By Julian Ong Endoscopy & Surgery (Pte Ltd)

05/20/2020

This is an Accepted Article that has been peer-reviewed and approved for publication in the Colorectal Disease, but has yet to undergo copy-editing and proof correction. Please cite this article as an “Accepted Article”; doi: 10.1111/j.1463-1318.2010.02261.x Received Date : 04-Nov-2009 Revised Date : 15-Jan-2010 Accepted Date : 21-Jan-2010 Article type : Original Article 590-2009.R1 Original Article Solitary Caecal Ulcer Syndrome: Our Experience with this Benign Condition Julian Ong, Lim KH, Lim JF, Eu KW Julian Ong, FRCSEd (Gen)1 Associate Consultant Surgeon Lim Kiat-Hon, FRCPath (UK)2 Consultant Pathologist Lim Jit-Fong, FRCS (Ed)1 Consultant Surgeon Eu Kong-Weng, FRCS (Ed)1 Senior Consultant Surgeon and Head of Department 1 Department of Colorectal Surgery, Singapore General Hospital, Outram Road, Singapore (169608) 2 Department of Pathology, Singapore General Hospital, Outram Road, Singapore (169608) Corresponding Author : Lim Jit Fong and reprint requests Consultant Surgeon Department of Colorectal Surgery Singapore General Hospital Outram Road Singapore (169608) Email address: lim.jit.fong@sgh.com.sg Running header : Solitary Caecal Ulcer Syndrome Key Words : Solitary Caecal Ulcer Syndrome, Benign, Idiopathic Abstract Purpose Solitary, sometimes termed idiopathic, caecal ulcer syndrome is rare. Its aetiology is unknown. We describe our experience of ten patients with the condition. Methods A prospectively collected database was reviewed from 1999 to 2008 of patients undergoing colonoscopy or surgery with histology reporting a solitary caecal ulcer . Patients with known carcinoma of the colon, cytomegalovirus infection, amoebiasis, inflammatory bowel disease, immunosuppression and history of non-steroidal anti-inflammatory drug use were excluded. Results Ten patients were found to have a solitary caecal ulcer. All were of Chinese ethnicity of median age of 61 years. The most common presenting symptoms were hematochezia and right sided abdominal pain. Five patients underwent surgery and five were treated with oral antibiotics. Histological findings include ulceration extending sharing some features of solitary rectal ulcer syndrome, but with significant differences to suggest a different etiology. Conclusion Solitary caecal ulcer syndrome should be included in the differential diagnosis of lower gastrointestinal haemorrhage, right iliac fossa pain or when CT imaging demonstrates caecal wall thickening. The diagnosis can only be made on histopathological examination. Background Solitary, sometimes termed idiopathic, caecal ulcer syndrome is rare. The aetiology is unknown, and there are no pathognomonic symptoms. The clinical presentation is varied, but bleeding and pain are common. Imaging with contrast studies or computed tomography (CT) may suggest carcinoma of the caecum. The definitive diagnosis is made only on histological examination, and in the absence of carcinoma, immunosuppression, non-steroidal anti-inflammatory drug (NSAID) use, cytomegalovirus infection, amoebiasis, and inflammatory bowel disease. Early and precise diagnosis allows a conservative approach to management. We describe our experience of 10 patients with histology confirming a benign caecal ulcer, in the absence of the above-mentioned conditions. Method Our prospective database of patients undergoing colonoscopy or surgery with histology reporting a solitary caecal ulcer from 1999 to 2008 was reviewed. Patients with known carcinoma of the colon, cytomegalovirus infection, amoebiasis, immunosuppression, NSAID medication and inflammatory bowel disease were excluded. Results Ten patients were found with solitary caecal ulcer between 1999 and 2008. Its benign nature on biopsy of the lesion at colonoscopy or on histologic examination of the resected specimen after surgery. All patients were of Chinese ethnicity (5 males,) of median age 61 (range 52-89) years. The medical, drug and surgical history is summarized in Table 1. Of note, no patient gave any history of NSAID or potassium channel blocker medication, The most common symptom at presentation was hematochezia, followed by right sided abdominal pain (Table 1). Colonoscopy was the most common diagnostic procedure performed (Table 2). Five patients underwent surgery for this condition, three because of a high suspicion of malignancy, one for increasing abdominal pain, and one for severe rectal bleeding. Four patients recovered well, but the patient presenting with severe rectal bleeding succumbed on the second post-operative day after total abdominal colectomy to an acute myocardial infarction secondary to anaemia. The other five patients who had an ulcer with a benign appearance on colonoscopy were treated with oral antibiotics. Healing was subsequently confirmed on a repeat colonoscopic examination. Histopathologic Findings The histology in all 10 patients showed ulceration with granulation tissue and fibrinous exudate extending into the submucosa, in one ulceration had reached the muscularis propria. Submucosal abscesses were noted in half (5) of the cases. Disruption of the muscularis mucosae (MM) was noted in 9 out of 10 patients with thickening in half the patients. In two patients there was smooth muscle proliferation. One case had hyperplastic mucosal glands, while the rest showed evidence of mucosal regeneration. No features of ischaemia were seen in any patient. Discussion Benign colonic ulcers have been found most commonly in the caecum and ascending colon and rarely involve the hepatic flexure (1). Solitary caecal ulcer is seen with increasing frequency as the use of colonoscopy becomes more common(2). An increased incidence has been reported in patients with chronic renal failure, especially those requiring hemodialysis or after renal transplantation(3). Our small series indicates that solitary caecal ulcer occurs in patients in the sixth to ninth decades of life (median 61 years) and has an equal gender distribution. Previous reports have described the condition to occur usually in the fourth to sixth decades of life with a slight female predominance(4,5). The solitary rectal ulcer syndrome (SRUS) is characterized histologically by hyperplastic glands accompanied by smooth muscle proliferation and ulceration with granulation tissue and fibrinous exudate. Disruption of the muscularis mucosae is not characteristic of SRUS and thickening is not always seen. The smooth muscle bundles that are present in SRUS are typically aligned perpendicular to the mucosal surface and not parallel to the muscularis mucosae. Although solitary caecal ulcer shows superficial similarities to SRUS, such as ulceration with granulation tissue and fibrinous exudates, extension of the ulcer into the submucosa is unusual for SRUS. Disruption and thickening of the muscularis mucosae often seen in caecal ulcer syndrome is not typical of SRUS. The hyperplastic glands and smooth muscle proliferation seen in SRUS are only very rarely seen in caecal ulcer syndrome. Possible causes of solitary caecal ulcer, such as infection, drugs and neoplasia, were discussed by Chi et al (6).They have been attributed to infections such as cytomegalovirus(7,8), campylobacter jejuni(9) and E. histolytica(10). Drugs implicated include non-steroidal inflammatory agents and oral contraceptives(11), particularly Ibuprofen(12,13) and oxyphenbutazone(14). Neoplastic conditions causing caecal ulceration are common and must always be considered as in the differential diagnosis (6) and primary carcinoma of the caecum mimicking idiopathic caecal ulceration have been described(15). Caecal ulcers caused by carcinoid tumours have also been reported (16). Chronic drug abuse, peptic ulcer disease, colonic stasis, local ischaemia, atherosclerosis, and strongyloidiasis should also be excluded before a diagnosis of idiopathic caecal ulcer syndrome is made. Solitary caecal ulcer is often located within 2cm of the ileocaecal valve on the antimesenteric wall of the colon(17). The patient can present with an acute abdomen due to perforation and abscess formation, or with chronic right sided abdominal pain, with or without an inflammatory mass. The condition may therefore be mistaken for acute appendicitis. Chronic abdominal pain or gastrointestinal hemorrhage may raise concern of diverticulosis or occult colonic carcinoma(2,4,6,18). In our series, the most common clinical presentation was bleeding and right sided abdominal pain. While solitary caecal ulcer shares some histological similarity to SRUS, significant differences in their appearance may suggest a different aetiology. Given that most of the ulcers are at the base of the appendix, mucosal prolapse does not seem likely. In our series, three cases show increased eosinophil infiltrition which may suggest an allergic or inflammatory etiology. The combination of physical, infectious, allergic or chemical causes remains a hypothesis. Solitary caecal ulcers may be diagnosed at the time of surgery for acute abdomen or suspected appendicitis(2). Investigations such as barium enema examination, are often unreliable and may not differentiate benign from malignant conditions. A benign ulcer can cause thickening of the caecal wall, making diagnosis on ultrasound or CT difficult (1,2,12,19). The management of patients with idiopathic caecal ulcer depends largely on the clinical presentation. Patients presenting with an acute abdomen should have an emergency surgical exploration. If a caecal ulcer is diagnosed on colonoscopy in a stable patient without evidence of peritonitis, the ulcer should be biopsied along with random biopsies throughout the colon to exclude an infective or inflammatory cause of ulceration(6). If there is a suspicion of malignancy in the initial or subsequent biopsies, surgery is then essential. The difficulty in differentiating benign from malignant ulceration has led many surgeons to advocate definitive surgical management with right hemicolectomy. If there is no overwhelming evidence of malignancy, an ulcer discovered at the time of surgery may be treated with limited resection or caecectomy with frozen section to confirm its benign nature (17). Close follow up with early repeat colonoscopy is recommended in patients with benign histology (2). Patients presenting with severe gastrointestinal bleeding secondary to caecal ulceration may require surgery if they are unstable with uncontrollable haemorrhage(6). In recent years, superselective embolization of visceral arterial branches has been available for the management of acute lower gastrointestinal (GI) hemorrhage. A recent review showed technical success in 97% of patients with 3% incidence of post-embolization ischaemia. This therapeutic approach was definitive in 63% of patients, with no recurrent haemorrhage(20). Conclusion References 1. Ona FV, Allende HD, Vivenzio R, Zaky DA, Nadaraja N. Diagnosis and management of nonspecific colon ulcer. Arch Surg 1982;117:888-94. 2. Blundell CR, Earnest DL. Idiopathic caecal ulcer. Diagnosis by colonoscopy followed by nonoperative management. Dig Dis Sci 1980;25:494-503 3. McCarthy JH, Beveridge BR. Solitary caecal ulcer as a cause of gastrointestinal bleeding. Med J Aust 141:530-531, 1984. 4. Koçdor MA, Aydin C, Astarcioğlu H, Küpelioğlu A, Bora S. Perforated solitary ulcer of the colon. Report of a case. Dis Colon Rectum 1998;41:1059-61. 5. Cameron JR. Simple nonspecific ulcer of the caecum. Br J Surg 1939;25:494-503. 6. Chi KD, Hanauer SB. Benign Solitary Caecal Ulcer: A case report and review of the literature. Dig Dis Sci 2003;48:2207-12. 7. Rosen-Levin EM, Schwartz IS. Solitary caecal ulcer due to cytomegalovirus in a leukaemic patient. Mt Sinai J Med 52:139-141, 1985. 8. Pfau P, Rothstein RD. Cytomegalovirus caecal ulcer in a patient awaiting cardiac transplantation. Am J Gastroenterol 91:2435-2436. 1996 9. Alloy AM, Santoro JJ, Lazarus BG, Chiesa JC, Pecora AA. Campylobacter fetus ss. Jejuni: a cause of solitary caecal ulcer. J Clin Gastroenterol 8:605, 1986 10. Yoshikawa I, Murata I, Yano K, Kume K, Otsuki M. Asymptomatic amebic colitis in a homosexual man. Am J Gastroenterol 94:2306-2308. 1999 11. Bernardino ME, Lawson TL. Discrete colonic ulcers associated with oral contraceptives. Am J Dig Dis 21:503-506, 1976. 12. Crosby JC, Ross GJ, Auchenbach RC, McCafferty MH. Nonsteroidal anti-inflammatory drug-induced caecal ulceration diagnosed on barium enema. J Clin Gastroenterol 1998;26:154-6. 13. Charuzi I, Ovnat A, Zirkin H, Peiser J, Sukenik S. Ibuprofen and benign caecal ulcer. J Rheumatol 12:188-189. 1985 14. Debenham GP. Ulcer of the caecum during oxyphenbutazone (Tandearil) therapy. Can Med Assoc J 94:1182-1184, 1966 15. Jae Myung Cha, Joung Il Lee, Jae Won Choe. A Case Report of Idiopathic Caecal Ulcer Mimicking Caecal Cancer. Dig Dis Sci 2008;53:3259-3262. 16. Pao-Huei C, Chuan-Pau S, Kuang-Yang L, Gong-Chin H, Jean-Dean L, Hsian-Chong K, Ting-Yao C. Carcinoids of the gastrointestinal tract. Endoscopy 12:299-305, 1980. 17. Shallman RW, Kuehner M, Williams GH, Sajjad S, Sautter R. Benign caecal ulcers. Spectrum of disease and selective management. Dis Colon Rectum 1985;28:732-7. 18. Damiani S, Ruscazio M, Ciulla A, Miceli G, Tomasello G, Cerasa G. The solitary benign ulcer of the colon: a report of a case located in the caecum. G Chir 1998;19:381-5. 19. Rao PM, Novelline RA, Zukerberg L. Solitary caecal ulcer syndrome, a benign condition which mimics the CT appearance of Caecal Carcinoma. Clin Radiol 1999;54:331-3. 20. Tan KK, Wong D, Sim R. Superselective embolization for lower gastrointestinal hemorrhage: an institutional review over 7 years. World J Surg. 2008 Dec;32(12):2707- 15. Table legend Table 1 Demography, concomitant medical conditions, drug history and clinical presentation Table 2 Summary of colonoscopy and biopsy findings, definitive treatment, and final histology Figure legend Figure 1 Endoscopic photograph of a solitary benign caecal ulcer Figure 2 Benign ulceration with fibrinous exudate Figure 3 Smooth muscle proliferation in the muscularis mucosae Figure 4 Disruption of the muscularis mucosae Table 1 Demography, concomitant medical conditions, drug history and clinical presentation. Pt Age Sex Medical history Surgical history Drugs Presenting complaints 1 52 Male Nil Right renal stone Antibiotics. No NSAIDs Bloody diarrhea 2 66 Male DM, HPT Appendicectomy Nil. No NSAIDs Right abdominal pain, CT abdomen/pelvis done 3 89 Male Old CVA, IHD, CRF, HPT, AAA Bilroth I gastrectomy Vasteral, Norvasc, GTN, Lactulose, Plavix, Losec, Carvedilol, Imdur, Pravachol. No NSAIDs Severe fresh per rectal bleeding 4 61 Female Nil Anterior resection for carcinoma rectum Nil. No NSAIDs Bloody diarrhea 5 79 Female Hyperthyroidism, CRF, HPT Nil Carbimazole, Adalat, Famotidine, Frusemide. No NSAIDs RIF pain. CT abdomen/pelvis showed thickening of the caecum 6 56 Female Hyperthyroidism. RAI given Previous LSCS, tubal ligation Thyroxine, previous RAI. No NSAIDs Black stools 7 61 Female HPT Nil Atenolol, Hydrochlorothiazide. No NSAIDs Bloody diarrhea 8 70 Male PTB, Ex smoker, alcohol drinker, Graves' disease, RAI given Appendicectomy Previous RAI, Carbimazole. No NSAIDs Abd pain, tender RIF mass. CT abdomen/pelvis done 9 54 Female Nil Nil Nil Rectal bleeding 10 58 Male HPT ESWL Nifedipine Asymptomatic, screening NSAIDs, nonsteroidal anti-inflammatory drugs DM, diabetes mellitus HPT, hypertension CVA, cerebrovascular accident IHD, ischemic heart disease CRF, chronic renal failure AAA, abdominal aortic aneurysm RAI, radioactive iodine PTB, pulmonary tuberculosis LSCS, lower segment caesarean section CT, Computer Tomography RIF, right iliac fossa Table 2 Summary of colonoscopy and biopsy findings, definitive treatment and final histology. Pt Presentation Colonoscopy findings Biopsy findings Definitive treatment Final Histology 1 Bloody diarrhea Large ileo-caecal ulcer Acute self-limited enterocolitis Right hemicolectomy Acute enterocolitis with perforation. Discrete ulcer in caecum 2 Right abdominal pain Hard irregular caecal lesion Acute on chronic inflammation with ulcer base material Right hemicolectomy Acute suppuration with mucosal ulceration 3 Fresh rectal bleeding Bleeding caecal ulcer, diverticulosis Nil done Total colectomy Benign colonic ulcer, no evidence of granulomas, malignancy or ischemia 4 Bloody diarrhea Acute ulcer on ileocaecal valve Ileocaecal ulcer: Small superficial erosion and edematous lamina propria Conservative NA 5 RIF pain, CT A/P showed thickening of caecum Caecal ulcer Ileocaecal ulcer: Ulcer, nonspecific, with repair effects Conservative NA 6 Black stools Hard ulcer in caecum Caecum: nonspecific colitis Right hemicolectomy Ulcer, caecum, extending to the muscularis propria with adjacent chronic inflammation of caecal wall and appendix. 7 Bloody diarrhea Benign looking ulcer on ileocaecal valve Benign ulcer Conservative NA 8 Abd pain, tender RIF mass RIF mass on CT abdomen/pelvis Right hemicolectomy Benign caecal ulcer with features suggestive of ischemia 9 Rectal bleeding Benign looking ulcer at ileocaecal valve Focal active chronic ulceration Conservative NA 10 Asymptomatic, screening Caecal ulcer 1 – 2cm away from ileocaecal valve Non-specific acute inflammation Conservative NA RIF, right iliac fossa


Received Date : 04-Nov-2009 Revised Date : 15-Jan-2010 Accepted Date : 21-Jan-2010 Article type : Original Article 590-2009.R1 Original Article Solitary Caecal Ulcer Syndrome: Our Experience with this Benign Condition Julian Ong, Lim KH, Lim JF, Eu KW Julian Ong, FRCSEd (Gen)1 Associate Consultant Surgeon Lim Kiat-Hon, FRCPath (UK)2 Consultant Pathologist Lim Jit-Fong, FRCS (Ed)1 Consultant Surgeon Eu Kong-Weng, FRCS (Ed)1 Senior Consultant Surgeon and Head of Department 1 Department of Colorectal Surgery, Singapore General Hospital, Outram Road, Singapore (169608) 2 Department of Pathology, Singapore General Hospital, Outram Road, Singapore (169608) Corresponding Author : Lim Jit Fong and reprint requests Consultant Surgeon Department of Colorectal Surgery Singapore General Hospital Outram Road Singapore (169608) Email address: lim.jit.fong@sgh.com.sg Running header : Solitary Caecal Ulcer Syndrome Key Words : Solitary Caecal Ulcer Syndrome, Benign, Idiopathic

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