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Paediatric Surgery
Urology
Paediatrics

Hypospadias

High EvidenceUpdated: 2025-12-23

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Red Flags

  • Hypospadias + Cryptorchidism (Undescended Testes) = Possible Disorder of Sex Development (DSD)
  • Congenital Adrenal Hyperplasia (CAH) must be excluded in severe cases
Overview

Hypospadias

[!WARNING] Medical Disclaimer: This content is for educational and informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional for diagnosis and treatment. Medical guidelines and best practices change rapidly; users should verify information with current local protocols.

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.

The Diagnostic Triad

  1. Ventral Urethral Meatus: 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.

[!IMPORTANT] Circumcision is contra-indicated. The foreskin is precious vascularised tissue used for the surgical reconstruction (urethroplasty).

Clinical Scenario: The Newborn Exam

During the NIPE (Newborn and Infant Physical Examination), you note that a male infant's foreskin looks unusual—it is bunched up on top of the penis. You cannot find the urethral opening at the tip.

Key Teaching Points

  • Do not retract the foreskin to look (it may be tight/adherent).
  • Check the position of the testes. If one or both are undescended, this is a red flag for a DSD (Disorder of Sex Development) or CAH (Congenital Adrenal Hyperplasia) in a virilised female.
  • Advise parents against circumcision.
  • Refer to Paediatric Urology/Surgery.

2. Epidemiology
  • Incidence: Approx 1 in 300 live male births. The incidence appears to be rising (possibly due to environmental endocrine disruptors).
  • Risk Factors:
    • Low birth weight / Prematurity.
    • Maternal age > 35.
    • Family history (7% risk for subsequent siblings).
    • IVF (Assisted Reproductive Technology).
    • Endocrine disruptors (pesticides, mild oestrogens).

3. Pathophysiology
  • Embryology: Failure of the urethral folds to fuse in the midline during weeks 8–14 of gestation.
  • Hormonal: Dependent on Testosterone and Dihydrotestosterone (DHT). Failure of androgen receptors or 5-alpha-reductase deficiency can cause severe hypospadias.

Associated Anomalies

  • Inguinal Hernia / Patent Processus Vaginalis: 9%.
  • Cryptorchidism (Undescended Testes): 8-10%.
  • Renal Anomalies: Rare (routine renal ultrasound not indicated unless other congenital anomalies present).

4. Clinical Presentation

DSD (Disorders of Sex Development)


Diagnosis
Usually made at birth (NIPE).
Spraying
Parents may report urine sprays or drips downwards when the boy passes urine.
Cosmesis
The hooded foreskin is visually distinct.
Curvature
May be noted during erection (Chordee).
5. Clinical Examination
  1. Meatus: Identify location.
  2. Glans: Often flattened or splayed.
  3. Prepuce: Dorsal hood.
  4. Chordee: Difficult to assess in flaccid state, but verify if penile shaft is straight.
  5. Testes: Palpate scrotum carefully.

6. Investigations
  • Mild/Distal: None required.
  • Severe/Proximal + Undescended Testis:
    • Karyotype.
    • Endocrine Workup (Testosterone, DHT, LH/FSH).
    • Ultrasound KUB.

7. Management

Indications for Surgery

  1. Functional: To allow standing to urinate (stream direction).
  2. Sexual: To straighten the penis (Chordee correction) for future intercourse.
  3. Cosmetic: To create a normal-looking penis.

Timing

  • Typically performed between 6 and 18 months of age.
  • Benefits: Amnesia of the event, easier catheter management (nappies), less psychological trauma than in older childhood.
  • Pre-op: Testosterone cream may be used for a few weeks to increase penile size in "micro-penis" cases to facilitate repair.

Surgical Techniques

  1. Distal Hypospadias:
    • TIP (Tubularised Incised Plate) / Snodgrass Repair: Most common. The urethral plate is incised longitudinally to widen it, then tubularised over a catheter.
    • MAGPI (Meatal Advancement and Glanuloplasty): For very distal glanular cases.
  2. Proximal Hypospadias:
    • Often requiring Two-Stage Repair.
    • Stage 1: Correct chordee and lay down a graft (e.g., buccal mucosa from cheek) to create a new urethral plate.
    • Stage 2 (6 months later): Tubularise the graft.

Post-Operative Care (The Double Nappy)

  • Catheter: A stent/catheter is left in for 5-10 days.
  • "Double Nappy" Technique:
    • Inner nappy for stool.
    • Catheter passes through a hole in the inner nappy.
    • Outer nappy collects the urine from the catheter.
  • Analgesia & Antibiotics.

8. Complications

Hypospadias surgery has a significant complication rate (up to 10-20% depending on severity).

Early

  • Bleeding / Haematoma.
  • Infection.
  • Catheter / Stent issues (blockage, falling out).

Late

  • Urethrocutaneous Fistula: A hole forms between the new urethra and the skin. Urine leaks out the side. Requires surgical repair.
  • Meatal Stenosis: Narrowing of the new opening. Causes straining/spraying.
  • Urethral Stricture: Narrowing of the tube.
  • Recurrent Chordee: Persistent curvature.
  • Diverticulum.

9. Prognosis & Outcomes
  • Most boys achieve excellent functional and cosmetic results.
  • Long-term follow-up (into puberty) is ideal to monitor for strictures or curvature recurrence as the penis grows.
  • Fertility is usually normal unless associated with testicular dysgenesis.

10. Evidence & Guidelines
  • BAPU (British Association of Paediatric Urologists): Consensus statements.
  • EAU (European Association of Urology): Paediatric Urology Guidelines.

11. Patient & Layperson Explanation

What is Hypospadias? In boys, the tube that carries urine (urethra) normally runs to the tip of the penis. In hypospadias, this tube stops short, meaning the opening is underneath the penis rather than at the end. The foreskin is usually "hooded" at the top but missing underneath.

Why did it happen? It happens early in pregnancy when the penis is forming. It is quite common (1 in 300 boys). It is not caused by anything the mother did or didn't do.

Can he be circumcised? No. Not at birth. The foreskin is very important "spare skin" that the surgeon will need to fix the tube later.

How is it fixed? An operation called a "urethroplasty" is done, usually around 1 year of age. The surgeon:

  1. Straightens the penis (if curved).
  2. Builds a new tube to reach the tip.
  3. Fixes the foreskin (reconstruction) or removes the excess (circumcision) at the same time.

What happens after surgery? He will have a small plastic tube (catheter) in the penis for about a week to let the new repair heal without urine touching it. We use a special "double nappy" system to manage this easily.


12. References
  1. Snodgrass W, Bush N. Hypospadias: Algorithm for repair. Nat Rev Urol. 2011.
  2. Manzoni G, et al. Hypospadias surgery: when, what and by whom? BJU Int. 2004;94(8):1188-1195.
  3. Spinoit AF, et al. Hypospadias repair at a tertiary care center: long-term follow-up is mandatory. Urology. 2013;81(5):1061-1065.

Last updated: 2025-12-23

At a Glance

EvidenceHigh
Last Updated2025-12-23

Red Flags

  • Hypospadias + Cryptorchidism (Undescended Testes) = Possible Disorder of Sex Development (DSD)
  • Congenital Adrenal Hyperplasia (CAH) must be excluded in severe cases

Clinical Pearls

  • **Circumcision is contra-indicated**. The foreskin is precious vascularised tissue used for the surgical reconstruction (urethroplasty).

Guidelines

  • NICE Guidelines
  • BTS Guidelines
  • RCUK Guidelines