Surgical treatment of hereditary adenomatosis of the colon and rectum in Sweden during the last 20 years. Part I. Introduction. Surgical procedures used. Pre-and postoperative measures. Arrangement of follow-up. Propositi group, primary and late results.

An investigation of the prevalence of hereditary adenomatosis of the colon and rectum (ACR) in Sweden was started in 1959. Up to the end of 1972, 97 families were traced and 80 of them were more fully investigated. In 56 of the latter families there were at least 2 affected members; in the other 24, only one. In all, 340 affected individuals were found, 150 of whom were operated upon with the aim of cure. Of the operated patients 82 had presented with symptoms, the propositi group, while 68 affected individuals were detected during the family investigations and underwent prophylactic operations, the call-up group. A comparison was made between the two groups of the primary and long-term results after operation. Major surgery with total proctocolectomy and ileostomy (PCI) or colectomy with ileorectal anastomosis (IRA) was performed in 135 cases, 67 propositi and 68 call-up patients; minor surgery with segmental resection (SR) in 15 propositi. The propositi group is presented in detail in this paper, the call-up group in a following paper. In the propositi group there were 3 surgical deaths (3.7%). At the time of operation 48 patients (58.5%), had already developed cancer. Up to the end of the period of observation 16 of them died from metastases, 2 of other malignancies, and 3 of nonmalignant disease.