Solitary rectal ulcer syndrome in children

P. GODBOLE, I. BOTTERILL*, S.J. NEWELL†, P.M. SAGAR* and M.D. STRINGER†
Departments of *Colorectal Surgery, Paediatric Surgery, †Paediatrics, Leeds Teaching Hospitals NHS Trust, Leeds U.K.

Solitary rectal ulcer syndrome (SRUS) is rare in children. Few reported cases have undergone detailed investigations, treatments have been extremely varied and outcome poorly documented. We report two cases of SRUS in children, each with a different macroscopic pathology. The diagnosis was delayed in both cases. The importance of appropriate investigation and the need to tailor treatment to the type of lesion are emphasised. One case of SRUS was associated with a complete, full thickness rectal prolapse and symptoms improved after an abdominal sutured rectopexy. The other patient responded well to endoanal excision of polypoid lesions. The diagnosis and management of this condition in children deserves wider recognition.

Solitary Rectal Ulcer Syndrome ( SRUS ) is a well-recognised entity in adults with a variety of macroscopic appearances.^1,2 Clinical and pathological findings may mimic other rectal pathologies thereby leading to an incorrect or delayed diagnosis. Tjandra et al (1992) reported a previously incorrect clinicopathological diagnosis in 25% of 80 adult cases from the Cleveland Clinic.² SRUS has been rarely reported in children. Treatment of such cases has been very variable and the outcome poorly documented. We describe the management and follow-up of two cases of SRUS in children, emphasizing the importance of appropriate investigation and the need to tailor treatment to the type of solitary rectal ulcer.

A 14-year-old boy presented with a two-year history of increased stool frequency ( 6-7/day ) associated with the passage of mucus and blood, central abdominal pain and a decreased appetite. Clinically he was prepubertal, with weight and height on the 0.4th and 2nd centile, respectively. His bone age was 12 years. Routine haematologic and biochemical parameters were normal. He was initially treated with nutritional supplements, bulk laxatives and loperamide with little effect. Colonoscopy revealed evidence of inflammation and a polypoid appearance in the rectum; mucosal biopsies were abnormal with glandular distortion, inflammation and granulation tissue. He was treated for presumed inflammatory bowel disease with corticosteroid enemas without benefit. Repeat endoscopy showed a single, firm, ulcerated rectal submucosal polyp, measuring 2 cm across, on the anterior wall 7 cm. from the anal verge (Figure 1). Numerous small, friable polypoid lesions were also noted just above the dentate line. Biopsies of the anterior rectal polyp showed fibrous obliteration of the lamina propria by smooth muscle fibres together with inflammatory changes, dilated capillaries and glandular distortion, features typical of the polypoid variant of SRUS.

Figure 1: Per-anal view of polypoid variant SRUS (with attached stay suture) and associated inflammatory cloacogenic polyps

Anorectal physiology demonstrated a normal sphincter pressure profile. A defaecating proctogram showed an anterior filling defect consistent with the polyp and anterior rectal wall prolapse. He underwent endoanal excision of all polyps. Follow up examination demonstrated a few small residual granulations at the anorectal junction which were diathermied. Two years after surgery, he has had no further rectal bleeding and his stool frequency has decreased to 5 times a day. He has no tenesmus, straining or soiling. Repeat anorectal manometry was again normal. His subsequent growth has improved with weight just below the 2nd centile and height on the 9th centile.

A 12-year-old boy presented with a five-year history of intermittent rectal bleeding and progressively worsening rectal prolapse. This had been previously treated several years before by injection sclerotherapy at another institution. The bleeding was accompanied by mucus, tenesmus, occasional incontinence, and a reported bowel frequency of up to 20 times per day and 3 times at night. On examination he was prepubertal with normal growth indices. He had evidence of a complete, full thickness rectal prolapse.

Anorectal physiology by vector volume manometry demonstrated slightly low resting and squeeze anal canal pressures and a marginally weakened sphincter posteriorly. Defaecating proctography confirmed the presence of a large complete full thickness rectal prolapse with an anterior lead point suggestive of SRUS. Examination under anaesthesia confirmed a large anterior rectal ulcer about 8cms from the anal verge (Figure.2). Histology revealed mucosal ulceration and architectural distortion with smooth muscle fibres in the lamina propria.

Figure 2: Anterior solitary rectal ulcer with full thickness rectal prolapse demonstrated prior to surgery in Case 2

An abdominal sutured posterior rectopexy, without division of the lateral ligaments, was carried out via a lower midline incision. Subsequent assessment six weeks later demonstrated a healed ulcer with no evidence of recurrent prolapse. Repeat anorectal physiology studies showed an improvement in resting and squeeze anorectal pressures, which were within normal limits for his age. One year following surgery, he remains symptomatically improved but still passes 6-10 stools per day.

In neither case was there a history of recent foreign travel, anal sexual activity, self-digitation, foreign body insertion or a family history of inflammatory bowel disease.

Solitary rectal ulcer is rare in children, with only nine documented cases.^3-9 There have been no previous reports of a child with SRUS associated with complete rectal prolapse. Few paediatric cases have undergone detailed investigations, treatment has been very variable and, in most cases, outcome has been poorly documented. Our two patients illustrate the importance of a detailed assessment and the need to tailor treatment to the type of lesion. In common with adults, there is often a long duration of symptoms prior to diagnosis in children and symptomatic control can be difficult despite ulcer healing (probably because the underlying pathophysiological mechanisms of SRUS persist).^3,10

The aetiology of SRUS is multifactorial.^2,11 Both ischaemia and trauma appear to be significant. Dysfunctional defaecation associated with an anterior rectal prolapse, either occult or overt, is important. Reflex contraction of the puborectalis may traumatise the mucosal prolapse, causing ischaemia and ulceration of the lead point, which in turn causes a further desire to defecate and strain.⁷ Others have postulated that internal rectal prolapse together with spasm of the external anal sphincter during voiding leads to high intrarectal pressures which causes the lesion.¹² The incidence of overt rectal prolapse in adults ranges from 15 to 59% in published series.^1,2 Occult rectal prolapse is the commonest radiographic abnormality found during videoproctography.¹² Trauma from rectal digitation has also been implicated in some patients but we could not elicit this history in our cases.⁹

There are several reports of inflammatory cloacogenic polyps in children which share similar histological features with SRUS.^3,13 Whilst both may be manifestations of a similar pathologic process, and, therefore, may occur together as in our first case, there are notable differences between these lesions. Inflammatory cloacogenic polyps arise from the anal transition zone and not the rectal wall and may be found in association with a variety of other pathological conditions including inflammatory bowel disease, haemorrhoids, and colorectal tumours.^3,13

In adults, SRUS occurs predominantly in the fourth to sixth decades and females are more commonly affected.^1,2,7 Principal symptoms are rectal bleeding, mucus discharge, tenesmus, straining and rectal pain.^2,7,12 Alteration in bowel habit is common but not invariable; this may include chronic constipation, diarrhoea, and faecal incontinence.⁷ Symptomatology in children is similar but almost all cases have been male. The endoscopic appearance of SRUS can be divided into three types: ulcerated (single or multiple), polypoid and plaque-like.^1,7 Lesions are typically sited on the anterior rectal wall, 5-8 cm from the anal verge but may occasionally be found elsewhere.^1,2 Microscopically, there is fibrous obliteration of the lamina propria with smooth muscle fibres splaying out into the lamina propria. The muscularis mucosa is hypertrophied with distorted crypts. The epithelium is hyperplastic and mucus glands may be displaced deep to the muscularis mucosa.

Endoscopy and biopsy are diagnostic of SRUS but video-proctography and anorectal manometry are useful additional investigations. A barium enema frequently fails to show any abnormality.^3,5,6 Evacuation proctography usually demonstrates abnormal pelvic floor function12 as in our two patients, but this investigation has only rarely been performed in paediatric cases.^3,5 Anorectal pressure profiles may be normal but some patients have reduced resting and squeeze pressures.¹¹

Treatment of SRUS aims to relieve symptoms and heal the lesion. Bulk laxatives and a high fibre diet may lead to symptomatic improvement but are of no proven long term benefit.¹ Topical steroids have not been helpful^7,8 but there is one report of response to topical sulfasalazine.⁸ Electromyographic biofeedback to correct paradoxical contraction of puborectalis is beneficial in some cases, either alone or as an adjunct to surgery.^5,14 However, ulcer healing is not consistently achieved and patients with rectal prolapse do not respond to biofeedback alone. As described by Tjandra et al (1992), there was only a partial correlation between healing of the lesion and symptomatic improvement in our two cases.²

Surgical treatments for SRUS are variably successful in adults. From a 12-year review of 98 adults with SRUS at the Cleveland Clinic, Tjandra et al (1993) concluded that local excisional procedures yielded better results in patients with polypoid SRUS and rectopexy offered the best chance of cure in those with overt rectal prolapse.¹⁰ Others have reported that rectopexy affords good short term results but persistent or recurrent symptoms are common in the longer term.¹⁵ Our second patient underwent an abdominal sutured rectopexy, not previously described for SRUS in children, and long-term follow up will be important.

Correspondence: M.D. Stringer, Department of Paediatric Surgery,, Gledhow Wing, St James’s University Hospital, Leeds LS9 7TF, U.K.

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