All of the patients (5) had at least 1 rectal biopsy with histologic findings that were consistent with SRUS or mucosal prolapse (Table 3). Multiple biopsies were available for review for 6 patients, with histologic features of SRUS seen in multiple biopsies for 4 of these patients. Common histologic findings included muscularization of the lamina propria with strands or bundles of smooth muscle extending between crypts, thickening or hyperplasia of the muscularis mucosae, surface ulceration with inflammation, focal hyperplastic changes in the epithelium, misshapen crypts, and vascular ectasia/congestion, which have been described in previous reports of SRUS or mucosal prolapse (6–11) (Figs. 2 and 3).
A mixed inflammatory infiltrate was often seen in areas of ulceration, but cryptitis or crypt abscesses and chronic changes characteristic of IBD were not seen in most biopsies. In the 2 patients with a concurrent diagnosis of IBD, 1 had a single biopsy available for review, which showed features of SRUS without features of IBD. Five biopsies were available for review for the other patient with IBD, 1 of which showed features of SRUS without features of IBD, 1 of which showed features of an inflammatory polyp, and 2 of which showed overlapping features (particularly surface ulceration with granulation tissue and dense inflammation). In addition, although reactive epithelial atypia was seen in biopsies of 3 of the patients, none of the biopsies had definitive evidence of dysplasia or adenocarcinoma.
The 13 patients without IBD were treated with laxatives, stool softeners, and mesalamine suppositories or enemas. Four underwent polypectomy at diagnosis. One patient required proctectomy for recurrent symptomatic polyps. The 2 patients with IBD were treated with stool softeners, oral or rectal aminosalicylates, oral or rectal corticosteroids, and antibiotics throughout the course of their follow-up. Several of the patients were lost to follow-up after initial diagnosis and treatment, precluding assessment of their response to treatment. In those patients who did have long-term follow-up, 6 of 9 responded to treatment, but at least 50% had recurrent symptoms, particularly after medication adherence lapsed.
Our review of pediatric patients at our institution revealed 15 cases of SRUS during a 13-year period. Presenting symptoms were consistent but nonspecific. Delay in diagnosis was common, likely because of the nonspecific symptoms and rarity of SRUS in the pediatric population. Our cases had a median 3.2-year delay between symptom onset and diagnosis. This is similar to the delay in diagnosis seen in pediatric patients with IBD, who also often present with similar symptoms (12). Treatment led to clinical improvement in most patients, but symptoms often recurred with poor medication adherence.
Our series confirms that SRUS is a misnomer because not all lesions are solitary or ulcers. Previous reports support this characterization (4,13). SRUS has also been referred to as mucosal prolapse to avoid this confusion (11). Endoscopically, the distal rectal mucosa typically appears erythematous, and lesions can be ulcerative or polypoid (3,14). Differential diagnosis in children includes juvenile polyps, infections, IBD, sexual abuse, or rectal digitations. Because our case search strategy relied on cases confirmed by pathology, we were not able to include cases suspected to be SRUS that did not have pathologic confirmation or were diagnosed as other disorders on histology.
Given the lack of symptom specificity and endoscopic findings seen in our cases, this series supports the necessity for histologic diagnosis. The histologic changes are similar to those seen in adults. Characteristic findings include muscularization of the lamina propria, hyperplastic muscularis mucosa, and distortion of crypt architecture (10,11,15). SRUS is distinguished from IBD by scarring of the lamina propria with only a relatively mild inflammatory infiltrate, as well as the accompanying muscular hyperplasia (15). Our patients without coexistent IBD had findings limited to the distal rectum.
Two of our cases, however, illustrate that SRUS and IBD can coexist in children and adolescents. Previous case reports in the adult literature have also noted concurrent SRUS and IBD, particularly UC (5,16). Thus, a diagnosis of IBD should not preclude consideration of SRUS, because management may differ. For those with concurrent SRUS and IBD, stool softeners and/or anti-inflammatory enemas to treat the SRUS may be necessary even when systemic medications are used to treat the IBD.
Because SRUS and IBD can present with identical symptoms—and have similar endoscopic features—SRUS can also be misdiagnosed as IBD. Misdiagnosis is concerning because the oral or intravenous immunosuppressive medications used for UC are unlikely to be effective in SRUS. In addition, because patients with long-standing IBD colitis can develop malignant lesions in the colon, SRUS can be misdiagnosed as tumor (16). Thus, histologic confirmation of the diagnosis is important to guide management. IBD with rectal ulcers could also be mistaken for SRUS; in general, features of systemic disease, such as weight loss, extraintestinal manifestations of IBD, or elevated serum inflammatory markers, should not be present in isolated SRUS.
Although its etiology is unclear, SRUS has been linked to direct mucosal trauma, rectal dysmotility, and ischemia. Strained defecation in some patients may lead to direct ulceration of the mucosa. In others, the anterior rectal mucosa is forced into the anal canal, causing rectal prolapse and mucosal injury (4,15). Rectal prolapse and high intraabdominal pressures may contribute to vascular compression, leading to mucosal ischemia (1). Tenesmus and straining related to IBD colitis have been theorized as contributors to the development of SRUS in patients with both disorders.
SRUS is not considered to be invasive or progressive; however, it tends to be refractory (6), as the outcomes of our patients suggest. Common treatments include laxatives, stool softeners, and rectal aminosalicylates (1,2,17). Surgery has also occasionally been used with minimal long-term success because the lesions often recur if the straining and dysmotility continue. In our patients, stool softeners and mesalamine suppositories were most often used. The small number of patients and variable follow-up prevent us from drawing robust conclusions about treatment efficacy.
Our analysis was limited because of the relatively small sample size and missing data. Multicenter prospective cohort studies would help identify treatment methods and outcome of pediatric patients with SRUS.
1. Keshtgar AS. Solitary rectal ulcer syndrome in children. Eur J Gastroenterol Hepatol
2. De la Rubia L, Ruiz Villaespesa A, Cebrero M, et al. Solitary rectal ulcer syndrome in a child. J Pediatr
3. 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
4. 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
5. Arhan M, Onal IK, Ozin Y, et al. Solitary rectal ulcer syndrome in association with ulcerative colitis: a case report. Inflamm Bowel Dis
6. Madigan MR, Morson BC. Solitary ulcer of the rectum. Gut
7. Warren BF, Dankwa EK, Davies JD. ‘Diamond-shaped’ crypts and mucosal elastin: helpful diagnostic features in biopsies of rectal prolapse. Histopathology
8. Franzin G, Scarpa A, Dina R, et al. “Transitional” and hyperplastic-metaplastic mucosa occurring in solitary ulcer of the rectum. Histopathology
9. Tendler DA, Aboudola S, Zacks JF, et al. Prolapsing mucosal polyps: an underrecognized form of colonic polyp—a clinicopathological study of 15 cases. Am J Gastroenterol
10. Chiang JM, Changchien CR, Chen JR. Solitary rectal ulcer syndrome: an endoscopic and histological presentation and literature review. Int J Colorectal Dis
11. Singh B, Mortensen NJ, Warren BF. Histopathological mimicry in mucosal prolapse. Histopathology
12. Heyman MB, Kirschner BS, Gold BD, et al. Children with early-onset inflammatory bowel disease (IBD): analysis of a pediatric IBD consortium registry. J Pediatr
13. Martin CJ, Parks TG, Biggart JD. Solitary rectal ulcer syndrome in northern Ireland. 1971–1980. Br J Surg
14. du Boulay CE, Fairbrother J, Isaacson PG. Mucosal prolapse syndrome—a unifying concept for solitary ulcer syndrome and related disorders. J Clin Pathol
15. Sharara AI, Azar C, Amr SS, et al. Solitary rectal ulcer syndrome: endoscopic spectrum and review of the literature. Gastrointest Endosc
16. Uza N, Nakase H, Nishimura K, et al. Solitary rectal ulcer syndrome associated with ulcerative colitis. Gastrointest Endosc
17. Bishop PR, Nowicki MJ, Subramony C, et al. Solitary rectal ulcer: a rare cause of gastrointestinal bleeding in an adolescent with hemophilia A. J Clin Gastroenterol
Keywords:Copyright 2012 by ESPGHAN and NASPGHAN
constipation; pediatrics; prolapse; rectal bleeding; rectum; solitary rectal ulcer syndrome