Problems in the Diagnosis and Management of Colonic Strictures in Chronic Ileocolitis: Report of a Case

Problems in the Diagnosis and Management of Colonic Strictures in Chronic Ileocolitis: Report of a Case

Vol. 49, No. 5 Printed in U.S.A. GASTROENTEROLOGY Copyright © 1965 by The Williams & Wilkins Co. PROBLEMS IN THE DIAGNOSIS AND MANAGEMENT OF COLONI...

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Vol. 49, No. 5 Printed in U.S.A.

GASTROENTEROLOGY

Copyright © 1965 by The Williams & Wilkins Co.

PROBLEMS IN THE DIAGNOSIS AND MANAGEMENT OF COLONIC STRICTURES IN CHRONIC ILEOCOLITIS: REPORT OF A CASE PAUL MANDELS'l'AM, M.D., H Au MING KwAAN, M.D., EDWIN PATRICK JENEVEI N, JR ., M.D., AND BENJAMIN

F.

RusH, JR. , M.D.

Departments of Medicine, Radiology, Pathology and Stagery, University of Kentucky College of Medicine, Lexington, Kentucky

The clinical and roentgen evaluation of strictures in patients with inflammatory disease of the colon has been studied by several groups who have reached diverse conclusions. Marshak et al.,l in reviewing an extensive clinical experience, conclude that "when all of the features of a benign stricture are demonstrated such as a concentric lumen, fusiform tapering margins and smooth contours, a carcinoma is unlikely." These authors and their associates1-5 consider ulcerative colitis and granulomatous colitis to be distinct entities which can often, t hough not always, be distinguished by differences in clinical manifestations, roentgen appearance, and pathological findings. They maintain, as do Cornes and Stecher, 6 that no cases of carcinoma of the colon have yet been identified in patients with granulomatous colitis; there are other groups 7-9 who do not concur. Indeed, t he distinction between granulomatous and ulcerative colitis, although recognized by many investigators 1-6 • 10 -12 is by no means acknowledged by all _l3- 17 In contrast to Marshak et al. , other workers state that the differential diagnosis between benign and malignant strictures of the colon is virtually impossible in patients with underlying inflammatory disease. Finkelstein,18 in an unpublished review of the literature and of cases from the University of Pennsylvania Graduate Hospital, noted that 43% of t he cases of carcinoma of the colon superimposed on ulcerative colitis demonstrated roentgen configurations of ill defined strictures. Bargen and Gage, 19 in

an analysis of the Mayo Clinic cases, found that in many patients, a long stretch of bowel ensheathed in carcinoma could not be distinguished from a benign stricture. In cases in which there is roentgen evidence of hour glass deformity in the bowel damaged by colitis they state that "the diagnosis [of carcinoma] can be reasonably well established only because of the stiffening of the bowel, the unusual narrowing of the lumen which was not present at a previous examination, the shortness of the time in which the stricture has developed and other associated signs." Hodgson and Sauer,2° in a review of the same Mayo Clinic material, similarly conclude that there is no reliable way to distinguish in this group of patients between benign stricture of the colon and scirrhous carcinoma. In their view, however, "annular, sessile, and polypoid lesions offer no serious problem in diagnosis." The difficulty in t he roentgen diagnosis is well exemplified by the University of Chicago data: 21 in a series of 10 patients in whom carcinoma was superimposed upon chronic colitis, correct diagnosis by roentgen examination was made in six, suspected in one, overlooked in three. The clinical course and serial roentgen examinations in the patient reported below illustrate some of the difficulties in diagnosis and management entailed in the care of such patients. It will be demonstrated that even in t he case of annular lesions the differential diagnosis between benign and malignant strictures is not possible by roentgen examination.

R eceived May 22, 1965. Accepted July 1, 1965. Address requests for reprints to : Dr. Paul M andelstam, Department of Medicine, University of Kentucky, Lexington, Kentucky 40506.

Case Report

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E. T., a 27-year-old Negro man, was in excellent health until age 18 (1955) . Over

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TABLE 1. Weight, laboratory findings, and the following 4 years there was progressive prednisone dosage anorexia, fatigue , and _weight_ loss fro~ 170 to 115 lb. There were mtermittent episodes Serum PredWeight Hemoglobin Date albumin nisone of diffuse abdominal pain accompanied by - - - · -- loose nonbloody stools. He was unable to lb. g/100 ml g/100 ml mg/24 ltr work for the latter 2 years. 11.4 8/8/59 The patient was admitted to Hospital 103 9.6 2/6/61 no. 1 in August 1959 with chills, fever, nau107 10.6 1/8/62 sea, and abdominal pain more severe than 102 10.6 3. 1 9/20/62 experienced previously. Temperature was 10/ 15/62 96 40 103 F. There was abdominal tenderness and 10/24/62 105 30 11/7/62 108 20 rigidity, most marked in the right low~r 110 11/ 14/62 15 quadrant. There was moderate anemia 117 11 .2 11/21/62 10 (table 1) and the white blood count was 121 12/5/62 5 15,750 per mm 3 with 76% polymorphon~­ 142 5 3/30/63 clear cells. Abdominal films showed dis144 12.4 5 7/19/63 tended loops of small bowel, with ladder 139 12.2 4.9 5 2/1/64 pattern and with multiple fluid levels. . 132 8.8 3.7 40 5/18/64 Limited exploration via a McBurney m138 40 5/26/64 cision revealed a normal appendix. The 141 40 6/5/64 14 .2 151 40 small intestine was slightly dilated and the 7/9/64 168 14.3 10 4/20/65 ileum thickened; 8 to 12 em proximal to the cecum a 3- by 5-cm globular mass was found pa;tially obstructing the ileum. No cause it appeared nonmalignant, only 7 em biopsies were taken. One week later, after of transverse colon were resected. Pathogastric drainage and antibiotic adminis- logical examination revealed the midportration, the abdomen was re-explored. Ery- tion of this segment to be too narrow to thema and swelling of both the small and admit a fingertip. The narrowing was seclarge intestine were noted and were most ondary to fibrosis of all layers, this being marked in those segments in the right lower most marked in the submucosa. Underquadrant. The mesenteric nodes were en- mining sinuses and fistulae with broad filarged and the mesenteric vessels dilate?. brous walls extended into the muscularis. The mass in the distal ileum was agam Many lymphocytes and a few plasma cells noted and the ileal segment just proximal were present in the submucosa. Glands was anastomosed to the transverse colon. maintained their usual simple pattern. EpiIleal resection was not performed and no thelial cells were not atypical. Lymph chanbiopsies were taken. nels were dilated. No amebae were found. Although the surgical bypass relieved the The patient did not improve postoperaobstruction the patient's chronic symptoms tively. Barium enema on 28 May 19~2 re' . continued. Small bowel roentgen examma- vealed the development of a long stncture tion in December 1960 disclosed in the in the area of the anastomosis performed 4 lower ileum irregularity of pattern, lack of months before. Irregularity and matting of pliability, and matting together of _loops. the lower ileal loops were again noted. The patient's progressive fatigue and The patient's condition continued to deweight loss continued. In January 1962, he teriorate and in September 1962 he was was admitted to Hospital no. 2. He ap- admitted to the University of Kentucky peared malnourished and chronically ill. Hospital. He appeared chronically ill and There was generalized abdominal tender- malnourished. The abdomen was thin, flat, ness most marked in the right lower quad- tense, and diffusely tender. Tenderness was rant.' Barium enema disclosed a stricture 4 most marked in the right lower quadrant, em in length in the mid transverse colon; lap- where there was a suggestion of a 4- by arotomy revealed a soft constriction. Be- 5-cm mass. Tuberculin and histoplasmin

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tests were negative. Stools were not grossly bloody but were positive for occult blood. Several stools were negative for ova and parasites. n-Xylose absorption and quantitative fecal fat determination (van den Kamer) were within normal limits. Sigmoidoscopy revealed bright red blood in the ampulla. The greater portion of the visualized mucosa was granular and hyperemic; in a few areas, the mucosa was pale and contained small clear vesicles. Barium enema showed diffuse inflammation of the entire colon, as well as the previously noted stricture in the transverse coIon. The ileocolostomy appeared narrowed, and the terminal ileum deformed. The ileal segment of the ileocolostomy and the terminal ileum appeared to communicate. Small bowel examination revealed normal appearing jejunum. In the ileum, several loops were bound together and areas of narrowing and of abnormal mucosal pattern were seen. Several sinus tracts interconnected the small bowel loops within the

Fm. 1. 19 May 1964. Complete and abrupt obstruction to the retrograde flow of barium, at the level of the proximal sigmoid colon.

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pelvis and the right lower quadrant. One of the sinus tracts appeared to communicate with the rectosigmoid. Prednisone therapy was instituted after 3Y2 weeks of hospitalization (table 1). Marked improvement began immediately, and was characterized by generalized well being, ravenous appetite, marked weight gain, and diminution in number of stools to two per day. This improvement continued despite gradual reduction of prednisone to a maintenance level of 5 mg per day. Sigmoidoscopic findings in January 1963 were within normal limits. In March 1963, the patient began full time upholstery work and continued to do well over the ensuing year. In February 1964, his weight was adequate and his over-all condition good. Three months thereafter, in mid-May 1964, the patient reported general malaise, poor appetite, and diffuse abdominal pain of a few weeks' duration. He still had but 1 to 2 bowel movements per day. He weighed 7 lb. less than when last seen. A tubular mass, consistent with colon, was present in the right paramedian area. There was marked rectal tenderness. Hemoglobin and serum albumin concentrations were substantially diminished. Sigmoidoscopy on 18 May 1964 revealed marked tenderness and diffuse mucosal edema and erythema. Marked granularity was noted in some areas. Many small ulcers were seen, some covered by clotted blood. There was complete obstruction to retrograde passage of barium 30 em from the anus (fig. 1). Repeat examination 1 hr later gave identical results, and thus diminished the likelihood of transitory spasm as the basis for the roentgen findings. Orally administered gastrografin traversed the area in question without difficulty. Prednisone was increased from 5 to 40 mg per day, and striking clinical improvement resulted (table 1). Within a week, the patient again felt very well, had a ravenous appetite, and gained 6 lb. On sigmoidoscopy on 5 June 1964, 18 days after the last examination, there was no tenderness and the rectosigmoid mucosa appeared normal. Barium enema on 6 June 1964, in

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CA SE REPORTS .

marked contrast to the findings 3 weeks earlier, demonstrated ready retrograde progression of barium beyond the site of previous obstruction. There was, however, in this area, a napkin ring lesion classically characteristic of carcinoma (fig. 2) . On this examination complete obstruction to t he retrograde flow of barium was noted just proximal to the splenic flexure, at the distal end of the strict ure noted earlier in the t ransverse colon. The increased prednisone level was continued for 3 more weeks, and the patient continued to improve and to gain weight. R epeat barium enema on 26 June 1964 showed no change in the roentgen characteristics of the sigmoid lesion, and there was still complete retrograde obstruction in the dist al transverse colon. Barium enema on July 9, 4 days prior to surgery, revealed no change in the area of stricture in t he sigmoid . Barium did , however, traverse the stricture in the transverse colon and this stricture now exhibited central ulceration (fig. 3). Abdominal exploration was performed on 13 July 1964. Despite the long history of small and large bowel involvement, past operations, and fi stulae formation, the peritoneal space was relatively open. There were numerous filmy adhesions, but the dense involvement of bowel which might have been expected was not present . The small bowel appeared grossly normal without evidence of thickening or fibrosis, and was supple and soft to palpation. The mesentery was not thickened and the mesenteric lymph nodes were not enlarged. The long area of stenosis in the transverse colon was easily identified ; it was quite soft to palpation and appeared benign. The diaphragm atic stenosis in the sigmoid colon, which had appeared identical to a carcinoma of t he bowel on roentgen examination only 4 days prior to surgery , was diffi cult to demonstrate by gross inspection and palpation. It was fin ally identified with certaint y by t he inj ection of air through a fine needle in the proximal portion of t he descending colon , the air then being milked toward t he distal portion. In t his way, by the appearance of an hour glass-type constriction, a very short but

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definite area of stenosis was ident ified. The stenosis seemed almost totally intraluminal. A colocolostomy was performed between t he proximal t ransverse and t he sigmoid colon , both strictures being resected en bloc. P athological examination of t he strictures in t he sigmoid (figs. 4 and 5) and transverse colon revealed abnormalities similar to those seen in t he stricture resected in J anuary 1962, but different in degree. The narrowing of t he sigmoid was caused by a complex "heaping up" of mucosa with the formation of sinus tracts and fistulae in the submucosa. Fibrosis had minimally involved t he muscularis and serosa. The central area of the stri cture in t he transverse colon was so narrowed by fibrosis and by polypoid "heaping up" of mucosa that a pencil-tip could not be admitted. Microscopic examination of both areas revealed in t he submucosa scattered fo ci of granulomatous reaction with mul tinucleated gi ant cells. In bot h segments, t he mucosal crypts were uniform and had an orderly arrangement despite active necrosis and inflammation in the subj acent fibrous tissue. Crypt abscesses were absent. In the t ransverse colon lesion at least t wo sinuses had extended t hrough t he fi brotic muscularis. The patient's postoperative course was benign. H e was discharged on the 8t h day, on a regimen of ferrous sulfate and of prednisone, 40 mg p.o. daily. Over the ensuing 7 weeks, prednisone was gradually decreased to t he present maintenance dosage of 10 mg per day. Roentgen examination of the small bowel on 26 September 1964, 10 weeks postoperatively, revealed no abnormalities, these findin gs being in marked contrast to t hose on t he last previous small bowel examination performed in September 1962. The patient has continued to feel well , to have excellent appetite, and to gain weight. At present (April 1965) , he weighs 168 lb., is without striae or edema, and works full t ime an an upholsterer. His hemoglobin and hematocrit have remained at normal levels. Sigmoidoscopic findin gs in April 1965 were normal and barium enema showed no evidence of colonic strictures.

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Fro. 2 (top). 6 June 1964. Barium enema demonstrates a napkin-ring lesion 3 em in length in the proximal sigmoid colon. Note the eccentric lumen, irregular contours, and overhanging margins characteristic of malignancy, There is no dilation proximal to the lesion. Fluoroscopically, the involved area exhibited rigidity. Fw. 3 (bottom). 9 July 1964. Barium enema reveals a long stricture in the transverse colon. The collection of barium over the central portion denotes superficial ulceration.

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November 1965

Discussion

This 28-year-old patient, after several years of anorexia, fatigue, weight loss, loose stools, and intermittent abdominal pain, presented with clinical manifestations of intestinal obstruction and operative findings of mesenteric adenopathy and diffuse inflammatory involvement of the ileum and colon. Surgical bypass of an obstructing lesion in the terminal ileum relieved the acute .situation, but resulted in no improvement m general clinical course. Twenty-nine months later, a stricture in the transverse colon was resected and was followed in less than 4 months by stricture formation in the area of the anastomosis. Subsequent roentgen and sigmoidoscopic examination revealed extensive ileal and colonic disease, fistulae, and sinus tracts. ''i." · Adrenal cortical steroid therapy resulted in prompt disappearance of abnormal sigmoidoscopic findings. There was dramatic clinical improvement and the patient resumed a normal pattern of living. Eighteen months later, while on a maintenance dose of .5 mg per day of prednisone, anorexia, wei.ght loss, and malaise recurred and sigmOidoscopy revealed ulcerative and inflammatory changes. Total obstruction to the retrograde passage of barium was noted in the proximal sigmoid. It is noteworthv that there were no symptoms or signs of ·intestinal obstruction. With increase in prednisone from 5 to 40 mg per day, there was rapid weight gain, marked general clinical improvement, and a return to normal sigmoidoscopic findings. Barium enema no longer showed the presence of complete obstruction to retrograde flow but, rather, in the same location an annular stricture with such classical ~tig­ mata of malignancy as irregular contours eccentric lumen, overhanging margins and stiffening manifest upon fluoroscopy: On this examination, however, there was complete obstruction to retrograde flow just proximal to the splenic flexure , in the region of the previously demonstrated stricture of the transverse colon. The roentgen findings in the sigmoid persisted despite further clinical improvement; barium enema performed shortly prior to surgery revealed,

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however, that barium now traversed the in. the transverse colon. The partial resolutiOn of the complete obstruction to retrograde flow in the sigmoid and in the dis~al transverse colon can reasonably be attributed to diminution in edema or inflammation secondary to the increase in prednisone dosage, or to both. At laparotomy, performed 4 days after the last barium enema, the sigmoid stricture was difficult to demonstrate; the stricture in the transverse colon was readily apparent. Both lesions were benign and were resected en bloc. There was no evidence of the extensive ileal involvement noted at previous operations and on earlier roentgen examinations. Small bowel radiological study 2 months postoperatively was also normal. The initial indolent course the extensive ileocolonic disease, ileal ob~truction and . ' mesen~enc adenopathy noted at the early operatiOns, the nonbloody diarrhea, fistulae and recurrent colonic strictures are all con. ' s1stent with a diagnosis of Crohn's granulomatous ileocolitis, as are the histological findings of granulomatous fibrosis and submucosal destruction by burrowing s~ricture

FIG. 4. 13 July 1964. Longitudinal section of the sigmoid stricture. The right half of the segment is normal colon with the serosa inferior. In the left half of the section is the constricted segment where there is "piling up" of the mucosa and fibrosis of the muscularis and serosa.

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Fra. 5. 13 July 1964. Magnification of an area of the sigmoid stricture demonstrates the complex arrangement of the mucosa, without atypia. The origin of a shallow sinus is noted in the left lower aspect.

sinuses which leave the mucosa largely intact. In Crohn's disease, however, the effect of adrenal cortical steroid therapy is usually far less satisfactory than in ulcerative colitis 22 -25 and the two dramatic clinical, roentgen and sigmoidoscopic response;,; of this patient to such therapy are distinctly unusual. We believe that differential diagnosis between benign and malignant strictures of the colon in patients with underlying inflammatory disease of that organ is virtually impossible and we cannot concur with Hodgson and Sauer20 that this distinction is not difficult when such patients present with roentgen manifestations of annular deformity of the colon. In the patient here reported, stiffening of the involved segment of sigmoid, irregular contours, overhanging margins all pointed strongly

toward malignancy; the pathological findings, both grossly and histologically, were clearly those of a benign stricture. Because one stricture of the colon has been benign, it does not follow that subsequent strictures in the same patient will also be nonmalignant. Exfoliative cytological examination of the colon is not, especially in patients with underlying inflammatory disease, sufficiently precise at present to rule out malignancy.2 6 We believe that patients with inflammatory disease of the colon should be subjected to sigmoidoscopy and to barium enema whenever there is progression of symptoms or signs or, if the clinical situation is unchanged, at 6-month intervals. Should there be evidence of stricture formation of recent onset, a short trial of ACTH or adrenal cortical steroid therapy

CASE REPORTS

November 1965

would appear justifiable, for the changes, if due to edema or inflammation, or both, may be reversible. Operative intervention is clearly in order, however, unless clear-cut roentgen disappearance of the stricture is demonstrable within several weeks.

8. Bersack, S. R., J . S. Howe, and E . M. Rehak. 1958. A unique case with roentgenologic evidence of regional enteritis of long duration and histologic evidence of diffuse adenocarcinoma. Gastroenterology 34: 703710. 9. Weingarten, B ., and J . Weiss. 1960. M alig-

Summary

Problems in the diagnosis and management of colonic strictures in a patient with chronic ileocolitis are considered and the difficulty in distinguishing benign and malignant strictures detailed. Such patients should be subjected to sigmoidoscopy and to barium enema whenever there is clinical deterioration or, if the status is unchanged, at 6-month intervals. A short trial of ACTH or adrenal cortical steroid therapy would appear justifiable in patients with stricture formation of recent onset . Should such a course not result in roentgen evidence of stricture resolution, definitive evaluation at laparotomy is indicated, since it is not possible at present to rule out malignancy in such strictures by other means. REFERENCES

10. 11.

12.

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chronic ulcerative colitis and its pathogenic implications. Gastroenterology 38 : 596604. 16. Valdes-Dapena, A.,

17.

90: 709-716.

2. Marshak, R . H., B . S. Wolf, and J. Eliasoph . 1959. Segmental colitis. Radiology 73 : 707-

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716. 3. Wolf, B. S., and R. H. Marshak. 1962. Gran-

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ulomatous colitis (Crohn's disease of the colon), roentgen features. Amer. J. Roentgen. 88: 662-670. Lindner, A. E., R. H. Marshak, B. S. Wolf, and H. D. J anowitz. 1963. Granulomatous colitis, a clinical study. N ew Eng. J. Med. 269: 379-385. Janowitz, H. D ., A. E. Lindner, and R. H. Marshak . 1965. Granuloma tous colitis, Crohn's disease of the colon. J. A. M. A. 191: 825-828. Comes, J . S., and M . Stecher. 1961. Primary Crohn's disease of the colon and rectum. Gut 2 : 189-201. Van Patter, W. N ., J . A. Bargen, M. B . Dockerty, W. H . Feldman, C. W. Mayo, and J. M. Waugh. 1954. Regional enteritis. Gastroenterology 26 : 347-450.

nant degeneration in chronic inflammatory disease of the colon and small intestine. Amer. J . Gastroent. 33: 203- 207. Brooke, B . N . 1959. Granulomatous diseases of the intestine. Lancet 2 : 745-749. Lockhart-Mummery, H. E ., and B. C. Morson. 1960. Crohn's disease (regional enteritis) of the large intestine and its distinction from ulcerative colitis. Gut 1: 87-105. Lockhart-Mummery, H. E ., and B . C. Morson . 1964. Crohn's disease of the large intestine. Gut 5: 493-509. Lumb, G. 1951. Cicatrizing enterocolitis. Brit. J. Surg. 39 : 233-243. Lumb, G., and R. H . B. Protheroe. 1958. Ulcerative colitis, a pathologic study of 152 surgical specimens. Gastroenterology 34 : 381-

407. 15. Goldgraber, M . B., J . B . Kirsner, and W. L. P almer. 1960. The histopathology of

1. Marshak, R. H ., C. Bloch, and B. S. Wolf. 1963. The roentgen findings in strictures of

the colon associated with ulcerative and granulomatous colitis. Amer. J. Roentgen.

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and F. Vilardell. 1962. Granulomatous lesions in ileocolitis. Gastroenterologia 97: 191- 204. Valdes-Dapena, A. 1964. Comments on enterocolitis, p. 240-242. In H. L. Bockus [ed.] Gastroenterology, Vol. 2, Ed. 2. W. B. Saunders Company, Philadelphia. Finkelstein, A. 1964. Roentgen features of carcinoma super-imposed on ulcera tive colitis, p. 852. Unpublished data, quoted by J. L . A. Roth . In H . L. Bockus [ed.] Gastroenterology, Vol. 2, Ed. 2. W. B. Saunders Company, Philadelphia. Bargen, J . A., and R. P . Gage. 1960. Carcinoma and ulcerative colitis : Prognosis. Gastroenterology 39: 385-392. Hodgson, J. R., and W. G. Sauer. 1961. The roentgenologic features of carcinoma in chronic ulcerative colitis. Amer. J. Roentgen. 86: 91-96. Goldgraber, M. B., E. M . Humphreys, J . B. Kirsner, and W. L . P almer. 1958. Carcinoma and ulcerative colitis. A clinical-pathologic study . I. Cancer deaths. Gastroenterology 34 : 809-839. Nevin, R. W. 1961. A review of granulomata of the large intestine. Proc. Roy. Soc. Med. 54: 137-142.

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23. M endeloff, A. I. 1962. p. 1630, 1651. In T. R.

Harrison, R . D. Adams, I. L. Bennett, Jr., W. H . Resnick, G. W. Thorn, and M. M. Wintrobe [ed.] Principles of internal medicine. McGraw-Hill Book Company, Inc., N ew York. 24. Zetzel, L. 1963. Regional ileitis, p. 960-968. In P. B. Beeson, and W. M cDermott. [ed.]

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Cecil-Loeb textbook of medicine, Ed. 11. W. B. Saunders Company, Philadelphia. 25. Bockus, H. L . 1964. p. 297. Gastroenterology, Vol. 2, Ed . 2. W. B. Saunders Company, Philadelphia. 26. R askin, H . F ., and S. Pleticka. 1964. The cytologic diagnosis of cancer of the colon. Acta Cytol. 8: 131-138.