*Department of Paediatric Gastroenterology, Great Ormond Street Hospital, London, UK
†Department of Pediatrics, Gastroenterology Service, University of Parma, Parma, Italy.
Address correspondence and reprint requests to Osvaldo Borrelli, MD, PhD, Department of Pediatric Gastroenterology, Division of Neurogastroenterology and Motility, Great Ormond Street Hospital for Sick Children, Great Ormond Street, London WC1N 3HZ, UK (e-mail: email@example.com).
Received 10 June, 2011
Accepted 26 July, 2011
The authors report no conflicts of interest.
See “Clinical Presentation of and Outcome for Solitary Rectal Ulcer Syndrome in Children” by Blackburn et al on page 263.
Solitary rectal ulcer syndrome (SRUS) is a rare benign rectal disorder characterized by a combination of symptoms, endoscopic findings, and histological abnormalities (1,2). It usually affects young adults with a slight predominance in women and an estimated prevalence of 1 in 100,000 people annually (3). Although its description in paediatrics is largely limited to case reports (4–12), in an Iranian series including 256 children who had undergone colonoscopy for rectal bleeding during a 2.5-year period, 12 (4.7%) children were diagnosed as having SRUS (11).
Clinical features include rectal bleeding, copious mucus discharge, prolonged excessive straining, perineal pain, feeling of incomplete defecation, and rarely rectal prolapse (11–13). However, because of the nonspecificity of these symptoms, diagnosis depends upon endoscopic and histological assessments. The macroscopic spectrum of SRUS may vary from simple hyperaemic mucosa to small or giant ulcers to broad-based polypoid lesions varying in size, number, and rectal wall position (14,15). Thus, the term SRUS is ambiguous and undoubtedly misleading because less than one-third of patients show a single rectal ulcer, and lesions are not necessarily ulcerated. Key histological features include fibromuscular obliteration of the lamina propria, hypertrophied muscularis mucosae with extension of muscle fibres upward between the crypts, and glandular crypt abnormalities (15–17). Other minor microscopic changes, including surface erosion, mild inflammation, distorted crypts, and reactive epithelial atypia, may lead to erroneous diagnoses such as inflammatory bowel disease, infectious colitis, and cancer (18,19). Diffuse collagen deposition in the lamina propria and abnormal smooth muscle fibre extensions are sensitive markers for differentiating SRUS from other conditions (20).
Consequently, because of the wide spectrum of clinical, endoscopic, and histological features, SRUS can mimic other rectal disorders, often leading to delayed or incorrect diagnosis and inappropriate treatment. Almost 25% of adults receive an initial incorrect diagnosis, and the mean time from symptom onset to diagnosis ranges between 3.5 and 5.5 years, whereas in children it is between a few months and 2 years (8–11,21).
In this issue of JPGN, Blackburn et al (22) retrospectively report the clinical characteristics and the short- and long-term outcomes of 8 children with pathologically proven SRUS. A behavioural approach involving reduction of time spent straining at defecation was instituted in all of the children, and 7 showed a significant clinical improvement, becoming asymptomatic. The child with treatment failure had a comorbid autism spectrum disorder. Of 6 children available at long-term follow-up (mean 44 months), 4 were asymptomatic and 2 had relapsed and were not compliant with the behavioural programme.
Present available treatments are suboptimal, and despite correct diagnosis, outcomes can be unsatisfactory, with symptom and rectal lesion persistence. Initial therapeutic approaches usually include family reassurance about the nature of the complaint, constipation avoidance, and behavioural measures. Topical treatments, including salicylate, sulfasalazine, steroid, and sucralfate, have been found to be effective in some paediatric case reports, but no obvious efficacy has been found in adults (23). Surgery is warranted in almost one-third of adults with associated rectal prolapse; in children this has only been described in case reports (24).
The pathophysiology of SRUS is incompletely understood; however, studies suggest a role for rectal hypersensitivity leading to the persistent desire to defecate and/or feelings of incomplete evacuation, which may in turn cause excessive straining and altered defecatory behaviour, resulting in pelvic dyssynergia (25). Over time, straining and mucosal intussusception may cause rectal mucosal trauma and bleeding (25). Based on this assumption, behavioural approach or biofeedback therapy that improves muscle activity during defecation represents the cornerstone in the treatment of SRUS (26,27). Blackburn et al offer precious information reporting the efficacy of behavioural therapy assessed in long-term follow-up, highlighting positive long-term results produced by compliance with therapy. Conversely, noncompliance with treatment is a substantial risk for relapse.
The research of Blackburn et al provides an opportunity to pay attention to an unusual cause of rectal disorders, requiring a high index of suspicion, and helps paediatric gastroenterologists to think of SRUS when evaluating patients with rectal bleeding. It also supports the need for additional larger multicenter studies to define its paediatric prevalence and to delineate the best treatment strategy for this condition.
1. Vaizey CJ, van den Bogaerde JB, Emmanuel AV, et al. Solitary rectal ulcer syndrome. Br J Surg 1998; 85:1617–1623.
2. Felt-Bersma RJ, Tiersma ES, Cuesta MA. Rectal prolapse, rectal intussusception, rectocele, solitary rectal ulcer syndrome, and enterocele. Gastroenterol Clin North Am 2008; 37:645–668.
3. Martin CJ, Parks TG, Biggart JD. Solitary rectal ulcer syndrome in northern Ireland. 1971–1980. Br J Surg 1981; 68:744–747.
4. Sondheimer JM, Slagle TA, Bryke CR, et al. Solitary rectal ulcer syndrome in a teenaged boy. J Pediatr Gastroenterol Nutr 1985; 4:835–838.
5. Figueroa-Colon R, Younoszai MK, Mitros FA. Solitary rectal ulcer syndrome in children. J Pediatr Gastroenterol Nutr 1989; 8:408–412.
6. De la Rubia L, Ruiz Villaespesa A, Cebrero M, et al. Solitary rectal ulcer in a child. J Pediatr 1993; 122:733–736.
7. Eigenmann PA, Le Coultre C, Cox J, et al. Solitary rectal ulcer: an unusual cause of rectal bleeding in children. Eur J Pediatr 1992; 151:658–660.
8. Ertem D, Acar Y, Karaa EK, et al. A rare and often unrecognized cause of hematochezia and tenesmus in childhood: solitary rectal ulcer syndrome. Pediatrics 2002; 110:e79.
9. Martin de Carpi J, Vilar P, Varea V. Solitary rectal ulcer syndrome in childhood: a rare, benign, and probably misdiagnosed cause of rectal bleeding. Report of three cases. Dis Colon Rectum 2007; 50:534–539.
10. Gabra HO, Roberts JP, Variend S, et al. Solitary rectal ulcer syndrome in children. A report of three cases. Eur J Pediatr Surg 2005; 15:213–216.
11. Dehghani SM, Haghighat M, Imanieh MH, et al. Solitary rectal ulcer syndrome in children: a prospective study of cases from southern Iran. Eur J Gastroenterol Hepatol 2008; 20:93–95.
12. Suresh N, Ganesh R, Sathiyasekaran M. Solitary rectal ulcer syndrome: a case series. Indian Pediatr 2010; 47:1059–1061.
13. Tjandra JJ, Fazio VW, Church JM, et al. Clinical conundrum of solitary rectal ulcer. Dis Colon Rectum 1992; 35:227–234.
14. Sharara AI, Azar C, Amr SS, et al. Solitary rectal ulcer syndrome: endoscopic spectrum and review of the literature. Gastrointest Endosc 2005; 62:755–762.
15. Chiang JM, Changchien CR, Chen JR. Solitary rectal ulcer syndrome: an endoscopic and histological presentation and literature review. Int J Colorectal Dis 2006; 21:348–356.
16. Madigan MR, Morson BC. Solitary ulcer of the rectum. Gut 1969; 10:871–881.
17. Malik AK, Bhaskar KV, Kochhar R, et al. Solitary ulcer syndrome of the rectum—a histopathologic characterisation of 33 biopsies. Indian J Pathol Microbiol 1990; 33:216–220.
18. Ford MJ, Anderson JR, Glimour HM, et al. Clinical spectrum of solitary ulcer of the rectum. Gastroenterology 1983; 84:1533–1540.
19. Haray PN, Morris-stiff GJ, Foster MS. Solitary rectal ulcer syndrome—an underdiagnosed condition. Int J Colorectal Dis 1997; 12:313–315.
20. Levine DS, Surawicz CM, Ajer TN, et al. Diffuse excess mucosal collagen in rectal biopsies facilitates differential diagnosis of solitary rectal ulcer syndrome from other inflammatory bowel disease. Dig Dis Sci 1988; 33:1345–1352.
21. Tjandra JJ, Fazio VW, Petras RE, et al. Clinical and pathologic factors associated with delayed diagnosis in solitary rectal ulcer syndrome. Dis Colon Rectum 1993; 36:146–153.
22. Blackburn C, McDermott M, Bourke B. Clinical presentation and outcome for solitary rectal ulcer syndrome in children. J Pediatr Gastroenterol Nutr, 2012;54:263–5.
23. Edden Y, Shih SS, Wexner SD. Solitary rectal ulcer syndrome and stercoral ulcers. Gastroenterol Clin North Am 2009; 38:541–545.
24. Bonnard A, Mougenot JP, Ferkdadji L, et al. Laparoscopic rectopexy for solitary ulcer of rectum syndrome in a child. Surg Endosc 2003; 17:1156–1157.
25. Rao SS, Ozturk R, De Ocampo S, et al. Pathophysiology and role of biofeedback therapy in solitary rectal ulcer syndrome. Am J Gastroenterol 2006; 101:613–618.
26. Vaizey CJ, Roy AJ, Kamm MA. Prospective evaluation of the treatment of solitary rectal ulcer syndrome with biofeedback. Gut 1997; 41:817–820.
27. Jarrett MED, Emmanuel AV, Vaizey CJ, et al. Behavioural therapy (biofeedback) for solitary rectal ulcer syndrome improves symptoms and mucosal blood flow. Gut 2004; 53:368–370.