Internal rectal intussusception—Fact or fancy?

PURPOSE: There is still considerable debate whether internal intussusception represents a functional disorder. We have reviewed our results in an effort to define its symptomatology and to assess defecography. METHODS: Rectopexy has been performed for internal intussusception in 37 patients. Eighteen had solitary rectal ulcer syndrome (SRUS), and 31 had anterior rectal wall prolapse. Defecography demonstrated anterior wall prolapse in 13, circular prolapse in 21, and no disorders in 3 patients. Pelvic floor function was normal. Follow-up varied from one to nine years. RESULTS: Twenty-six patients became asymptomatic. Anterior wall prolapses could not be palpated anymore. All SRUS lesions healed. Patients with SRUS (P<0.001) or circular prolapse (P<0.001) became significantly more asymptomatic. Results in patients with anterior rectal wall prolapse were significantly worse (P<0.001). CONCLUSIONS: Internal intussusception is a distinct functional rectal disorder. Its symptomatology and findings during physical examination are aspecific. Characteristic defecographic features and presence of SRUS are indications for surgery, provided pelvic floor function during straining is normal.     

Diagnosis of functional disorders of defecation causing the solitary rectal ulcer syndrome

The solitary rectal ulcer and colitis cystica profunda are different manifestations of the solitary rectal ulcer syndrome. The cause of solitary rectal ulcer syndrome remains unknown. Since defecation disorders are common among patients with solitary rectal ulcer syndrome, defecography is indicated. Defecography was performed on 19 patients with solitary rectal ulcer syndrome. In five patients, the spastic pelvic floor syndrome had occurred. Twelve patients had internal intussusception of the rectum, and one patient had an anterior rectal wall prolapse. In one patient, no abnormalities could be detected. These abnormalities led to severe straining, which can damage the anterior rectal wall. Findings strongly support the hypothesis that solitary rectal ulcers are traumatic lesions caused by straining. Defecography is a suitable procedure for detecting the causative disorder of defecation and for selecting patients for treatment. Read at the meeting of the American Society of Colon and Rectal Surgeons, San Diego, California, May 5 to 10, 1985. No reprints are available.     

Anorectal Physiology in Solitary Ulcer Syndrome: A Case-Matched Series

PURPOSE Solitary ulcer syndrome is a rare condition characterized by inflammation and chronic ulcer of the rectal wall in patients suffering from outlet constipation. Despite similar surgical options (rectopexy, anterior resection), solitary ulcer syndrome may differ from overt rectal prolapse with regard to symptoms and pathogenesis. The present work analyzed differences between these conditions in a case-control physiology study. METHODS From 1997 to 2002, 931 consecutive subjects were investigated in a single physiology unit for anorectal functional disorders. Standardized questionnaires, anorectal physiology, and evacuation proctography were included in a prospective database. Diagnosis of solitary ulcer syndrome was based on both symptoms and anatomic features in 25 subjects with no overt rectal prolapse (21 females and 4 males; mean age, 37.2 ± 15.7 years) and no past history of anorectal surgery. They were compared with age-matched and gender-matched subjects: 25 with outlet constipation (also matched on degree of internal procidentia), 25 with overt rectal prolapse without any mucosal change, and 14 with overt rectal prolapse and mucosal changes. RESULTS Subjects with solitary ulcer syndrome reported symptomatic levels (digitations, pain, incontinence) similar to those of patients with outlet constipation, but they had significantly more constipation and less incontinence than patients with overt rectal prolapse. Compared with each of the three control groups (dyschezia, rectal prolapse without mucosal change, and rectal prolapse with mucosal change), subjects with solitary ulcer syndrome more frequently had an increasing anal pressure at strain (15 vs. 5, 3, and 1, respectively ; P < 0.01) and a paradoxical puborectalis contraction (15 vs. 9, 1, and 1, respectively; P < 0.05). With respect to evacuating proctography, complete rectal emptying was achieved less frequently in this group (5 vs. 12, 23, and 10, respectively; P < 0.05). Compared with patients with overt rectal prolapse, mean resting and squeezing anal pressures were significantly higher in both groups of subjects with solitary ulcer syndrome and with outlet constipation. Prevalence and levels of anatomic disorders (perineal descent, rectocele) did not differ among the four groups except for rectal prolapse grade and prevalence of enterocele (higher in overt rectal prolapse group). Interestingly, and despite matched controls for degree of intussusception, individuals with solitary ulcer syndrome had circular internal procidentia more often compared with those suffering from outlet constipation without mucosal lesions (15 vs. 8, P < 0.05). CONCLUSION This case-controlled study quantifies functional anal disorders in patients suffering from solitary ulcer syndrome. Despite no proven etiologic factor, sphincter-obstructed defecation and circular internal procidentia both may play an important part in the pathogenesis and an exclusive surgical approach may not be appropriate in this context. Presented at the meeting of the American Gastroenterology Association, New Orleans, Louisiana, May 18, 2004.     

The pathogenesis and pathophysiology of rectal prolapse and solitary rectal ulcer syndrome

Rectal prolapse and solitary rectal ulcer syndrome are both benign conditions affecting the rectum, mainly in women; prolapse tends to occur late in life, while solitary rectal ulcer syndrome has a predilection for the younger adult. Complete rectal prolapse probably starts as a mid-rectal intussusception, although a combination of this theory and the 'sliding hernia' theory has been proposed by Altemeier et al (1971). The pelvic floor weakness associated with prolapse, which gives rise to incontinence, is most likely due to a traction injury to the pudendal nerve. Anorectal manometry will indicate those incontinent patients likely to benefit from rectopexy. Abnormal descent of the perineum may be found in rectal prolapse and solitary rectal ulcer syndrome as well as descending perineum syndrome per se. The clinical features of these three conditions can overlap. Solitary rectal ulcer syndrome is essentially due to prolapse and traumatization of the rectal mucosa. Inappropriate puborectalis contraction, abnormal perineal descent, and overt rectal prolapse have all been cited as possible mechanisms of development of the condition. Defecography is the radiologic investigation of choice. Electromyography, as in rectal prolapse, may show evidence of pudendal nerve damage although incontinence is rare.     

Transrectal ultrasound study of the pathogenesis of solitary rectal ulcer syndrome.

Transrectal ultrasonography is of clinical value in anorectal carcinoma and in inflammatory diseases of the anorectum. In this study a rigid linear endorectal probe was used to examine 15 patients with endoscopically and biopsy proved diagnosis of solitary rectal ulcer syndrome. In 13 of the 15 patients the rectal wall was thicker (mean (SEM) 5.7 (0.4) mm; normal values: 2.8 (0.1) mm) near the rectal ulcer. In all these cases the muscularis propria layer exceeded the maximum normal diameter of 2 mm. In nine of the 15 patients the normal rectal wall echo-structure, with five distinct layers, was disturbed and there was fading of the borders between the mucosa and the muscularis propria. Poor relaxation of the puborectalis muscle during straining was seen on ultrasound in 11 patients, as was intussusception of the rectal wall. The obvious enlargement of the muscularis propria points to a chronic mechanical load on the rectal wall. The ulcerative lesions are formed in this area of overloaded rectal wall. The direct visualisation of the puborectalis muscle during dynamic transrectal ultrasonography indicates that the fact that it does not relax is an important element in the pathogenesis of solitary rectal ulcer syndrome.     

Solitary rectal ulcer and complete rectal prolapse: one condition or two?

We studied the physiological features of patients with complete rectal prolapse and different degrees of solitary rectal ulcer syndrome to determine whether these conditions are likely to form part of the same disorder. 52 solitary rectal ulcer patients (median age 31, 40 females), and 15 complete rectal prolapse patients (median age 31, 12 females) were studied. Solitary rectal ulcer patients were divided into 3 groups, based on the extent of accompanying rectal prolapse (no prolapse, internal prolapse, or external prolapse). Both solitary rectal ulcer patients without prolapse and with internal prolapse had significantly higher maximum anal resting (p<0.01 for both groups) and squeeze pressure (p<0.05 for both groups) than complete rectal prolapse patients. In contrast, solitary rectal ulcer patients having external prolapse were similar to those with complete rectal prolapse. Solitary rectal ulcer patients without rectal prolapse had significantly decreased anal and rectal electrosensitivity (p<0.01 for both) when compared to healthy control subjects. Solitary rectal ulcer patients therefore have a spectrum of clinical and physiological features — this condition may comprise a range of different disease entities. The findings also suggest a different underlying aetiopathophysiology of solitary rectal ulcer from that of complete rectal prolapse.

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Résumé Nous avons étudié les données physiologiques de patients présentant un prolapsus rectal complet et différents degrés du syndrome d'un ulcère solitaire du rectum afin de déterminer si ces conditions constituent des composantes d'un même trouble. Cinquante-deux patients porteurs d'un ulcère solitaire du rectum (médiane 31 ans, 40 femmes) et 15 prolapsus complets du rectum (âge médian 31; 12 femmes) ont été étudiés. Les ulcères solitaires du rectum ont été répartis en 3 groupes selon l'extension du prolapsus rectal (pas de prolapsus, prolapsus interne, prolapsus extériorié). Les patients porteurs d'un ulcère solitaire du rectum sans prolapsus et ceux porteurs d'un prolapsus interne ont une pression anale de repos maximale élevée de manière significative (P<0.001 dans les deux groupes) et une pression de rétention (P<0.005) dans les deux groupes ainsi qu'un canal anal plus long (P<0.005 dans les deux groupes comparativement aux patients porteurs d'un prolapsus rectal complet). A l'inverse, les patients porteurs d'un ulcère solitaire du rectum accompagné d'un prolapsus externe présentaient des valeurs similaires à celles observées en cas de prolapsus rectal complet. Les patients porteurs d'un ulcère solitaire du rectum sans prolapsus ont une diminution significative de l'électrosensitivité anale et rectale (P<0.001 dans les deux groupes) en comparaison à des sujets sains. Les patients porteurs d'un ulcère solitaire du rectum ont donc un large spectre d'anomalies cliniques et physiologiques pouvant comprendre un nombre très varié d'entités pathologiques différentes. Ces constatations suggèrent une étiopathophysiologie différente en cas d'ulcère solitaire du rectum qu'en cas de prolapsus rectal complet.

     

Solitary rectal ulcer syndrome accompanied by submucosal invasive carcinoma

We report a case of carcinoma in solitary rectal ulcer syndrome. The diagnosis was made by colonoscopic appearance and biopsy. A tumor measuring  0.9 × 0.6 cm  was found in a resected solitary rectal ulcer. The lesion exhibited typical histological features of solitary rectal ulcer syndrome, with a well differentiated adenocarcinoma invading submucosal layers and some dysplastic glands. We believe that the adenocarcinoma represents a malignant transformation from solitary rectal ulcer syndrome, because similar to longstanding chronic idiopathic colitis, colorectal dysplasia and carcinoma may develop.     

Pressure and prolapse--the cause of solitary rectal ulceration

The cause of solitary rectal ulceration has been investigated using a method that radiologically visualises rectal voiding whilst simultaneously measuring intrarectal pressure and external anal sphincter electromyographic activity. Control subjects and patients with the solitary rectal ulcer syndrome, both with and without mucosal ulceration, have been studied. A high incidence of rectal prolapse (94%) was present in the patients who voided. Overactivity of the anal sphincter during evacuation contributed to the fact that patients with mucosal ulceration required higher intrarectal pressures to void than the controls and the patients without mucosal ulceration. The results indicate that a combination of rectal prolapse and a high voiding pressure may act to cause the mucosal ulceration in this syndrome by exposing the rectal wall to a high transmural pressure gradient.     

The solitary rectal ulcer syndrome

The solitary rectal ulcer syndrome is a rare debilitating disorder of the rectum characterized by perianal chronic pain with passage of blood and mucus. The pathogenesis remains uncertain, rectal prolapse and trauma from straining are the main hypothesis. The diagnosis includes clinical symptoms associated with endoscopic lesion (erythema, ulcer or polypo?d lesion) and histological features. Mano-metric studies and defecography are helpful to determinate an underlying defecation disorder or rectal prolapse. The treatment is controversial including a conservative option (medications and behavioural therapy) with poor long term results, and the surgical option (treatment of a rectal prolapse with or without resection of the lesion), more aggressive with uncertain results in a long follow-up period.     

A case of rectal cancer arising from long-standing prolapsed mucosa of the rectum

Mucosal prolapse syndrome (MPS) has been recognized as a chronic benign inflammatory disorder, characterized mainly by rectal mucosal prolapse. Disorders representing this condition include solitary rectal ulcer syndrome (SRUS), rectal prolapse, proctitis cystica profunda, and inflammatory cap polyps. The gross appearance of rectal MPS can be occasionally misinterpreted as rectal cancer. In contrast, there have been a few reports of colorectal cancer originating from prolapsed mucosa. Herein, we report a case of MPS associated with two independent rectal cancers extending into the submucosal layer. We speculate that long-standing MPS may increase the risk of malignant transformation.     

Barium enema and defaecography in the diagnosis and evaluation of the solitary rectal ulcer syndrome

Forty-three patients with histologically proven solitary ulcer syndrome of the rectum were examined by defaecography and 33 by barium enema. Barium enema showed changes in the rectum in all cases. Thickening of the rectal folds and spasm were most common, followed by ulceration and pseudopolypoid change. None of these changes is individually pathognomonic of the solitary ulcer syndrome, but viewed in conjunction they are highly suggestive of the condition. During defaecography, intussusception of the rectum was observed in 34 cases (79%). In 19 (44%) a complete external prolapse was present while intra-anal and intra-rectal intussusception was found in 15 (35%). Intussusception arose in most cases from the mid-rectum, and rarely from a rectal mucosal prolapse of the ampulla. Awareness of the abnormalities of the solitary ulcer syndrome on barium enema enables the radiologist to suggest the diagnosis and recommend defaecography to establish the functional disorder, which may help determine the appropriate medical or surgical treatment.     

Solitary rectal ulcer syndrome presenting with rectal prolapse, severe mucorrhea and eroded polypoid hyperplasia: case report and review of the literature.

A case of solitary rectal ulcer syndrome in a 36-year-old woman presenting with severe, persistent mucorrhea and eroded polypoid hyperplasia as the predominant clinical features, who was ultimately noted to have symptoms of rectal prolapse, is presented. Endoscopically, she had multiple (50 to 60) small, whitish polypoid lesions in the rectum that were initially misinterpreted as being a carpeted villous adenoma, juvenile polyposis or atypical proctitis. The lesions were treated with argon plasma coagulation with resolution, but a solitary rectal ulcer developed. The patient then admitted to a history of massive rectal prolapse over the preceding six months and underwent surgical treatment. Severe mucorrhea as the presenting feature and the presence of multiple polypoid lesions consistent with a histological diagnosis of eroded polypoid hyperplasia make the present case unique.     

Transanal repair of rectocele and full rectal mucosectomy with one circular stapler: a novel surgical technique

Abstract We present a new surgical stapling technique for treatment of rectocele when associated with internal mucosal prolapse or haemorrhoids using only one circular mechanical stapler. Eight female patients, mean age 53 years (range, 42–70), complaining of obstructed defecation with vaginal digitation because of rectocele associated with internal mucosal prolapse underwent transanal repair of rectocele and rectal mucosectomy using one circular stapler between April and July 2004. A running horizontal mattress suture was placed through the base of the rectocele including mucosa, submucosa and the muscle layer of the whole anterior anorectal junction wall. The prolapsed mucosa and the muscular layer were then excised with an electrical scapel. Acontinuous pursestring rectal mucosa suture was placed 0.5 cm before the previous anterior mucosa and muscle layers resected wound, including the anorectal junction wall which was kept separate from the posterior vaginal wall by a Babcock forceps. Posteriorly, the pursestring suture included only mucosal and submucosal layers. The stapled suture was positioned between normal anterior rectal wall and the anal canal, 0.5 cm above the pectinate line. The stapler was then closed, fired and withdrawn. One patient complained of a perianal hematoma on the seventh postoperative day, requiring surgical excision. Postoperative defecography showed correction of the rectocele and outlet obstruction disappeared in all patients. This novel combined manual-stapled technique for rectocele and rectal internal mucosal prolapse seems to be a safe procedure and the preliminary results are encouraging. Further investigations have to be performed to assess long-term outcome in a larger number of patients.     

Is solitary rectal ulcer a manifestation of a systemic disease?

In a prospective study of 22 patients with solitary rectal ulcer, we tried to define the features of this condition, especially the associated systemic features, that may give some clues to its etiopathogenesis. In 15 of these patients a single rectal ulcer was found, whereas in seven patients two ulcers were present in each. Of the total 29 ulcers in these patients, 19 were located on the anterior or anterolateral wall of the rectum and 10 were on the posterior or posterolateral wall. The sigmoidoscopic appearance of the ulcer was quite characteristic, yet a biopsy was considered essential to rule out other pathologic processes. Histological features of the solitary rectal ulcer comprised fibrous obliteration of the lamina propria with disorientation of the muscularis mucosa and extension of muscle fibers into the lamina propria. Evident rectal prolapse was present in only three patients. Recurrent oral ulcerations occurred in four (18.2%) patients and erythema nodosum in one of them (4.5%). Sacroiliitis was present in six of 19 (31%) patients studied radiologically, and human leukocyte antigen (HLA)-B27 occurred in four of the 20 patients (20%) tested for HLA class I antigens. All the four HLA-B27-positive patients had associated sacroiliitis and showed good response to sulfasalazine. These associations raise the possibility that solitary rectal ulcers may be a part of a systemic disease or of several diseases with varied etiology.     

Anorectal ergotism: another cause of solitary rectal ulcers

Anorectal ulceration was observed in 6 patients who excessively used suppositories containing ergotamine tartrate. The mucosal lesions of the rectum resembled those observed in the "solitary rectal ulcer syndrome." However, characteristic features of ergotamine-induced ulcers are absence of a mucosal prolapse, lack of a history of constipation, and rapid healing after discontinuation of the drug. Furthermore, the rectal lesion may be associated with anal ulceration, which occasionally presents as the only clinical manifestation of "anorectal ergotism."     

Clinical and manometric features of the solitary rectal ulcer syndrome

We report 33 patients with the solitary rectal ulcer syndrome seen between 1975 and 1982. Twenty-four were women and the median age was 32 years. The principal symptoms were bleeding (89 per cent), chronic constipation (64 per cent), rectal pain (42 per cent), tenesmus (42 per cent), and mucous discharge (45 per cent). Twenty-eight patients gave a history of straining (85 per cent). A full-thickness rectal prolapse was present in six patients, an anterior rectal prolapse was observed in 11, and 12 patients had clinical evidence of perineal descent. Defecation was only possible by digital evacuation in six women, and three male patients were passive homosexuals. Manometric studies were performed on 16 patients; eight patients were unable to tolerate rectal distension with 200 cc of air and impaired rectal sensation was present in two. Rectal distention was associated with bursts of involuntary external sphincter activity in three patients, and the distention reflex was absent in six, despite normal ganglia on rectal biopsy. In only four patients was there electromyographic evidence of contraction of the puborectalis during attempted defecation. Fourteen patients were treated by rectopexy; healing of the ulcer occurred in five of six with a full-thickness prolapse compared with only two of eight without a complete prolapse. Work performed at the General Hospital, Birmingham, England.     

Solitary rectal ulcer

Two cases of solitary rectal ulcer syndrome occurred in an 18-year-old woman and a 24-year-old man. Each had a history of bleeding on defecation, together with constipation alternating with diarrhea. In each case, a solitary rectal ulcer was noted by sigmoidoscopy and confirmed by both light and electron microscopic evaluations of biopsy material. Both clinical and pathologic features of these cases are compatible with classic rectal ulcer syndrome, which has been suggested to result from trauma followed by an ischemic event leading to fibrosis of the mucosa. Fibrosis in these two cases was confirmed by electron microscopy.     

Solitary rectal ulcer syndrome

A retrospective study of 20 cases of biopsy-proven solitary rectal ulcer syndrome seen at a large oncology hospital which highlight the clinicopathologic features is presented. Thirty five percent of patients were in their sixth decade and there was a male preponderance of 2.3∶1. All patients presented with anorectal symptoms. Eighty five percent had normal abdominal findings and seventy five percent had positive findings on rectal examinations. Fifty four percent had a diagnosis of carcinoma of the rectum on barium enema studies. On sigmoidoscopy, a solitary ulcer was found in 80 percent; 50 percent had lesions between 6 and 10 cm from the anal verge, and 40 percent had the lesion on the anterior rectal wall. All patients were treated conservatively and 35 percent were disease free at the end of five years, while 30 percent had persisting symptoms.     

Solitary rectal ulcer syndrome: a series of 13 patients operated with a mean follow-up of 4.5 years

Solitary rectal ulcer syndrome (SRUS) is an infrequent pathology often associated with pelvic floor disorders. The aim of this retrospective study was to review the long-term results of a surgical series of SRUS. Between 1988 and 1998, 13 patients were operated on for SRUS. Seven patients had associated internal rectal prolapse (58%), two had associated total rectal prolapse (15%), and two had associated mucosal prolapse (15%). We performed simple resection of the SRUS in one case (8%), a stoma as primary operation in one (8%), three rectopexies according to Orr-Loygue (23%), and eight Delorme's operations as modified by Berman (62%). Mean follow-up was 57 months (range 15-112). Simple resection of the solitary rectal ulcer syndrome did not improve symptoms. Colostomy permitted relief of symptoms and healing of the SRUS. Two of the three rectopexies achieved good results, and the third patient relapsed at the 6th postoperative month. A secondary modified Delorme's operation permitted relief of symptoms and healing of the SRUS. Five of the eight patients (62.5%) who received modified Delorme's operations had improved at a follow-up of 46 months. We conclude that, considering the high failure rate after surgery, operations should be performed only in patients with total rectal prolapse or intractable symptoms not amenable to behavioral therapy. Delorme's operation and abdominal rectopexy help in about 60% of cases.     

Anorectal function in the solitary rectal ulcer syndrome

The anorectal function of nine patients with solitary rectal ulcer syndrome (SRUS) (5 F: 4 M, median age, 27 (range, 19–41 years) and nine control subjects (5 F: 4 M, median age, 47 (35–66)P<0.01) has been investigated by a new technique that radiologically visualizes the anorectum during voiding of a semisolid contrast medium, while simultaneously measuring intrarectal pressure and anal sphincter EMG activity. A degree of rectal prolapse was demonstrated in eight of the SRUS patients; six of these lesions were clinically occult. Abnormal failure of the anal sphincter to relax on voiding was present in seven of the SRUS patients. These abnormalities resulted in the SRUS patients requiring a greater increase in intrarectal pressure (median, 100 cm water) to void than the control subjects (median, 65 cm water,P<0.01). This combination of high intrarectal pressure and rectal prolapse during straining seems to be the cause of SRUS This work was supported by a grant from the Medical Research Council.