Half century of followup after ureterosigmoidostomy performed in early childhood.

PURPOSE

We studied clinical outcomes, especially regarding colorectal adenocarcinoma, in patients who underwent ureterosigmoidostomy in early childhood between 1944 and 1961.

MATERIALS AND METHODS

A total of 25 consecutive patients underwent ureterosigmoidostomy at a mean age of 3.1 years. The most common indication for ureterosigmoidostomy was bladder exstrophy-epispadias complex. The study period ended in 2010. Patient files were retrospectively evaluated, personal telephone interviews were performed and colorectal histology was reevaluated. One girl who died 4 days postoperatively was excluded.

RESULTS

Of the 24 patients 17 were alive in 2010 with a mean age of 59 years (range 48 to 67), and 2 still had a functioning ureterosigmoidostomy. A total of 20 patients with a mean age of 33 years had undergone re-diversion at a mean of 30 years postoperatively. Invasive colorectal adenocarcinoma developed in 7 patients and colorectal adenocarcinoma in situ in 1. Five patients died due to generalized colorectal adenocarcinoma. Mean time from ureterosigmoidostomy to diagnosis of invasive colorectal adenocarcinoma was 38 years (range 23 to 55). Three cases were diagnosed at 1, 21 and 25 years after re-diversion. One patient with colorectal adenocarcinoma in situ was 22 years old at polyp resection, which was 20 years after re-diversion. A carcinoid tumor developed in 1 patient. Of the 7 cases of invasive colorectal adenocarcinoma 6 were low differentiated.

CONCLUSIONS

After a half century of followup in 25 individuals undergoing ureterosigmoidostomy during childhood 17 were still alive and 20 had undergone re-diversion. Compared to the general Swedish population, the risk of colorectal adenocarcinoma was increased 42 times and the incidence of low differentiation was extremely high.