Ectopia vesicae
(syn. exstrophy of the bladder)
This is thought to be caused by the incomplete
development of the infra-umbilical part of the anterior abdominal wall,
associated with incomplete development of the anterior wall of the bladder owing
to delayed rupture of the cloacal membrane.
Clinical features of ectopia vesicae
•
One in 50 000 births (four male: one female)
•
Characteristic appearance (Fig. 65.2) because of the pressure of the
viscera behind it
•
Edges of abdominal wall can be felt
•
Umbilicus is absent
In the male the completely epispadiac penis is broader and shorter than normal, and
bilateral inguinal herniae may be present; the prostate and seminal vesicles are
rudimentary, whereas the testes are normal and have usually descended.
In the female the clitoris is bifid and the labia minora are separated anteriorly,
exposing the vaginal orifice. In both sexes, there is separation of the pubic
bones (Fig. 65.3),
which are connected
by a strong ligament. This bony defect causes no disability and subsequent
delivery is normal. The linea alba is also broad. In the rare, incomplete form
of penile epispadias or female epispadias, the pubes are united and the external
genitalia are almost normal, although in the female the clitoris is bifid (Fig. 65.4).
Treatment
Iliac osteotomy,
closure of the bladder and closure of abdominal wall
In the first year of life, the bladder is
closed following osteotomy of both iliac bones just lateral to the sacroiliac
joints. Later reconstruction of the bladder neck and sphincters is required. In
some patients, the reconstructed bladder remains small and requires
augmentation.
Another
option is urinary diversion which may be necessary if continence is poor
following bladder reconstruction. This can be done by means of a ureterosigmoid
anastomosis or the formation of an ileal conduit, colonic conduit or continent