The solitary rectal ulcer syndrome, localized colitis cystica profunda, and inflammatory cloacogenic polyp
are closely allied conditions that have been linked to bowel prolapse.1-10 Affected patients often
demonstrate abnormal function of the anal and pelvic floor musculature during defecation 2,11,12 that
leads to rectal mucosal prolapse or even intussusception. The resulting trauma is thought to cause the
clinical symptoms and the pathologic changes. The term solitary rectal ulcer syndrome is quite a misnomer
because the ulcers are often multiple, there is preulcer polypoid phase, and similar lesions occur in the
anal canal and sigmoid colon.1-3 Additionally, colitis cystica profunda and inflammatory cloacogenic
polyp are also misnomers. Since all three conditions share a common histologic appearance, clinical
presentation, clinical course, and pathogenesis, I prefer to consider them together under the heading
mucosal prolapse syndromes.
Patients with mucosal prolapse syndrome range in age form 10-83 years with the majority presenting in the
third and fourth decade of life. The condition occurs more commonly in women.2 Table 1 outlines the
various clinical presentations of mucosal prolapse syndrome along with their relative frequencies. All
patients report difficulties in defecation. This can manifest as a constant need for laxatives 1,2 or
excessive straining. Some patients claim to have spent up to 200 minutes per day straining on the toilet.13
Furthermore, a surprising number of patients admit to the use of digital manipulation, spoons, or
other instruments to assist in rectal evacuation. Rectal bleeding occurs commonly and is usually reported
as mild to moderate.1,8 Other common presenting symptoms include pruritus ani, abdominal or rectal pain,
and intermittent bowel habits in which periods of constipation interrupt bouts of loose stools.
The diagnosis of mucosal prolapse syndromes can be difficult because the history often suggests primary
inflammatory or ischemic bowel disease. In addition, mucosal prolapse is often covert requiring special
techniques for its demonstration.
Investigators generally agree that barium enema is not helpful in the diagnosis of mucosal prolapse
syndromes.1,12,11,13 Although newer radiologic techniques such as evacuation cineproctography, and
defecography often demonstrate abnormalities in these patients, the definitive diagnosis requires the
demonstration of characteristic changes in the biopsy specimen.1,2,11
Ulcers, when present, typically occur on the anterior or anterolateral wall of the rectum, are irregular in
shape, and often appear well demarcated. In patients without ulceration, the mucosa appears polypoid,
roughened, or erythematous. Inflammatory cloacogenic polyp 4,5 involves the lower rectum and anal
transition zone. The clinical impression in patients with mucosal prolapse syndromes is often incorrect and
includes ulcer, Crohn's disease, nonspecific proctitis, carcinoma, or villous adenoma.9
CLINICAL PRESENTATIONS OF PATIENTS
THE MUCOSAL PROLAPSE SYNDROMES*
|Feature ||Relative Frequency|
|Difficulty with defecation ||100%|
|Rectal bleeding ||68 - 91%|
|Passage of mucus ||43 - 68%|
|Diarrhea ||19 - 27%|
|Pruritus ani ||21%|
|Intermittent bowel habits ||6 - 28%|
|Anorectal pain ||9 - 24%|
|Abdominal cramps ||12 - 16%|
|Feeling of rectal fullness or a sense of bearing down||8%|
|Incomplete rectal evacuation ||6%|
|Overt rectal prolapse ||4%|
* Modified from References 1, 6-9
The characteristic histopathologic changes are found in the mucosa adjacent to the ulcers or in the polypoid
areas and consist of fibromuscular obliteration of the lamina propria associated with mucosal architectural
distortion often with a hyperplastic or villiform appearance.2,3,6,8,9 Inflammation is typically mild or
absent and mucosal capillaries can be ectatic. Superficial erosions occasionally occur and can be
associated with acute inflammation and the formation of inflammatory "pseudomembranes." On occasion,
colonic glands may be misplaced into the muscularis mucosae or submucosa, a histology referred to as
"localized colitis cystica profunda." Submucosal vessels may also be ectatic and hyalinized. The histology
of specimens obtained from ulcerated areas appears nonspecific and usually shows fibrinopurulent debris,
fibrosis, and granulation tissue.
Differential diagnostic considerations include mucinous adenocarcinoma, chronic ulcerative colitis, Cowden's
disease, and ulcers due to ergotamine suppositories. The misplaced glands of mucosal prolapse syndrome
(colitis cystica profunda) can be associated with dissecting mucous pools and can be easily mistaken for
invasive mucinous adenocarcinoma. Table 2 illustrates histologic features that aid in this differential.14-16
The mucosal abnormalities of mucosal prolapse syndrome also closely mimic chronic ulcerative colitis.
Knowledge of the clinical picture and recognition of the characteristic fibromuscular obliteration of the
lamina propria (not usually present in inflammatory bowel disease) aid in this distinction. The histologic
appearance of colorectal polyps from patients with Cowden's disease (multiple hamartoma syndrome) appears
identical to mucosal prolapse syndrome.17 Mucosal prolapse syndrome and Cowden's must be separated on
clinical grounds. Clinical history is also required to separate the rectal ulcers associated with the use
of ergotamine suppositories from mucosal prolapse. These can be grossly and microscopically identical to
mucosal prolapse syndrome.18
DIFFERENTIAL FEATURES BETWEEN DISSECTING MUCUS
OF MUCOSAL PROLAPSE SYNDROME (COLITIS CYSTICA PROFUNDA)
AND INVASIVE MUCINOUS ADENOCARCINOMA*
|Feature||Mucosal Prolapse Syndrome (Colitis Cystica Profunda)||Invasive Mucinous Adenocarcinoma|
|Shape of mucous pools ||Rounded ||Irregular, infiltrating|
|Location of epithelium ||Periphery of pool ||Floating in pool|
|Configuration of epithelium ||Single often discontinuous layer, basal polarity of nuclei||Cellular piling up, complex glandular, proliferation gland in gland configuration|
|Cytologic features ||No dysplasia ||Atypia sometimes pronounced|
|Tumor desmoplasia ||Absent ||Usually present|
|Hemorrhage and hemosiderin deposits||Sometimes ||Usually absent|
|Supporting lamina propria ||Sometimes present ||Absent|
* Modified from References 14-16
Treatment and Follow-up
The few studies with long-term follow up report a chronic stable appearance to the lesions.1,2,19 The
majority of mucosal prolapse syndrome patients tolerate their symptoms following reassurance that they do
not have cancer, the addition of fiber to the diet, and instruction to reduce straining and to avoid digital
manipulation. The rare patient with severe bleeding or obstruction requires excisional therapy. Medical
remedies such as Sulfasalazine, local or systemic corticosteroids and antibiotics are useless.1,2
Unusual patients with severe incapacitating symptoms have been treated by resection, diverting colostomy, or
rectal prolapse repair. Results of these operations have varied and since approximately 100 different
operations have been described for mucosal prolapse syndrome, comparisons are difficult.
- Madigan MR, Morson BC. Solitary ulcer of the rectum. Gut 10:871-881, 1969.
- Levine DS. "Solitary" rectal ulcer syndrome: Are "solitary" rectal ulcer syndrome and "localized"
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- Rutter KRP, Riddell RH. The solitary ulcer syndrome of the rectum. Clin Gastroenterol 4:505-530, 1975.
- Lobert PF, Appelman HD. Inflammatory collagenic polyp. A unique inflammatory lesion of the anal
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- Womack NR, Williams NS, Holmfield JH, Morrison JF. Anal rectal function in the solitary rectal ulcer
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- Sun WM, Read NW, Carmel Donnelly C, Bannister JJ, Shorthouse AJ. A common pathophysiology for full
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- Mackle EJ, Manton Mills JO, Parks TG. The investigation of anorectal dysfunction in the solitary rectal
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- Silver H, Stoler J. Distinguishing features of well differentiated mucinous adenocarcinoma of the rectum
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- Petras RE. Adenomas and malignant polyps of the colon and rectum: Invasive carcinoma versus
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- Wayte DM, Helwig EB. Colitis cystica profunda. Am J Clin Pathol 48:159-169, 1967.
- Carlson GJ, Nivatvongs S, Snover DC. Colorectal polyps in Cowden's disease (multiple hamartoma
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- Eckardt VF, Kanzler G, Remmele W. Anal rectal ergotism: Another cause of solitary rectal ulcers.
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- Allen-Mersh TG, Henry MM, Nichols RJ. Natural history of anterior mucosal prolapse. Brit J Surg