Ulcerative colitis is an inflammatory disease primarily involving the rectum and distai colon, although it usually affects the entire colon in children. The condition tends to be more severe during childhood; about 90% of children with this disease have moderate to severe inflammation, almost twice that observed in adults. Temporary remission with prednisone and intense medical therapy can be achieved in approximately 95% of cases, but at least 40% will eventually require proctocolectomy because of persistent symptoms, bleeding, or complications due to medical therapy. Removal of the diseased rectum and colon is curative, permits normal growth and development, and obviates the complications of chronic steroid administration. Children with severe chronic ulcerative colitis are rarely cured of the disorder without surgery.
Pediatricians and close relatives have often been reluctant to support the recommendation for proctocolectomy for children and young adults because of the lifetime commitment to an abdominal ileostomy, as well as the low but significant incidence of postoperative bladder dysfunction and/or possible impotence. During the past decade, new operative techniques have become available for treating patients with ulcerative colitis which obviate the need for continuous ileostomy appliance and minimize the risk of injury to the nerves surrounding the rectal muscles.
In 1969, Kock1 first described the use of a "continent ileostomy" with an internal ileal reservoir proximal to the stoma. In 1973, a nipple valve was constructed between the pouch and the stoma by intussuscepting the terminal ileum in a retrograde fashion into the pouch for a distance of 3 cm to 5 cm to improve continence. The pouch is emptied several times daily as necessary by inserting a soft Silastic catheter through the stoma. Several modifications of the procedure, including securing the nipple valve to the abdominal wall and using several rows of staplesto hold the nipple valve in place, have achieved continence in approximately 75% of cases.1 In the past decade, the Kock internal reservoir has gained wide popularity for adults with ulcerative colitis. 3 The advantage of wearing a small gauze patch over the stoma rather than a bulky collecting appliance is readily apparent.
Complications with the continent ileostomy have included nipple valve retraction (30%); enteritis of the reservoir (in excess of 50%); stoma too large, not level or too low (40%); prolonged hospital stay (over three weeks); reservoir fistulae; inability to empty the reservoir completely with the catheter, and others. Reporting on the Mayo Clinic experience with more than 325 operations, Dozois and associates2 indicated that approximately 70% are completely continent for stool and about 65% are completely continent for gas. Ninety-four percent do not wear an ileostomy appliance. Twenty-five percent of the patients required at least one re-operation, chiefly for nipple retraction. Although the Kock reservoir has been used for many children who have undergone proctocolectomy, the results have been less gratifying than in adults.
Inasmuch as ulcerative colitis is primarily a mucosal disease and removal of the entire rectum with its closely adherent nerves in the pelvis supplying the bladder and genitalia may cause bladder dysfunction and/ or impotence (all serious consequences in the young adult), attention has recently been directed toward removing the rectal mucosa and preserving the anal sphincter mechanism. The feasibility of removing the rectal mucosa without disturbing the rectal muscle was initially described by Ravitch and Sabiston in 1947. 4 A more extensive clinical application occurred with its use as a colonie pullthrough operation for Hirschsprung's disease,5 as well as high imperforate anus malformations. If the rectal mucosa is removed in patients with ulcerative colitis, the upper end of the rectum may be oversewn and the anal sphincter divided in two sites; a drain is then inserted into the rectal muscular cavity, enabling more rapid healing than if the rectum is completely excised.6 In our experience with 39 patients undergoing this procedure, the operative blood loss was considerably lower than with the standard proctectomy, the patients were ambulated earlier, and no bladder dysfunction or impotence developed. Two patients required removal of residual fragments of mucosa that caused mild bleeding and persistent drainage, indicating the importance of removing all segments of rectal mucosa. In patients with severe ulcérations of the rectal mucosa, which make it infeasible to remove the tissue completely, chemical debridement has proven to be a promising procedure in laboratory studies.7
Endorectal pullthrough of the ileum subsequent to colectomy and mucosal proctectomy with ileoanal anastomosis has been popularized by Martin and associates,8 Telander and Perrault,9 Ferrari and Fonkalsrud,10 Parks," and others, with varying degrees of success. While this procedure eliminates the need for an intestinal stoma on the abdomen and is likely to ensure continence, it results in frequent loose stools and a sense of fecal urgency. The neurogenic sensory and discriminatory mechanism remain essentially intact if the rectal mucosa is removed down to the dentate line, providing that the muscularis is not disturbed. It is therefore imperative that all rectal mucosa be removed in patients with ulcerative colitis; otherwise, inflammation will recur, delaying satisfactory healing of the ileoanal anastomosis with resultant fistulae, stricture, etc. The ileal pullthrough operation is contraindicated in patients with Crohn's disease since recurrent inflammation in the ileal pullthrough segment is likely, leading to serious consequences.
In order to facilitate removal of the rectal mucosa, the dissection is carried downward into the pelvis as low as feasible from the abdomen. The rectal muscle may be removed 2 cm to 3 cm below the peritoneal reflection without affecting continence. Because most patients with ulcerative colitis suffer moderate to severe anal sphincter spasm due to chronic diarrhea, gentle dilatation is required prior to complete removal of the mucosa. The lower 2 cm to 4 cm of rectal mucosa is removed from below, facilitated by a self-retaining retractor and injection of dilute epinephrine solution between the mucosa and the muscularis. A circumferential incision is made through the mid-dentate line in order to remove all rectal mucosa. Thorough hemostasis is mandatory in the rectal muscle canal before mobilizing the terminal ileum down to the anus. The area is also thoroughly irrigated with antibiotic solution. The ileoanal anastomosis is performed in one layer, encompassing full thickness of the terminal ileum and approximating it to the anal mucosa, taking bites of the underlying muscularis. In most cases, absorbable suture material is preferred. The space between the ileal pullthrough and the rectum should be drained for three to four days to minimize accumulation of blood. It is our practice to irrigate this space with dilute antibiotic solution three times daily through a Jackson-Pratt catheter which is brought through the anterior abdominal wall. The ileoanal anastomosis should be protected with a completely diverting cutaneous ileostomy for four to six months before allowing intestinal contents to pass through the anus.
Although preservation of an intact anorectai sphincter has made it possible to achieve continence, the absence of a reservoir for storage of fecal contents has resulted in frequent loose or watery stools, fecal urgency and perianal cutaneous excoriation. These problems can be reduced substantially by giving the patient lmodium (loperimide) and Metamucil (psyllium hydrophyllic mucilloid). Martin and associates,8 Telander and Perrault9 and others have reported that many patients without an ileal reservoir experience spontaneous dilatation of the JJeum proximal to the ileoanal anastomosis, eventually achieving a satisfactory clinical result. Such dilatation of the proximal ileum varies considerably among patients and may be minimal even after several months. To encourage the consistent development of an ileal reservoir, Telander et al'2 have recommended dilating the ileum proximal to the ileoanal anastomosis using a balloon catheter with repeated mechanical distension. A more recent report by Heppell and associates11 has indicated a clear linear relationship between the number of bowel movements per 24 hours and the distension of the terminal ileum proximal to the ileoanal anastomosis.
In order to provide a fecal reservoir at an early stage after the ileoanal anastomosis, Ferrari and Fonkalsrud10 and Parks" constructed an internal S-shaped ileal reservoir immediately proximal to the ileoanal anastomosis. Although this procedure has worked well in several patients, with minimal fecal urgency and defecation as infrequent as three to six times daily, often the reservoir distends so much that muscular contraction is insufficient to induce complete defecation. In others, the ileal outlet became kinked, causing partial obstruction. Parks" found it necessary in almost half of his patients to drain the reservoir by means of a rectal tube at least once daily, resembling the technique used for draining a Kock ileostomy reservoir. Furthermore, the large reservoir may cause upward traction on the ileal pullthrough segment, favoring anastomotic separation. Construction of the reservoir simultaneously with the ileal pullthrough may jeopardize the blood supply to the terminal ileum and retard healing of the ileoanal anastomosis.
Utsinomiya and associates'4 have utilized a "J -shaped reservoir" in more than 20 patients with familial polyposis who have undergone colectomy and mucosal proctectomy. While this procedure has the advantage of placing the reservoir at the level of the anus, there is the disadvantage of long suture lines in the rectal muscle canal which are prone to inflammation, particularly in patients with ulcerative colitis. These authors have reported a larger incidence of "cuff abscess" than has been observed in patients without a reservoir extending into the rectal canal. This procedure has provided a high degree of continence and low incidence of fecal urgency in patients who have not experienced complications.
A "lateral reservoir" with two isoperistaltic segments of ileum anastomosed along the antimesenteric border has been used successfully in our hospital in 19 patients during the past three years. ' Approximately four months after construction of the ileal pullthrough, the diverting ileostomy is taken down and the end oversewn and brought well into the pelvis below the level of the peritoneal reflection. The anastomosis is constructed over a 25 cm to 30 cm distance ( Figure I ). The segment of ileum without anastomosis extending to the anus is limited to only 5 cm to 7 cm. This reservoir has the advantage of two isoperistaltic segments propelling the intestinal contents to the anus. A temporary drainage catheter is placed into the reservoir to permit expulsion of gas and liquid content as the bacterial flora gradually approximates that of the normal colon. The use of Flagyl routinely during the first six weeks following the operation has minimized the inflammatory response in the reservoir. Patients are encouraged to expel fecal contents at least three to four times daily in order to avoid stasis. A mean of four continent bowel movements per 24 hours has been achieved within four weeks in patients with the lateral reservoirs. Almost all have returned to school or work within four weeks.
Figure 1. Endorectal ileal pullthrough with ileoanal anastomosis showing isoperistaltic lateral ileal reservoir. Mucosal proctectomy and total colectomy were performed prior to the ileal pullthrough construction.
The endorectal ileal pullthrough operations are technically difficult to perform and there is a "steep learning curve." Low morbidity can be achieved by anticipating complications. Reports from the several hospitals where more than 15 pullthrough operations have been performed indicate that no mortalities have occurred. The endorectal pullthrough operations are promising for future management of patients with ulcerative colitis who require proctocolectomy; however, many technical improvements must be effected before the operation can be widely recommended.
The continence-preserving operations available for children and adults with ulcerative colitis have provided an optimistic alternative to standard proctocolectomy. The entire rectum should rarely be removed in a patient with benign rectal disease.
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