Malformations of the genitourinary system: Hypospadias. Epispadias-D

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Malformations of the Genitourinary System: Hypospadias & Epispadias

I. HYPOSPADIAS

Definition

Hypospadias is a congenital malformation in which the urethral meatus opens on the ventral (inferior) surface of the penis — anywhere from the glans to the perineum — rather than at the tip of the glans. It is often accompanied by chordee (ventral penile curvature) and a dorsally hooded prepuce.

Embryology

Normal urethral development depends on androgen-driven fusion of the urethral folds along the ventral midline (weeks 8–14 of gestation). Failure of complete fusion leads to hypospadias. The more proximal the arrest, the more severe the defect.

Incidence

Approximately 1 in 200–300 male births (one of the most common congenital penile anomalies)
Higher incidence in first-degree relatives

Classification

Classified by the location of the ectopic meatus:

Type	Meatal Position
Distal (anterior)	Glanular, coronal, distal shaft (~70%)
Middle	Midshaft
Proximal (posterior)	Proximal shaft, penoscrotal, scrotal, perineal (~30%)

Hypospadias classification diagram showing distal vs. proximal types with meatal positions from glanular to perineal

Clinical Features

Abnormal urinary stream (deflected downward/ventral)
Chordee — ventral curvature of the penis (more common in proximal types)
Dorsal hooded prepuce (incomplete foreskin ventrally)
Difficulty with micturition in proximal types
Psychosocial / sexual dysfunction if untreated

Associations

Cryptorchidism (~10%)
Inguinal hernia
In severe cases: disorders of sex development (DSD) should be excluded

Management

(Bailey & Love's, p. 1561)

Surgical correction is the mainstay:

Timing: Ideally before 18 months of age
Distal hypospadias:
- Often repaired for cosmetic and functional reasons
- Technique of choice: Tubularised Incised Plate (TIP) urethroplasty (Snodgrass procedure)
Proximal hypospadias with chordee: Two-stage repair
- Stage 1: Correct penile curvature (chordee release ± skin grafting)
- Stage 2: Urethral reconstruction (urethroplasty using preputial or buccal mucosa)
Circumcision should be avoided prior to repair — preputial skin is valuable tissue for reconstruction

Complications of Surgery

Urethrocutaneous fistula (most common)
Meatal stenosis
Urethral stricture
Wound dehiscence
Persistent/recurrent chordee

II. EPISPADIAS

Definition

Epispadias is a rare congenital dorsal penile defect in which the urethral meatus opens on the dorsal (superior) surface of the penis. The opening may lie anywhere from the glans to the penopubic junction (Bailey & Love's, p. 300).

A useful conceptual mnemonic: if hypospadias results from a ventral opening (scissors blade into the urethra, cutting ventrally), epispadias is the dorsal counterpart — cutting dorsally through the penis and pubis into the bladder.

Incidence

Much rarer than hypospadias: ~1 in 117,000 males, ~1 in 484,000 females

Embryology

Results from failure of the cloacal membrane to be replaced by mesenchymal tissue during embryogenesis, leading to premature rupture of the cloacal/urogenital membrane.

Classification (Males)

Type	Description
Glanular	Meatus on dorsal glans (mildest)
Penile	Meatus on dorsal shaft
Penopubic (complete)	Meatus at penopubic junction; sphincter usually incompetent → incontinence

Epispadias–Exstrophy Complex

Epispadias exists on a spectrum with bladder exstrophy:

Condition	Features
Isolated epispadias	Dorsal penile opening only; bladder intact
Bladder exstrophy	Bladder and bladder neck open on lower abdominal wall; epispadias always present
Cloacal (ileocaecal) exstrophy	Most severe variant: exomphalos + everted caecum/ileum separating two bladder halves + split penis

Clinical photograph of a neonate with classical bladder exstrophy: exposed bladder mucosa, foreshortened penis with dorsal chordee and open urethral plate characteristic of the exstrophy-epispadias complex

Clinical Features

Dorsal urethral opening on the penis
Dorsal chordee (upward penile curvature)
Urinary incontinence — common, especially in penopubic type (incompetent bladder neck)
Widened pubic symphysis (diastasis) in exstrophy
Fertility issues — retrograde ejaculation, epididymo-orchitis risk

In Females

Short, wide urethra with cleft clitoris
Incontinence is the predominant symptom
Bifid clitoris

Management

Complex multistep surgical reconstruction, often in a staged approach:

Penile/bladder neck reconstruction — restore continence
Urethral reconstruction — tubularize the dorsal urethral plate
Pelvic osteotomy — for bladder exstrophy (to close the pubic diastasis)
Bladder augmentation / urinary diversion — in severe exstrophy

Boys with epispadias and a functioning bladder neck may achieve continence with reconstructive surgery alone (Bailey & Love's, p. 300)

III. COMPARISON: Hypospadias vs. Epispadias

Feature	Hypospadias	Epispadias
Meatal location	Ventral surface	Dorsal surface
Chordee direction	Ventral (downward)	Dorsal (upward)
Incidence	~1:200–300	~1:117,000
Incontinence	Rare	Common (penopubic type)
Associated anomaly	Cryptorchidism, hernia	Bladder exstrophy, diastasis pubis
Prepuce	Dorsal hood	Often absent/abnormal
Surgical timing	Before 18 months	Staged from neonatal period
Key repair	TIP urethroplasty	Bladder/urethra reconstruction ± osteotomy

Sources: Bailey and Love's Short Practice of Surgery, 28th Edition (pp. 300, 1561)

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