A Rare and Often Unrecognized Cause of Hematochezia and Tenesmus in Childhood: Solitary Rectal Ulcer Syndrome

Abstract

ABSTRACT. Solitary rectal ulcer syndrome (SRUS) is an unusual disorder of childhood, which usually presents with rectal bleeding, mucous discharge, prolonged straining, tenesmus, and localized pain in the perineal area. After the first description by Cruveilhier, Madigan and Morson further detailed the clinical and pathologic features of SRUS in 1969. The pathogenesis of the syndrome is not well-understood. The postulated mechanism responsible for rectal prolapse in most cases seems to be excessive straining efforts during which high intraabdominal pressure forces the anterior rectal mucosa firmly into the contracting puborectalis muscle. The anterior rectal mucosa is frequently forced into the anal canal and as a consequence becomes strangulated, causing congestion, edema, and ulceration.Histologically, the presence of fibromuscular obliteration of the lamina propria with disorientation of muscle fibers is characteristic, which could be secondary to chronic mechanical and ischemic trauma and inflammation by hard stools, and intussusception of the rectal mucosa. Although the syndrome is well-recognized in adults, the pediatric experience with this condition is limited and often remains unrecognized or misdiagnosed. A misdiagnosis has been reported in one fourth of adult cases, and the correct diagnosis usually delayed approximately 5 to 7 years. There are few pediatric case reports in English literature. Here, we describe 2 children with SRUS, aged 11 and 14 years, whose SRUS was diagnosed 2 and 6 years, respectively, after the onset of the first signs and symptoms. Pediatrics 2002;110(6). URL: http://www.pediatrics.org/cgi/content/full/110/6/ e79; rectal bleeding, solitary rectal ulcer syndrome.

A Rare Cause of Chronic Rectal Bleeding in Children: Solitary Rectal Ulcer (Case Report)

“…[4,5,7,16,17,19,20] In previous reports, the macroscopic appearance of the hyperplastic type could not be distinguished from inflammatory polyp of the rectum or villous adenoma. [4,7,19] The characteristic histopathological findings of SRUS that provide differentiation from other diseases are obliteration with increased fibroblastic activity and presence of collagen deposition in the lamina propria, distortion of crypt architecture, lack of epithelial dysplasia, regenerative changes in crypt epithelium, and muscle fibers derived from muscularis mucosa in the surrounding tissue. [1,7,9,12,13,16,17] The diagnosis of SRUS is established by appraising symptoms together with endoscopic appearance and histopathologic findings.…”

“…[3,7,8] Its etiology is still unclear. [4,[9][10][11] Previously, rectal mucosal ischemia and trauma were reported to be related in the etiology of SRUS. [7,[11][12][13] Defecation straining causes puborectalis relaxation allowing the stool passage in the normal situation.…”

“…[12,13,[16][17][18][19] However, none of the affirmed hypotheses can exactly explain the pathogenesis. [4,9,11] Although SRUS presents generally with rectal bleeding, mucus passage, rectal pain, and tenesmus, additional complaints such as constipation or diarrhea, prolonged straining, altered bowel habits, and rectal prolapsus may be discovered once a detailed history is obtained. [5,7,8,10,18] Gabra et al [20] reported a case of SRUS who presented with severe rectal stricture.…”

“…Defecation training including avoidance of straining and anal digitations, biofeedback, high-fiber diet, and some medication are principal components in the conservative treatment. [4,7,8,12,13] Some authors have proposed topical steroids, sulfasalazine or local excision of the polypoid-type lesion in patients with SRUS; however, there are studies reporting these treatments as being insufficient. [5,[7][8][9] Zargar et al [21] obtained clear improvement in clinical symptoms and the endoscopic appearance with sucralfate enemas.…”

“…[4,5] Tjandra and Rao et al reported the mean period between the onset of the symptoms and correct diagnosis to be 7.8 years and 4.5 years, respectively. [5,6] We report a 10-year-old boy with SRUS diagnosed two years after the onset of the symptoms and we discuss his clinical course in light of the literature.…”