Bladder extrophy
Bladder exstrophy is a rare congenital birth defect where the bladder wall
is turned inside out, so that the ureteric orifices and the interior bladder
wall are exposed. The urethra and external genitalia are not completely
formed. The anal verge and the vagina in females appear to be displaced
forward, in addition to widely separated pubic bones . The first
mention of this abnormality was seen in an Assyrian tablet from 2000 BC
preserved in the British museum. It was first described by Grofenberg in
1597.
Bladder exstrophy was thought to occur as a result of an abnormal
over-development of the cloacal membrane, (Marshall and Muecke)
The abnormality occurs in a range of spectrum, from epispadias to
cloacal exstrophy The estimated incidence of bladder exstrophy is
1:30000-1:50000 live births with a 2:1 male/female ratio. There is no
clear evidence for genetic predisposition of this anomaly, however, it can
re-occur in the same family by a ratio of 1:100 and there is 500 times
greater chance of having bladder exstrophy in the offspring of individuals
having bladder exstrophy or epispadias than in the general population
Bladder exstrophy can be associated with other anomalies like
spinal defects, but in general they are confined to structures around the
open bladder and urethra as vesicoureteric reflux, inguinal hernias,
undescended testes, rectal prolapse, and foreshortened penis in males and
stenosed vagina with bifid clitoris in females. The upper urinary tract is
usually spared. The main problems associated with this abnormality are:
Skin irritation from continuous uncontrolled urine leakage and ascending
urinary infection. It is managed surgically in a single or multiple stages.
Staged Management of Bladder Exstrophy
These involved closure of the bladder and abdominal wall defects,
tightening of the bladder neck, and genitoplasty. The procedures can be
facilitated by pelvic osteotomy The overall results of the surgical
management are still beyond satisfaction with no definite solution and
urinary diversion in some cases are warranted
Stage 1
Bladder closure
stage 2
Repair of epispadius
stage 3
Bladder neck reconstruction