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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 6  |  Issue : 4  |  Page : 146-150

Bizarre presentation of solitary rectal ulcer syndrome mimicking rectal malignancy with impending bowel obstruction


1 Department of General Surgery, King Fahd Central Hospital, Jazan, Saudi Arabia
2 Department of Histopathology, King Fahd Central Hospital, Jazan, Saudi Arabia

Date of Web Publication13-Dec-2018

Correspondence Address:
Dr. Khaled Elsayed Elshaar
Department of General Surgery, King Fahd Central Hospital, Jazan 45196, B.O. 8123
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ssj.ssj_30_18

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  Abstract 

Solitary rectal ulcer syndrome (SRUS) is a rare benign and chronic rectal disease that has a wide spectrum of clinical presentations with variable endoscopic findings. It mostly occurs between 20 and 29 years of ages and gives a significant female preponderance. A 23-year-old female presented with 2 years history of constipation and bleeding per rectum, with a recent complaint of tenesmus, inability to defecate along with abdominal distention, and vomiting. She was on mesalazine as advised by the gastroenterologist, without improvement. Contrast-enhanced computed tomography (CECT) abdomen showed circumferential poorly enhancing 8 cm rectal mass starting 10 cm from the anal verge with multiple mesorectal lymphadenopathy. Colonoscopy showed hard, ulcerating rectal mass obstructing the lumen, highly suspicious for malignancy. Endoscopic biopsy showed the features suggesting SRUS. However, as the patient had impending colonic obstruction along with the doubtful presence of underlying malignancy, we prepared her for exploratory laparotomy, which revealed a hard midrectum 5 cm × 5 cm × 6 cm mass, so low anterior resection had done with stapled colorectal anastomosis. Paraffin section proved the diagnosis of SRUS. SRUS should always be considered in young patients with malignant-mimicking rectal mass. A differential diagnosis of SRUS should always be kept in mind for mural thickening of the rectum on CT. However, it is important not to miss a diagnosis of rectal cancer over the diagnosis of SRUS.

Keywords: Colonic obstruction, rectal malignancy, solitary rectal ulcer syndrome


How to cite this article:
Elshaar KE, AbuAleid LH, Mikhail NT. Bizarre presentation of solitary rectal ulcer syndrome mimicking rectal malignancy with impending bowel obstruction. Saudi Surg J 2018;6:146-50

How to cite this URL:
Elshaar KE, AbuAleid LH, Mikhail NT. Bizarre presentation of solitary rectal ulcer syndrome mimicking rectal malignancy with impending bowel obstruction. Saudi Surg J [serial online] 2018 [cited 2019 Jan 18];6:146-50. Available from: http://www.saudisurgj.org/text.asp?2018/6/4/146/247417


  Introduction Top


Solitary rectal ulcer syndrome (SRUS) is a rare benign and chronic rectal disease that has a wide spectrum of clinical presentations and variable endoscopic findings,[1] including mucosal ulcerations, polypoid, and mass lesions mimicking rectal cancer.[2]

The underlying etiology of SRUS is not fully understood, but it is likely to be secondary to ischemic changes in the rectum associated with paradoxical contraction of the puborectalis muscle.[3] The most common symptoms are rectal bleeding (56%), straining (28%), and pelvic fullness (23%).[4]

We report a rare complex case of SRUS who had a circumferential ulcerated rectal mass that mimicked the inflammatory bowel disease at initial presentation, based on endoscopic and histopathological picture, and mimicked rectal cancer on further follow-up, based on clinical, gross endoscopic as well as radiologic findings, with impending bowel obstruction, ended by low anterior resection.


  Case Report Top


Twenty three years old female patient, not known to have chronic medical illness, presented initially to gastroenterology clinic, during her first trimester, complained of bleeding per rectum associated with chronic constipation with loss of 13 kg in the preceding 2 years without diet regimen. Because of pregnancy status, colonoscopy not done.

After delivery, she attended the gastro clinic again; colonoscopy done for her showed severe inflammatory process involved the rectum and sigmoid colon with hyperemic mucosa. That picture at that time was in favor of inflammatory bowel disease. Histopathology of the rectal biopsy showed chronic active inflammation with regenerative atypical glands, rare cryptitis, and superficial ulceration, but no granuloma or crypt abscess and no malignancy seen, again the picture was suspicious for inflammatory bowel disease. Hence, her physician started her on oral 5-aminosalicylic acid (mesalazine).

After few weeks, she came for follow-up in the gastro clinic and explained that her complaints did not improve, besides, she developed hair loss as a side effect of the medication.

Her physician repeated the colonoscopy [Figure 1]a, which showed at that time an ulcerating rectal mass with narrowed lumen. This picture raised the suspicion of rectal malignancy versus SRUS.
Figure 1: Endoscopic imaging and corresponding histological findings.(a) Endoscopic view shows the ulcerated rectal mass with narrowed lumen. (b) Hematoxylin and eosin stain of endoscopic biopsy showed fibrosis of the lamina propria, thickened muscularis mucosae, and haphazardly arranged crypts

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Colonoscopic biopsy from the ulcerating lesion showed fibrosis of the lamina propria, thickened muscularis mucosae, haphazardly arranged crypts, and no evidence of malignancy. The overall features were in favor of solitary rectal ulcer [Figure 1]b.

The gastroenterologist put the patient on budesonide foam, per rectal corticosteroid, once daily, and mesalazine enema (5-aminosalicylic acid derivatives) along with conservative measures in the form of avoidance of straining, use of a high-fiber diet, in combination with stool softeners and bulk-forming laxatives. After few months of treatment, the patient did not feel much improvement, although the frequency of rectal bleeding had decreased, she developed a new complaint of tenesmus. At that stage, the systemic examination of the patient was unremarkable apart from loss of weight and she refused per rectal examination.

All routine laboratory investigations were within normal values apart from iron deficiency anemia.

CECT abdomen and pelvis done showed a circumferentially poorly enhancing rectal mass 10 cm above the anal verge which extended 8 cm cranially with shoulder sign and multiple locoregional pelvic lymphadenopathy [Figure 2]. These radiological features were coinciding with neoplastic process of the rectum.
Figure 2: Contrast-enhanced computed tomography abdomen and pelvis shows. (a) Sagittal view with evidence of the rectal mass, marked by arrows, in the midrectum. (b) Coronal view shows circumferential narrowing of the rectum. (c) Axial cut shows the “shoulder sign” effect of the mass

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The gastro team admitted the patient to exclude an underlying malignancy and repeat colonoscopy, for the 3rd time, was done [Figure 3], which showed an ulcerative rectal mass 10 cm above the anal verge with friable surface. Biopsy showed again SRU with marked fibrosis. At that stage, a surgical consultation had been sent to us as the patient developed inability to pass stool, abdominal distension and recurrent vomiting, and features of low colonic obstruction.
Figure 3: Endoscopic pictures show the hard nodular ulcerated rectal mass

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After obtaining informed consent, the patient had been prepared for low anterior resection of the rectum with proximal colostomy.

Operative notes: Under general anesthesia and in lithotomy position, rigid sigmoidoscopy done showed an obstructing hard mass 10 cm above the anal verge, the scope did not pass beyond that point. Exploratory laparotomy through lower midline incision revealed a 5 cm × 6 cm × 8 cm hard midrecral mass involved the whole circumference of the rectum but more prominent in the anterior rectal wall [Figure 4].
Figure 4: Operative findings. (a) The instrument points the upper margin of the rectal mass. (b) The instrument points the lower margin of the mass.(c) The stapler transects through lower rectum distally

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We proceeded for low anterior resection of the rectum, with end-to-end colorectal anastomosis using circular stapler. The rectal mesentery, which harbored significantly enlarged lymph nodes, >2 cm size, had been resected also. Proximal protecting loop colostomy was performed.

The rectal specimen and both donuts sent for paraffin as well as frozen section, which confirmed the diagnosis of SRUS with marked hypertrophy of muscularis propria [Figure 5].
Figure 5: Trichrome stain of the resected rectal mass shows crypts distortion with hyperplasia, accumulation of muscle fibers and fibroblast in lamia propria, submucosal thickening with fibrosis, and marked hypertrophy of muscularis propria

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Postoperative course was uneventful and the patient discharged home after few days. Later on, her colostomy had been reversed.


  Discussion Top


SRUS is an infrequent and underdiagnosed disorder, with an estimated annual prevalence of one in 100,000 persons. It is a disorder of young adults, occurring most commonly in the third decade in men and in the fourth decade in women. Men and women are affected equally, with a small predominance in women.[5]

SRUS might be a misnomer because patients can present with lesions that are neither solitary nor ulcerated and not necessarily restricted to the rectum.[4]

The pathogenesis of SRUS is incompletely understood. However, a common observation in a number of reports is rectal prolapse and paradoxical contraction of the puborectalis muscle, which can result from rectal trauma or chronic constipation as well as excessive straining that generates a high-intrarectal pressure, which in part pushes the anterior rectal mucosa onto the contracting puborectalis muscle, resulting in pressure necrosis of the mucosa, congestion, edema, and ulceration.[6],[7] However, excessive puborectalis contraction is not found in all patients with SRU.[8]

Clinically, SRU may be asymptomatic or associated with rectal bleeding, copious mucus discharge, prolonged excessive straining, perineal and abdominal pain, feeling of incomplete defecation, constipation, and rarely, rectal prolapse.[9],[10]

The most distressing complaints of our patient were bleeding per rectum, constipation, and tenesmus; later she developed symptoms of bowel obstruction. This worsening of her condition could be attributed to delayed diagnosis at early stage of her disease or due to the worsening of constipation which aggravated by her pregnancy.

The diagnosis of SRUS is usually based on clinical symptoms combined with the endoscopic findings; however, the cornerstone for diagnosis is the histopathological findings with the characteristic appearance of a thickened mucosal layer, with distortion of the crypt architecture leading to hypertrophy and disorganization of the muscularis mucosa, an appearance that has been referred to as “fibromuscular obliteration.”[11]

SRU can be misdiagnosed for a lot of other pathologies affecting the rectum such as inflammatory bowel syndrome and rectal malignancy, as in our case.

In a retrospective review of 80 patients with SRUS, Tjandra et al. noted that 25% of patients' conditions were misdiagnosed for other pathological entities at the time of initial assessment, proving that this syndrome can be easily missed.[12]

Our patient initially misdiagnosed as inflammatory bowel disease, the cause underlying might be, in our opinion, due to obtaining a superficial biopsy during endoscopy.

The options of treatment selected will differ depending on the clinical and endoscopic findings and on the severity of symptoms. Conservative management is appropriate for patients who experience only mild symptoms but do not have rectal prolapse. This may include stool bulking agents and retraining of bowel habits using biofeedback. The goals of therapy are to manage constipation and to prevent paradoxical puborectalis contraction, if present. In one study, these measures were successful in 19% of patients.[12]

Topical treatments, including sucralfate, salicylate, corticosteroids, sulfasalazine, mesalazine, and topical fibrin sealant, have been reported to be effective with various responses and improvement of symptoms.[10] Sucralfate enema contains aluminum complex salts, which coat the rectal ulcer and form a barrier against irritants, allowing the ulcer to heal. Corticosteroids and sulfasalazine enemas may also help ulcer healing by reducing the inflammatory responses. However, these treatments are empirical and have been applied in uncontrolled studies, and their long-term benefits deserve further investigation.[13],[14]

Certainly, our patient did not respond to conservative and local treatment, in spite, her condition deteriorated more.

Surgery is appropriate for patients with full-thickness or mucosal rectal prolapse or with symptoms unresponsive to conservative management.[15] Surgical options include local excision, diversion, or rectopexy.[16]

In our patient, however, the presentation was bizarre from the point of clinical and endoscopic finding as well as the radiological appearance on CECT. All suspicions pointed to rectal malignancy starting from the complaint of per rectal bleeding and weight loss, to the endoscopic finding of a friable circumferential rectal mass, to the radiological finding which showed the shoulder sign of the malignant rectal ulcerative neoplasm, with pelvic significant lymphadenopathy. Our patient, however, did not respond to conservative management and developed symptoms and signs of impending bowel obstruction which directed us for the surgical option in the form of low anterior resection of the rectum.


  Conclusion Top


Despite the histopathological confirmation, still SRUS can mimic rectal malignancy clinically, endoscopically, and radiologically. It should be considered in the differential diagnosis of uncommon pathologic findings in the rectum. However, we believe that it is important not to miss a diagnosis of rectal cancer over the diagnosis of SRUS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Abid S, Khawaja A, Bhimani SA, Ahmad Z, Hamid S, Jafri W, et al. The clinical, endoscopic and histological spectrum of the solitary rectal ulcer syndrome: A single-center experience of 116 cases. BMC Gastroenterol 2012;12:72.  Back to cited text no. 1
    
2.
Blanco F, Frasson M, Flor-Lorente B, Minguez M, Esclapez P, García-Granero E, et al. Solitary rectal ulcer: Ultrasonographic and magnetic resonance imaging patterns mimicking rectal cancer. Eur J Gastroenterol Hepatol 2011;23:1262-6.  Back to cited text no. 2
    
3.
Mackle EJ, Parks TG. The pathogenesis and pathophysiology of rectal prolapse and solitary rectal ulcer syndrome. Clin Gastroenterol 1986;15:985-1002.  Back to cited text no. 3
    
4.
Tjandra JJ, Fazio VW, Petras RE, Lavery IC, Oakley JR, Milsom JW, et al. Clinical and pathologic factors associated with delayed diagnosis in solitary rectal ulcer syndrome. Dis Colon Rectum 1993;36:146-53.  Back to cited text no. 4
    
5.
Martin CJ, Parks TG, Biggart JD. Solitary rectal ulcer syndrome in Northern Ireland 1971-1980. Br J Surg 1981;68:744-7.  Back to cited text no. 5
    
6.
Rao SS, Ozturk R, De Ocampo S, Stessman M. Pathophysiology and role of biofeedback therapy in solitary rectal ulcer syndrome. Am J Gastroenterol 2006;101:613-8.  Back to cited text no. 6
    
7.
Ong J, Lim KH, Lim JF, Eu KW. Solitary caecal ulcer syndrome: Our experience with this benign condition. Colorectal Dis 2011;13:786-90.  Back to cited text no. 7
    
8.
Halligan S, Nicholls RJ, Bartram CI. Evacuation proctography in patients with solitary rectal ulcer syndrome: Anatomic abnormalities and frequency of impaired emptying and prolapse. AJR Am J Roentgenol 1995;164:91-5.  Back to cited text no. 8
    
9.
van den Brandt-Grädel V, Huibregtse K, Tytgat GN. Treatment of solitary rectal ulcer syndrome with high-fiber diet and abstention of straining at defecation. Dig Dis Sci 1984;29:1005-8.  Back to cited text no. 9
    
10.
Edden Y, Shih SS, Wexner SD. Solitary rectal ulcer syndrome and stercoral ulcers. Gastroenterol Clin North Am 2009;38:541-5.  Back to cited text no. 10
    
11.
Madigan MR, Morson BC. Solitary ulcer of the rectum. Gut 1969;10:871-81.  Back to cited text no. 11
    
12.
Tjandra JJ, Fazio VW, Church JM, Lavery IC, Oakley JR, Milsom JW, et al. Clinical conundrum of solitary rectal ulcer. Dis Colon Rectum 1992;35:227-34.  Back to cited text no. 12
    
13.
Zargar SA, Khuroo MS, Mahajan R. Sucralfate retention enemas in solitary rectal ulcer. Dis Colon Rectum 1991;34:455-7.  Back to cited text no. 13
    
14.
Ederle A, Bulighin G, Orlandi PG, Pilati S. Endoscopic application of human fibrin sealant in the treatment of solitary rectal ulcer syndrome. Endoscopy 1992;24:736-7.  Back to cited text no. 14
    
15.
Torres C, Khaikin M, Bracho J, Luo CH, Weiss EG, Sands DR, et al. Solitary rectal ulcer syndrome: Clinical findings, surgical treatment, and outcomes. Int J Colorectal Dis 2007;22:1389-93.  Back to cited text no. 15
    
16.
Niv Y, Bat L. Solitary rectal ulcer syndrome – clinical, endoscopic, and histological spectrum. Am J Gastroenterol 1986;81:486-91.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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