Symposium on Surgery at the Lahey Clinic

Ulcerative Colitis or Crohn's Colitis Is Differentiation Necessary?

Malcolm C. Veidenheimer, M.D.,* F. Warren Nugent, M.D.,t and Rodger C. Haggitt, M.D:+

More than 100 years have passed since colitis was first described in the English literature in 1875.14 Inflammatory bowel disease was studied and treated for more than 50 years before observers realized that more than one type of "colitis" might exist. In 1930, Bargen.and Weber2 described a type of colitis that seemed to affect different segments of the large bowel at varying times. Crohn and Berg,3 eight years later, reported their experience with right-sided colitis. Wells 13 of Liverpool, in 1952, drew attention to a new type of colitis, and, in 1954, Neuman and Dockerty9 of the Mayo Clinic described segmental colitis and granulomas in 25 patients. The realization that Crohn's disease could affect the colon resulted from the work of Lockhart-Mummery and Morson. s These authors described the distinctions between Crohn's colitis and nonspecific ulcerative colitis. The impact oftheirreport was tremendous, and since 1960 an increasing understanding of these two diseases- has been offered in medical, surgical, and pathologic literature. With this presentation it is our intent to outline some of the differences between chronic ulcerative colitis and Crohn's colitis. Some important differences are apparent between these two diseases, especially with regard to the risk of development of cancer of the colon, the likelihood of associated anal disease, and the potential for the development of toxic megacolon. The similarities in the two diseases in regard to outlook after proctocolectomy and ileostomy need to be emphasized, and we believe that our experience as presented here will demonstrate this and other similarities.

*Section of Colon and Rectal Surgery, Lahey Clinic Foundation, Boston, Massachusetts tDepartment of Gastroenterology, Lahey Clinic Foundation, Boston, Massachusetts !Laboratory of Pathology, New England Deaconess Hospital, Boston, Massachusetts Surgical Clinics of North America-Vol. 56, No.3, JUne 1976

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WARREN NUGENT, AND RODGER

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HAGGITT

DIFFERENTIATION BETWEEN CHRONIC ULCERATIVE COLITIS AND CROHN'S COLITIS The final differentiation of chronic ulcerative colitis from Crohn's colitis is usually based on pathologic criteria. Clinical differences also exist between these two diseases which permit an accurate clinical diagnosis in the majority of patients.

Pathologic Features The major pathologic features of the two diseases are summarized in Table 1. We now know that crypt abscesses, formerly considered a classic pathologic feature of chronic ulcerative colitis, occur with equal frequency in patients having Crohn's colitis. The findings of a sarcoid-like granuloma in a patient with colitis is pathognomonic of Crohn's disease. Random sampling of the colon removed for Crohn's colitis will reveal granulomas in about 60 per cent of cases. Transmural inflammation results in thickening of the bowel wallin Crohn's colitis, whereas thickening of the bowel wall is rarely seen in chronic ulcerative colitis.

Clinical Features The very nature of the pathologic change has resulted in clinical differences between Crohn's disease of the colon and chronic ulcerative colitis which are usually readily recognized. Chronic ulcerative colitis causes extensive mucosal destruction associated with marked hyperemia of the mucosal and submucosal layers of the bowel wall, and, when active, it always results in bloody stools. Crohn's colitis has much less mucosal destruction. Although bleeding may occur, bloody stools are not a constant clinical feature-absent in 25 per cent and occasional or intermittent in the majority of patients. The deep fissures of the bowel wall that occur in Crohn's colitis may penetrate into the pericolic tissues with consequent sinus tract and fistula formation; these changes are never seen in chronic ulcerative colitis. Anal sepsis may be found in any patient with chronic diarrhea; however, in Crohn's colitis, characteristic changes including granulomas occur in the anal skin and mucosa. These changes predispose to induration and Table 1. Relative Frequency of Pathologic Findings PATHOLOGIC CHANGE

Gross thickening of wall Inflammation and ulceration restricted to mucosa and submucosa Transmural inflammation and fibrosis Ulceration into muscularis propria (including fissures) Granulomas Crypt abscesses Carcinomas or atypical epithelium

ULCERATIVE

CROHN'S

COLITIS

COLITIS

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ULCERATIVE COLITIS OR CROHN'S COLITIS

anal sepsis with fissures, abscesses, and fistulas as well as to hypertrophied anal papillas and thickened skin tags. In chronic ulcerative colitis, the rectum is always involved by the inflammatory process and, when active, the diagnosis can always be made at the time of proctosigmoidoscopy. Conversely, in Crohn's disease, the rectum may be normal (40 per cent) or may be involved. When the rectum is involved, its appearance may be similar to that in ulcerative colitis or the changes may be spotty. These changes may be present regardless of the presence or absence of anal disease. Rectal biopsy may prove to be a useful means of differentiating the two diseases. Table 2 summarizes the differences between clinical features of chronic ulcerative colitis and Crohn's colitis. In about 10 per cent of patients with colitis, clinical and pathologic features do not permit a definitive diagnosis of either disease; these patients are classified as having indeterminate colitis. Carcinoma In 1928, Bargen1 first noted the increased risk of malignancy associated with chronic ulcerative colitis. The relationship between chronic ulcerative colitis and cancer of the colon has since been well documented. 6 Although carcinoma of the colon has been reported to develop in patients with Crohn's colitis,4.10. 12 the risk is not nearly so great as with ulcerative colitis. We have not seen any instance of carcinoma of the colon associated with Crohn's disease at the Lahey Clinic. Recurrent Disease We reviewed records of 194 patients having had colectomy between the years 1957 and 1963 and classified these patients using clinical and histologic criteria (Table 3). All patients who had a diagnosis of chronic ulcerative colitis had total proctocolectomy with ileostomy. Of the 76 patients with a diagnosis of Crohn's colitis, 48 had total proctocolectomy with ileostomy as the initial surgical procedure, and 28 patients had some type of colonic resection associated with anastomosis. Ten patients in this latter group required eventual total proctocolectomy with ileostomy. Four others in the group having anastomosis required further resection with anastomosis, and disease recurred in four additional patients after anastomosis which has been successfully controlled by medical means (Table 4). Thus, Crohn's disease recurs in 64 per cent of patients with Crohn's colitis having resection with anastomosis. Table 2. Relative Frequency of Clinical Findings ULCERATIVE

CROHN'S

FINDINGS

COLITIS

COLITIS

Anal disease Rectal involvement Bleeding Fistulas Segmental involvement

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Table 3. Classification ofColitis-194 Patients

Ulcerative colitis Crohn's colitis Indetenninate colitis

NUMBER

PER CENT

99

51.3 39.2 9.8

76 19

A total of 58 patients required total proctocolectomy with ileostomy for the management of Crohn's colitis (48 .as an initial procedure and 10 after failure of an anastomosis). Of these patients, 53 have had follow-up studies for an average of 13 years. Three of these patients have recurrent Crohn's disease in the small intestine. This represents an incidence of recurrence of 5.7 per cent after ileostomy (Table 5), A comparison of chronic ulcerative colitis and Crohn's colitis must therefore emphasize the possibility of anastomotic surgery in Crohn's disease, but at the same time points out the high rate of recurrence after such a procedure. Anastomoses have not been useful in the surgical management of chronic ulcerative colitis in our experience. A very small number of patients have such minor rectal involvement to permit ileoproctostomy. 1 Our low incidence of recurrent Crohn's disease after ileostomy has made us feel as secure in suggesting total proctocolectomy to our patients with Crohn's disease with definite rectal involvement as to our patients with chronic ulcerative colitis. This recurrence rate, however, is lower than that reported by others.s, 7 The need for late ileostomy revision, the incidence of postoperative obstruction, and the incidence of persisting perineal sinus was the same for both chronic ulcerative colitis and Crohn's disease. Anorectal Disease In patients with chronic ulcerative colitis, because of their chronic diarrhea, minor inflammatory anal changes are not uncommon. However, marked changes are commonly seen in patients with Crohn's colitis. Of our patients with Crohn's colitis, 60 per cent either had evidence of significant anal disease at the time of colectomy or had a history of previous anal disease (excluding hemorrhoids and tissues).

Table 4. Anastomotic Procedures in Crohn's Colitis-28 Patients NUMBER

Required subsequent proctocolectomy Required further resection with anastomosis Recurrent disease treated medically No recurrence after 11-17 years (average, 13 years) Postoperative death

10 4 4 9 1

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Table 5. Proctocolectomy with Ileostomy in Crohn's Colitis58 Patients NUMBER

Died postoperatively At risk for recurrence Recurrent ileitis No recurrence

5 53 3 (5.7%) 50

Megacolon Toxic megacolon has been recognized as a serious complication of chronic ulcerative colitis. However, in our experience megacolon has only occurred in the presence ofCrohn's colitis in the early days after the onset of symptoms. CONCLUSIONS Three types of colitis are now recognized. In the late 1950's and early 1960's, 51 per cent of our patients having colectomy had classic chronic ulcerative colitis. Of our patients operated on at that time, 39 per cent had Crohn's colitis and 10 per cent had colitis of indeterminate type. These three types of colitis should be differentiated because of the high risk of cancer of the colon in patients with long-standing chronic ulcerative colitis. We have not seen an increased risk of carcinoma associated with Crohn's colitis. We performed resection with anastomosis in approximately one third of patients with Crohn's colitis, but recurrent disease developed in two thirds. In contrast, recurrent Crohn's disease developed in only 5.7 per cent of patients having colectomy with ileostomy. Toxic megacolon associated with Crohn's colitis has been seen only in the early clinical stage of the disease. From the standpoint of management and prognosis, attempts to differentiate between the two major types of colitis are appropriate. We have outlined some of the basic clinical and pathologic differences between these two types of colitis.

REFERENCES 1. Bargen, J. A.: Chronic ulcerative colitis complicated by malignant neoplasia. Arch. Surg., 17:561-576 (Oct.) 1928. 2. Bargen, J. A., Weber, H. M.: Regional migratory chronic ulcerative colitis. Surg. Gynecol. Obstet., 50:964-972 (June) 1930. 3. Crohn. B. B., Berg, A. A.: Right-sided (regional) colitis. J.A.M.A., 110:32-38 (Jan. 1) 1938. 4. Darke, S. G., Parkes, A. G., Grogono, J. L., et al: Adenocarcinoma and Crohn's disease. A report of 2 cases and analysis of the literature. Br. J. Surg., 60: 169-175 (March) 1973. 5. De Dombal, F. T., Burton, I., Goligher, J. C.: Recurrence ofCrohn's disease after primary excisional surgery. Gut., 12:519-527 (July) 1971. 6. Devroede, G. J., Taylor, W. F., Sauer, W. G., et al: Cancer risk and life expectancy of children with ulcerative colitis. N. Engl. J Med., 285:17-21 (July 1) 1971.

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7. Korelitz, B. 1., Present, D. R., Alpert, L. 1., et al: Recurrent regional ileitis after ileostomy and colectomy for granulomatous colitis. N. Engl. J. Med., 287: 110-115 (July 20) 1972. 8. Lockhart-Mummery, R. E., Morson, B. C.: Crohn's disease (regional enteritis) of the large intestine and its distinction from ulcerative colitis. Gut, 1 :87-105 (June) 1960. 9. Neuman, R. W., Dockerty, M. B.: The pathology of regional (segmental) colitis. Surg. Gynecol. Obstet., 99:572-579 (Nov.) 1954. 10. Saeed, W., Kim, S., Burch, B. R.: Development of carcinoma in regional enteritis. Arch. Surg., 108:376-379 (March) 1974. 11. Veidenheimer, M. C., Dailey, T. R., Meissner, W. A.: Ileorectal anastomosis for inflammatory disease of the large bowel. Am. J. Surg., 119:375-378 (April) 1970. 12. Weedon, D. D., Shorter, R. G., Ilstrup, D. M., et al: Crohn's disease and cancer. N. Engl. J. Med., 289:1099-1103 (Nov. 22) 1973. 13. Wells, C.: Ulcerative colitis and Crohn's disease. Ann. Roy. ColI. Surg. Engl., 11 :105-120 (Aug.) 1952. 14. Wilks, S., Moxon, W.: Lectures on Pathological Anatomy. 2nd ed., London, J., and A. Churchill, Ltd., 1875, 681 pp. Lahey Clinic Foundation 605 Commonwealth A venue Boston, Massachusetts 02215

Ulcerative colitis or Crohn's colitis. Is differentiation necessary?

Symposium on Surgery at the Lahey Clinic Ulcerative Colitis or Crohn's Colitis Is Differentiation Necessary? Malcolm C. Veidenheimer, M.D.,* F. Warr...
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