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Solitary rectal ulcer syndrome in children

Keshtgar, Alireza S.

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European Journal of Gastroenterology & Hepatology: February 2008 - Volume 20 - Issue 2 - p 89-92
doi: 10.1097/MEG.0b013e3282f402c1
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Abstract

Introduction

Solitary rectal ulcer syndrome (SRUS) is an uncommon benign disease of rectum, which affects predominantly young adult females aged between 30 and 50 years with a prevalence of 1 in 100 000 people per year [1,2]. SRUS is rare in children and its description is largely limited to case reports. The condition was first described by Cruveilhier in 1830, and detailed clinical and histopathological features were reported by Madigan and Morson in 1969 [1,3]. SRUS is often diagnosed late or incorrectly because of its similarity with other rectal diseases. The term solitary ulcer was first described by Lloyd-Davies at St Mark's Hospital in London but this expression can be misleading because there may be more than one rectal ulcer and there is a preulcerative stage of the disease [1].

Etiology and pathophysiology

The underlying etiology and pathogenesis are not fully understood but multiple factors may be involved. It has been suggested that descent of perineum and abnormal contraction of puborectalis muscle during straining on defecation or defecation in squatting position result in trauma and compression of anterior rectal wall on upper anal canal, and internal intussusception or prolapse of rectum [4,5]. This may lead to venous congestion, poor blood flow and edema in the mucosal lining of rectum and ischemic changes with resultant ulceration. Self-digitation maneuver to reduce a rectal prolapse or to evacuate an impacted stool may also cause direct trauma and ulceration [6,7].

Clinical features and diagnosis

Children with SRUS usually present with a symptom complex consisting of rectal bleeding, passage of mucus and straining on defecation, tenesmus, perineal and abdominal pain, sensation of incomplete defecation, constipation and rectal prolapse [8–11]. In a series of 256 children with rectal bleeding from Iran, Dehghani and colleagues [5] diagnosed SRUS in 12 (5%) on colonoscopy and histology examinations. All children had severe straining on squatting defecation and six (50%) had a sense of incomplete defecation, presumably related to internal intussusception or prolapse of anterior rectal wall, although overt rectal prolapse was seen in only two (17%) children [5]. It is not unusual that a child may present with recurrent rectal bleeding and anemia requiring blood transfusions. A detailed history and physical examination should be undertaken to exclude sexual abuse in children. Diagnosis of SRUS is based on clinical features, findings on proctosigmoidoscopy and histology examinations, imaging investigations including defecating proctography, dynamic MRI and anorectal functional studies including manometry and electromyography. An early diagnosis requires a high index of suspicion by the clinician and the pathologist. In adults, up to 26% of patients are asymptomatic and a rectal ulcer is discovered incidentally while investigating other diseases [12]. In a quarter of patients a delay in diagnosis or misdiagnosis of SRUS might occur because of inadequate rectal biopsy and failure to recognize the histopathological features of the disease.

Colonoscopy and histology

Colonoscopy and biopsy of normal and abnormal looking mucosa of rectum and colon should be performed. On proctosigmoidoscopy a single ulcer is found in 70% of cases that is usually located anteriorly or laterally astride valves of Houston [1,7]. The distance of ulcer from the anal margin varies from 3 to 10 cm [1,12,13]. The appearance of SRUS on endoscopy may vary from preulcer hyperemic changes of rectal mucosa to established ulcers measuring from 0.5 to 5 cm in diameter with the base of ulcer covered by a white, grey or yellowish slough [1,14]. The ulceration is shallow and the adjacent mucous membrane may appear nodular, lumpy or granular [1,8]. The ulcer may appear as a polypoid or multiple lesions in 25 and 30% of cases, respectively [1,2]. The characteristic histological features of SRUS are fibromuscular obliteration of lamina propria and replacement by collagen derived from fibroblasts and muscle fibers from muscularis mucosae [1]. The muscularis mucosae appear thicker than normal and the lamina propria shows no significant number of inflammatory cells. The epithelial component of mucous membrane shows erosion of the superficial mucosa, which is covered by mucus, pus and detached epithelial cells, and may show reactive hyperplasia with distortion of the crypt architecture [1,15]. The submucosa shows dense fibrosis accounting for the whitish appearance on proctosigmoidoscopy. The histological features of SRUS should be differentiated from other diseases of rectum including ulcerative colitis, Crohn's disease and infective or nonspecific colitis, which may show chronic and acute inflammatory cells in lamina propria, cryptitis, crypt abscesses and granuloma formation, with distortion of epithelial and glandular structures. SRUS has been misdiagnosed on occasions for adenomatous polyp and adenocarcinoma of rectum in adults. This is because of variation in its macroscopic appearance from hyperemia to ulceration or a polypoid lesion and also misplaced epithelial elements and lamina propria in the submucosa, which undergo cystic dilatation due to retention of mucin [1,11].

Laboratory and imaging investigations

Routine laboratory tests including red and white blood cell count, platelets count, hemoglobin, liver function tests, coagulation tests, C-reactive protein and erythrocyte sedimentation rate are usually normal. Features of microcytic anemia with low values of hemoglobin, hematocrit and mean corpuscular volume may, however, be seen in a child with a history of recurrent bleeding per rectum. Stool examination for bacteriology, virology and parasites should be done. A sweat test should also be done to exclude cystic fibrosis in children with rectal prolapse. In children, dynamic MRI is valuable to obtain information related to pelvic floor descent and dysfunction, rectocele, internal rectal intussusception and prolapse. Although dynamic MRI is safe and does not carry risk of radiation exposure, it does not provide information about completeness of stool evacuation from rectum, which can be obtained from defecation proctography. Anal endosonography has demonstrated thickening of the internal anal sphincter (IAS) in children with chronic constipation and megarectum [16]. This is analogous to thickening of the IAS in adults with SRUS who have associated high-grade rectal intussusception and prolapse [17]. We have observed that constipated children with fecal impaction have higher amplitude and frequency of rectal and anal contractions compared with children without impaction [18]. This over activity of smooth muscles because of fecal impaction or rectal prolapse in time leads to secondary hypertrophic changes in rectal and the IAS muscles, which appear thickened on endosonography [16]. Anorectal manometry and electromyography provide useful information about rectoanal inhibitory reflex, pressure profiles, defecation dynamics and rectal compliance and sensory thresholds. On awake anorectal manometry, 42–55% of children with chronic constipation show dyssynergia and abnormal contraction of voluntary muscles of pelvic floor and external anal sphincters (EAS) during an attempt to expel a rectal balloon [19,20]. In adults excessive straining and uncoordinated defecation, caused by dyssynergia of pelvic floor muscles have been attributed to development of SRUS [21,22]. This all suggests a relationship between dyssynergia of the pelvic floor and the EAS muscles, constipation, rectal prolapse and SRUS.

Treatment

The treatment of SRUS in children is guided by symptoms, findings on physical examination and anorectal investigations. SRUS can be difficult to treat and the ulcer may recur. In the absence of overt rectal prolapse, however, a conservative treatment includes high intake of fluids and fibers, laxatives and bowel retraining to avoid straining on defecation. Different treatments have been used with various responses and improvement of symptoms using enemas containing sucralfate, salicylate, corticosteroid, sulfasalazine, mesalazine and topical fibrin sealant [1,5,23–25]. Sucralfate enema contains aluminum complex salts, which coats the rectal ulcer and forms a barrier against irritants, allowing the ulcer to heal. Corticosteroid and sulfasalazine enemas may also help ulcer healing by reducing the inflammatory responses. These treatments, however, are empirical, and noncontrolled studies and their long-term benefits deserve further investigation. Behavior modification therapy by biofeedback training in adults with SRUS has shown improvement of symptoms in 75% of patients who had uncoordinated defecation habits, excessive straining and stool frequency, and high rectal sensory threshold. Laser Doppler study in these patients showed increased rectal mucosal blood flow after treatment with resolution of ulcer in 31% of patients [21,22]. Experience of biofeedback therapy for SRUS has, however, been limited in children. Surgical treatments include excision of the ulcer, treatment of internal or overt rectal prolapse and defunctioning colostomy. The indication for surgery is failure of conservative treatments to control severe symptoms, and the aim is to avoid formation of colostomy as a primary operation. Neodymium Yttrium-Aluminium-Garnet laser therapy has been used successfully to excise solitary rectal ulcer in the absence of rectal prolapse [26]. In a majority of children, the rectal prolapse resolves spontaneously by treating the underlying causes of constipation and straining on defecation. Sclerotherapy injection into submucosa or retrorectal space with 5% phenol, 30% hypertonic saline or 25% glucose and perianal cerclage has been effective in treating the rectal prolapse. Long-term results, however, are not known because these procedures do not deal with underlying causes of chronic straining and abnormal defecation dynamics. Surgery may be indicated for recurrent rectal prolapse refractory to conservative treatments. In children laparoscopic rectopexy using a polypropylene mesh on each side of rectum, fixed to sacral promontory with nonabsorbable suture, has been used successfully to treat SRUS [27]. In a series of 66 adult patients with SRUS, rectopexy was done in 49, Delorm's operation in nine, restorative anterior resection in two, postanal repair and division of puborectalis in two and primary colostomy in four [28] patients. Subsequent colostomy was required for failed operation in 16 patients giving an overall stoma rate of 30%. Local excision of polypoid rectal ulcer and rectopexy for overt rectal prolapse, however, have a higher long-term cure rate [7,11,28]. In another early series of 68 patients, three required formation of proximal colostomy for SRUS with persistence of the ulcer up to 12 years later [1]. Resection proctectomy may be required in patients with intractable rectal pain and bleeding, who have not responded to other surgical treatments [1,28,29].

Summary and future direction

The current evidence from the literature indicates that SRUS is a rare condition in children, which is likely to be secondary to ischemic changes in the rectum, and is associated with paradoxical contraction of pelvic floor and the EAS muscles, rectal prolapse, obstructive defecation, constipation and rectal bleeding. Although conservative measures may be beneficial to treat constipation and dyssynergia of defecation dynamics, excision of rectal ulcer and surgery of overt rectal prolapse may be required in refractory cases.

Keshtgar and others [30] have recently reported a new treatment modality of botulinum toxin injection into the IAS for chronic idiopathic constipation in children. Evidence from our research trial of the needle-free injection of botulinum toxin into the EAS, however, has proved to be more effective for the treatment of childhood constipation and withholding habits, caused by dyssynergia of defecation dynamics [31]. A therapeutic role might exist for botulinum toxin injection into the EAS for treatment of SRUS associated with constipation and paradoxical contraction of pelvic floor and EAS muscles. The effect of botulinum toxin lasts approximately 3 months, which may be more beneficial than biofeedback therapy. Further prospective studies and long-term follow-up are, however, required to establish the optimum treatment modality for SRUS in children.

Acknowledgement

The author thanks Mr. Harry Ward, consultant pediatric surgeon at Barts and the Royal London Hospital, NHS Trust for reading the manuscript and providing comments.

Conflict of interest: none declared.

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Keywords:

anorectal manometry; botulinum toxin; children; endosonography; rectal bleeding; solitary rectal ulcer

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