Total Colectomy and Mucosal Proctectomy with Preservation of Continence in Ulcerative Colitis LESTER W. MARTIN, M.D., CLAUDE LECOULTRE, M.D., WILLIAM K. SCHUBERT, M.D.

Since ulcerative colitis is a mucosal disease, it would appear possible to remove the diseased rectal mucosa and preserve all anorectal musculature. When performed in conjunction with total colectomy, the terminal ileum could then be placed inside the retained muscular wall of the rectum and anastomosed to the anus. This would remove all of the disease and yet preserve anorectal continence. Seventeen patients with chronic ulcerative colitis have undergone this operation with satisfactory results in 15 and no deaths. Many details of preoperative, operative and postoperative management are presented which are imperative for a successful result. Sufficient experience has been gained that the operation can now be recommended.

Wl rHEN SURGERY IS REQUIRED for the treatment of chronic idiopathic ulcerative colitis, the usual operation is total colectomy with construction of a permanent ileostomy. The diseased tissues are removed and the prognosis for life expectancy is good. Nevertheless, some patients, particularly teenagers and young adults, are reluctant to accept a permanent ileostomy. They fear that it will cause embarrassment in relation to courtship, marriage, participation in sports and other activities. Consequently, surgery is often deferred until the risk is prohibitive because of the advanced stage of the disease and poor general condition of the patient. If it is possible to retain rectal control, this would be preferable to an abdominal ileostomy. Since ulcerative colitis is primarily a mucosal disease, it would appear possible to remove the diseased rectal mucosa and preserve all the anorectal musculature. When performed in conjunction with total colectomy, the terminal ileum could be placed inside the retained muscular wall of the rectum anastomosed to the anal canal. This would remove all the disease and yet preserve anorectal continence. This philosophy was the basis for the operative technique described by Ravitch and Sabiston3 in 1947. The technique was applied to at least one patient with ulcerative colitis. Soave,5 in 1963, reported a similar 'k 7

From the Pediatric Surgery and the Pediatric Gastroenterology Services of The Children's Hospital and the Departments of Surgery and Pediatrics, The College of Medicine, The University of Cincinnati, Cincinnati, Ohio

operation for the management of Hirschsprung's disease. He indicated that the technique had been employed in Brazil by Simonson, Mendonca and Raia in the management of megacolon of the acquired variety due to Chagas disease. Pomerantz and Sabiston,2 in 1968, reported satisfactory results with one patient with ulcerative colitis employing this technique. Other reports1'4 have likewise been encouraging and the operation has subsequently become popular for Hirschsprungs disease. More recent advances in the areas of broad spectrum antibiotics, steroid therapy and total parenteral nutrition lend additional support to minimize the risk of major surgical undertakings. During the past ten years, we have attempted to preserve rectal continence in 17 patients undergoing total colectomy for chronic ulcerative colitis. A method of management has evolved which would appear to be successful and can be recommended for selected patients.

Presented at the Annual Meeting ofthe American Surgical Association. Boca Raton, Florida, March 23-25, 1977.

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Clinical Material Seventeen patients, 11 to 20 years of age, with symptoms for three months to 12 years are included and represent all patients coming to surgery at the Children's Hospital over the past ten years with a diagnosis of chronic ulcerative colitis. All had been evaluated by the Pediatric Gastroenterology Service because of unsatisfactory response to medical management including dietary restriction, systemic steroids, steroid enemas, Azulfidine (sulfasalazine, Pharmacia Laboratories, Inc.), Lomotil (diphenoxylate hydrochloride with atropine sulfate, Searle and Co.) and other supportive measures. The diagnosis had been established by radiographic examination with barium enema, sigmoidoscopy and biopsy with histologic examination. All had symptoms of bloody diarrhea

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MARTIN, LE COULTRE AND SCHUBERT TABLE 1. Complications

Pelvic infection Cuff abscess Wound infection Stricture Bleeding Bowel obstruction

3 3 5 3 I 3

and as much as 30 pounds of weight loss. Growth retardation was attributed to a combination of the effects of the disease and the steroid therapy. In each instance, the objective of the operative approach was to perform a total colectomy and yet preserve anorectal continence.

Operation It is essential that the rectal mucosa be free of gross disease at the time of operation. This may be achieved by intensive medical management, total parenteral alimentation, steroid therapy, establishment of a preliminary diverting ileostomy with subtotal colectomy, or a combination of these measures. Often, four to six weeks of preoperative total parenteral alimentation is required to adequately prepare the colon. The first step of the operation is a sigmoidoscopic examination. In the event of active gross disease, a mucosal protectomy should not be attempted. If the mucosa is free of gross disease, the rectum is thoroughly irrigated with physiologic saline solution. If a subtotal colectomy has not been previously performed, a large rectal tube is inserted to aid in further irrigation after the abdomen is open. The patient is then placed supine with the legs flat on the operating table. If the patient has an ileostomy, it is closed, the instruments discarded and the abdomen again prepared and draped. The abdomen is opened through a long left paramedian incision. A constricting tape is placed snugly about the sigmoid colon and the rectosigmoid thoroughly irrigated through the rectal tube by an assistant. The abdominal colon is mobilized in routine manner for resection. The peritoneal floor about the rectum is opened. A circumferential incision is made through the muscular wall of the colon just below the peritoneal reflection. Dissection is then continued downward separating the muscular wall of the rectum from the mucosa and the submucosa. Many small vessels will be encountered and can be fulgurated before they are divided or disrupted. This portion of the operation requires two to three hours of careful dissection. It is important not to perforate the mucosa. The resulting mucosal cylinder can be dissected free all the way to the anus. A second ligature is placed about the sigmoid colon distal to the

Ann. Surg. a October 1977

previous one and the colon transected between the two. The terminal ileum is next divided between anastomotic clamps and the colon passed from the operative field. The terminal ileum is closed with interrupted sutures taking care to avoid contamination. The mesentery ofthe terminal ileum is then incised to afford sufficient length for the ileum to reach the anus, and yet care must be taken to preserve an adequate blood supply. The surgeon then proceeds to the perineal portion of the operation with an assistant remaining at the abdominal field. The legs of the patient are elevated to a lithotomy position, the anus gently dilated and the rectal mucosa, previously mobilized from above, is everted and delivered outside the anus. The entire perineal field is then prepared and draped. The rectal mucosa is transected with electrocautery one centimeter above the mucocutaneous junction and the terminal ileum brought down through the rectal canal which has been preserved intact but denuded of mucosa. A single Penrose drain is placed between the wall of the ileum and the muscular cuff of rectum. The end of the terminal ileum is anastomosed to the anorectal mucosa completing the perineal portion ofthe operation. The patient's legs are returned to the operating table and the surgeon changes gown and gloves and returns to the abdominal portion of the operation. A diverting ileostomy is established through a separate opening in the right abdomen but not opened until the working incision has been closed and sealed. We prefer the Turnbull diverting loop ileostomy and apply an adherent ileostomy bag before the patient leaves the operating room. Following operation, the systemic steroid dosage is gradually decreased, then discontinued. The ileostomy is closed three to six months later following complete healing of the rectal anastomosis. Results The results in general have been encouraging with no deaths and only two failures. It is now ten years since our first patient's operation. He has two to three stools daily, has complete control, and is a full-

time college student. All patients have frequent, watery stools for several weeks following ileostomy closure and stools at nighttime are not an uncommon occurrence. Immediately following operation, medication designed to decrease intestinal peristalsis has been of value. As long as six or 12 months have been required for adaptation, after which patients have complete control of two to eight semiformed stools daily. All 17 patients recovered and are currently free of

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disease. The two failures were both due to pelvic sepsis. Both have permanent ileostomies. Two patients have undergone the definitive operation but their proximal ileostomy has not yet been closed. The other 13 all have complete bowel control. Four still have frequent watery stools but their ileostomy closure was recent and their course is satisfactory for this stage. Seven patients recovered with no complications but the other ten, including the two failures, developed a total of 18 significant complications (Table 1). Early in our experience, we performed the operation in the presence of active gross disease and without proximal diversion in three patients. All three developed serious pelvic infection. The initial operation in four patients was subtotal colectomy with ileostomy. The definitive operation was performed at a later date with excellent results in three. In the other, the operation was performed in the presence of persistent rectal disease, and was followed by infection. A so-called "cuff abscess" developed in three patients. This is an abscess forming inside the rectal cuff between it and the wall of the ileum and in the absence of an anastomotic leak. All three subsequently developed a stricture which required release or dilatation under anesthesia. The cuff abscess has not been encountered since drainage of the cuff area was included as a part of the operative routine. Wound infections occurred in five patients. All were in patients in whom perforation of the rectal mucosa occurred during the operation. Four were prior to adoption of thorough rectal irrigation just prior to the operation. Bleeding from the superior hemorrhoidal artery in one patient four weeks following operation, necessitated hospitalization and laparotomy for its control. This patient is still awaiting ileostomy closure. Small bowel obstruction due to adhesions occurred as a late complication in three patients and necessitated laparotomy.

systemic and rectal steroids, or by a combination of these methods. Inflamed, ulcerated mucosa is difficult to dissect from the muscle layer of the rectal wall, perforation is more likely and the resulting complications can be devastating. It is of utmost importance that the rectum be thoroughly irrigated and be completely free of fecal contamination at the time of operation. Otherwise, a small perforation of the rectal mucosa during the course of the dissection will lead to destructive postoperative pelvic infection. Drainage of the space between the rectal muscular cuff and the wall of the ileum within it is important to eliminate collection of serum in the space, subsequent "cuff abscess" and eventual stenosis. The establishment of a proximal diverting ileostomy until rectal healing has occurred represents a basic surgical principle which must be strictly observed in this group of poor-risk patients. Both of our failures were in patients who did not have a protective diverting ileostomy. Strict observance of aseptic surgical technique with complete avoidance of contamination is essential. The patients have lost weight; their resistance is poor; their immune response is further depressed by long-term steroid therapy, and they are particularly vulnerable to infection. Antibiotics must be employed liberally and any blood lost during the operation must be accurately replaced.

Conclusions

References

Our experience with 17 patients with ulcerative colitis demonstrates that it is possible, at least in selected patients, to perform a total colectomy and yet retain rectal continence. The procedure is, however, subject to multiple complications unless certain precautions are rigidly observed. The most significant of all precautions is that gross rectal disease as visualized by proctoscopic examination must be completely cleared prior to operation. This may be accomplished by total parenteral alimentation, by subtotal colectomy and ileostomy, by use of

1. Ekesparre, W.: Wietere Erfahrungen mit der Chirurgischen Behandlung der Colitis Ulcerosa. Z. Kinderchir., 5:84, 1968. 2. Pomerantz, M. and Sabiston, D.C.: Modified Operation for the Treatment of Hirschsprung's Disease. Am. J. Surg., 115:198,

Summary Our experience with 17 patients with chronic ulcerative colitis demonstrates that it is possible to perform a total colectomy with mucosal proctectomy and preserve anorectal continence. Acknowledgments The authors wish to thank William R. Richardson, M.D. and Jens G. Rosenkrantz, M.D. for permission to include one patient of each of them in this series.

1968. 3. Ravitch, M. M. and Sabiston, D. C.: Anal Iliostomy with Preservation of the Sphincter. Surg. Gynecol. Obstet., 84: 1095, 1947. 4. Ravitch, M. M. and Handelsman, J. C.: One Stage Resection of entire Colon and Rectum for Ulcerative Colitis and Polypoid Adenomatosis. Bull. Johns Hopkins Hosp., 88: 59, 1951. 5. Soave, F.: A New Surgical Technique for the Treatment of Hirschsprung's Disease. Surgery, 56:1007, 1964.

Total colectomy and mucosal proctectomy with preservation of continence in ulcerative colitis.

Total Colectomy and Mucosal Proctectomy with Preservation of Continence in Ulcerative Colitis LESTER W. MARTIN, M.D., CLAUDE LECOULTRE, M.D., WILLIAM...
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