Is Ileoanal the Proper Operation for Indeterminate Colitis: The Case Against

Ulcerative colitis is now commonly definitively treated surgically by ileoanal anastomosis rather than by proctocolectomy with permanent external ileostomy. It has generally been thought that confirmed cases of Crohn's disease should not be treated with ileal reservoir because of the unacceptably high risk of recurrence and possible loss of substantial bowel length in the case of pouch failure caused by recurrence. Debate continues regarding the advisability of ileal pouch anal anastomosis (IPAA) for indeterminate colitis, with some advocating aggressive IPAA construction with indeterminate colitis (IC) and others discouraging consideration of restorative proctocolectomy because of the possibility of subsequent Crohn's in patients with IC. The term “colitis indeterminate” was introduced in 1978 by Price (1), referring to an inability to differentiate Crohn's disease from ulcerative colitis with strictly pathologic criteria. It was estimated that the incidence of IC was 10% to 20%. Price made the point that differentiation was particularly difficult when the colectomy was performed under urgent circumstances because of the depth of the inflammatory infiltrate within the wall of the colon in these specimens. He stressed the need to examine mucosal biopsy specimens from the same patients when their colitis had been quiescent to help refine the diagnosis. In a subsequent study from the same institution, Wells et al. (2) from St. Mark's Hospital examined the case records of 46 patients with IC and combined the pathology with the preoperative clinical information and radiological reports. On that basis, 19 patients were reclassified to probable Crohn's disease, 11 to probable ulcerative colitis, and 16 remained as having IC. Crohn's disease did not develop over a median of 10 years of followup in those patients who continued to be classified as having IC after extensive investigation. This article is an important reminder that the diagnosis of IC is a clinicopathologic rather than a strictly histologic exercise and requires using all clinical and pathologic resources to arrive at the most appropriate diagnosis. It is generally acknowledged that Crohn's disease recurs after resection and anastomosis. Regardless of whether the anastomosis is small bowel-to-small bowel, small bowel-to-colon or colon-to-colon, the reported recurrence rates over time vary between 66% and 80%. Conversely, if there is not an anastomosis, the recurrence rates are substantially less. Before the popularity of IPAA, idiopathic IBD of the colon was treated with proctocolectomy. All cases of ulcerative colitis were cured and approximately 80% of cases of Crohn's disease did not recur. As a result, the accuracy of the preoperative diagnosis would have to be overestimated, because a percentage of those with ulcerative colitis would have had Crohn's disease but would not have experienced recurrence, which would have led to the discovery of the incorrect diagnosis. When the ileal-to-anal anastomosis of the IPAA was made, data began to appear that indicated that the diagnosis was not always correct. This was evidenced by the development of Crohn's disease in the ileal pouch in patients who initially underwent operation for ulcerative colitis. The experience with IC at the Lahey Clinic began with the observation of a higher incidence of late perineal complications such as abscess, fistula, and ulcer in patients with indeterminate colitis and IPAA (3). Also, chronic pouchitis was observed more commonly in patients with a preoperative diagnosis of IC than in those with ulcerative colitis (4). Further followup of the same cohort of patients in our institution revealed that pouch failure was more common in patients with fistulas and Crohn's disease (5). In a study of 543 patients undergoing IPAA for inflammatory bowel disease, IC was the initial diagnosis in 42 and Crohn's disease was the initial diagnosis in only two. With prospective followup, based on observed clinical course and subsequent pathologic analyses when available, there was a tendency to change the pathologic diagnosis from ulcerative colitis to IC to Crohn's as late complications developed necessitating rediversion and pouch excision (6). These observations have recently been confirmed and extended by the Mayo Clinic (7), demonstrating significantly more pelvic sepsis, pouch fistula, and pouch failure in the IC patients compared with ulcerative colitis. The evolution of pathologic diagnosis was also confirmed in that a significantly higher percentage of patients with IC ultimately proved to have Crohn's disease. It is important to interpret these data considering that this experienced group of surgeons, gastroenterologists, and pathologists will have exercised due diligence in attempting to resolve the exact nature of the colitis before proceeding to IPAA. The diagnosis of IC in such institutions represents a pure group of patients whose colitis is truly indeterminate. The critical question is whether it is advisable to recommend IPAA in this group of patients with a diagnosis of IC. The fact that those patients who do not prove to have Crohn's disease have a similar outcome as patients with ulcerative colitis does not justify the routine performance of a series of operations with a 33% to 50% failure rate. Patients must understand the risks involved, the patterns of failure if the diagnosis is Crohn's disease, and the consequences of the loss of 50 cm of small bowel if the pouch must be removed. The alternative of proctocolectomy must be presented honestly as a functional alternative that offers significant advantages in these circumstances.

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