management of epispadia

Management of Epispadias

Regi Septian

Anatomy

Defenition

• Epispadia is an abnormality in which the opening of the urethra is localized on the dorsum (upper side) of the penis or clitoris.

• Seen in only 1:300.000 newborns.• Occur in 1 in 117.000 newborns boys and 1 in

484.000 newborns girls.• In females: Bifid Clitoris.

Embryology

• There is a disorder of the mesoderm cell migration during the 4th developmental week.

• There is a defective migration of the paired primordia of the genital tubercle that fuse on the midline to form the genital tubercle at the fifth week of embryologic development.

• The urethra does not develop into a full tube.• Epispadia can occur with Exstrophy of the

bladder; in which the anterior wall of the abdomen and bladder are absent.

Sign & Symptomps

• Males : o usually have a short, wide penis with an abnormal

curve.o Urethra usually opens on the top or side of the

penis instead on the tip, may open along the whole length of the penis.

• Females:o Abnormal clitoris and labiao The opening is usually between the clitoris and

labia, but it may be in the belly area.

Sign & Symptomps

• Backward flow of urine into kidney (reflux nephropathy).

• Urinary incontinence• Urinary tract infections• Widened pubic bones

Classification

• Glans → glanular• Penile → Along the Shaft penis• Penopubic → near the pubic bone

Complication

• Stress Incontinence → because the bladder neck can not close completely, and the result is leakage of urine.

• Urinary Tract Infections (UTIs)• Infertile → the bladder neck may not close

completely during ejaculation → Retrograde ejaculation.

• Difficult sexual intercourse → because of dorsal chordae and a short, stubby penis.

Goal of Surgery

• Correction of dorsal chordae• Creation of straight urethra to allow easy negotiation

during catheterization or cystoscopy.• Satisfactory cosmetic• Minimal complications, especially regarding

urethrocutaneous fistulas• Maintenance of erectile function• Creation of urinary continence

Management of Surgery

• Glandular epispadias → corrected with reposition of the distal urethra and creation of a symmetric glans (glanuloplasty) → for cosmetic or psychological reasons.

• Penile epispadias → corrected with penile straightening by resection of the chordae and creation of a new urethra of adequat caliber and length (urethroplasty).

• Penopubic epispadias → corrected to close the abdominal wall and the bladder exstrophy.

Management of Surgery

• Surgical Technique in Male:1. Modified Cantwell-Ransley Technique

o Involves partial disassembly of the penis2. Mitchell Technique

o involves complete disassembly of the penis

Mitchell Technique

• Most recent evolution of the modern epispadias repair.

• Complete disassembly of penis into its three separate components:o Two corpora cavernosao Single corpora spongiosum

• Has a lower complication rate.• Facilitates bladder and bladder neck repair

Mitchell Technique

1. Initial Dissection

Mitchell Technique

2. Penile Disassembly

Mitchell Technique

2. Penile Disassembly

Mitchell Technique2. Penile Disassembly

Mitchell Technique

3. Proximal Dissection

Mitchell Technique4. Urethral Tubularization

Mitchell Technique

Mitchell Technique

Management of Surgery

Surgical Technique in Female:• Genital reconstruction in girls with bladder exstrophy is

less complex compared to the reconstruction in boys.• The urethra and vagina may be short and near the

front of the body and the clitoris is in two parts.• If diagnosed at birth, the two parts of the clitoris can be

brought together and the urethra can be placed into normal position.

Management of Surgery

Surgical Technique in Female:• If repaired early enough: lack of urinary control

(incontinence) may not be a problem.• If the vaginal opening is narrow in older girls or younger

women, reconstruction can be performed after puberty.

Management of Surgery

Management of Surgery

Management of Surgery

Management of Surgery

Management of Surgery

• If surgery is performed within the first few months of life, the child may have a better chance of having a normal bladder.

• There is a reasonable increase in tha bladder capacity after epispadias reconstruction

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