgical repair after 6-12 months (allow inflammation to settle)."
  • "Technique: Fistula excision, multi-layer closure with flap interposition."

2. Meatal Stenosis:

• Definition: Narrowing of neourethral meatus.
• Incidence: 5-10% (higher with TIP repair). [16]
• Presentation: Thin urinary stream, straining, spraying.
• Management:
  • "Mild: Observation; meatal dilation (controversial efficacy)."
  • "Severe: Meatoplasty (surgical widening)."

3. Urethral Stricture:

• Definition: Narrowing of neourethra along its length.
• Incidence: 2-5%.
• Presentation: Weak stream, straining, recurrent UTIs.
• Diagnosis: Urethrography, cystoscopy.
• Management: Urethral dilation, optical urethrotomy, or urethroplasty revision.

4. Recurrent Chordee (Penile Curvature):

• Incidence: 5-10%.
• Presentation: Noted during erections in adolescence.
• Management: Surgical correction (plication or grafting).

5. Urethral Diverticulum:

• Definition: Outpouching of neourethra (ballooning during voiding).
• Incidence: 1-5%.
• Cause: Distal stenosis with proximal dilatation; excess redundant neourethra.
• Management: Excision of diverticulum; ensure meatus is widely patent.

6. Glans Dehiscence:

• Breakdown of glansplasty; glans wings separate.
• Incidence: 3-7%.
• Management: Revision glansplasty.

7. Hairy Urethra:

• If hair-bearing scrotal or perineal skin used in proximal repairs.
• Presentation: Recurrent UTIs, obstructive symptoms, hematuria.
• Prevention: Use non-hair-bearing tissue (buccal mucosa, bladder, preputial inner skin).
• Management: Laser hair ablation; rarely, urethral reconstruction.

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Factors Increasing Complication Risk

• Proximal meatus location (penoscrotal, scrotal, perineal). [15]
• Severe chordee (> 30 degrees).
• Redo surgery (revision after prior failed repair). [20]
• Poor tissue quality (scarred, ischemic).
• Surgeon experience (higher complication rates with low-volume surgeons).

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12. Prognosis & Long-Term Outcomes

Functional Outcomes

Voiding:

• > 90% of boys achieve normal voiding with good forward stream after successful repair. [1,2]
• Standing micturition possible in distal/mid repairs.
• Proximal repairs may have more variable outcomes.

Sexual Function:

• Most men with corrected hypospadias report normal sexual function and satisfaction. [21]
• Straight erections achieved in > 85-90%.
• Ejaculation and orgasm typically normal.

Fertility:

• Isolated hypospadias: Fertility generally normal.
• Severe hypospadias with testicular dysgenesis: May have impaired spermatogenesis.
• Proximal hypospadias with persistent curvature: May affect semen deposition.

Psychosocial Outcomes

• Early repair (before 18 months) associated with better psychosocial outcomes (no memory of surgery). [14]
• Adolescents with uncorrected or poorly corrected hypospadias may experience:
  • Body image concerns.
  • Anxiety regarding sexual relationships.
• Counselling and reassurance important.

Long-Term Follow-Up

Recommendations: [1,2]

• Follow-up into puberty and adolescence essential.
• Monitor for:
  • Recurrent chordee (penile growth may unmask curvature).
  • Stricture development.
  • Psychosexual adjustment.
• Transition to adult urology: If ongoing issues (stricture, fistula) or complex reconstructive needs.

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13. Disorders of Sex Development (DSD)

When to Suspect DSD

Hypospadias with any of the following features warrants urgent DSD investigation: [11]

• Bilateral cryptorchidism (palpable gonads in inguinal canals or non-palpable).
• Unilateral cryptorchidism + severe hypospadias.
• Micropenis (stretched penile length less than 2.5 cm in term neonate). [12]
• Bifid scrotum.
• Penoscrotal/scrotal/perineal hypospadias.
• Palpable gonad in labioscrotal fold (suggests ovotestis or ovary).

Differential Diagnosis

46,XX DSD (Virilized Female):

• Congenital Adrenal Hyperplasia (CAH): Most common cause.
  • 21-hydroxylase deficiency (> 90% of CAH).
  • Presents with ambiguous genitalia, virilization, salt-wasting crisis.
  • "Diagnosis: Elevated 17-OHP, hyponatremia, hyperkalemia."
  • Life-threatening salt-wasting crisis in first 2 weeks of life.

46,XY DSD (Undervirilized Male):

• 5α-reductase deficiency: Severe hypospadias, micropenis; virilization at puberty.
• Partial androgen insensitivity syndrome (PAIS): Variable undervirilization.
• Leydig cell hypoplasia: Reduced testosterone production.
• LH receptor defects.

Ovotesticular DSD:

• Presence of both ovarian and testicular tissue (ovotestis).
• Ambiguous genitalia; asymmetric gonadal development.

Gonadal Dysgenesis:

• Mixed gonadal dysgenesis (45,X/46,XY mosaicism).
• Complete or partial dysgenesis.

Investigation of DSD

Urgent (within 24-48 hours):

• Karyotype (or rapid FISH for sex chromosomes).
• Serum electrolytes (Na+, K+) to detect salt-wasting CAH.
• 17-hydroxyprogesterone (17-OHP): Screen for CAH.
• Glucose: Hypoglycemia may occur in CAH.

Endocrine Panel (within 1 week):

• Testosterone, DHT, LH, FSH, AMH.
• Cortisol, ACTH (if CAH suspected).

Imaging:

• Pelvic/abdominal ultrasound: Look for Müllerian structures (uterus, vagina), gonads.
• Genitography (contrast study): Delineate urogenital anatomy.

Genetic Testing:

• Targeted gene panels (AR, SRD5A2, NR5A1, etc.).
• Whole exome sequencing if diagnosis unclear.

Multidisciplinary Team:

• Paediatric endocrinologist.
• Paediatric urologist/surgeon.
• Geneticist.
• Psychologist/psychiatrist.
• Ethicist (gender assignment in ambiguous cases).

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14. Guidelines & Evidence

International Guidelines

European Association of Urology (EAU) Paediatric Urology Guidelines (2024): [1]

• Recommend surgery at 6-18 months of age.
• TIP repair as first-line for distal hypospadias.
• Two-stage repair for severe proximal hypospadias.
• Mandatory DSD workup for proximal hypospadias with cryptorchidism.

American Urological Association (AUA) Guidelines (2021):

• Similar recommendations to EAU.
• Emphasize long-term follow-up into adolescence.

British Association of Paediatric Urologists (BAPU) Consensus (2020):

• National standards for hypospadias surgery.
• Advocate centralization of complex cases to specialist centers.

Key Evidence

Snodgrass TIP Repair: [16]

• Landmark technique introduced 1994.
• Multiple systematic reviews confirm low complication rates (5-10% fistula, 5-10% stenosis).
• Now gold standard for distal hypospadias.

Two-Stage Bracka Repair: [17]

• Superior outcomes for severe proximal hypospadias vs. single-stage.
• Buccal mucosa graft preferred (lower complication rates than skin).

Complications: [15,20]

• Overall complication rate 10-30%.
• Higher in proximal hypospadias, redo surgery.
• Fistula most common complication.

Long-Term Outcomes: [21]

• Most men report satisfactory cosmetic and functional outcomes.
• Sexual function and fertility generally normal in isolated hypospadias.

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15. Patient & Layperson Explanation

What is Hypospadias?

In boys, there is a tube inside the penis called the urethra that carries urine from the bladder to the outside. Normally, the opening of this tube (the "pee hole") is at the very tip of the penis. In hypospadias, this opening is on the underside of the penis instead of at the tip. The foreskin is also different—it looks like a "hood" at the top of the penis but is missing underneath.

How Common is It?

Hypospadias is quite common. About 1 in every 200-300 baby boys is born with it. It is one of the most frequent birth differences affecting boys.

Why Did It Happen?

Hypospadias happens very early in pregnancy (around weeks 8-14) when the penis is forming. The tube that becomes the urethra doesn't completely close all the way to the tip. It is not caused by anything the mother did or didn't do. Sometimes it runs in families (if dad or a brother has it, the risk is higher).

Can My Baby Be Circumcised?

No, not right away. The foreskin is very important "spare tissue" that the surgeon will use later to fix the tube and reconstruct the penis. If the foreskin is removed by circumcision, it makes the repair much more difficult or impossible. Your doctor will advise against circumcision at birth.

Does My Baby Need Tests?

For most boys with hypospadias, no tests are needed. However, if the opening is very far back (near the scrotum) or if one or both testicles have not descended, then your doctor may order tests to check hormones and chromosomes. This is to rule out rare conditions affecting sex development.

How Is It Fixed?

An operation called urethroplasty (or hypospadias repair) is done, usually when your child is around 6-18 months old. The surgeon will:

1. Straighten the penis if it is curved.
2. Build a new tube (urethra) from the current opening all the way to the tip of the penis.
3. Reconstruct the foreskin or remove the excess skin.

The operation is done under general anesthesia (your child will be asleep and feel no pain). It usually takes 1-3 hours depending on how complex the repair is.

What Happens After Surgery?

After the operation:

• Your child will have a small plastic tube (catheter) in the penis for about 5-10 days. This tube drains urine into the nappy so that the repair can heal without urine touching it.
• We use a special "double nappy" system: one nappy for poo, and the catheter passes through a hole to an outer nappy that collects the urine.
• Your child will need pain medicine (paracetamol, ibuprofen) for about a week.
• The catheter is removed in clinic after 5-10 days (this is usually quick and not very painful).

Will There Be Complications?

Most boys do very well, but hypospadias surgery can have complications in about 10-20% of cases. The most common problems are:

• Fistula (a small hole forms and urine leaks from the side): This may need another operation to fix.
• Narrowing of the opening (stenosis): The new opening may become tight; this can be widened.
• Infection or bleeding: Rare; treated with antibiotics or observation.

Your surgeon will discuss the risks with you in detail.

What About the Long Term?

Most boys grow up with a normal-looking penis and normal function. They can:

• Stand to urinate.
• Have normal erections and sexual function as adults.
• Father children (fertility is usually normal).

Your child will need follow-up appointments for several years (sometimes into the teenage years) to make sure everything is healing well and working properly.

Will My Child Remember the Surgery?

If the surgery is done at 6-18 months, your child will have no memory of it. This is one reason we do the operation at this age—it is less traumatic psychologically.

What If We Don't Do the Surgery?

If hypospadias is not repaired:

• Your child may have difficulty standing to urinate (the stream goes downward).
• The penis may be curved, which can cause problems with sexual function later.
• There may be concerns about appearance and self-esteem.

Most doctors recommend surgery for these reasons, but you can discuss your options with your surgeon.

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16. Clinical Pearls & Exam Tips

High-Yield Exam Points

For MRCS/FRCS(Paeds) Vivas:

• Triad: Ventral meatus, dorsal hood, chordee.
• Classification: Anterior (distal), middle, posterior (proximal).
• Red flag: Hypospadias + cryptorchidism = DSD workup (CAH, AIS).
• Surgery timing: 6-18 months (amnesia, easier catheter care, before toilet training).
• TIP/Snodgrass: Most common technique for distal hypospadias.
• Two-stage Bracka: For severe proximal hypospadias; Stage 1 = graft, Stage 2 = tubularize.
• Complications: Fistula (5-15%), stenosis (5-10%), stricture (2-5%).
• Post-op: "Double nappy" technique for catheter management.

For MRCPCH Clinical Exam:

• Newborn exam: Note hooded prepuce, locate meatus, palpate testes.
• Do NOT circumcise: Counsel parents.
• Refer to paediatric urology.

For OSCEs (Communication Station):

• Explain to parents: common, not their fault, needs surgery around 1 year, good outcomes, avoid circumcision.

Clinical Pearls

• "Never circumcise a hypospadias": The foreskin is essential for repair.
• "Proximal hypospadias + cryptorchidism = DSD until proven otherwise": Urgent endocrine workup.
• "Chordee is underestimated in the flaccid state": Artificial erection test intraoperatively is essential.
• "Fistula is the most common complication": Parents should be warned pre-operatively.
• "TIP repair is the workhorse": For distal hypospadias, TIP gives excellent cosmetic results.
• "Testosterone cream for micropenis": Increases penile size pre-operatively to facilitate repair.
• "Follow-up into puberty": Late strictures and recurrent chordee may only manifest with penile growth.

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17. Related Topics

Prerequisites

• Embryology of the genitourinary system
• Disorders of Sex Development (DSD)
• Congenital Adrenal Hyperplasia (CAH)

Differentials

• Epispadias (dorsal urethral defect; associated with bladder exstrophy)
• Buried penis
• Micropenis
• Ambiguous genitalia (DSD)

Consequences

• Urethrocutaneous fistula
• Urethral stricture
• Psychosexual dysfunction (if uncorrected)

Related Procedures

• TIP (Snodgrass) urethroplasty
• Two-stage Bracka repair
• Chordee correction
• Meatoplasty

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18. References

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3. Nassar N, Bower C, Barker A. Increasing prevalence of hypospadias in Western Australia, 1980-2000. Arch Dis Child. 2007;92(7):580-584. doi:10.1136/adc.2006.112730

4. Belva F, Bonduelle M, Tournaye H. Endocrine and reproductive profile of boys and young adults conceived after ICSI. Curr Opin Obstet Gynecol. 2019;31(3):163-169. doi:10.1097/GCO.0000000000000535

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7. Sinnecker GH, Hiort O, Dibbelt L, et al. Phenotypic classification of male pseudohermaphroditism due to steroid 5 alpha-reductase 2 deficiency. Am J Med Genet. 1996;63(1):223-230. doi:10.1002/(SICI)1096-8628(19960503)63:1less than 223::AID-AJMG38> 3.0.CO;2-O

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9. Yuri P, Warli SM, Rodjani A. The ratio of COL2A1:COL1A1 in dartos tissue patients with hypospadias. BMC Urol. 2024;24(1):272. doi:10.1186/s12894-024-01688-1

10. Duckett JW. Hypospadias. In: Walsh PC, Retik AB, Vaughan ED, Wein AJ, eds. Campbell's Urology. 7th ed. WB Saunders; 1998:2093-2119.

11. Oswald J, Schwentner C, Lunacek A, Radmayr C. Disorders of sex development and proximal hypospadias. Urologe A. 2016;55(2):225-232. doi:10.1007/s00120-015-0004-2

12. Khadilkar V, Khadilkar A, Kajale N, Lahoti A, Unni J, Gondhalekar K. Micropenis. Indian J Pediatr. 2023;90(8):809-817. doi:10.1007/s12098-023-04540-w

13. Bush NC, Villanueva C, Snodgrass W. Glans size is an independent risk factor for urethroplasty complications after hypospadias repair. J Pediatr Urol. 2015;11(6):355.e1-5. doi:10.1016/j.jpurol.2015.05.029

14. Timing of elective surgery on the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesthesia. American Academy of Pediatrics. Pediatrics. 1996;97(4):590-594.

15. Hild R, Schroth J, Seitz C, et al. Primary hypospadias repair outcomes: results from a retrospective cohort of 292 children. World J Urol. 2024;42(1):132. doi:10.1007/s00345-024-04811-x

16. Snodgrass W, Bush N, Cost N. Tubularized incised plate (TIP) hypospadias repair: A pediatric urology indicator operation. J Pediatr Urol. 2016;12(1):16-23. doi:10.1016/j.jpurol.2015.08.016

17. Bracka A. A versatile two-stage hypospadias repair. Br J Plast Surg. 1995;48(6):345-352. doi:10.1016/0007-1226(95)90024-1

18. Caione P, Capozza N, Zavattaro M. Catheter or no catheter in hypospadias repair: a randomized study. BJU Int. 2002;89(9):955-957. doi:10.1046/j.1464-410X.2002.02791.x

19. Polaner DM, Ramamoorthy C, Suresh S. Caudal analgesia, hypospadias, and urethrocutaneous fistula: Does association mean causality? Paediatr Anaesth. 2017;27(8):785-788. doi:10.1111/pan.13167

20. Morrison T, Gallagher T, Bush NC, Keays M, Snodgrass W. Surgical Approaches and Long-Term Outcomes in Adults with Complex Reoperative Hypospadias Repair. J Urol. 2018;199(4):1082-1087. doi:10.1016/j.juro.2017.11.078

21. Andersson M, Sjöström S, Wängqvist M, et al. Psychosocial and sexual outcomes in adolescents following surgery for proximal hypospadias in childhood. J Urol. 2018;200(6):1362-1370. doi:10.1016/j.juro.2018.06.078