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Pseudopolyps of the small intestine in Crohn's disease
Case Studies PSEUDOPOLVPS OF THE SMALL INTESTINE IN CROHN'S DISEASE ELLEN KAHN, MD, AND FREDRIC DAUM, MD
The morphologic features of two types of pseudopolyps of the terminal ileum in a patient with Crohn's disease are described. One form of pseudopolyp is indistinguishable from that observedin the large intestine in Crohn's colitis. while the second, nodular lymphangiectasia, seems to be unique to the small bowel. HUM PATROL 15:84-86,1984.
a patient with Crohn's ileocolitis, which had microscopic features of colonic pseudo polyps or localized nodular lymphangiectasia. REPORT OF A CASE A 19-year-old woman was initially diagnosed in 1976 as having Crohn's disease of the terminal ileum, cecum, and ascending colon on the basis of findings from upper gastrointestinal and small bowel series and barium enema. The patient had been clinically well without medication until four months prior to admission in 1981, when she developed right-lower-quadrant pain and anorexia and began to lose weight. A right-lower-quadrant abdominal mass was palpable. Radiographic studies revealed involvement of the terminal ileum and right colon with an ileocecal fistula. The patient was treated with sulfasalazine (Azulfidine), 1 g three times daily, without improvement. Prednisone, 40.0 mg by mouth, was started and the sulfasalazine discontinued. Sub-
Although pseudopolyps of the colon are common in inflammatory bowel disease, these lesions have not been observed in the small bowel of patients with Crohn's disease. We report multiple polypoid masses in the ileum of Received from the Departments of Laboratories and Pediatrics, North Shore University Hospital, Manhasset, New York, and Cornell University Medical College, New York, New York. Accepted for publication August 11, 1982. Address correspondence and reprint requests to Dr. Kahn: Department of Laboratories, North Shore University Hospital, Manhasset, NY 11030.
FleuRE 1. Pseudopolvp of Ileum, (Hematoxylln--eosln stain. x 155.) Left granulation tissue wIth Incipient epithelial regeneration (solid arrow). Notice the pyloric gland metaplasia (open arro~. Right pseudopolyp composed of mucosa, and muscularis mucosa with moderate Inflammation.
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CASE STUDIES
FIGURE 2. Nodular lymphangiectasia as a polypoid mass. NotIce the edema and lymphangiectasia of submucosa and mucosa. (Hematoxylin-eosin stain. x 155.) Inset musaca l lymphangiectasIa, x 500.
sequently, the patient developed a warm, erythematous fluctuant mass in the right groin, which was drained surgically. After four weeks of total parenteral nutrition and intravenous administration of hydrocortisone succinate, 20 mg q 6 h , a right hemicolectomy with an ileotransverse colostomy was performed. Pathological Features. The resected specimen measured 70 em and consisted of ileum, cecum, and ascending colon. The grossly involved 39-cm segment of bowel was characterized by a flat, shiny mucosa and a 2-cm ulceration at the proximally involved ileal segment. Numerous single or confluent finger-like polypoid masses were present in both the small and the large bowels and had the typical configuration of pseudopolyps. The ileum also contained sessile, nodular polypoid masses, isolated or confluent with a shiny pink surface. Yellow polyps characteristic of submucosal lipoma involved the ileocecal valve and ascending colon. The remaining changes included creeping of mesenteric fat over the ileal serosa, thickening of the bowel wall with narrowing of the lumen, and focal serosal fibrosis corresponding to the fistulous tract 1.5 em distal to the ileocecal valve.
Microscopically, there was moderate active inflammation of the mucosa without ulceration, except for the small area noted on gross examination, pyloric metaplasia in the ileal mucosa, submucosal fibrosis and hyperplasia of the muscularis mucosa, and serosal fibrosis . The fistulous tract extended into pericolic fat and was partially lined by colonic mucosa with moderate active inflammation. No evidence of granulomatous inflammation or dysplasia was seen. The finger-like polyps in both the small and the large intestines either were composed of granulation tissue alone or were at times partially covered by regenerating surface epithelium (fig. 1, top) or by complete mucosa and muscularis mucosa with a moderate degree of inflammation (fig. 1, bottom). The nodular polyps of the small intestine consisted of complete mucosa, muscularis mucosa, and edematous submucosa. Lymphangiectasia in both the lamina propria and the submucsoa was associated with widening and blunting of the villous structures (fig. 2). Marked submucosal fibrosis and smooth muscle hyperplasia were present in the adjacent intestinal wall. The lipomas of the ileocecal valve and colon had the typical pattern associated with these tumors.
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HUMAN PATHOLOGY
Volume 15, No, 1 (January 1984)
DISCUSSION
as polypoid masses. Therefore, the presence of nodular masses in the small intestine involved by Crohn's disease does not necessarily indicate a proliferative neoplastic process. This observation is pertinent in view of increasing reports of adenocarcinoma of the small intestine associated with Crohn's disease.e-t-
We describe two types of ileal pseudopolyps: those with a "classic" appearance and those with a pattern of nodular lymphangiectasia. Colonic pseudopolyps, associated with inflammatory bowel disease, are thought to reflect a reparative change without neoplastic potential. 1 Macroscopically, they usually appear as delicate finger-like projections, with branching and bridging, in a focal or diffuse distributionz-s or, less frequently, as tumor-like masses up to 15 em in diameter (giant pseudopolyps).1,4-6 In our patient, the finger-like polypoid masses were composed of either granulation tissue, probably representative of a more acute stage, or complete mucosa and muscularis mucosa, with varying degrees of inflammation, These patterns are indistinguishable from those of pseudopolyps of the large intestine frequently observed in patients with Crohn's colitis. The second group of polyps found exclusively in the ileum consists of mucosa and submucosa with marked submucosal edema and both submucosal and mucosal lymphangiectasia. Diffuse lymphangiectasia is a common finding in Crohn's disease of the small intestine." A nodular configuration of lymphangiectasia has not been described. The mechanism for the polypoid appearance of these lesions is unclear. The marked submucosal fibrosis at the base of the pseudopolyp, indicative of antecedent severe inflammation, and the preservation of a delicate muscularis mucosa within the pseudopolyp itself are characteristic of lesser involvement. This suggests the following three stages in pathogenesis. First, the margin of the area where the future pseudopolyp is going to develop is the site of severe submucosal inflammation with ulceration. Subsequent repair of the ulceration results in attenuated mucosa and submucosal fibrosis surrounding a better preserved mucosa and submucosa. Finally, the fibrosis is complicated by stasis in the central, less involved mucosa and submucosa, with subsequent edema, lymphangiectasia, and pseudopolyp formation, Regardless of the pathogenesis, nodular lymphangiectasia seems to represent a unique form of pseudopolyp restricted to the small bowel, perhaps related to its rich lymphatic supply. This report points out that the "classic" pseudopolyps in Crohn's disease are not restricted to the large bowel and that lymphangiectasia of the small intestine may also appear
Acknowledgments. The authors thank Dr. Sheldon C. Sommers for reviewing the histologic sections and Dr. Stanley Fisher for his comments on the manuscript. Addendum, Since the acceptance of this paper, pseudo polyps of the
ileum have been noted in seven additional patients with Crohn's disease. These findings suggest that pseudopolyps are not uncommon in small bowel Crohn's disease.
REFERENCES I. Martinez CR, Siegelman 55, Saba GP, et al: Localized tumor-like lesions
in ulcerative colitis and Crohn's disease of the colon. Johns Hopkins Med J 140:249, 1977 2. Rotterdam H, Sommers SC: Biopsy Diagnosis of the Digestive Tract. Biopsy Interpretation Series, Raven Press, New York, H181, p 366 3. Goldgraber MB: Pseudopolyps in ulcerative colitis. Dis Colon Rectum 8;355, 19G5 4. Schneider R, Dickersin R, Patterson JF: Localized giant pseudopolyposis: a complication of granulomatous colitis. Dig Dis \8:265. 1973 5. Willis JS, Han SS: Localized giant pseudopolyposis complicating granulomatous ileocolitis. Radiology 122:320, 1977 6. Jones B, Abbruzzese AA; Obstructing giant pseudopolyps in granulomatous colitis. Gastrointest Radiol 3:437, 1978 7. Bennington WB: Histopathological spectrum of regional enteritis and ulcerative colitis. In Major Problems in Pathology, Vol 2. Philadelphia, WB Saunders Co, 1971, pp 68, 76 8. Frank In, Shorey BA: Adenocarcinoma of the small bowel as a complication ofCrohn's disease. Gut 14:120,1973 9. Fleming KA, Pollock AC: A case of "Crohn's carcinoma." Gut 16:533, 1975 10. Newman RD, Bennett SJ, Pascal RR: Adenocarcinoma of the small intestine arising in Crohn's disease. Cancel' 36:2016, 1975 11. Floch HF, Slattery LR, Hazzi CG: Carcinoma of the small intestine in regional enteritis. Am J Gastroenterol 70:520, 1978 12. Greenstein AJ, Sachar DB, Pucillo A, et al: Cancer in Crahn's disease after diversionary surgery: a report of seven carcinomas occurring in excluded bowel. Am J Surg 135:86, 1978 13. Holter A, Fischer JE: Adenocarcinoma of the small bowel associated with Crohn's disease. Arch Surg 113:991. 1978 14. Greenstein AJ, Sachar DB, Smith H, et al: A comparison of cancer risk in Crohn's disease and ulcerative colitis. Cancer 48:2742, 1981
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The morphologic features of two types of pseudopolyps of the terminal ileum in a patient with Crohn's disease are described. One form of pseudopolyp is indistinguishable from that observedin the large intestine in Crohn's colitis. while the second, nodular lymphangiectasia, seems to be unique to the small bowel. HUM PATROL 15:84-86,1984.
a patient with Crohn's ileocolitis, which had microscopic features of colonic pseudo polyps or localized nodular lymphangiectasia. REPORT OF A CASE A 19-year-old woman was initially diagnosed in 1976 as having Crohn's disease of the terminal ileum, cecum, and ascending colon on the basis of findings from upper gastrointestinal and small bowel series and barium enema. The patient had been clinically well without medication until four months prior to admission in 1981, when she developed right-lower-quadrant pain and anorexia and began to lose weight. A right-lower-quadrant abdominal mass was palpable. Radiographic studies revealed involvement of the terminal ileum and right colon with an ileocecal fistula. The patient was treated with sulfasalazine (Azulfidine), 1 g three times daily, without improvement. Prednisone, 40.0 mg by mouth, was started and the sulfasalazine discontinued. Sub-
Although pseudopolyps of the colon are common in inflammatory bowel disease, these lesions have not been observed in the small bowel of patients with Crohn's disease. We report multiple polypoid masses in the ileum of Received from the Departments of Laboratories and Pediatrics, North Shore University Hospital, Manhasset, New York, and Cornell University Medical College, New York, New York. Accepted for publication August 11, 1982. Address correspondence and reprint requests to Dr. Kahn: Department of Laboratories, North Shore University Hospital, Manhasset, NY 11030.
FleuRE 1. Pseudopolvp of Ileum, (Hematoxylln--eosln stain. x 155.) Left granulation tissue wIth Incipient epithelial regeneration (solid arrow). Notice the pyloric gland metaplasia (open arro~. Right pseudopolyp composed of mucosa, and muscularis mucosa with moderate Inflammation.
84
CASE STUDIES
FIGURE 2. Nodular lymphangiectasia as a polypoid mass. NotIce the edema and lymphangiectasia of submucosa and mucosa. (Hematoxylin-eosin stain. x 155.) Inset musaca l lymphangiectasIa, x 500.
sequently, the patient developed a warm, erythematous fluctuant mass in the right groin, which was drained surgically. After four weeks of total parenteral nutrition and intravenous administration of hydrocortisone succinate, 20 mg q 6 h , a right hemicolectomy with an ileotransverse colostomy was performed. Pathological Features. The resected specimen measured 70 em and consisted of ileum, cecum, and ascending colon. The grossly involved 39-cm segment of bowel was characterized by a flat, shiny mucosa and a 2-cm ulceration at the proximally involved ileal segment. Numerous single or confluent finger-like polypoid masses were present in both the small and the large bowels and had the typical configuration of pseudopolyps. The ileum also contained sessile, nodular polypoid masses, isolated or confluent with a shiny pink surface. Yellow polyps characteristic of submucosal lipoma involved the ileocecal valve and ascending colon. The remaining changes included creeping of mesenteric fat over the ileal serosa, thickening of the bowel wall with narrowing of the lumen, and focal serosal fibrosis corresponding to the fistulous tract 1.5 em distal to the ileocecal valve.
Microscopically, there was moderate active inflammation of the mucosa without ulceration, except for the small area noted on gross examination, pyloric metaplasia in the ileal mucosa, submucosal fibrosis and hyperplasia of the muscularis mucosa, and serosal fibrosis . The fistulous tract extended into pericolic fat and was partially lined by colonic mucosa with moderate active inflammation. No evidence of granulomatous inflammation or dysplasia was seen. The finger-like polyps in both the small and the large intestines either were composed of granulation tissue alone or were at times partially covered by regenerating surface epithelium (fig. 1, top) or by complete mucosa and muscularis mucosa with a moderate degree of inflammation (fig. 1, bottom). The nodular polyps of the small intestine consisted of complete mucosa, muscularis mucosa, and edematous submucosa. Lymphangiectasia in both the lamina propria and the submucsoa was associated with widening and blunting of the villous structures (fig. 2). Marked submucosal fibrosis and smooth muscle hyperplasia were present in the adjacent intestinal wall. The lipomas of the ileocecal valve and colon had the typical pattern associated with these tumors.
85
HUMAN PATHOLOGY
Volume 15, No, 1 (January 1984)
DISCUSSION
as polypoid masses. Therefore, the presence of nodular masses in the small intestine involved by Crohn's disease does not necessarily indicate a proliferative neoplastic process. This observation is pertinent in view of increasing reports of adenocarcinoma of the small intestine associated with Crohn's disease.e-t-
We describe two types of ileal pseudopolyps: those with a "classic" appearance and those with a pattern of nodular lymphangiectasia. Colonic pseudopolyps, associated with inflammatory bowel disease, are thought to reflect a reparative change without neoplastic potential. 1 Macroscopically, they usually appear as delicate finger-like projections, with branching and bridging, in a focal or diffuse distributionz-s or, less frequently, as tumor-like masses up to 15 em in diameter (giant pseudopolyps).1,4-6 In our patient, the finger-like polypoid masses were composed of either granulation tissue, probably representative of a more acute stage, or complete mucosa and muscularis mucosa, with varying degrees of inflammation, These patterns are indistinguishable from those of pseudopolyps of the large intestine frequently observed in patients with Crohn's colitis. The second group of polyps found exclusively in the ileum consists of mucosa and submucosa with marked submucosal edema and both submucosal and mucosal lymphangiectasia. Diffuse lymphangiectasia is a common finding in Crohn's disease of the small intestine." A nodular configuration of lymphangiectasia has not been described. The mechanism for the polypoid appearance of these lesions is unclear. The marked submucosal fibrosis at the base of the pseudopolyp, indicative of antecedent severe inflammation, and the preservation of a delicate muscularis mucosa within the pseudopolyp itself are characteristic of lesser involvement. This suggests the following three stages in pathogenesis. First, the margin of the area where the future pseudopolyp is going to develop is the site of severe submucosal inflammation with ulceration. Subsequent repair of the ulceration results in attenuated mucosa and submucosal fibrosis surrounding a better preserved mucosa and submucosa. Finally, the fibrosis is complicated by stasis in the central, less involved mucosa and submucosa, with subsequent edema, lymphangiectasia, and pseudopolyp formation, Regardless of the pathogenesis, nodular lymphangiectasia seems to represent a unique form of pseudopolyp restricted to the small bowel, perhaps related to its rich lymphatic supply. This report points out that the "classic" pseudopolyps in Crohn's disease are not restricted to the large bowel and that lymphangiectasia of the small intestine may also appear
Acknowledgments. The authors thank Dr. Sheldon C. Sommers for reviewing the histologic sections and Dr. Stanley Fisher for his comments on the manuscript. Addendum, Since the acceptance of this paper, pseudo polyps of the
ileum have been noted in seven additional patients with Crohn's disease. These findings suggest that pseudopolyps are not uncommon in small bowel Crohn's disease.
REFERENCES I. Martinez CR, Siegelman 55, Saba GP, et al: Localized tumor-like lesions
in ulcerative colitis and Crohn's disease of the colon. Johns Hopkins Med J 140:249, 1977 2. Rotterdam H, Sommers SC: Biopsy Diagnosis of the Digestive Tract. Biopsy Interpretation Series, Raven Press, New York, H181, p 366 3. Goldgraber MB: Pseudopolyps in ulcerative colitis. Dis Colon Rectum 8;355, 19G5 4. Schneider R, Dickersin R, Patterson JF: Localized giant pseudopolyposis: a complication of granulomatous colitis. Dig Dis \8:265. 1973 5. Willis JS, Han SS: Localized giant pseudopolyposis complicating granulomatous ileocolitis. Radiology 122:320, 1977 6. Jones B, Abbruzzese AA; Obstructing giant pseudopolyps in granulomatous colitis. Gastrointest Radiol 3:437, 1978 7. Bennington WB: Histopathological spectrum of regional enteritis and ulcerative colitis. In Major Problems in Pathology, Vol 2. Philadelphia, WB Saunders Co, 1971, pp 68, 76 8. Frank In, Shorey BA: Adenocarcinoma of the small bowel as a complication ofCrohn's disease. Gut 14:120,1973 9. Fleming KA, Pollock AC: A case of "Crohn's carcinoma." Gut 16:533, 1975 10. Newman RD, Bennett SJ, Pascal RR: Adenocarcinoma of the small intestine arising in Crohn's disease. Cancel' 36:2016, 1975 11. Floch HF, Slattery LR, Hazzi CG: Carcinoma of the small intestine in regional enteritis. Am J Gastroenterol 70:520, 1978 12. Greenstein AJ, Sachar DB, Pucillo A, et al: Cancer in Crahn's disease after diversionary surgery: a report of seven carcinomas occurring in excluded bowel. Am J Surg 135:86, 1978 13. Holter A, Fischer JE: Adenocarcinoma of the small bowel associated with Crohn's disease. Arch Surg 113:991. 1978 14. Greenstein AJ, Sachar DB, Smith H, et al: A comparison of cancer risk in Crohn's disease and ulcerative colitis. Cancer 48:2742, 1981
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