Frank L. Weakley

Desmoid Tumors in Familial Polyposis Coli

Desmoid tumors are locally invasive, nonmctastasizing fibrous tumors most frequently seen in patients with familial polyposis coli (FPC). Of 325 patients with FPC treated at the Cleveland Clinic, 29 (8.9%) were found to have a total of 36 desmoid tumors. These tumors occur in young patients (mean age: 29.8 years), particularly women (ratio 3:1), and most appeared after previous colectomy (86%). The majority (72% of all desmoids, 90% of patients) were located within the abdomen, specifically within the mesentery of the small intestine. In most cases, attempts at surgical resection were followed by recurrence, and other previous treatments were similarly ineffective. Six of the 29 patients (21%) died from the desmoid and three died from other causes. The recent use of sulindac (Clinoril

Radiation injury of the rectum: evaluation of surgical treatment.

) has produced some early encouraging results in four patients with these tumors that have proven so difficult to treat in the past.

The long-term outcome in Crohn's disease.

Internal fistulas in diverticular disease are uncommon and have a reputation of being difficult to treat. Eighty four patients treated from 1960 to April 1986, representing 20.4 percent (84 of 412) of the surgically treated diverticular disease patients, were reviewed. Eight patients had multiple fistulas. Sixty-five percent (60 of 92) of fistulas were colovesical, 25 percent (23 of 92) colovaginal, 6.5 percent (6 of 92) coloenteric, and 3 percent (3 of 92) colouterine fistulas. There were 66 percent (35 of 53) males and 34 percent (18 of 53) females with colovesical fistulas only. Hysterectomies had been performed in 50 percent (12 of 24) and 83 percent (19 of 23) of females with colovesical and colovaginal fistulas, respectively. Operative management included: resection anastomosis, resection with anastomosis and diversion, Hartmann procedure, and three-stage procedure. In the latter half of the series there was a significant decrease in stating procedures with no significant statistical difference in complications. There were three deaths (3.5 percent) in the series. Other complications included: wound infection, 21 percent (18 of 84), enterocutaneous fistula, 1 percent (4 of 84), and anastomotic dehiscence, 5 percent (4 of 84). Primary anastomosis can be performed with acceptable morbidity and mortality and today is the procedure of choice, leaving staging procedures to selected patients.

The risk of cancer following colectomy and ileorectal anastomosis for extensive mucosal ulcerative colitis.

One hundred four patients, 80 women and 24 men, with radiation injury of the rectum following treatment for gynecologic and urologic malignancy were studied. In 50 patients, the rectal injury was treated surgically; 54 patients were treated conservatively. The age and sex distributions were the same in each group. In 63 patients, symptoms developed one month to one year after radiotherapy. The longest latent interval was 17 years. Of the 50 surgical patients, 23 had associated small bowel injury. The indications for surgery for the rectal injury were 1) proctitis unresponsive to conservative measures in 14 patients, 2) rectal stricture or fistula or both in 32, and 3) rectosigmoid perforation in four. Forty-one patients had external diversions. Eleven had intestinal continuity restored; six of the 11 had required the stoma for proctitis unresponsive to medical measures. Nineteen patients did not undergo colostomy closure, although symptoms wer greatly improved. Diversion alone was insufficient treatment in the remaining 11 patients. Twenty-six patients died. The 12 deaths in the surgical group comprised four due to residual malignancy, four from postoperative complications, and four from unrelated causes. Of the 14 deaths in the nonsurgical group, 11 died of the primary malignancy and three of unrelated causes. Diversion is considered the safest form of treatment for rectovaginal fistulae, rectal strictures, and proctitis unresponsive to medical measures. Intestinal resection resulted in sharp rise in the morbidity and mortality rates.

The Ripstein procedure: A 16-year experience

The long-term outcome of Crohns disease was reviewed in 139 patients who were treated at the Cleveland Clinic for a minimum of 15 years. At the time of diagnosis, 38 (27 percent), 39 (28 percent) and 62 (43 percent) patients had small-bowel, large-bowel, and ileocolic patterns of disease, respectively. The disease progressed with time and, eventually, 104 (75 percent) patients had ileocolic disease. One hundred twenty-two patients (88 percent) underwent at least one definitive operation for the disease. Forty-four (32 percent) patients had proctocolectomies and 65 (47 percent) have ileostomies. Associated manifestations of Crohns disease occurred in a high proportion of patients; perianal disease in 78 (56 percent), intestinal fistulas in 45 (32 percent), extraintestinal disease in 49 (35 percent). Six patients died of causes directly related to the disease. Specific complications tend to occur at definite times in the course of the disease. Crohns disease is not a benign condition. There is a relentless progression of the disease and a high incidence of complications when patients are followed over a long period.

The outcome of loop ileostomy closure in 293 cases

A retrospective study was performed on 89 patients who underwent total colectomy and ileorectal anastomosis for extensive mucosal ulcerative colitis between the years 1957 and 1977 in order to determine the risk of developing cancer of the rectum. The 30-day operative mortality rate was 0%. Of the 84 patients available for follow-up study, four patients, (4.8%) developed a carcinoma of the rectum. The risk of cancer per patient-year was zero in the first decade, 1/206 in the second decade, and 1/116 in the third decade. The cumulative risk of developing cancer was 0% at 10 years, 2.1% +/- 2.1% at 15 years, 5.0% +/- 3.5% at 20 years, and 12.9% +/- 8.3% after 25 years of disease. Patients with cancer or precancer in the colon at the time of colectomy appear to be at high risk for the later development of rectal cancer.

Complications and quality of life after ileorectal anastomosis for ulcerative colitis

A retrospective study was undertaken, evaluating 54 patients who underwent 57 Ripstein procedures at the Cleveland Clinic Foundation during the years 1963–1978. The patients were evaluated for their preoperative characteristics as well as early and late postoperative results. Patients who experience procidentia are generally middle-aged women who have had significant disorders of bowel function. Although the operative mortality was zero, and the majority of patients were satisfied with the results of their Ripstein procedure, there was significant operative morbidity (26 per cent). Twelve and one-half per cent of patients had recurrent rectal prolapse, and 18 per cent of patients had significant long-term obstructive symptoms. The Ripstein procedure still remains the treatment of choice for rectal prolapse.

Proximal recurrence and the fate of the rectum following excisional surgery for Crohn's disease of the large bowel.

Our experience with closure of loop ileostomies between the years 1975–1986 was reviewed. Ninety-three percent of stoma closures were done by simple transverse suture. The overall complication rate was 17%. Of the early postoperative complications (13%), the major complication was small bowel obstruction especially in patients where the stoma was protecting a pelvic ileal reservoir. Abdominal septic complications (postclosure) were rare (1%). These were generally caused by unrecognized enteric tears during the mobilization of the stoma rather than anastomotic leakage. A careful operative technique is required. The wound infection rate after healing by both secondary intention and primary skin closure was low (3%) and mainly superficial. Only one incisional hernia was observed in the late postoperative period. In three patients a posterior rectus sheath defect at the stoma site was found incidentally at laparotomy, without clinical evidence of an incisional hernia. Closure of a loop ileostomy is a safe operation with a low morbidity. In patients with a previous total colectomy there was a significant risk of small bowel obstruction after ileostomy closure.

Survival with carcinoma arising in mucosal ulcerative colitis.

Ileorectal anastomosis is a safe operation with low mortality and morbidity and offers a good prospect for success in many patients with ulcerative colitis. The functional results are good in the majority of patients, and there is a high level of patient acceptance. There is a low cancer risk with regular surveillance, but there is a relative contraindication for ileorectal anastomosis in patients with colon cancer or dysplasia present at the time of colectomy. For many patients, especially children and adolescents in their formative years and for young adults, it avoids or delays an ileostomy and avoids the risk of postoperative sexual dysfunction, while at the same time still permits the elective use of some other continence-preserving operation at a later date.