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Crohn's Disease of the Anal Region

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Crohn's Disease of the Anal Region Gut: first published as 10.1136/gut.6.6.515 on 1 December 1965. Downloaded from Gut, 1965, 6, 515 Crohn's disease of the anal region B. K. GRAY, H. E. LOCKHART-MUMMERY, AND B. C. MORSON From the Research Department, St. Mark's Hospital, London EDITORIAL SYNOPSIS This paper records for the first time the clinico-pathological picture of Crohn's disease affecting the anal canal. It has long been recognized that anal lesions may precede intestinal Crohn's disease, often by some years, but the specific characteristics of the lesion have not hitherto been described. The differential diagnosis is discussed in detail. In a previous report from this hospital (Morson and types of anal lesion when the patients were first seen Lockhart-Mummery, 1959) the clinical features and were as follows: pathology of the anal lesions of Crohn's disease were described. In that paper reference was made to Anal fistula, single or multiple .............. 13 several patients with anal fissures or fistulae, biopsy Anal fissures ........... ......... 3 of which showed a sarcoid reaction, but in whom Anal fissure and fistula .................... 3 there was no clinical or radiological evidence of Total 19 intra-abdominal Crohn's disease. The opinion was expressed that some of these patients might later The types of fistula included both low level and prove to have intestinal pathology. This present complex high level varieties. The majority had the contribution is a follow-up of these cases as well as clinical features described previously (Morson and of others seen subsequently. Lockhart-Mummery, 1959; Lockhart-Mummery Involvement of the anus in Crohn's disease has and Morson, 1964) which suggest Crohn's disease, http://gut.bmj.com/ been seen at this hospital in three different ways: that is, the lesions had an indolent appearance with 1 Patients who presented with symptoms of irregular undermined edges and absence of indura- intestinal Crohn's disease who, at the same time, ation. They were surprisingly painless. Oedema and also had disease of the anus in the form of ulceration, a watery discharge were other features. In a few fistulae, or fissure-in-ano. These patients were patients the clinical appearances could not be referred because of their intestinal symptoms, the distinguished from the average fissure or fistula due involvement of the anus being an associated finding. to a non-specific infection; the diagnosis of Crohn's 2 Patients referred to the hospital because of disease in these cases was suggested only by the on September 28, 2021 by guest. Protected copyright. symptoms of anal disease but who were found also presence of a sarcoid reaction in biopsy material. to have intestinal pathology on clinical or radio- Five of the patients gave a history ofrecurrent anal logical investigation at that time. In this group the lesions requiring surgery over years, before their symptoms caused by the anal disease overshadowed first attendance at St. Mark's. Three of these pres- those due to intestinal involvement or else the ented with unhealed anal wounds which were latter caused no symptoms. indolent and remarkably painless. 3 Patients who presented with anal lesions which suggested Crohn's disease both clinically and HISTOLOGY histologically, who had at the time neither clinical nor radiological evidence of intestinal pathology. Figures 1, 4, and 6 all show sarcoid granulomata, as The object of this paper is to present the clinical seen in this site. features, pathology, and subsequent course of this Pieces of tissue for histological examination were last group of patients. taken from all 19 patients at the time of operation for the anal lesion. In every case the anal tissues CLINICAL FEATURES contained a sarcoid reaction consistent with a diagnosis of Crohn's disease or tuberculosis. The There were 19 patients with anal lesions in whom a histological sections have been reviewed and in only sarcoid reaction was found on biopsy. The clinical one case is there any reason to question the original 515 2 Gut: first published as 10.1136/gut.6.6.515 on 1 December 1965. Downloaded from 516 B. K. Gray, H. E. Lockhart-Mummery, and B. C. Morson opinion. It is possible that this one patient had a cultural methods. It is rarely possible to find bacilli foreign-body giant cell reaction in his tissues rather in non-caseating tuberculosis by Ziehl-Neelsen than a sarcoid or tuberculoid response. He has staining of histological preparations of ano-rectal remained well for seven years since his operation. lesions. In the present series of cases attempts to A 'sarcoid reaction' is a purely descriptive term demonstrate tubercle bacilli in histological prepar- applied to collections of epithelioid cells and giant ations have invariably failed. A few had guinea-pig cells of the Langhans type without any central inoculation of fresh tissue from the anal lesion, but caseation. In the cases reported here the sarcoid foci these were reported as negative. always contained epithelioid cells, but occasionally Giant cells of the foreign-body type are commonly giant cells were absent or very poorly developed. seen in tissue removed from anal fistulae. They The numbers of lymphocytes and plasma cells usually appear spherical with a tendency for their within and surrounding the foci were also very many nuclei to be centrally placed, in contrast to the variable, and it was exceptional for these to be peripheral position of the nuclei in Langhans giant completely absent. The size of these sarcoid granulo- cells. They are isolated or in clumps of a few. The mata varied from less than a dozen epithelioid cells giant cells may be placed around foreign matter, such with or without one or two giant cells to larger as vegetable material derived from the faeces. lesions containing many dozens of epithelioid cells. However, many seem to be formed from striated The anal tissues also contained chronic inflam- muscle cells which have been broken up and matory changes, abscess formation, and fibrosis of destroyed by inflammation and fibrosis. The a non-specific character. The sarcoid granulomata appearances are reminiscent of the giant cells were in some patients very sparse but in others derived from muscle in myocardial fibrosis. Epi- relatively large pieces of tissue from the anal region, thelioid cells are not a feature of an ordinary usually the perianal skin, were riddled with lesions. foreign-body reaction. Their absence is a useful, if They were found beneath the epidermis, deep in the arbitrary, distinction from the sarcoid type of tissue dermis, subcutaneous fat and muscle or in the response. granulation tissue lining the fistulous track, ulcer, or abscess cavity. The chances of finding sarcoid granulomata in FOLLOW-UP biopsy material from an anal lesion to some extent depend on the amount of tissue available for Thirteen of the 19 patients have remained well http://gut.bmj.com/ examination. For this reason small biopsies are since their discharge from hospital for periods usually inadequate and it is best to have larger varying from four to eight years. Their anal lesions pieces of tissue removed at the time of operation, at have not recurred and there is no clinical or radio- which most or all of the track or lesion can be logical evidence of intestinal disease. One patient excised. died from a carcinoma of the stomach two months A confident distinction between a sarcoid reaction after treatment for her anal lesion. due to Crohn's disease, sarcoidosis, or tuberculosis The remaining six patients have developed on September 28, 2021 by guest. Protected copyright. is impossible on histopathological evidence alone intestinal Crohn's disease at intervals varying from (Jones-Williams, 1964). The presence of caseation in 17 months to six years from the first appearance of the centre of a focus of epithelioid cells and giant the anal lesion (Table I). One of these has died as a cells is suggestive of tuberculosis, but it is still result of her intestinal disease. The clinical histories essential to confirm the diagnosis by demonstrating and pathology of these six patients are now des- the tubercle bacillus in histological sections or by cribed. TABLE I SUMMARY OF ILLUSTRATIVE CASES Patient Age Sex Anal Lesion Treatment Time Interval Location of lntestinal Disease from First Anal Symptoms to Diagnosis of Intestinal Lesion 13 M Fissures Excision 3 yr. 8 mth. Terminal ileum and colon 2 16 M Fistu1a (unhealed wound) Biopsy 5 yr. 2 mth. Terminal ileum 3 24 M Fistula Lay open 3 yr. 6 mth. Terminal ileum 4 37 F Fistula Lay open 1 yr. 5 mth. Terminal ileum 5 39 F Fistula and tags Lay open 1 yr. 10 mth. Terminal ileum and ascending colon 36 F Fistula, fissure, and tags Lay open I yr. 5 mth. Terminal ileum and caecum Gut: first published as 10.1136/gut.6.6.515 on 1 December 1965. Downloaded from Crohn's disease of the anal region 517 ILLUSTRATIVE CASES was extremely slow and required 16 months in all, during which period he had a further excision of the unhealed CASE 1 A boy, aged 13 at his first attendance, gave two wounds, but healing eventually occurred after 18 months, months' history of bleeding from the anus at stool, when he was discharged from attendance at the hospital painful swollen tags at the anal margin, and some with the anal region soundlyhealed andwithno symptoms. watery discharge from the anus. He had no alteration of The patient remained perfectly well for a further two bowel habit and no symptoms suggesting any abdominal years when, now aged 17, he again attended the hospital disorder and his general health was excellent. with a three months' history of right-sided abdominal Local examination of the anal region revealed two colic, diarrhoea up to four times daily, loss of weight of oedematous tags dorsally, each associated with a rather over a stone in two months, anorexia, and lassitude.
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