Pelvic Floor Disorders: Surgical Approach

10. Solitary Rectal Ulcer Syndrome and Obstructed Defecation: Common Pathology

Niels A. T. Wijffels  and William Chambers

(1)

Department of Surgery, Zuwe Hofpoort Hospital, Woerden, The Netherlands

Niels A. T. Wijffels

Email: nwijffels@zuwehofpoort.nl

Abstract

Solitary rectal ulcer syndrome (SRUS) is a condition combining disturbances to bowel function and ulcer-related symptoms such as the passage of blood and mucus rectally. Ulceration is not always present but the main feature of the condition is erythema or ulceration of the anterior rectal wall [1]. It was first described by Cruveilhier in 1829 [2]; however, it was not until 1969 that Madigan and Morson [3] proposed the clinicopathological features accepted today. It is an uncommon condition with an annual incidence of 1 in 100,000 to 3.6 in 100,000. It affects young adults, occurring most commonly in the third decade in men and in the fourth decade in women [4], with a slightly higher rate of occurrence in women [5]. As a result of the low incidence/prevalence of SRUS, good-quality articles on etiology and therapy are scarce or even lacking. It is associated with other pelvic floor disorders with overlapping symptomatology. Although a variety of both conservative and surgical therapies have been described, a lack of consensus regarding the exact underlying etiology remains.

10.1 Introduction

Solitary rectal ulcer syndrome (SRUS) is a condition combining disturbances to bowel function and ulcer-related symptoms such as the passage of blood and mucus rectally. Ulceration is not always present but the main feature of the condition is erythema or ulceration of the anterior rectal wall [1]. It was first described by Cruveilhier in 1829 [2]; however, it was not until 1969 that Madigan and Morson [3] proposed the clinicopathological features accepted today. It is an uncommon condition with an annual incidence of 1 in 100,000 to 3.6 in 100,000. It affects young adults, occurring most commonly in the third decade in men and in the fourth decade in women [4], with a slightly higher rate of occurrence in women [5]. As a result of the low incidence/prevalence of SRUS, good-quality articles on etiology and therapy are scarce or even lacking. It is associated with other pelvic floor disorders with overlapping symptomatology. Although a variety of both conservative and surgical therapies have been described, a lack of consensus regarding the exact underlying etiology remains.

10.2 Etiology

Physiological and histological studies have illustrated a spectrum of findings suggesting a possible variety of causes [69]. Most authors describe at least internal or external rectal prolapse and a simultaneous opposing force on the rectal mucosa; the downward force of defecation countered by upward force from the pelvic floor generates the trauma required for the formation of SRUS. The forces may lead to mucosal ischemia and subsequent ulceration. In addition, the prolapsed mucosa may be traumatized against the closed anal canal.

While the presence of rectal prolapse, external or internal, is clearly understood, the nature of the opposing upward physiological force is unclear. Some authors suggest another, contributing factor to explain the ischemia hypothesis: an opposing upward physiological force. Anismus or paradoxical puborectalis contraction may cause an obstruction during defecation that necessitates excessive straining to evacuate stool. Increased activity in the external anal sphincter during evacuation, together with high voiding pressures, has been measured [10]. However, it can be questioned whether these findings reflect a true anismus. A reflex secondary to the accompanying pain sensation during the act of defecation, as seen in fissura ani, might be an alternative explanation.

10.3 Pathological Features

Though usually single, SRUS is occasionally multiple. The ulcers are usually situated on the anterior rectal wall. The microscopic histological features include thickening of the mucosa and distortion of the underlying glands. The lamina propria is edematous and may contain an abundance of fibroblasts. There is thickening of the muscularis mucosae and extension of its fibers upward through the glandular crypts. Often, there is erosion of surface epithelium. This is accompanied by a fibrinous exudate and engorged vessels in the superficial lamina propria, but perhaps, surprisingly, no significant inflammatory cell infiltrate [1].

Chiang et al [11] summarized the histological findings of 158 patients: 56% showed an ulcerated pattern, 24% a polypoid pattern, and 20% a flat lesion (also called erythematous lesions). Glandular crypt abnormalities were seen in 91% of patients, fibromuscular obliteration of the lamina propria in 98%, hypertrophied and splayed muscularis mucosae upwards into the lamina propria in 96%, inflammatory cells and granulation tissue infiltration in the lamina propria in 75%, mucosal capillary abnormalities in 48%, hemosiderin deposition in the lamina propria in 53%, surface erosion with fully mature and normal epithelium in 56%, and misplaced glands in the submucosa in 7%.

10.4 Clinical Features

Classically, the symptoms of SRUS are said to be rectal bleeding, passage of mucus, pain or tenesmus, and excessive straining [2]. Chiang et al [11] reported bleeding in 91% of patients, mucous discharge in 77%, rectal pain in 61%, excessive straining in 63%, tenesmus in 64%, digitations in 29%, incontinence in 38%, constipation in 47%, and diarrhea in 18%. Up to 26% of patients can be asymptomatic [1]. Patients often present long after the onset of symptoms. The mean reported duration of symptoms before diagnosis ranges from 3.5 to 5.3 years [11213].

10.5 Diagnosis

Diagnosis is made on sigmoidoscopy. Typically the rectal ulcer is small with a white sloughy base and a hyperemic rim of mucosa, often located anteriorly [14]. Lesions may be multiple (in 30% of patients) [3]. The ulcerated lesion (present in 57% of patients) is not always present, and polypoid lesions (25% of patients) or erythema of the mucosa (18% of patients) are seen instead [12].

An endoanal ultrasound can be considered, especially if fecal incontinence is present, to rule out sphincter injury. In patients with a rectal prolapse, a typical asymmetric thickening of the internal anal sphincter can be seen. If a symmetrical thickening of the internal anal sphincter is seen and patients present with obstructed defecation, a rare condition called hereditary myopathy of the internal anal sphincter can be diagnosed by biopsy of the internal anal sphincter followed by pathological investigation with electron microscopy [15].

Anorectal physiology testing yields variable results in patients with SRUS. The tests neither make the diagnosis nor predict the response of the patient to therapy. The most consistent finding with anorectal physiology is that of relatively high maximum resting pressures, excessive perineal descent, and pudendal neuropathy [16].

Patients with a rectal ulcer deserve a full diagnostic work-up to exclude other causes for excessive straining, even when a relapse prolapse has been shown on defecography (or magnetic resonance [MR] defecography).

Defecography is the gold standard to diagnose occult or internal rectal prolapse. The Oxford rectal prolapse grading system is very useful to determine the severity of prolapse (Fig. 10.1Table 10.1). Simultaneous existence of a rectocele and enterocoele will also be demonstrated on defecography. Although anismus can also be shown on defecography, it has been suggested that defecography is likely to overdiagnose anismus [17]. MR defecography has been shown to be feasible and reveal the same anatomic entities as compared to normal defecography. The (lying) position of examined patient is not physiologic though and because defecating feels unnatural in a lying position it might under diagnose the degree of rectal prolaps( which is best diagnosed with a complete evacuation of stool/contrast).

A978-88-470-5441-7_10_Fig1_HTML.jpg

Fig. 10.1

Oxford rectal prolapse grade: a radiologic grading system (visual appearance)

Table 10.1

Oxford rectal prolapse grade: a radiologic grading system (types and characteristics)

Type of rectal prolapse

Grade of rectal prolapse

Radiological characteristics of rectal prolapse

Internal rectal prolapse

   

Rectorectal intussusception

I (high rectal)

Descends no lower than proximal limit of the rectocele

 

II (low rectal)

Descends into the level of the rectocele, but not onto sphincter/anal canal

Rectoanal intussusception

III (high anal)

Descends into sphincter/anal canal

 

IV (low anal)

Descends into sphincter/anal canal

External rectal prolapse

V (overt rectal prolapse)

Protrudes from anus

A colonic transit study or colonic transit scintigraphy can show or exclude slow transit constipation. For its practical advantages, Arhan’s method [18] is recommended: 10 markers or pellets are ingested on 6 consecutive days (60 markers in total) and an X-ray of the abdomen is performed on the seventh day. The number of markers is counted and this number is multiplied by 2.4 h to give the colonic transit time. Differentiation between right-sided (suggestive of true or primary slow transit constipation) and left-sided (suggestive of secondary slow transit constipation caused by outlet obstruction) slow transit constipation is possible. A colonic transit of more than 50 h is considered abnormal.

10.6 Treatment

Many different treatment strategies have been published. Since SRUS seems to be a symptom caused by other pelvic floor disorders rather than being a pathology by itself, treatment should be focused on the underlying disorders. To treat the solitary rectal ulcer is to treat the (predominant) underlying disorder causing the ulceration. It is mandatory that conservative measurements are comprehensively explored before surgical procedures are offered to the patient.

10.7 Conservative Management

10.7.1 Dietary Fiber

When using dietary fiber, the response rate varies from 19% to 70%, with rectal prolapse patients seeming to benefit least from the use of bulking agents alone [19]. Most investigators have combined the use of additional dietary fiber with behavioral modification to reduce straining. Combination therapy was used successfully for symptom control in 14 of 21 patients.

10.7.2 Topical Agents

Local agents may contribute to healing of the mucosa but they do not address the underlying defecatory disorder and/or anatomy. Topical steroids and sulphasalazine enemas have not been shown to be effective. In contrast, a small study has demonstrated that sucralfate enemas (2 g twice daily for 3–6 weeks) have produced symptomatic improvement and even macroscopic healing on sigmoidoscopy, but the histological changes have persisted [20].

The application of human fibrin glue has also been shown to stimulate fibroblast and vascular proliferation leading to tissue regeneration and mucosal healing. In a small study, all six subjects treated with topical fibrin, increased dietary fiber, and behavioral correction of straining had ulcer healing at 14 days. The healing remained at the 1-year follow-up. In the group of control subjects, treated with fiber and correction of straining alone, none had achieved ulcer healing by 14 days, yet half demonstrated healing at 1 year [21].

10.7.3 Biofeedback

Malouf et al. [22] showed short-term benefit from using biofeedback in 8 of 12 patients, but longer-term benefit in only half this number. Jarrett et al. [23] showed that biofeedback led to a significant rise in mucosal blood flow and postulated that this showed improved extrinsic autonomic nerve activity. Binnie et al. [24] demonstrated a higher recurrence rate in 14 patients treated with surgery (posterior rectopexy) alone compared with a group of 17 who were treated with surgery and biofeedback, either before or immediately after surgery.

10.8 Surgery

The surgical options that have been used for SRUS include perineal treatments (local excision, Delorme’s procedure, stapled transanal rectal resection [STARR]) and abdominal rectopexies of various kinds, almost always posterior, and sometimes combined with resection. Anterior resection and stoma formation have also been used.

10.8.1 STARR

Boccasanta et al. [25] reported the results of STARR in patients who had all received biofeedback and remained refractory to treatment. While ulcer healing was reported as occurring in 100% in this series, 20% of patients remained symptomatic to some degree.

10.8.2 Posterior Rectopexy

Success with posterior rectopexy ranges from 50% to 100% (median 70%). It should be noted that all published series report posterior rectopexy, involving posterior rectal mobilization and rectal denervation. Posterior rectopexy does not support the anterior rectal wall, the usual origin an internal prolapsed. Sitzler et al. [26] described results of SRUS at St Mark’s Hospital, Harrow, London in which 81 patients underwent surgical treatment over a 10-year period and 66 were followed up for at least 12 months. Forty-nine patients underwent posterior rectopexy, nine underwent Delorme’s procedure, two underwent anterior resection, and four patients a primary stoma. Rectopexy succeeded in 27/49 patients (55%); the procedure failed in 22 patients, and of the 19 underwent further surgery including rectal resection and colostomy formation. Eventually, 14 of these patients required permanent colostomy. For the nine patients treated initially with Delorme’s procedure there were four failures at a median follow-up of 38 months. Two of these patients ultimately required a stoma. Seven patients underwent an anterior resection as their initial treatment or as second-line therapy for SRUS, and four of these eventually required a colostomy. Anterior resection was not a successful salvage procedure. The overall stoma rate for the treatment of SRUS in this large series was 30%. Posterior rectopexy resulted in a satisfactory long-term outcome in only 55-60% of patients. The poor outcome after surgery was related to two main factors: incontinence and incomplete evacuation, probably due to rectal denervation associated with posterior rectal dissection.

10.8.3 Ventral Rectopexy

When ventral rectopexy is employed, the success rate is improved [2728]. Ventral mesh rectopexy supports the anterior rectum where intussusception originates, without disturbing the autonomic innervation of the rectum, as happens with posterior rectopexy. However, the number of series detailing the use of this novel treatment for SRUS is limited and further studies are needed to confirm the promising early results.

10.9 Summary

A proportion of patients with SRUS respond to conservative measures such as advice to stop straining, and stool modification with bulking agents and stool softeners. For those who do not respond the main choice is between biofeedback and surgery.

It can be argued that any structural abnormality should be corrected before defecatory re-education commences, but it is reasonable to try biofeedback before surgery. Rectopexy should be a laparoscopic ventral rectopexy rather than a posterior rectopexy.

Perineal procedures such as Delorme’s procedure may result in ulcer healing, but they are also likely to lead to poor function and recurrence. The STARR procedure may be suitable for those in whom an abdominal approach is likely to be difficult.

References

1.

Tjandra JJ, Fazio VW, Church IM et al (1992) Clinical conundrum of solitary rectal ulcer. Dis Colon Rectum 35:227–234PubMedCrossRef

2.

Cruveilhier J (1829) Ulcere chronique du rectum. In: Anatomiepathologique du corps humain. JB Baillière, Paris

3.

Madigan MR, Morson BC (1969) Solitary ulcer of the rectum. Gut 10:871–881PubMedCrossRef

4.

Sharara AI, Azar C, Amr SS et al (2005) Solitary rectal ulcer syndrome: endoscopic spectrum and review of the literature. Gastrointest Endosc 62:755–762PubMedCrossRef

5.

Haray PN, Morris-Stiff GJ, Foster ME (1997) Solitary rectal ulcer syndrome: an underdiagnosed condition. Int J Colorectal Dis 12:313–315PubMedCrossRef

6.

Rao S, Ozturk R, De Ocampo S, Stessman M (2006) Pathophysiology and role of biofeedback therapy in solitary rectal ulcer syndrome. Am J Gastroenterol 101:613–618PubMedCrossRef

7.

Schweiger M, Alexander-Williams J (1977) Solitary-ulcer syndrome of the rectum: its association with occult rectal prolapse. Lancet 309:170–171CrossRef

8.

Sun WM, Read NW, Donnelly TC et al (1989) A common pathophysiology for full thickness rectal prolapse, anterior mucosal prolapse and solitary rectal ulcer. Br J Surg 76:290–295PubMedCrossRef

9.

Kang YS, Kamm MA, Nicholls RJ (1995) Solitary rectal ulcer and complete rectal prolapse: one condition or two? Int J Colorectal Dis 10:87–90PubMedCrossRef

10.

Womack NR, Williams NS, Holmfield JHM, Morrison JFB (1987) Pressure and prolapse the cause of solitary rectal ulcer. Gut 28:1228–1233PubMedCrossRef

11.

Chiang JM, Changchien CR, Chen JR (2006) Solitary rectal ulcer syndrome. Int J Colorectal Dis 21:348–356PubMedCrossRef

12.

Martin CJ, Parks TG, Biggart JD (1981) Solitary rectal ulcer syndrome in Northern Ireland, 1971–80. Br J Surg 68:744–747PubMedCrossRef

13.

Ford MJ, Anderson JR, Gilmour HM et al (1983) Clinical spectrum of “solitary ulcer” of the rectum. Gastroenterology 84:1533–1540PubMed

14.

Madigan MR (1964) Solitary ulcer of the rectum. Proc R Soc Med 47:603

15.

de la Portilla F, Borrero JJ, Rafel E (2005) Hereditary vacuolar internal anal sphincter myopathy causing proctalgia fugax and constipation: a new case contribution. Eur J Gastroenterol Hepatol 17:359–361PubMedCrossRef

16.

Ho YH, Ho JM, Parry BR (1995) Solitary rectal ulcer syndrome: The clinical entity and anorectal physiology findings in Singapore. Aust N Z J Surg 65:93–97PubMedCrossRef

17.

Hompes R, Harmston C, Wijffels N (2012) Excellent response rate of anismus to botulinum toxin if rectal prolapse misdiagnosed as anismus (‘pseudoanismus’) is excluded. Colorectal Dis 14:224–230PubMedCrossRef

18.

Arhan P, Devroede G, Jehannin B et al (1981) Segmental colonic transit time. Dis Colon Rectum 24:625–629PubMedCrossRef

19.

van den Brandt Gradel V, Huibregtse K, Tytgat GN (1984) Treatment of solitary rectal ulcer syndrome with high fibre diet and abstention of straining at defaecation. Dig Dis Sci 29:1005–1008PubMedCrossRef

20.

Zagar SA, Khuroo MS, Mahajan R (1991) Sucralfateretension enemas in solitary rectal ulcer. Dis Colon Rectum 34:455–457CrossRef

21.

Ederle A, Bulighin G, Orlandi PG, Pilati S (1992) Endoscopic application of human fibrin sealant in the treatment of solitary rectal ulcer syndrome [letter]. Endoscopy 24:736–737PubMedCrossRef

22.

Malouf AJ, Vaizey CJ, Kamm MA (2001) Results of behavioural treatment (biofeedback) for solitary rectal ulcer syndrome. Dis Colon Rectum 44:72–76PubMedCrossRef

23.

Jarrett M, Vaizey CJ, Emmanuel AV et al (2004) Behavioural therapy (biofeedback) for solitary rectal ulcer syndrome improves symptoms, mucosal blood flow. Gut 53:368–370PubMedCrossRef

24.

Binnie NR, Papachrysostomou M, Clare N, Smith AN (1992) Solitary rectal ulcer: the place of biofeedback and surgery in the treatment of the syndrome. World J Surg 16:836–840PubMedCrossRef

25.

Boccasanta P, Venturi M, Calabro G et al (2008) Stapled transanal rectal resection in solitary rectal ulcer associated with prolapse of the rectum: a prospective study. Dis Colon Rectum 51:348–354PubMedCrossRef

26.

Sitzler PA, Kamm MA, Nicholls RJ (1996) Surgery for solitary rectal ulcer syndrome. Int J Colorectal Dis 11:136

27.

Evans C, Jenes OM, Cunningham C, Lindsey I (2010) Management of solitary rectal ulcer syndrome: Ignore the ulcer, treat the underlying posterior compartment prolapse [abstract]. Colorectal Dis 12:23

28.

Badrek-Amoudi A, Roe T, Mabey K et al (2012) Laparoscopic ventral mesh rectopexy (LVMR) in the management of solitary rectal ulcer syndrome (SRUS): a cause for optimism? Colorectal Dis 15:575–581CrossRef