EPISPADIAS

NIZZAH AFINA
1310211011

DEFINISI
Kelainan kongenital berupa tidak adanya dinding uretra bagian atas,
kelianan ini terjadi pada laki-laki maupun perempuan, tetapi lebih sering
pada laki-laki, ditandai dengan terdapatnya lubang uretra di suatu tempat
pada permukaan dorsum penis. (Dorland)
Kelainan yg jarang dijumpai (1:30000) berupa ostium uretrae eksternum yg
ditemukan di dorsum penis. Tuberkulum genital tidak terbentuk di batas
kranial membrane kloakalis, tetapi terbentuk di region septum urorektale.
Karena itu, sebagian dari membrane kloakalis terdapat disebelah kranial
dari tuberkulum genital, dan ketika membrane ini pecah, muara sinus
urogenitalis menjadi terletak di aspek kranial penis. Umumnya disertai
ekstrofi kandung kemih. ( Langman)

EPIDEMIOLOGI
Epispadias occurs more commonly in males than in females, with a
prevalence of 1 case in 10,000-50,000 persons. The male-to-female ratio is
2.3:1
(Medscape)

CAUSES
The causes of epispadias are not known. It may occur because the pubic
bone does not develop properly.
Epispadias can occur with a rare birth defect called bladder exstrophy. In
this birth defect, the bladder is inside out and sticks through the abdomen
wall. Epispadias can also occur with other birth defects.
The condition occurs more often in boys than girls. It is most often
diagnosed at birth or soon afterward.
(U.S national library of medicine)

ETIOLOGY
Unlike hypospadias, epispadias can be explained by 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.
Epispadias and exstrophy of the bladder are considered varying degrees of
a single disorder.
Another hypothesis relates the defect to the abnormal development of the
cloacal membrane.
Epispadias is rarely observed in 2 members of the same family.
(Medscape)

CLASSIFICATIONS

glandul
ar

penile

penopu
bis

CLASSIFICATIONS
Epispadias vary in severity according to the time of the pathognomic insult during
embryologic development and can be classified as glandular, penile, or complete (ie,
penopubic).
1. With the glandular type, the malformation affects the distal part of the urethra.
2. With the penile type, the entire penile urethra is affected, with an external meatus
on the dorsal shaft of the penis.
3.

With the complete or penopubic type, a total deficiency of the dorsal wall of the
urethra and the anterior wall of the bladder is present.

. The glans is often spatulated, and the prepuce is clefted dorsally with ventral
transposition. All forms of epispadias are associated with chordee. The extent of
chordee varies.
. In females, epispadias consists of bifid clitoris with diastases of the corpora
cavernosa, flattening of the mons, and separation of the labia.
(Medscape)

SYMTOMPS
Males will have a short, wide penis with an abnormal curve. The urethra
most often opens on the top or side of the penis instead of the tip.
However, the urethra may be open along the whole length of the penis.
Females have an abnormal clitorus and labia. The urethral opening is often
between the clitoris and the labia, but it may be in the belly area. They may
have trouble controlling urination (urinary incontinence)
(U.S national library of medicine)

GEJALA KLINIS
Tidak terjadi sendirian, biasanya disertai anomaly saluran kemih
Penis pipih dan kecil
Melengkung ke dorsal -> urin backward
Inkontenensia urin

EXAMS AND TEST

1. Signs include:
. Abnormal opening from the bladder neck to the area above the normal
urethra opening
. Backward flow of urine into the kidney (reflux nephropathy)
. Urinary incontinence
. Urinary tract infections
. Widened pubic bone
(U.S national library of medicine)

2. Tests may include:

Blood test
Intravenous pyelogram (IVP), a special x-ray of the kidneys, bladder, and
ureters
MRI and CT scans, depending on the condition
Pelvic x-ray
Ultrasound of the urinary system and genitals
(U.S national library of medicine)

TREATMENT
Patients who have more than a mild case of epispadias will need surgery.
Leakage of urine (incontinence) can often be repaired at the same time.
However, a second surgery may be needed.
(U.S national library of medicine)

INDICATIONS
Correction of glandular epispadias with reposition of the distal urethra and creation of a
symmetric glans (glanuloplasty) is indicated in childhood or adolescence at the patient's
request for cosmetic or psychological reasons.
Penile epispadias is corrected in childhood with penile straightening by resection of the
chordee and creation of a new urethra of adequate caliber and length (urethroplasty).
In females, bifid clitoris and normal genitalia appearance can be restored during
adolescence.
The aim is to permit normal voiding and erection and to avoid urinary tract infections.
The complete (penopubic) form of the malformation is treated early in childhood to close
the abdominal wall and the bladder exstrophy
(Medscape)

CONTRAINDICATIONS
Urethroplasty and restoring the normal appearance of the genitalia are
contraindicated in infancy because of the small size of the structures
(Medscape)

OUTLOOK (PROGNOSIS)
Surgery can help the person control the flow of urine. It will also fix the
appearance of the genitals.
(U.S national library of medicine)

POSSIBLE COMPLICATION
Some people with this condition may continue to have urinary incontinence,
even after surgery.
Ureter and kidney damage and infertility may occur.
(U.S national library of medicine)

REFERENCES
Gearhart JP, Mathews RI. Exstrophy-epispadias complex. In: Wein AJ, ed.Gearhart JP,
Mathews RI. Exstrophy-epispadias complex. In: Wein AJ, ed. Campbell-Walsh Urology.
10th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 124.
Elder JS. Anomalies of the bladder. In: Kliegman RM, Behrman RE, Jenson HB, Stanton
BF, eds.Elder JS. Anomalies of the bladder. In: Kliegman RM, Behrman RE, Jenson HB,
Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders
Elsevier; 2011:chap 535.
Update Date 1/12/2014
Updated by: Scott Miller, MD, Urologist in private practice in Atlanta, GA. Also reviewed
by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
Medscape