Solitary rectal ulcer syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Mugilan Poongkunran M.B.B.S [2]

Overview

Solitary rectal ulcer syndrome (SRUS, SRU), is a disorder of the rectum and anal canal, caused by straining and increased pressure during defecation. This increased pressure causes the anterior portion of the rectal lining to be forced into the anal canal (an internal rectal intussusception). The lining of the rectum is repeatedly damaged by this friction, resulting in ulceration. SRUS can therefore considered to be a consequence of internal intussusception (a sub type of rectal prolapse), which can be demonstrated in 94% of cases. It may be asymptomatic, but it can cause rectal pain, rectal bleeding, rectal malodor, incomplete evacuation and obstructed defecation (rectal outlet obstruction).

Epidemiology and Demographics

The condition is thought to be uncommon. It usually occurs in young adults, but children can be affected too.[1]

Causes

  • The essential cause of SRUS is thought to be related to too much straining during defecation.
  • Overactivity of the anal sphincter during defecation causes the patient to require more effort to expel stool. This pressure is produced by the modified valsalva manovoure (attempted forced exhalation against a closed glottis, resulting in increased abdominal and intra-rectal pressure). Patiest with SRUS were shown to have higher intra-rectal pressures when straining than healthy controls.[2] SRUS is also associated with prolonged and incomplete evacuation of stool.[3]
  • More effort is required because of concomitant anismus, or non-relaxation/paradoxical contraction of puborectalis (which should normally relax during defecation).[4] The increased pressure forces the anterior rectal lining against the contracted puborectalis and frequently the lining prolapses into the anal canal during straining and then returns to its normal position afterwards.
  • The repeated trapping of the lining can cause the tissue to become swollen and congested. Ulceration is thought to be caused by resulting poor blood supply (ischemia), combined with repeated frictional trauma from the prolapsing lining, and exposure to increased pressure are thought to cause ulceration. Trauma from hard stools may also contribute.
  • The site of the ulcer is typically on the anterior wall of the rectal ampulla, about 7–10 cm from the anus. However, the area may of ulceration may be closer to the anus, deeper inside, or on the lateral or posterior rectal walls. The name "solitary" can be misleading since there may be more than one ulcer present. Furthermore, there is a "preulcerative phase" where there is no ulcer at all.[5]
  • Pathological specimens of sections of rectal wall taken from SRUS patients show thickening and replacement of muscle with fibrous tissue and excess collagen.

[6] Rarely, SRUS can present as polyps in the rectum.[7][8]

  • SRUS is therefore associated and with internal, and more rarely, external rectal prolapse.[3] Some believe that SRUS represents a spectrum of different diseases with different etiologies.[9]
  • Another condition associated with internal intussusception is colitis cystica profunda (also known as CCP, or proctitis cystica profunda), which is cystica profunda in the rectum. Cystica profunda is characterized by formation of mucin cysts in the muscle layers of the gut lining, and it can occur anywhere along the gastrointestinal tract. When it occurs in the rectum, some believe to be an interchangeable diagnosis with SRUS since the histologic features of the conditions overlap.[10][11] Indeed, CCP is managed identically to SRUS.

Differentiating Solitary Rectal Ulcer Syndrome from other Diseases

The differential diagnosis of SRUS (and CCP) includes:

Natural History, Complications and Prognosis

Complications are uncommon, but include massive rectal bleeding, ulceration into the prostate gland or formation of a stricture. Very rarely, cancer can arise on the section of prolapsed rectal lining.

Diagnosis

History and Symptoms

Symptoms include:

Laboratory Findings

  • SRUS is commonly misdiagnosed, and the diagnosis is not made for 5–7 years.[1] Clinicians may not be familiar with the condition, and treat for Inflammatory bowel disease, or simple constipation.[12][13]
  • The thickened lining or ulceration can also be mistaken for types of cancer.[14][15][16][17]

Treatment

Although SRUS is not a medically serious disease, it can be the cause of significantly reduced quality of life for patients. It is difficult to treat, and treatment is aimed at minimizing symptoms.

  • Surgery may be considered, but only if non surgical treatment has failed and the symptoms are severe enough to warrant the intervention. Improvement with surgery is about 55-60%.[22]
  • Ulceration may persist even when symptoms resolve.[23]

References

  1. 1.0 1.1 Ertem, D; Acar, Y; Karaa, EK; Pehlivanoglu, E (December 2002). "A rare and often unrecognized cause of hematochezia and tenesmus in childhood: solitary rectal ulcer syndrome". Pediatrics. 110 (6): e79. PMID 12456946.
  2. Womack, NR; Williams, NS; Holmfield, JH; Morrison, JF (October 1987). "Pressure and prolapse--the cause of solitary rectal ulceration". Gut. 28 (10): 1228–33. PMC 1433454. PMID 3678951.
  3. 3.0 3.1 Halligan, S; Nicholls, RJ; Bartram, CI (January 1995). "Evacuation proctography in patients with solitary rectal ulcer syndrome: anatomic abnormalities and frequency of impaired emptying and prolapse". AJR. American journal of roentgenology. 164 (1): 91–5. doi:10.2214/ajr.164.1.7998576. PMID 7998576.
  4. Van Outryve, MJ; Pelckmans, PA; Fierens, H; Van Maercke, YM (October 1993). "Transrectal ultrasound study of the pathogenesis of solitary rectal ulcer syndrome". Gut. 34 (10): 1422–6. PMC 1374554. PMID 8244113.
  5. Madigan, MR; Morson, BC (November 1969). "Solitary ulcer of the rectum". Gut. 10 (11): 871–81. PMC 1553062. PMID 5358578.
  6. Kang, YS; Kamm, MA; Engel, AF; Talbot, IC (April 1996). "Pathology of the rectal wall in solitary rectal ulcer syndrome and complete rectal prolapse". Gut. 38 (4): 587–90. PMC 1383120. PMID 8707093.
  7. Brosens, LA; Montgomery, EA; Bhagavan, BS; Offerhaus, GJ; Giardiello, FM (November 2009). "Mucosal prolapse syndrome presenting as rectal polyposis". Journal of clinical pathology. 62 (11): 1034–6. doi:10.1136/jcp.2009.067801. PMC 2853932. PMID 19861563.
  8. Saadah, OI; Al-Hubayshi, MS; Ghanem, AT (2010-08-15). "Solitary rectal ulcer syndrome presenting as polypoid mass lesions in a young girl". World journal of gastrointestinal oncology. 2 (8): 332–4. doi:10.4251/wjgo.v2.i8.332. PMC 2999680. PMID 21160895.
  9. Kang, YS; Kamm, MA; Nicholls, RJ (1995). "Solitary rectal ulcer and complete rectal prolapse: one condition or two?". International journal of colorectal disease. 10 (2): 87–90. PMID 7636379.
  10. Vora, IM; Sharma, J; Joshi, AS (April 1992). "Solitary rectal ulcer syndrome and colitis cystica profunda--a clinico-pathological review". Indian journal of pathology & microbiology. 35 (2): 94–102. PMID 1483723.
  11. Levine, DS (January 1987). ""Solitary" rectal ulcer syndrome. Are "solitary" rectal ulcer syndrome and "localized" colitis cystica profunda analogous syndromes caused by rectal prolapse?". Gastroenterology. 92 (1): 243–53. PMID 3536653.
  12. Blackburn, C; McDermott, M; Bourke, B (February 2012). "Clinical presentation of and outcome for solitary rectal ulcer syndrome in children". Journal of pediatric gastroenterology and nutrition. 54 (2): 263–5. doi:10.1097/MPG.0b013e31823014c0. PMID 22266488.
  13. Umar, SB; Efron, JE; Heigh, RI (2008-09-30). "An interesting case of mistaken identity". Case reports in gastroenterology. 2 (3): 308–13. doi:10.1159/000154816. PMC 3075189. PMID 21490861.
  14. Amaechi, I; Papagrigoriadis, S; Hizbullah, S; Ryan, SM (November 2010). "Solitary rectal ulcer syndrome mimicking rectal neoplasm on MRI". The British journal of radiology. 83 (995): e221–4. doi:10.1259/bjr/24752209. PMC 3473720. PMID 20965892.
  15. Lokuhetty, D; de Silva, MV; Mudduwa, L (December 1998). "Solitary rectal ulcer syndrome (SRUS) masquerading as a carcinomatous stricture". The Ceylon medical journal. 43 (4): 241–2. PMID 10355182.
  16. Blanco, F; Frasson, M; Flor-Lorente, B; Minguez, M; Esclapez, P; García-Granero, E (November 2011). "Solitary rectal ulcer: ultrasonographic and magnetic resonance imaging patterns mimicking rectal cancer". European journal of gastroenterology & hepatology. 23 (12): 1262–6. doi:10.1097/MEG.0b013e32834b0dee. PMID 21971372.
  17. Levine, DS; Surawicz, CM; Ajer, TN; Dean, PJ; Rubin, CE (November 1988). "Diffuse excess mucosal collagen in rectal biopsies facilitates differential diagnosis of solitary rectal ulcer syndrome from other inflammatory bowel diseases". Digestive diseases and sciences. 33 (11): 1345–52. PMID 2460300.
  18. Bishop, PR; Nowicki, MJ (June 2002). "Nonsurgical Therapy for Solitary Rectal Ulcer Syndrome". Current treatment options in gastroenterology. 5 (3): 215–223. PMID 12003716.
  19. van den Brandt-Grädel, V; Huibregtse, K; Tytgat, GN (November 1984). "Treatment of solitary rectal ulcer syndrome with high-fiber diet and abstention of straining at defecation". Digestive diseases and sciences. 29 (11): 1005–8. PMID 6092015.
  20. Jarrett, ME; Emmanuel, AV; Vaizey, CJ; Kamm, MA (March 2004). "Behavioural therapy (biofeedback) for solitary rectal ulcer syndrome improves symptoms and mucosal blood flow". Gut. 53 (3): 368–70. PMC 1773992. PMID 14960517.
  21. Vaizey, CJ; Roy, AJ; Kamm, MA (December 1997). "Prospective evaluation of the treatment of solitary rectal ulcer syndrome with biofeedback". Gut. 41 (6): 817–20. PMC 1891593. PMID 9462216.
  22. Sitzler, PJ; Kamm, MA; Nicholls, RJ; McKee, RF (September 1998). "Long-term clinical outcome of surgery for solitary rectal ulcer syndrome". The British journal of surgery. 85 (9): 1246–50. doi:10.1046/j.1365-2168.1998.00854.x. PMID 9752869.
  23. Vaizey, CJ; van den Bogaerde, JB; Emmanuel, AV; Talbot, IC; Nicholls, RJ; Kamm, MA (December 1998). "Solitary rectal ulcer syndrome". The British journal of surgery. 85 (12): 1617–23. doi:10.1046/j.1365-2168.1998.00935.x. PMID 9876062.


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