Departments of Gastroenterology and Histopathology, Children's Research Centre, Our Ladys Children's Hospital Crumlin, and Conway Institute, University College Dublin, Ireland.
Address correspondence and reprint requests to Dr Billy Bourke, Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland (e-mail: email@example.com).
Received 18 October, 2010
Accepted 12 May, 2011
The authors report no conflicts of interest.
Background and Aims: Solitary rectal ulcer syndrome (SRUS) is an uncommon but troublesome and easily misdiagnosed condition of childhood. We have reviewed the presentation and outcome following conservative management of a group of children with SRUS attending a single national paediatric gastrointesinal referral unit.
Methods: Eight children were identified with histology-proven SRUS. Chart review was conducted for relevant history and examination at diagnosis. Patients were contacted to assess success of treatment at the time of follow-up.
Results: Symptoms at presentation included repeated prolonged and ineffectual straining at stool, passage of blood/mucous per rectum, diarrhoea, and constipation. Most children were referred with suspected constipation, diarrhoea, or inflammatory bowel disease. On the basis of retrospective chart review, 7 of 8 children responded well to conservative management (behavioural modification programme involving reduction of time spent straining at defecation). The child failing treatment could not comply with advice because of comorbid autism. Six of the initial responders were available for follow-up. Four were asymptomatic. Two had relapsed and were not compliant with the management programme.
Discussion/Conclusions: SRUS can masquerade as more common childhood intestinal conditions such as inflammatory bowel disease or constipation. A biopsy is required for diagnosis, because ulceration may not be apparent at the time of endoscopy. Most patients with SRUS in childhood have a satisfactory outcome using a simple behavioural modification approach. Ongoing follow-up to reinforce behavioural modification is important and may avoid long-term, treatment-resistant disease into adulthood.
See “Solitary Rectal Ulcer Syndrome: It's Time to Think About It” by Borrelli and de’ Angelis on page 167.
Solitary rectal ulcer syndrome (SRUS) results from obstructed defecation secondary to internal rectal prolapse. Although it is uncommon, it is well recognized in adult populations, with a prevalence of around 1:100,000 (1). SRUS is rare in childhood (2–5) and may masquerade as other more common conditions, causing difficult-to-manage lower gastrointestinal symptoms. For example, obstructive symptoms (anismus) in children may be interpreted by parents as constipation. Concomitant haematochezia may be misinterpreted as originating from an anal fissure caused by constipation or as other causes of rectal bleeding such as a juvenile polyp. Furthermore, in our experience, some children present with apparent diarrhoea (because of prolonged visits to the bathroom) and the associated bleeding, abdominal pain, and tenesmus may suggest to clinicians the presence of inflammatory bowel disease.
Opinion differs regarding the best treatment for this troublesome condition, varying from laxatives and enema preparations to more invasive surgical procedures such as rectopexy (6). We discuss our experience of an exclusively conservative approach to management, focusing on avoidance of behaviours, which may lead to SRUS or exacerbate it.
It is thought that SRUS occurs as a result of high intrarectal voiding pressure, resulting in prolapse of the anterior rectal wall and ischaemic damage with ultimately formation of an ulcer. This rectal prolapse may be complete or merely involve prolapse into the anal canal and has been demonstrated in adult patients via defecography to occur in symptomatic patients with and without rectal ulceration (7).
The pathological features of SRUS are those of mucosal prolapse and do not always include ulceration. Therefore, clinical findings at endoscopy may be absent. Histological examination of biopsy material is necessary to confirm a diagnosis of SRUS. In the present study, we describe a cohort of children attending a single national tertiary referral service with biopsy-proven SRUS and their subsequent outcome.
Children with a diagnosis of SRUS were identified from the hospital histology database (Winpath Ward Enquiry V5). Only children investigated via colonoscopy/proctoscopy with biopsy-proven SRUS were included. Patient charts were reviewed for age and history at diagnosis, duration of symptoms, investigations, treatments, and differential diagnosis at the time of referral. Following hospital ethics committee approval, families were then contacted via post to invite them to partake in the study with an option to decline. Follow-up telephone calls were then made to ascertain patient symptoms.
Eight children (4 boys and 4 girls) with SRUS were identified (Table 1). The mean age at referral was 9.87 years. The mean duration of symptoms before referral was 1.73 years (range 1 month–7 years). The presumed diagnoses by referring physicians included constipation, inflammatory bowel disease, and SRUS. Symptoms at referral (Table 2) typified those previously described in children with SRUS. (2–4,8–10).
All of the children had a history of prolonged time at stool with persistent ineffectual straining; however, these symptoms were commonly not volunteered by children or their parents and were elicited only following specific enquiry. Seven patients had passage of blood PR. Six patients each had feelings of incomplete evacuation (tenesmus), prolonged periods at defecation, and passage of mucous per rectum. Two presented with “diarrhoea” and 3 with constipation. One described digital evacuation of stool.
Histological material from 10 large-bowel endoscopic series was available for review, representing 8 patients. The gross endoscopic appearances were available for 9 of these procedures and were described as normal in 4. Three endoscopies showed the presence of a typical solitary ulcer, whereas minor mucosal changes (erythema) were present in 2.
Frequently, the attending gastroenterologist did not suspect the diagnosis of SRUS, even following colonoscopy. In only 5 of the 10 endoscopies, SRUS was mentioned as a differential diagnosis on the pathology request form that accompanied the specimen, although 2 of these were repeat endoscopies in patients in whom the original endoscopy had established the diagnosis. In the remainder, inflammatory bowel disease, proctitis, and autism were offered as the clinical differentials.
One of the biopsy series showed no definite histological features associated with a diagnosis of SRUS, although a previous endoscopy from the same patient had shown diagnostic changes. The remainder showed crypt hyperplasia (8/9), lamina propria fibrosis (9/9), thickening of muscularis mucosa with muscular extension into lamina propria (9/9), ulceration (4/9), and surface architectural changes with mucin depletion or villous transformation (7/9). None of the cases showed misplaced colonic glands (colitis cystica) and none of the cases supported a histological diagnosis of an inflammatory fibrin cap polyp or inflammatory cloacogenic polyp. In 3 cases, the constellation of histological findings supported a clinical diagnosis of SRUS. In the other 5 cases, the histological findings provided the initial prompt for the diagnosis of SRUS, which had not been considered clinically.
A conventional behavioural modification treatment approach was instituted in all of the children. This comprised parent-supervised limitation of time spent at stool from 3 to 4 times per day with a maximum time limit on the order of 10 minutes. Children were encouraged not to persist with ineffective straining. Stool softeners (liquid paraffin or Macrogol 3350) were not used routinely unless the children described firm stools. Follow-up was on a clinical need basis, but typically visits occurred at 3- to 4-month intervals. Seven of 8 children (88%) showed improvement in symptoms of bleeding, mucus, and tenesmus. The child with primary treatment failure was unable to comply with the programme because of autism.
Of the 7 children with improvement in symptoms, 6 were available for telephone follow-up. Four of these 6 children were asymptomatic. The remaining patient (no. 5 [Table 1]), who was asymptomatic at the last clinic appointment, was uncontactable.
Of the 3 children with symptoms at the time of telephone follow-up, 1 was the child with autism and primary treatment failure. The other 2 children, both girls, had relapsed and were not attempting to comply with the treatment regimen.
The clinical course for patient 5 is instructive. She first presented to a general paediatric clinic, aged 10 years, with a history of loose movements, abdominal pain, tenesmus, and hematochezia. She was referred to the gastroenterology clinic, where suspicion of inflammatory bowel disease was raised and endoscopy arranged. Endoscopy was grossly normal. She was treated initially with stool softeners but showed no improvement in symptoms. She defaulted on follow-up and, 2 years later, re-presented with worsening symptoms. Repeat lower endoscopy demonstrated a single rectal ulcer (Fig. 1). The histological appearance was typical of SRUS (Fig. 2). Behavioural modification was introduced. She was advised to limit time spent on the toilet to a maximum of 10 minutes to avoid straining and to reduce attempts at defecation to 3/day. At subsequent clinic visits, her toileting regimen was further restricted towards normal, with ultimate complete resolution of symptoms.
SRUS is uncommon in children and for this reason can easily be overlooked or misinterpreted (5). Typically, children present with symptoms suggestive of more common conditions such as constipation or inflammatory bowel disease. In our experience, children and their parents often do not volunteer a history of prolonged ineffectual straining at stool. Eliciting this information is crucial to accurately identify SRUS and discriminate it from other conditions.
There are few data on treatment and its outcome in children with SRUS. In most reported case series, active intervention using enemas (10), laxatives (4), and surgical approaches (3,6,9) have been used more frequently than behavioural modification, mainly biofeedback therapy in adults (8). This may reflect a selection bias towards reporting more severely affected, treatment-resistant patients. Against this background, we sought to review the success of our conventional approach in clinical practice, which seeks to limit the child's tendency to repeatedly strain ineffectually and thereby break the vicious cycle that perpetuates the condition. Our results from a small but unselected group of children suggest that most will do well with conservative management; however, involvement of parents and ongoing medical supervision appear to be necessary to maintain progress and prevent relapse. In general, these patients were seen on 1 to 2 occasions in the outpatient department following introduction of the behavioural modification plan and discharged when success was apparent. We speculate that maintaining compliance in children may prevent progression to the type of long-term morbidity and treatment resistance sometimes seen in adults with this condition. Given our experience of late relapse, a longer follow-up period than occurred in this cohort may be more appropriate.
SRUS is uncommon in childhood. It typically masquerades as more common conditions such as constipation or inflammatory bowel disease. Prolonged periods spent at defecation with persistent ineffectual straining are typical but should be specifically sought on clinical history. Compliance with simple behavioural modification appears to produce a good outcome in childhood SRUS, likely because of the short disease duration compared with adults. Rectal mucosal biopsy may reveal histological changes of prolapse, facilitating a diagnosis in patients with atypical clinical history. Early recognition and management of these patients may avoid some of the chronic long-term morbidity often associated with this condition in adulthood; however, late relapse because of noncompliance is a substantial risk and children should be followed up long term.
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