Bladder exstrophy is a birth defect where the bladder and abdominal wall do not close properly in the womb, leaving the bladder exposed. It occurs in about 1 in 3,000 births and is more common in males. Risk factors include young maternal age and exposure to high doses of progesterone during the first trimester. Clinically, the abdominal wall is absent and the bladder lining is exposed and bright red. Surgical correction is required to repair the abdominal wall and close the bladder. Long term risks include incontinence, infection, and rarely cancer due to changes in the bladder lining.
3. ANATOMY OF URINARY BLADDER
It is a muscular reservoir of urine situated in the
pelvis, posterior to the pubic bone separated by the
retro-pubic space.
The size, shape, position of bladder vary depends on
amount of urine it contains
Made up of detrusor and trigone muscles
The neck of bladder is of 3-4 cm
5. BLOOD SUPPLY
Superior vesical artery, a branch of internal iliac
artery.
Inferior vesical artery.
– Venous drainage is by the vesical plexus of veins,
which drains into the internal iliac veins.
6. INNERVATION AND LYMPHATIC DRAINAGE
Lymphatics drain into internal iliac and external iliac
nodes.
The efferent parasympathetic fibres (S2-S4):
contraction of the detrusor muscle and relaxation of
the sphincter vesicae.
The sympathetic input, this arises in the 11th
thoracic to the second lumbar segments;
7. By definition:is defined as incomplete development of
the infraumbilical part of the anterior abdominal
wall and anterior wall of the bladder .
This is one among the commonest congenital
abnormality lower urinary and genital track
9. RISK FACTORS
It occurs in infants of young mother
Inrease 10 folds to those mothers who received high
dose of progestrone during the first trimester
10. PATHOPHYSIOLOGY
Abnormal large cloacal membrane prevents the
mesodermal to growth and lower abdominal midline
fusion. So when the cloacal membrane rupture the
anterior abdominal wall,pubis,bladder and urethra
fail to develop normally
11. TYPES
There are two types
1.complete
2. incomlete:
the pubic bones are united and the external
genitalia normal or there is epispadias.
12. CLINICAL PRESENTATION
Anterior abdominal wall absent
Posterior bladder linning which is bright red color is
exposed
Urine seeps into the posterior bladder wall from
abnormal ureteral outlets
At time of birth the mucosa of bladder may be thin
and smooth but it is later become edemetous
13. ASSOCIATED ANOMALIES:
1. The rami of pubic bones are widely separated.
There is a thick fibrous band. The femurs are rotated
externally and the child waddles like a duck
(waddling gait).
2. The umbilicus is absent. There may be umbilical
hemia.
14. 3. Genitalia.—
(a) In the males :
(i) Epispadias is almost always present.
(ii) The penis is broader and shorter than normal. It is
drawn up and fixed to the abdominal wall.
(iii) The scrotum may be ill-developed.
(iv) The testes may be normal or ectopic or
maldescended.
(v) The prostate and seminal vesicles are often
rudimentary or absent.
15. (b) In the females :
(i) The clitoris is usually cleft.
(ii) The labia minora are separated.
(iii) There may be anomalies in the development of
uterus and vagina.
4. Anal sphincter is often lax.
5. Hernia :
(a) Umbilical hernia may be present.
(b) There may be inguinal hernia of one or both
sides
17. DIAGNOSIS
1 Diagnosis: can be confirmed before delivery via uss
and or MRI. Also clinically
2 Diffential diagnosis:
patent urachus
18. INVESSTIGATION
prenatal USS:
include a lower abdominal wall mass,
absent bladder filling,
low-set umbilicus,
small genitalia,
abnormal iliac crest widening.
Diagnosis can help planning of delivery in a centre
with facilities to perform early surgical correction.
19. MANEGEMENT
At birth:
cover the bladder with plastic fi lm and irrigate
regularly with sterile saline.
Surgical:
Newborn:
pelvic osteotomy (cutting bone to correct deformity)
with external fixation and closure of bladder,
abdominal wall, and posterior urethra.
20. 6–18 months: epispadias repair
4–5yrs: bladder neck reconstruction (Young– Dees–
Leadbetter procedure) and anti-reflux surgery
(ureteric re-implantation)is performed when there is
adequate bladder capacity and children can
participate in voiding protocols. Where bladder
capacity is too small, bladder augmentation and/or
urinary diversion is required
21. SURGICAL COMPLICATION
:increased risk of malignancy in urinary or
orthotopic bladder
fistula
hypospadias
bladder stones
infection (UTI, epididymitis)
incontinence