See “Solitary Rectal Ulcer Syndrome in Children and Adolescents” by Perito et al on page 266, and “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 benign condition of the rectum that is usually diagnosed in adolescents in pediatric age group (1,2). The present evidence from the literature indicates that SRUS is either a rare condition in children or usually goes unrecognized or misdiagnosed in pediatric practice (3–6). Diversity of the clinical presentation of SRUS requires a high index of suspicion of both the clinician and the pathologist for the definite diagnosis (3,4).
The rarity of the condition could be explained by the lack of experience of both the endoscopic and the histopathologic features of SRUS during childhood (4,7); however, larger series of children with SRUS were being published in the literature secondary to the heightened awareness of the condition (1,8–10). The most common clinicopathological diagnostic confusion may be inflammatory bowel disease, infectious proctocolitis, intussusception, hemorrhoids, prolapsing rectal polyp, or sexual abuse in children, whereas villous adenoma or adenocarcinoma also should be included in the differential diagnosis of SRUS in adults (2). In this issue of JPGN, Perito et al (9) reported the largest series of childhood SRUS, studied across 13 years in a single institution. Interestingly, 2 of 15 children with SRUS also had previous diagnosis of inflammatory bowel disease (IBD). Because there are few case reports showing this association in adults, the coexistence of SRUS and IBD in children is remarkable. There is usually a delay in the establishment of an accurate diagnosis of SRUS even in adult patients (2,3,8,10), and the time interval between the onset of symptoms and the diagnosis was reported as 3.2 years by the same authors (9). Rectal prolapse and bleeding are the most startling symptoms for the parents, and a prolonged period of misdiagnosis may have important consequences, such as anemia secondary to unremitting hemorrhage or poor appetite in a growing child and distress to the child's family (1,8,11).
The postulated mechanism of SRUS in most cases seems to be excessive straining efforts, during which high intra-abdominal pressure forces the anterior rectal mucosa firmly into the contracting puborectalis muscle (2,12). The anterior rectal mucosa is frequently forced into the anal canal, and as a consequence becomes strangulated, causing congestion, edema, and ulceration. Hence, the endoscopic spectrum of SRUS may vary from hyperemia to ulceration or polypoid lesion, which may be multiple or circumferential, usually occurring on the anterior or anterolateral wall of the rectum at 5 to 10 cm from the anal verge (1,2,5,9). Because of the wide spectrum of the endoscopic appearance of SRUS and being unfamiliar with the endoscopic features of SRUS, the condition may go unrecognized or, more commonly, misdiagnosed. Hence, it is crucial to take biopsy specimens from the lesion and normal-appearing mucosa to confirm the diagnosis, and to make a full colonoscopy in doubtful cases for the exclusion of IBD.
The most prominent histological features of SRUS are fibromuscular obliteration of lamina propria by fibroblasts and muscle fibers derived from muscularis mucosa, which is thicker than normal and devoid of inflammatory cell infiltration (2). The epithelium may show regenerative changes with distortion and hyperplasia of mucosal crypts. All of these architectural changes in SRUS may mimic IBD, ileocecal intussusception, prolapsing rectal polyp, or adenocarcinoma. The presence of collagen infiltration of the lamina propria distinguishes SRUS from IBD and chronic ischemic colitis (2,3). The tendency toward the presence of misplaced glands in the submucosa in SRUS may mislead clinicians to the consideration of carcinoma of the rectum. These mucus-filled glands are lined by normal colonic epithelium, devoid of cytological dysplasia, and allow the pathologist to be sure about the benign nature of the lesion (3,4). It has been suggested that these morphologic changes could be explained by rectal prolapse and ischemic mucosal injury, which may be secondary to the occlusion of the anal canal by the anterior rectal wall during excessive straining on defecation (2,12).
The treatment of SRUS depends on the severity of symptoms and the presence or absence of a concomitant rectal prolapse. Although there are no definitive treatment recommendations for SRUS, the primary endpoint remains the resolution or the improvement of symptoms (1,2,9). Hence, a stepwise, individualized, conservative approach would be the most reasonable method of therapy. Several treatment options have been used in the management of SRUS, ranging from behavioral modification to topical treatment, biofeedback, and surgery (2). The initial clinical approach to SRUS in children should be reassurance of the child and the parents about the benign nature of the disease and to suggest conservative measures, which include avoidance of straining, use of a high-fiber diet, and intermittent use of laxatives to prevent constipation (1,5,9). Surgical treatment is usually reserved for patients who are refractory to conservative treatment and biofeedback or in those who have significant mucosal rectal prolapse.
A comprehensive understanding of the variations of endoscopic features of pediatric SRUS and drawing the attention of the pathologist to the morphologic description of the endoscopic lesions and obtaining adequate colonic biopsies for the histopathological examination will enable us as pediatric gastroenterologists to discover overlooked cases with SRUS in children.
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