This syndrome is associated with erythema and ulceration of the rectal wall. In some cases, the inflammation may produce a mass lesion, prompting suspicion of a malignancy. On histopathologic examination, there are classic features which should enable a clear distinction from a malignancy. However, in some cases, there is considerable atypia and multiple biopsies may be needed to establish a diagnosis. The lesion is thought to arise from ischemic changes in the bowel resulting in repeated ulceration with healing and subsequent re-injury.
| EPIDEMIOLOGY |
CHARACTERIZATION |
| SYNONYMS |
Mucosal prolapse syndrome
Colitis cystica profunda |
INCIDENCE/
PREVALENCE |
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| AGE |
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| PEDIATRIC |
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Solitary rectal ulcer syndrome in children. A report of three cases.
Gabra HO, Roberts JP, Variend S, Shawis RN.
Paediatric Surgical Unit, Sheffield Children's Hospital, Sheffield S10 2TH, UK.
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| Eur J Pediatr Surg. 2005 Jun;15(3):213-6. Abstract quote |
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Solitary rectal ulcer syndrome (SRUS) is a rare condition in children. It is well recognised in the adult literature.
We report our experience with 3 patients; two boys presented at a very young age (18 and 24 months, respectively) with a clinical picture simulating inflammatory bowel diseases.
The other patient was a fifteen-year-old girl presenting with severe rectal stricture as a result of the syndrome, which is the first to be reported in the paediatric literature.
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A rare and often unrecognized cause of hematochezia and tenesmus in childhood: solitary rectal ulcer syndrome.
Ertem D, Acar Y, Karaa EK, Pehlivanoglu E.
Division of Pediatric Gastroenterology and Nutrition, Marmara University School of Medicine, Istanbul, Turkey.
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| Pediatrics. 2002 Dec;110(6):e79. Abstract quote |
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Solitary rectal ulcer syndrome (SRUS) is an unusual disorder of childhood, which usually presents with rectal bleeding, mucous discharge, prolonged straining, tenesmus, and localized pain in the perineal area. After the first description by Cruveilhier, Madigan and Morson further detailed the clinical and pathologic features of SRUS in 1969. The pathogenesis of the syndrome is not well-understood. The postulated mechanism responsible for rectal prolapse in most cases seems to be excessive straining efforts during which high intra-abdominal pressure forces the anterior rectal mucosa firmly into the contracting puborectalis muscle. The anterior rectal mucosa is frequently forced into the anal canal and as a consequence becomes strangulated, causing congestion, edema, and ulceration.
Histologically, the presence of fibromuscular obliteration of the lamina propria with disorientation of muscle fibers is characteristic, which could be secondary to chronic mechanical and ischemic trauma and inflammation by hard stools, and intussusception of the rectal mucosa. Although the syndrome is well-recognized in adults, the pediatric experience with this condition is limited and often remains unrecognized or misdiagnosed. A misdiagnosis has been reported in one fourth of adult cases, and the correct diagnosis usually delayed approximately 5 to 7 years. There are few pediatric case reports in English literature.
Here, we describe 2 children with SRUS, aged 11 and 14 years, whose SRUS was diagnosed 2 and 6 years, respectively, after the onset of the first signs and symptoms.
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| SEX |
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| GEOGRAPHY |
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| EPIDEMIOLOGIC ASSOCIATIONS |
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| HISTOLOGICAL TYPES |
CHARACTERIZATION |
| GENERAL |
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Pathology of the rectal wall in solitary rectal ulcer syndrome and complete rectal prolapse.
Kang YS, Kamm MA, Engel AF, Talbot IC.
St Mark's Hospital, Harrow, Middlesex.
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| Gut. 1996 Apr;38(4):587-90. Abstract quote |
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BACKGROUND--The aetiology and pathology of rectal prolapse and solitary rectal ulcer are poorly understood.
AIMS--To examine the full thickness rectal wall in these two conditions.
METHODS--The pathological abnormalities in the surgically resected rectal wall were studied from nine patients with solitary rectal ulcer syndrome, 11 complete rectal prolapse, and nine cancer controls. Routine haematoxylin and eosin and Van Gieson staining for collagen were performed.
RESULTS--The rectal wall from solitary rectal ulcer syndrome specimens was thickened compared with complete rectal prolapse and controls. The major difference was in the muscularis propria (2.2 v 1.1 v 1.2 mm, medians, p < 0.005) and particularly the inner circular muscular layer, and to a lesser extent the submucosal and outer longitudinal muscular layers. Some solitary rectal ulcer syndrome specimens showed unique features such as decussation of the two muscular layers (four of nine), nodular induration of inner circular layer (four of nine) and grouping of outer longitudinal layer into bundles (three of nine); these were not seen in complete rectal prolapse or control specimens.
CONCLUSIONS--These features, which resemble the features of high pressure sphincter tissue, may be of aetiological importance, and suggest a different pathogenesis for these two disorders. Excess collagen was seen in both disorders, was more severe in solitary rectal ulcer syndrome specimens, and probably reflects a response to repeated trauma.
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Solitary ulcer syndrome of the rectum--a histopathologic characterisation of 33 biopsies.
Malik AK, Bhaskar KV, Kochhar R, Bhasin DK, Singh K, Mehta SK, Datta BN.
Department of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh.
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| Indian J Pathol Microbiol. 1990 Jul;33(3):216-20. Abstract quote |
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Histological analysis of 33 rectal biopsies from 26 patients of solitary ulcer syndrome (SUS) of rectum was undertaken. Fibrous obliteration and smooth muscle extension into the lamina propria were the characteristic and most constant findings. The limitation of the biopsy procedure in procuring deeper tissue made the detection of colitis cystica profunda rare in our study.
A combination of fibrosis of the lamina propria, superficial mucosal ulceration, muscularis mucosae hypertrophy and submucosal fibrosis, along with the clinical suspicion should clinch the diagnosis of SUS.
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| DIFFERENTIAL DIAGNOSIS |
KEY DIFFERENTIATING FEATURES |
| ACUTE HEMORRHAGIC RECTAL ULCER SYNDROME |
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Acute hemorrhagic rectal ulcer syndrome: a new clinical entity? Report of 19 cases and review of the literature.
Tseng CA, Chen LT, Tsai KB, Su YC, Wu DC, Jan CM, Wang WM, Pan YS.
Department of Internal Medicine, Buddhist Tzu Chi General Hospital, Dalin, Chia-Yi, Taiwan.
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| Dis Colon Rectum. 2004 Jun;47(6):895-903; discussion 903-5. Epub 2004 May 4. Abstract quote |
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PURPOSE: Acute hemorrhagic rectal ulcer syndrome is characterized by sudden onset, painless, and massive hemorrhage from rectal ulcer(s) in patients with serious underlying illnesses. It is a matter of controversy whether acute hemorrhagic rectal ulcer syndrome is a distinct clinical entity. This is the first Asian report on acute hemorrhagic rectal ulcer syndrome to be made outside Japan.
METHODS: From January 1989 to December 1999, 8085 patients underwent total colonoscopy at our institution. We retrospectively analyzed the medical records and colonoscopic files. The diagnosis of acute hemorrhagic rectal ulcer syndrome was made by means of the clinical, histologic, and colonoscopic findings.
RESULTS: Among the 8085 patients, 19 patients (11 males; mean age, 71.2 +/- 10.1 years) were diagnosed with acute hemorrhagic rectal ulcer syndrome, which accounted for 2.8 percent of the patients with massive lower gastrointestinal bleeding. The duration from hospitalization to the onset of massive bleeding ranged from 3 to 14 (mean, 9 +/- 3.3) days. Characteristics of colonoscopic appearance were solitary or multiple rectal ulcer(s), with round, circumferential, geographical, or Dieulafoy-like lesions located within a mean of 4.7 cm +/- 1.5 cm from the dentate line. Histopathologically, the lesions appeared as necrosis with denudation of covering epithelium, hemorrhage, and multiple thrombi in the vessels of the mucosa and underlying stroma, which is considered to be similar to stress-related mucosa injury. Successful hemostasis was obtained in 74 percent (14/19) of patients with direct therapeutic maneuvers. Prognosis was largely dependent on accurate diagnosis and management of the underlying disorders.
CONCLUSIONS: We assert that acute hemorrhagic rectal ulcer syndrome is a rare but important entity and stress that awareness of this clinical entity should lead to a high index of suspicion resulting in early detection, diagnosis, and appropriate therapy.
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| CARCINOMA |
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Malignant tumors in the rectum simulating solitary rectal ulcer syndrome in endoscopic biopsy specimens.
Li SC, Hamilton SR.
Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21205-2196, USA.
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| Am J Surg Pathol. 1998 Jan;22(1):106-12. Abstract quote |
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Patients with solitary rectal ulcer syndrome (SRUS) frequently present with a mass that can be misinterpreted as cancer. In contrast, the occurrence and characteristics of SRUS-like histopathology produced by underlying malignancy have not been reported in detail.
We report seven patients whose rectal mass that was induced by infiltrating carcinoma showed only histopathologic changes of SRUS on initial mucosal biopsy specimens. Carcinoma was evident in subsequent specimens after one to five repeat biopsies with delay in diagnosis from 1 week to 18 months in six patients. In one patient, infiltrating carcinoma was suggested on the first biopsy specimen by immunohistochemistry for cytokeratin. Three of the patients had primary rectal adenocarcinoma, two had metastatic carcinoma from stomach or ovary, and two had direct invasion of anal squamous cell carcinoma or prostatic adenocarcinoma.
We conclude that the histopathology of SRUS may occasionally represent a characteristic but nonspecific mucosal reactive change to a deeper seated malignancy. The terminology "solitary rectal ulcer syndrome/mucosal prolapse changes" with a cautionary note may be useful for reporting biopsy results to emphasize the possibility of underlying primary or metastatic malignancy in the differential diagnosis.
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| COLITIS |
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Biopsy diagnosis of colitis: possibilities and pitfalls.
Tsang P, Rotterdam H.
Department of Pathology, Cornell University Medical College, New York, New York, USA.
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| Am J Surg Pathol. 1999 Apr;23(4):423-30. Abstract quote |
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The histopathologic diagnosis of inflammation is common in colorectal biopsies but is of limited value, if not further specified.
We reviewed 280 endoscopic colorectal biopsy specimens for nonneoplastic disease from 100 consecutive patients in order to assess (a) the frequency of inflammation in excess of the physiologic infiltrate, (b) the frequency with which the cause of the inflammation could be specified, and (c) the interobserver variability in diagnosing inflammation.
Based on the reviewers' impression, each case was classified into one of three categories: (I) normal or nonspecific change, (II) nonspecific inflammation, and (III) inflammation suggestive or diagnostic of specific cause. Inflammation was diagnosed in 68% of cases. The majority of these cases (75%) showed features typically associated with specific types of colitis, including Crohn's disease (n = 16), ulcerative colitis (n = 13), inflammatory bowel disease not otherwise specified (n = 5), infectious colitis (n = 6), ischemic colitis (n = 4), solitary rectal ulcer syndrome (n = 3), radiation colitis (n = 2), and lymphocytic colitis (n = 2). Interobserver variability was greatest in biopsy specimens interpreted by the reviewers as normal or showing nonspecific changes, most of which had been diagnosed as mild inflammation by the original pathologists. Etiologic classification of colitis was lacking in 59% of the cases interpreted by the reviewers as suggestive or diagnostic of a specific cause.
We conclude that (a) the majority of colorectal biopsy specimens from patients with nonneoplastic disease in this series show inflammation, (b) the majority of such cases allow a specific cause of colitis to be suggested or firmly diagnosed, and (c) pathologists tend to overdiagnose the physiologic inflammatory infiltrate as evidence of colitis and underdiagnose specific etiologic types of colitis.
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| PROLAPSED MUCOSAL POLYP |
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Prolapsing mucosal polyps: an underrecognized form of colonic polyp--a clinicopathological study of 15 cases.
Tendler DA, Aboudola S, Zacks JF, O'Brien MJ, Kelly CP.
Division of Gastroenterogy, Duke University Medical Center, Durham, North Carolina 7710, USA.
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| Am J Gastroenterol. 2002 Feb;97(2):370-6. Abstract quote |
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OBJECTIVE: Prolapsing intestinal mucosa occurs in many forms throughout the GI tract. We describe 15 patients with polypoid masses in the sigmoid colon and histological features of mucosal prolapse.
METHODS: Fifteen patients with colon polyps demonstrating endoscopic and histological features of mucosal prolapse were retrospectively identified from our database.
RESULTS: Twelve patients presented with signs and symptoms that were nonspecific, but consistent with mucosal prolapse, such as occult or gross intestinal bleeding and lower abdominal pain. Three patients were asymptomatic. The polyps occurred in the sigmoid colon, usually in association with diverticular disease, and appeared more often in men. Endoscopically, the polyps appeared to be well-circumscribed, hyperemic masses that contrasted sharply with normal-appearing adjacent mucosa. Histological features include glandular crypt abnormalities, fibromuscular obliteration of the lamina propria, and thickened and splayed muscularis mucosa.
CONCLUSIONS: Prolapsing mucosal polyps of the colon are histologically similar to other mucosal prolapsing conditions in the GI tract, such as the solitary rectal ulcer syndrome, inflammatory cloacogenic polyps, inflammatory "cap" polyps, and gastric antral vascular ectasia, and should therefore be designated as part of the "mucosal prolapse syndrome."
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| SERRATED ADENOMA |
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Sessile serrated polyp mimicry in patients with solitary rectal ulcer syndrome: is there evidence of preneoplastic change?
Ball CG, Dupre MP, Falck V, Hui S, Kirkpatrick AW, Gao ZH.
Department of Surgery, Foothills Medical Center, University of Calgary, Calgary, Alberta, Canada.
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| Arch Pathol Lab Med. 2005 Aug;129(8):1037-40. Abstract quote |
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CONTEXT: Solitary rectal ulcer syndrome (SRUS) is associated with erythema and ulceration of the rectal wall. Serrated lesions of the colon are divided into conventional hyperplastic polyps and a new set of lesions that are variably called sessile serrated polyps (SSPs) and sessile serrated adenomas. The SSPs are epithelial proliferative lesions that appear to act as a unique pathway to colorectal carcinogenesis. No association between SRUS and SSPs has been previously reported.
OBJECTIVE: To assess a possible association between SRUS and morphologic features that mimic SSPs.
DESIGN: Twenty-six patients with SRUS, who presented to our institution between January 1, 1999, and November 14, 2004, were retrospectively reviewed for SSP-type morphologic features by 3 pathologists. Ki-67 and hMLH1 immunohistochemical stains were used. Control tissues included 10 conventional left-sided hyperplastic polyps, 10 right-sided large SSPs, 7 adenocarcinomas with known loss of hMLH1 gene expression, and 4 normal human tonsil tissues.
RESULTS: Ten (38%) of 26 SRUS specimens demonstrated histologic features consistent with SSPs. These features included exaggerated serration within the lower crypt compartments, crypt branching, hypermucinous appearance of epithelium, and horizontal extension of crypt bases along the muscularis mucosa. All 10 cases of SRUS had positive basal Ki-67 staining in 10% to 20% of cells. Two (20%) of the 10 cases demonstrated focal superficial loss of hMLH1 mismatch repair gene expression within areas of serrated morphologic features. One hyperplastic polyp superimposed on SRUS showed a reduced number of surface epithelial cells that express hMLH1 protein.
CONCLUSIONS: Up to 38% of patients with SRUS have histologic changes that mimic SSPs. More importantly, 20% of these serrated lesions were found to have focal loss of hMLH1 gene expression, indicating a potential of preneoplastic change. This phenomenon may reflect an increased propensity for neoplastic progression in response to repeated trauma and repair process in certain cases of SRUS.
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| PROGNOSIS |
CHARACTERIZATION |
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Solitary rectal ulcer syndrome: a series of 13 patients operated with a mean follow-up of 4.5 years.
Marchal F, Bresler L, Brunaud L, Adler SC, Sebbag H, Tortuyaux JM, Boissel P.
Department of Gastro-Intestinal Surgery C, Brabois Hospital, Vandoeuvre-les-Nancy, France.
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| Int J Colorectal Dis. 2001 Aug;16(4):228-33. Abstract quote |
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Solitary rectal ulcer syndrome (SRUS) is an infrequent pathology often associated with pelvic floor disorders. The aim of this retrospective study was to review the long-term results of a surgical series of SRUS. Between 1988 and 1998, 13 patients were operated on for SRUS. Seven patients had associated internal rectal prolapse (58%), two had associated total rectal prolapse (15%), and two had associated mucosal prolapse (15%).
We performed simple resection of the SRUS in one case (8%), a stoma as primary operation in one (8%), three rectopexies according to Orr-Loygue (23%), and eight Delorme's operations as modified by Berman (62%). Mean follow-up was 57 months (range 15-112). Simple resection of the solitary rectal ulcer syndrome did not improve symptoms. Colostomy permitted relief of symptoms and healing of the SRUS. Two of the three rectopexies achieved good results, and the third patient relapsed at the 6th postoperative month. A secondary modified Delorme's operation permitted relief of symptoms and healing of the SRUS. Five of the eight patients (62.5%) who received modified Delorme's operations had improved at a follow-up of 46 months.
We conclude that, considering the high failure rate after surgery, operations should be performed only in patients with total rectal prolapse or intractable symptoms not amenable to behavioral therapy. Delorme's operation and abdominal rectopexy help in about 60% of cases.
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| GRADING/STAGING |
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A new grading of rectal internal mucosal prolapse and its correlation with diagnosis and treatment.
Pescatori M, Quondamcarlo C.
Coloproctology Unit, UCP Villa Claudia, Rome, Italy.
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| Int J Colorectal Dis. 1999 Nov;14(4-5):245-9. Abstract quote |
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The management of rectal internal mucosal prolapse (RIMP) is not based on an accepted classification of the lesion which helps to choose the appropriate treatment.
The aim of this prospective study was to report a new endoscopic grading of RIMP and to evaluate its clinical value. Thirty-two patients (7 men, 25 women; mean age 56 years, range 28-72) affected by symptomatic RIMP were prospectively classified as follows: RIMP was defined as first degree when detectable below the anorectal ring on straining, as second degree when it reached the dentate line, and as third degree when it reached the anal verge. Anal manometry was carried out in 26 patients, and anal ultrasound and defecography in 6 prior to surgery.
A correlation was found between the occurrence and severity of symptoms and the degree of the prolapse as obstructed defecation, bleeding and fecal soiling affected mainly patients with third-degree RIMP. At manometry the maximal resting tone was 60+/-23 mm Hg and voluntary contraction 96+/-41 mm Hg (mean+/-SEM). At anal ultrasound the mean internal sphincter thickness was 2.1+/-0.2 mm, and external sphincter thickness was 7.0+/-0.8 mm. A significant rectocele and rectal intussusception (n = 2) and a nonrelaxing puborectalis muscle on straining (n = 2) were observed at defecography in cases with third-degree RIMP. The anorectal angle was 100+/-75 degrees at rest, 63+/-20 degrees on squeezing, and 115+/-9 degrees on straining.
A conservative treatment with high-fiber diet and/or rubber band ligation was carried out in all cases of first and in most patients with second-degree RIMP (n = 26). Those who required surgery, i.e., stapled transanal excision of the prolapse (n = 6), had either severely symptomatic third-degree RIMP with solitary ulcer syndrome (n = 4) or second-degree RIMP (n = 2). A positive outcome was achieved in 71% of cases.
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The proposed classification evaluated by the present study may be of clinical value in managing rectal internal mucosal prolapse. |
| MALIGNANCY |
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Solitary rectal ulcer syndrome accompanied by submucosal invasive carcinoma.
Tsuchida K, Okayama N, Miyata M, Joh T, Yokoyama Y, Itoh M, Kobayashi K, Nakamura T.
First Department of Internal Medicine, Nagoya City University Medical School, Nagoya, Japan.
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| Am J Gastroenterol. 1998 Nov;93(11):2235-8. Abstract quote |
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We report a case of carcinoma in solitary rectal ulcer syndrome. The diagnosis was made by colonoscopic appearance and biopsy.
A tumor measuring 0.9 x 0.6 cm was found in a resected solitary rectal ulcer. The lesion exhibited typical histological features of solitary rectal ulcer syndrome, with a well differentiated adenocarcinoma invading submucosal layers and some dysplastic glands.
We believe that the adenocarcinoma represents a malignant transformation from solitary rectal ulcer syndrome, because similar to longstanding chronic idiopathic colitis, colorectal dysplasia and carcinoma may develop.
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