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Case 13

Ureterosigmoidostomy complicated by sigmoid carcinoma

A young woman with haematuria

This 30 year old woman described a history of intermittent haematuria over the previous year. She had undergone surgery for a urinary disorder as an infant, but had been well since. Investigations undertaken for the haematuria a year earlier, including IVP and CT, apparently found no cause, but her symptoms persisted.

A repeat IVP was performed, and a significant change was noted from the previous "normal" examination. A 15 minute film is provided.

The IVP demonstrates a non functioning kidney on the right - no excretion was seen, even at 2 hours. This kidney had appeared normal previously.

Also of note is the abnormal "bladder" contour, and its elevation out of the pelvis. In fact the patient had no bladder, and the contrast was within the colon; the previous operation was a cystectomy and ureterosigmoidostomy.

Two images from the CT scan are shown, one at the level of the kidneys, one at the level of the lower ureters.

Findings

On the IVP film there is a faint nephrogram on the right, and the renal outline is normal. However there is no pyelogram - the right kidney is not functioning.

The bladder outline is abnormal, and the "bladder" is very high in the pelvis. This appearance is due to contrast collecting in the sigmoid colon. This appearance was similar on previous studies, and is consistent with a ureterosigmoidostomy.

The upper CT image shows a faint nephrogram on the right - the contrast enhancement is much less than on the left. The cause is the renal obstruction, as evidenced by the marked hydronephrosis.

The lower CT image demonstrates contrast opacified urine lying posteriorly in the rectosigmoid (star). The dilated unopacified lower right ureter is visible (short black arrow); compare with the normal opacified left ureter (thick white arrow). In between, is an abnormal soft tissue mass, which is intimately related to the sigmoid in the region of the ureteric insertions (thin white arrow). This obstructs the right ureter.

The second of the images shows the appearance one year earlier, at which time she had already complained of haematuria. In retrospect the same mass was probably present, albeit smaller at that time (black arrow).

Diagnosis

Cystectomy and ureterosigmoidostomy.
Adenocarcinoma of sigmoid colon.

Discussion

This patient developed a bladder sarcoma when aged 2 years. She was treated by cystectomy and the ureters were diverted into the sigmoid colon. This procedure had the advantage that continence of urine could be maintained by the anal sphincter, while the rectum and sigmoid colon developed the ability to distend and contain a large volume of urine.

The disadvantage was the high incidence of colonic carcinoma in the region of the anastomosis. This was attributed to the reabsorption of water by the colon and the high resultant concentration of toxins causing metaplasia in the colonic mucosa. The latent period is at least 15-30 years. The presence of faeces has been considered important in developing this complication, but this is not always so; cases have been reported in isolated colonic loops also. For this reason the operation is no longer popular, and an ileal conduit is more commonly used.

This is precisely what happened in this case. At surgery the adenocarcinoma was confirmed. There was microscopic spread to local nodes, which were excised. A new, continent, ileal pouch was created.

Rhabdomyosarcoma of the bladder

Rhabdomyosarcoma is the commonest soft tissue tumour in childhood. The lower genitourinary tract is a common site (The head, neck and orbit are the commonest). It may present with a pelvic mass, urinary frequency, dysuria and strangury (painful inability to void).

Modern treatment is a combination of surgery and chemotherapy.

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