Case 5 -

Solitary Rectal Ulcer and its Associated Syndrome Henry D. Appelman University of Michigan Ann Arbor, MI

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Clinical Summary For the past 2 years, this 25 year old man has had painless bright red rectal bleeding with every bowel movement. He has about 2 to 4 soft bowel movements per day, and he has no other symptoms such as tenesmus or abdominal pain. During defecation, he does not have to strain or bear down. He sometime feels tissue prolapsing through his anus, and occasionally he has to self-reduce it. He spent three years lifting heavy weights, but he has not worked out in the last eight months. Three colonoscopies were performed over this 2 year period, and during all of them, 3 large, multilobulated polyps, from 1 to 2.5 cm across, were found in the rectum, just above the anorectal junction, but there were no ulcers. These polyps were partly removed at each endoscopy, but were too large to remove completely, so eventually, because of continuing bleeding, surgical excision was required. Case 5 - Figure 1 - Low power of complex architecture Case 5 - Figure 2 - Ulcerated surface Case 5 - Figure 3 - Villiform surface, fibromuscular lamina propria, crypt distortion Case 5 - Figure 4 - Muscle bundles in lamina propria Case 5 - Figure 5 - Distorted crypts, some with pointed ends Microscopic Description: These lumps of mucosa are architecturally chaotic. The surface varies from flat to villiform, and large areas are eroded. There is profound crypt distortion with dilated, branching, pointed and serrated forms. The muscularis mucosae is irregularly thickened and splayed, and bundles of smooth muscle extend from it into the base of the mucosa between the crypts. In places, the lamina propria has lost its usual inflammatory cell population and contains instead collagen fibers, spindle cells and small vessels. Discussion: These polyps have all the changes that we blame on mucosal prolapse, including fibromuscular replacement of the lamina propria, disordered hyperplastic muscularis mucosae, and architectural distortion involving both the surface contours and the crypts. Erosions or ulcers are often superimposed, adding granulation tissue to the bulk of the polyps. On occasions, the basal crypts herniate into the submucosa, possibly through breaks in the disorganized muscularis mucosae, and become deep cysts, at which point they get a different name, "colitis cystica profunda." These changes can complicate any polyp, including adenomas and hyperplastic polyps, and any anastomosis be it internal or to the skin. They can be found next to and overlying neoplasms. Some polypoid prolapsed mucosae have specific names, such as the polypoid prolapsed folds in diverticular disease. The infamous inflammatory cloacogenic polyp is lumpy mucosal prolapse that crosses the anorectal junction, but it is the essence of poor naming, since it is neither inflammatory nor in the cloacogenic zone. Don't forget the non-ulcerated polypoid variant of the solitary ulcer syndrome. A group of polyps named "inflammatory myglandular polyp" shares many of these features, and other polyps with caps of exudates, called "cap polyps" are probably the same thing. When there are a lot of them in both the rectum and sigmoid colon, they have been called "eroded polypoid hyperplasia". In the colon, there is even a switch in goblet cell mucin from the normal sulphomucins to sialomucins, comparable to what happens in colonic transitional mucosa. In the gastric antrum, prolapse changes accompany the large superficial vessels in gastric antral vascular ectasias. There are even some isolated polyps that occur throughout the gut that have only these prolapse changes, that is, localized polypoid prolapsed mucosa, the cause of the prolapse being unknown. Du Boulay et al even invented the term "mucosal prolapse syndrome" as a unifying concept, but putting these disparate entities into a syndrome makes for an unmanageable syndrome. In the present case, the lumps were in the distal rectum where the solitary rectal ulcer and its associated syndrome occurs, so maybe these are part of that syndrome. If so, there must be a way to prove that, and such proof undoubtedly depends on the diagnostic criteria for that syndrome. Finding such criteria in print is frustrating. In one of the major textbooks of gastroenterology, there is no definition, simply some confusing non-specific information which leads to the obvious conclusion that this is one of the least well defined syndromes on record. First, it need not have an ulcer, but it can have polypoid prolapsed mucosa instead. Second, it need not be solitary, but there can be multiple ulcers or polyps. Third, in some patients, the ulcer is not even confined to the rectum. Fourth, the symptoms can be virtually anything including severe straining during defecation, no straining, tenesmus, diarrhea, constipation, bleeding, passage of mucus or no symptoms at all. It occurs in both sexes and at all ages. The cause is unknown but trauma or ischemia leading to rectal prolapse are the frontrunners. Some people have a functional obstruction in the puborectalis muscle part of the anal sphincter. Therefore this syndrome is broad enough that virtually any prolapse in the rectum or nearby can be included in it, so there is no reason that the patient in this presentation should not fit into this syndrome. He is a young man without an ulcer but with multiple polyps that are in the rectum and he had no defecatory problems, but he had bleeding.. His polyps have world class prolapse changes. However, it is possible that he has a different risk factor, weight lifting, which does not seem to be high on the risk list for the solitary rectal ulcer. Maybe the best thing to do with this case is just call in "rectal mucosal prolapse, not otherwise specified, but possibly weight-lifting induced." References Abrahams NA, Vesoulis Z, Petras RE. Angiogenic polypoid proliferation adjacent to ileal carcinoid tumors: a nonspecific finding related to mucosal prolapse. Mod Pathol. 14:821-7, 2001 Burke AP, Sobin LH: Eroded polypoid hyperplasia of the rectosigmoid. Am J Gastroenterol. 85:975-980, 1990 Du Boulay CE, Fairbrother J, Isaaacson PG. Mucosal prolapse syndrome—a unifying concept for solitary ulcer syndrome and related disorders. J Clin Pathol. 36:1264-8, 1983. Ehsanullah M, Filipe MI, Gazzard B. 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