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 urinary diversion. Long-term complications are frequent after ureterosigmoidostomy. These include: (1) stricture at the site of anastomosis with bilateral hydronephrosis and infection; (2) hyperchloraemic acidosis; and (3) there is an increased risk (20-fold) of tumour formation (adenomas aid adeno­carcinoma) at the site of the ureterocolic anastomosis.