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  • Colitis Cystica Profunda*

    j . KIRK MARTIN, JR., M.D. , CLYDE E. CULP, M.D. , Louis n. WEILAND, M.D.

    Martin JK Jr, Culp CE, Weiland LH. Colitis cystica profunda. Dis Colon Rectum 1980;23:488-491. Colitis cystica profunda is a be- nign disease of the colon. Its importance lies in differentiating it from mucus-producing adenocarcinoma. It has rarely been de- scribed in the surgical literature. A review of records of patients seen at the Mayo Clinic produced 66 clinical cases of localized colitis cystica profunda, and in 21 patients the diagnosis was confirmed histologically. Follow-up, which was available in all patients, ranged from 2 months to 29 years, with a mean follow- up of more than 8 years. The data suggest that local excision is the preferred initial therapy. [Key words: Colitis cystica pro- funda; Colon; Cyst(s), submucosal; Polyp(s), rectal]

    CoH'rts CYSTICA PROFUNDA is a benign lesion of the colon. Once considered to be rare, it is being diag- nosed with increasing frequency, so the true inci- dence and prevalence are unknown. It is important that the lesion be d i f fe rent ia ted f rom a mucus- producing adenocarcinoma and that the rectal bleed- ing and diarrhea that accompany colitis cystica pro- funda be properly treated. In reviewing the English medical l iterature, we found only 66 cases reported. A review of the Mayo Clinic experience of 21 patients with histologically proved lesions forms the basis for this report.

    Mater ia l s and Methods

    The records of all patients seen at the Mayo Clinic between 1968 and 1979 with colitis cystica profunda were retrospectively reviewed. From these records, 66 cases were identified in which the diagnosis had been made by history, physical examination, and procto- scopic examination, with or without the aid of bar ium enema study. Between 1968 and 1975, there were 45 cases, and dur ing the final 4 years of the study period, 21 cases were found. To establish a more homogene- ous group of patients and to eliminate any doubt about the diagnosis, only patients who had histologic p roo f of colitis cystica pro funda were chosen for

    * Read at the meeting of the American Society of Colon and Rectal Surgeons, Hollywood, Florida, May 11 to 16, 1980.

    Address reprint requests to Dr. Martin: Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55901.

    From the Department of Surge U, the Section of Colon and Rectal Surge' U , and the Department of

    Surgical Patholo~, Ma'r Clinic and M~o Foundation, Rochester, Minnesota

    further study. This group consisted of 21 patients, including 2 from our early experience.

    The ages of the 21 patients (14 women and 7 men) ranged from 14 to 66 years and averaged 39 years. The women patients tended to be slightly younger than the men, 35.9, compared with 45.6 years. Fre- quent symptoms were rectal bleeding (81 per cent), mucus discharge (35 per cent), d iarrhea (30 per cent), and pain, either perineal or abdominal (10 per cent). Only one patient had associated chronic ulcerative colitis. The duration of symptoms before evaluation at our clinic ranged f rom 1 week to 22 years and averaged 37 months. Only, 2 of the 21 patients, both male, were asymptomatic, and the lesions in both were found incidentally on routine examination.

    Most of the lesions were described at proctosig- moidoscopy as firm nodules with an overlying ery- thematous mucosa, occasionally with a white "cap" (Fig. 1). In 19 patients, the location of the lesions varied f rom just above the dentate line to 15 cm above it. In the remaining two patients, the nodules were located in the sigmoid colon, without rectal involve- ment. The most frequent site of involvement was on the anterior rectal wall between 6 and 7 cm above the dentate line. In fact, 60 per cent of the lesions oc- curred on the anterior rectal wall, whereas the re- mainder occurred with equal frequency on the poste- r ior wall, lateral walls, and sigmoid.

    In both patients with sigmoid lesions, colonoscopy was attempted. In one, tissue showed an atypical polyp with areas of mucous gland hyperplasia and foci of ulceration and inf lammation. In this patient, bar ium enema study also demonstrated mucosal in- f lammatory bowel disease involving the sigmoid colon and rectum, and abdominoper inea l resection was performed. In the other patient with sigmoidal in-

    0012-3706/80/1000/0488/$00.70 9 American Society of Colon and Rectal Surgeons

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  • Volume 23 Number 7 COL IT IS CYST ICA PROFUNDA 489

    volvement, colonoscopy failed to reach the lesion, so operation was advised, and a sigmoid colotomy with excision of the localized area of colitis cystica pro- funda was performed.

    Pathologic Findings

    Grossly, the lesion in colitis cystica profunda is a broad-based polyp or nodule covered over by intact mucosa. Cutting across the mass reveals mucus-filled cystic spaces in the classic lesion, but only thickening of the submucosa in lesions that are at an earlier stage.

    On microscopic examination, several features are prominent. The most important is the presence of benign colonic epithel ium beneath the muscularis propria. In some lesions, an actual communication with the overlying mucosa can be seen (Fig. 2B). This ectopic epithelium produces mucus that forms cystic spaces. Usually, the mucus escapes and dissects into the loose connective tissue of the submucosa (Fig. 2A). A frequent feature is hyperplasia of the overly- ing mucosa (Fig. 2B). This villous hyperplasia is prob- ably a reactive process related to the submucosal thick- ening; we do not believe that it is related to villous adenoma or other neoplasia. It might lead to an er- roneous diagnosis if biopsy specimens are superficial and do not include submucosa.

    Treatment and Follow-up

    Follow-up was 100 per cent and ranged from 2 months to 29 years, With an average of 8.4 years. T reatment var ied from excisional biopsy to ab- dominoper inea l resection. There were no deaths after treatment.

    Of the 21 patients, 19 underwent only biopsy of the rectosigmoid lesions. Just 2 of the 12 patients were asymptomatic at tbllow-up. The symptoms ranged from infrequent rectal bleeding to more pronounced diarrhea and bleeding with anemia. Extensive local disease in two patients involved ahnost circumferen- tial rectal nodules and precluded local excision. One patient in this treatment group died from cancer of the cervix during the follow-up period.

    Six patients underwent excision of the colitis cystica profunda, five transanally and one transabdominally. Of these six patients, two with transanal excision were asymptomatic, one 4 months and the other 5 years after operation. The patient who underwent sigmoid colotomy and excision was asymptomatic 8 years after operat ion. One asymptomatic patient t reated by transanal excision was asymptomatic preoperatively, as was the patient who underwent colotomy. The three other patients treated by transanal excision

    FIO. 1. Proctoscopic appearance of' colitis cystica profunda.

    were all symptomatic: two had rectal bleeding that required iron supplementation, and the other had in f requent rectal b leed ing that improved post- operatively.

    One patient was treated with five fulgurations of the lesions, which involved the anterior two-thirds of the rectal circumference. She continues to have occa- sional rectal bleeding, and the lesions have persisted, unchanged, during the last 3 years. Interestingly, this patient was given corticosteroid therapy for chronic active liver disease and experienced a temporary im- provement in rectal bleeding and mucous diarrhea.

    The two pat ients who underwent abdomino- perineal resection were both asymptomatic 4 and 29 years after their procedures, the more recent patient undergoing operation for chronic ulcerative colitis with coexisting colitis cystica profunda.

    Carcinoma of the colon did not develop in any of our patients, and no patient in our series died from complications directly related to colitis cystica pro- funda. Additionally, no patient had a hemoglobin level of less than 11 g/dl; the average hemoglobin level for the group was 13.0 g/dl. Of the patients who complained of mucous diarrhea, none had a total protein value of less than 6.4 g/dl, with the average being 6.9 g/dl. In one patient, rectal stenosis de- veloped after a 4-year history of disease and after multiple biopsy specimens were taken. This patient was considered to have colloid carcinoma and was treated by abdominoperineal resection. No patient had rectal prolapse.

    Discussion

    Although colitis cystica profunda was apparently first described by Stark, 1'2 later descr ipt ions by

  • 490 MARTIN, ET AL. Di~. Col. & Rect. October 1980

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    FIG. 9. Colitis cystica profunda. A. Pools of mucus dissect the loose connective tissues of submucosa. Epithelial cells that produce mucus may be obscure (hematoxylin and eosin; 60). B. Points of communication from mucosa across muscularis mucosa and into submucosa may occasionally" be seen. Villous hyperplasia of mucosal glands is a frequent finding (hematoxylin and eosin; x 42).

    Rokitansky a (1839), Virchow 4 (1863), Woodward 5 (1879), and Vanzetti 6 (1992) did little to stimulate interest in the disease. Goodall and Sinclair r (1957), in Britain, reviewed the l iterature and presented two cases, reviving appreciation of the entity. Epstein and colleagues, s in 1966, presented the first description of the disease in the United States and discussed four cases seen at Columbia U