Solitary Rectal Ulcer Syndrome in Children: A Report of Six Cases

Solitary rectal ulcer syndrome (SRUS) is a rare, benign disorder in children that usually presents with rectal bleeding, constipation, mucous discharge, prolonged straining, tenesmus, lower abdominal pain, and localized pain in the perineal area. The underlying etiology is not well understood, but it is secondary to ischemic changes and trauma in the rectum associated with paradoxical contraction of the pelvic floor and the external anal sphincter muscles; rectal prolapse has also been implicated in the pathogenesis. This syndrome is diagnosed based on clinical symptoms and endoscopic and histological findings, but SRUS often goes unrecognized or is easily confused with other diseases such as inflammatory bowel disease, amoebiasis, malignancy, and other causes of rectal bleeding such as a juvenile polyps. SRUS should be suspected in patients experiencing rectal discharge of blood and mucus in addition to previous disorders of evacuation. We herein report six pediatric cases with SRUS.     

Defecation disorders: An important subgroup of functional constipation, its pathophysiology, evaluation and treatment with biofeedback.

Functional defecation disorders are common and affect approximately 50% of patients with chronic constipation. The etiology of functional defecation disorders is not well known, but several pathophysiologic mechanisms have been described, including failure of rectoanal coordination, paradoxical anal contraction or insufficient relaxation of anal sphincter during defecation and impairment of rectal sensation as well as secondary slowing of colonic transit. Symptoms alone are inadequate to distinguish patients with defecation disorders from those with other types of constipation. Detailed clinical evaluation and anorectal physiologic tests are required for definitive diagnosis. There is also a pathophysiological association between functional defecation disorders and other anorectal conditions such as solitary rectal ulcer syndrome. Among the various treatment modalities, biofeedback therapy has emerged as an effective and specific treatment method for functional defecation disorders. The main goals of biofeedback therapy are to relax the pelvic floor and anal sphincter muscles during defecation and to improve rectoanal coordination and sensory perception. Today, it is possible to diagnose most functional disorders of defecation and either effectively treat or ameliorate symptoms in a majority of these patients.     

Studies of Manometric Abnormalities of the Rectoanal Region during Defecation in Constipated and Soiling Children: Modification through Biofeedback Therapy

Anorectal manometry was performed on 12 normal children and 18 patients suffering from constipation and soiling. In both groups, the results of the rectoanal inhibitory reflex and the squeezing anal pressure were similar. The pressure recording in all the normal children showed that the anal canal relaxed during defecation. Fourteen (78%) constipated children closed the anal canal while straining by contracting the anal sphincter. This paradoxical contraction appears to be the cause of chronic constipation. Twelve children with paradoxical anal closure were treated by biofeedback therapy. The results show that all these children were successfully conditioned to relax their anal sphincter during defecation. This therapy improved their bowel habits and relieved them from constipation and soiling. It is proposed that the paradoxical anal closure itself is the result of a self-conditioning process. In this process, the patient learns to paradoxically contract the external anal sphincter in response to the urge and the act of defecation. Biofeedback therapy seems to be the appropriate treatment in such cases.     

慢性功能性便秘肛门直肠敏感性和排便动力学研究

目的探讨慢性功能性便秘患者肛门直肠感觉功能的改变及排便时肛门直肠动力学特征。方法采用PC Polygraf HR高分辨多道胃肠功能测定仪检测46例功能性便秘患者的肛门直肠压力、直肠容量感知、疼痛阈值、耐受阈值及排便功能等指标，并与16例健康人做对照。结果功能性便秘组直肠静息压、肛管括约肌静息压、最大缩窄压及静态肛管长度与对照组比较差异无显著性，但功能性便秘患者的初始感觉阈值、疼痛阈值、排便阈值、引起肛门直肠抑制反射的最低充气量、排便状态下肛管功能长度和肛管内括约肌松弛压均高于对照组。模拟排便时，功能性便秘组34．78％的患者在直肠收缩时伴有肛管括约肌的矛盾收缩。结论功能性便秘患者直肠黏膜对容量刺激的反应性降低和排便时肛管括约肌的反向矛盾收缩可能是形成便秘的原因之一。     

Factors determining outcome in children with chronic constipation and faecal soiling.

To evaluate factors which might contribute to treatment failure in children with chronic constipation and soiling, we evaluated the history, physical findings, defecation dynamics, and anorectal function in 97 patients. We treated them with milk of magnesia, high fibre diet, and bowel training techniques and evaluated outcome at one year when 43% had recovered. Recovery rates were similar for boys and girls. Fifty seven per cent of the patients had not recovered. This group at the outset had more frequent soiling episodes, more severe constipation, were less likely to defecate water filled rectal balloons and to relax the external sphincter during defecation. In general girls had more severe constipation, abdominal pain, and a previous urinary tract infection than boys. Girls were more compliant during treatment and had less frequent soiling episodes at one year. Stepwise logistic regression showed that severe constipation, abnormal contraction of the external sphincter and pelvic floor during attempted defecation, and inability to defecate the 100 ml balloon in less than or equal to 1 min was significantly related to treatment failure. Defecation of smaller balloons, volumes for threshold of rectal sensation, critical volume and rectal contraction, and compliance with treatment could not predict treatment failure.     

Solitary rectal ulcer syndrome:Clinical features,pathophysiology,diagnosis and treatment strategies

Solitary rectal ulcer syndrome(SRUS)is an uncommon benign disease,characterized by a combination of symptoms,clinical findings and histological abnormalities.Ulcers are only found in 40%of the patients;20%of the patients have a solitary ulcer,and the rest of the lesions vary in shape and size,from hyperemic mucosa to broad-based polypoid.Men and women are affected equally,with a small predominance in women.SRUS has also been described in children and in the geriatric population.Clinical features include rectal bleeding,copious mucus discharge,prolonged excessive straining,perineal and abdominal pain,feeling of incomplete defecation,constipation,and rarely,rectal prolapse.This disease has well-described histopathological features such as obliteration of the lamina propria by fibrosis and smooth muscle fibers extending from a thickened muscularis mucosa to the lumen.Diffuse collage deposition in the lamina propria and abnormal smooth muscle fiber extensions are sensitive markers for differentiating SRUS from other conditions.However,the etiology remains obscure,and the condition is frequently associated with pelvic floor disorders.SRUS is difficult to treat,and various treatment strategies have been advocated,ranging from conservative management to a variety of surgical procedures.The aim of the present review is to summarize the clinical features,pathophysiology,diagnostic methods and treatment strategies associated with SRUS.     

排便的生理和便秘的病理生理

慢性便秘可以看作是不同病理生理过程的最终症状表现 ,其胃肠运动主要出现以下病理生理变化 :( 1)结肠运动功能异常 :表现为运动亢进、减弱、不协调。结肠非推进性收缩幅度、频率增加 ,肠传输时间延长 ,肠内容物水分吸收过多 ,粪便干燥 ;结肠推进性收缩的幅度频率减少、结肠蠕动无力 ,肠传输时间增加 ,粪便无法正常推送至直肠 ;不协调运动 ,结肠某一部分收缩增强、逆向蠕动 ,影响传输时间 ;( 2 )直肠运动异常 :直肠张力下降 ,顺应性增加 ,感觉功能下降 ;( 3 )肛门括约肌功能异常 ;( 4 )盆底肌群收缩功能下降 ,持续收缩、不协调收缩或松弛。本文对排便的生理和便秘的病理生理进行综述     

Outpatient protocol for biofeedback therapy of pelvic floor outlet obstruction

Pelvic floor outlet obstruction is a rare cause of severe constipation. Anal myectomy, subtotal colectomy, and medical therapy have limited success. The purpose of this study was to develop a short outpatient treatment using biofeedback techniques. Nine patients with severe constipation and straining resulting from pelvic floor outlet obstruction underwent complete investigation of the pelvic floor musculature and anal sphincter mechanism. Patients were unable to expel a 60-cc rectal balloon and had nonrelaxing puborectalis on defecography. The treatment protocol utilized anal surface electromyography to document improper straining and retrain pelvic floor muscles to relax during defecation. Sensory retraining with a rectal balloon, behavioral relaxation techniques, and defecation of simulated stool using a 120-cc Metamucil^® (Procter & Gamble, Cincinnati, OH) slurry in the rectum allowed re-establishment of normal defecation in all nine patients. Repeat training was required in three patients during follow-up. Treatment of pelvic floor outlet obstruction with outpatient retraining techniques is possible.Read at the meeting of The American College of Gastroenterology, San Francisco, California, October 1990.     

Biofeedback therapy for dyssynergic defecation

INTRODUCTION Chronic constipation is a common self-reported bowel symptom that affects 2%-30% of people in Western countries and has considerable impact on health expenses and quality of life[1]. Most patients respond either to fiber- fluid supplementatio…     

Effects of biofeedback on obstructive defecation--reconditioning of the defecation reflex?

Twenty two patients with obstructive defecation were recruited for relaxation training by domiciliary self regulatory biofeedback. Each patient served as his or her own control for anorectal and proctographic assessments. Biofeedback training improved the obstructive symptoms of the patients and showed significant change in various parameters related to the obstructive defecation syndrome. As examined by isotope dynamic proctography: the defecation rate (% of evacuation/defecation time) was significantly increased (p < 0.05), the anorectal angles at rest and during attempted defecation were made more obtuse (p < 0.05), and the pelvic floor movements were made more dynamic on voluntary contraction of the anal sphincter (p < 0.03). The external anal sphincter electromyographic voltage recorded during defecation was significantly reduced (p < 0.0005) as was the surface anal plug electromyographic electrode voltage (p < 0.0001), which was associated with a greatly reduced anismus index (p < 0.0001). The rectal sensation was improved (p < 0.05), concomitantly. Biofeedback thus improves the defecation act in patients suffering from inappropriate contraction of the pelvic floor and sphincter musculature. Furthermore, this study has shown that biofeedback objectively influences the defecation reflex leading to an improved quality of higher control of bowel function.     

Update of tests of colon and rectal structure and function

This review deals with the indications, methods, strengths, and limitations of anorectal testing in clinical practice. In chronic constipation, anal manometry and a rectal balloon expulsion test, occasionally supplemented by defecography, are useful to identify a functional defecatory disorder, because symptoms may respond to pelvic floor retraining. In patients with fecal incontinence, diagnostic testing complements the clinical assessment for evaluating the pathophysiology and guiding management. Manometry measures anal resting and squeeze pressures, which predominantly reflect internal and external anal sphincter function, respectively. Defecation may be indirectly assessed by measuring the recto-anal pressure gradient during straining and by the rectal balloon expulsion test. Endoanal ultrasound and magnetic resonance imaging (MRI) can identify anal sphincter structural pathology, which may be clinically occult, and/or amenable to surgical repair. Only MRI can identify external sphincter atrophy, whereas ultrasound is more sensitive for internal sphincter imaging. By characterizing rectal evacuation and puborectalis contraction, barium defecography may demonstrate an evacuation disorder, excessive perineal descent or a rectocele. Dynamic MRI can provide similar information and also image the bladder and genital organs without radiation exposure. Because the measurement of pudendal nerve latencies suffers from several limitations, anal sphincter electromyography is recommended when neurogenic sphincter weakness is suspected.     

Defecometry

The parameters of defecation,i.e., maximum rectal pressure increase during straining, duration of effective evacuation, and the work performed to evacuate a simulated stool, can be quantified by defecometry, a new method to evaluate the defecation act. Simultaneous anal pressure records demonstrate the nature of the sphincter activity during simulated defecation. The test was performed on 19 patients with constipation and on 14 controls. Five patients could not evacuate the simulated stool, while five others could, but more laboriously than the remaining nine patients whose defecation was comparable with the controls. Laborious defecation is characterized by longer duration and more performed work during evacuation. Every patient with difficult or ineffective evacuation had sphincter contraction during defecation, whereas this phenomenon was not observed in patients with normal defecation. Defecometry permits more adequate identification and characterization of the outlet-obstruction-type constipated patients than the simple balloon expulsion test and the analysis of sphincter activity during straining with empty rectum in lateral decubitus. Early diagnosis and treatment of patients with outlet obstruction is important to avoid late neuromuscular damage to the pelvic floor.     

Behavioral medicine treatment in chronic constipation with paradoxical anal sphincter contraction

Nine women and five children with severe chronic constipation received behavioral medicine therapy. Before treatment, all patients had a paradoxical contraction of the external anal sphincter at defecation attempts as demonstrated with electromyography and/or anorectal manometry. An electromyographic biofeedback device connected to an anal probe was used for the training that was performed on a regular toilet seat during five 1-hour sessions. Thirteen of the patients improved considerably and could learn to defecate spontaneously, and the use of laxatives ceased or diminished. Simultaneously with improvement, the paradoxical anal contraction disappeared. The results remained after 6 months, although two of the patients had received booster sessions of biofeedback training during follow-up.This study was supported financially by the Örebro County Research Committee.     

Pathogenesis of slow transit and pelvic floor dysfunction: from bench to bedside

The colon and anorectum function together to provide intraluminal mixing, absorption of water, electrolytes, and short chain fatty acids, dehydration of fecal material, storage, and ultimately, elimination in a socially appropriate manner. Normal function and continence require accommodation of the colon and rectum to the entry of fecal materials, which includes receptive relaxation, perception, and discrimination of rectal contents, and voluntary and reflex motor function of the anorectum. Defecation, on the other hand, requires the reflex relaxation of the internal anal sphincter, voluntary and reflexive relaxation of the external anal sphincters and pelvic floor structures, and adequate rectosigmoid tone to allow funneling of contents through the anal canal. The sensation of urgency with rectal filling, and the motivation and prior learning of the appropriate responses are also required. Continence and defecation, therefore, involve complex sensory, structural, and motor mechanisms that involve both the colon and pelvic floor. These mechanisms and their relative importance to the pathogenesis of slow-transit constipation and pelvic floor dysfunction will be reviewed.     

女性气虚型功能性出口梗阻型便秘的测压特点分析

目的探讨女性气虚型功能性出口梗阻型便秘（OOC）患者肛门直肠动力及直肠感知功能的特点。 方法回顾性分析南京市中医院2018年1月至2018年12月期间在便秘平台收治的53例OOC患者及60名健康志愿者的肛门直肠动力及直肠感知功能资料。 结果53例患者的平均肛管静息压高于健康志愿者（t＝4.985,P<0.01）,平均最大收缩压低于健康志愿者（t＝7.555,P<0.01）。直肠排便压中,所有患者的平均排便压也是低于健康志愿者的（t=4.297,P<0.01）。肛门括约肌长度OOC患者较女性健康者增长（t=4.825,P<0.01）;不同合并症女性OOC患者肛管静息压与健康志愿者相比差异均有统计学意义（t_前突=5.257,t_会阴下降=6.091,t_内套叠=4.23;P<0.05）。总体平均最大收缩压低于正常参考值,各型患者与健康志愿者差异均有统计学意义（t_前突=6.277,t_会阴下降=5.306,t_内套叠=3.293;P<0.05）;直肠排便压中,各型患者均表现排便压较低,但仅直肠前突及会阴下降与健康者相比差异均有统计学意义（t_前突=3.313,t_会阴下降=4.637,P均<0.05）;患者排便阈值及平均排便阈值均高于健康志愿者,差异均有统计学意义（t＝2.818,P<0.01）。虽然53例患者的平均初始阈值高于健康志愿者,但差异没有统计学意义（P>0.05）。最大耐受量与健康志愿者相比,差异也没有统计学意义（P>0.05）;4种合并症便秘患者的初始阈值与健康志愿者相差不大,差异均无统计学意义（均P>0.05）。 结论OOC动力特点主要表现为高肛管静息压,低收缩压,以及直肠低敏感性,这说明肛门外括约肌及盆底肌的收缩及协调功能障碍、直肠敏感性下降及对容量刺激的反应较迟钝可能是造成功能性便秘的重要原因。     

Magnetic resonance imaging of anatomic and dynamic defects of the pelvic floor in defecatory disorders

OBJECTIVE: Endoanal ultrasound identifies anal sphincter anatomy, and evacuation proctography visualizes pelvic floor motion during simulated defecation. These complementary techniques can evaluate obstructed defecation and fecal incontinence. Our aim was to develop a single, nonionizing, minimally invasive modality to image global pelvic floor anatomy and motion. METHODS: We studied six patients with fecal incontinence and seven patients with obstructed defecation. The anal sphincters were imaged with an endoanal magnetic resonance imaging (MRI) coil and endoanal ultrasound (five patients). MR fluoroscopy acquired images every 1.4-2 s, using a modified real-time, T2-weighted, single-shot, fast-spin echo sequence, recording motion as patients squeezed pelvic floor muscles and expelled ultrasound gel; no contrast was added to other pelvic organs. Six patients also had scintigraphic defecography. RESULTS: Endoanal ultrasound and MRI were comparable for imaging defects of the internal and external sphincters. Only MRI revealed puborectalis and/or external sphincter atrophy; four of these patients had fecal incontinence. MR fluoroscopy recorded pelvic floor contraction during squeeze and recorded relaxation during simulated defecation. Corresponding comparisons for angle change and perineal descent during defecation were not significant; only MRI, but not scintigraphy, identified excessive perineal descent in two patients. CONCLUSIONS: Pelvic MRI is a promising single, comprehensive, nonradioactive modality to measure structural and functional pelvic floor disturbances in defecatory disorders. This method may provide insights into mechanisms of normal and disordered pelvic floor function in health and disease.     

Persistence of chronic constipation in children after biofeedback treatment

We investigated the efficacy of biofeedback treatment and evaluated anorectal factors that might be responsible for persistence of chronic constipation with or without encopresis in a group of 38 children with abnormal contraction of the pelvic floor during straining and persistence of chronic constipation with encopresis after conventional treatment. Nine children were unsuccessful in learning to relax the pelvic floor during straining with biofeedback treatment, and one patient had contraction of the pelvic floor on follow-up despite successful biofeedback treatment; none recovered. Twenty-eight children were able to relax the pelvic floor on follow-up; 14 recovered and 14 did not recover from chronic constipation. Nonrecovered patients who learned to relax the pelvic floor had significantly decreased rectal and anal responsiveness to rectal distension as compared to recovered patients during the initial and follow-up anorectal manometric study. Psychological factors such as social competence and behavior problems did not appear to be responsible for recovery or nonrecovery from chronic constipation and encopresis.This work was supported by grant M01-RR-00069 from the General Clinical Research Center Program, Division of Research Resources, National Institutes of Health.Results of this study were published in part as an abstract inJournal of Gastrointestinal Motility 1:69, 1989; and presented at the 12th International Symposium on Gastrointestinal Motility, Gmunden, Austria, September 13, 1989.     

Outlet obstruction constipation (anismus) managed by biofeedback.

Fifteen subjects presenting with intractable constipation due to obstructive defecation, mean (SEM) duration 8.8 (1.8) years, had the inappropriate contraction and electromyographic changes in the pelvic floor muscles and external and sphincter typical of this condition. An electromyographically derived index was used to grade its severity. A self applied biofeedback device was used to allow electromyographic recording of the abnormal external anal sphincter. The subjects were encouraged to reduce the abnormal electromyographic activity on straining after instruction and training. The procedure was intended as a relearning process in which the non-relaxing activity of the pelvic floor was gradually suppressed. Biofeedback training was maintained on a domiciliary basis for a mean time of 3.1 weeks and resulted in a significant reduction in the anismus index (mean (SEM) 69.9 (7.8)% before biofeedback, mean 14 (3.9)% after biofeedback, p less than 0.01). There was an associated reduction in the time spent straining at stool and in the difficulty of defecation and an increased frequency of defecation. Defecatory video proctograms in six subjects showed improvements in the anorectal angle during straining and evacuation. The clinical benefit to the patients persisted after a mean follow up of 6.2 months.     

Clinical picture and pelvic floor physiology in the solitary rectal ulcer syndrome

Clinical examination, proctosigmoidoscopy, rectal biopsies, barium enema and pelvic floor physiology studies were performed in four patients with solitary rectal ulcer syndrome. All patients had chronic constipation and rectal bleeding. Resting tone and voluntary contraction were found to be decreased at anal manometry in two patients (maximal squeeze pressures were ten and 35 mm Hg, respectively). Balloon proctogram showed an increased rectoanal angle in these two patients (90° and 93° at rest, unchanged on squeezing) with a poor striated sphincter function at EMG; their deficient anal reflex, slight fecal incontinence and perineal descent seemed consistent with pudendal neuropathy. Fibromuscular obliteration of the lamina propria was found at histology. All subjects had successful conservative treatment, including topical corticosteroids in one patient; normalization of bowel habit was the most effective therapy for the disease. The present study seems to confirm the role of chronic constipation and abnormal pelvic floor physiology in the pathogenesis of the syndrome.     

A concept of the anatomy of the anal sphincter mechanism and the physiology of defecation

A review of the new concepts of the anatomy of the anal sphincter mechanism and the physiology of defecation is presented. The external sphincter is a triple-loop system; each loop can function as a separate sphincter through voluntary inhibition action and mechanical compression. Stress defecation resulting from internal sphincter damage is described. A new technique for repair of rectal incontinence is presented, which depends on inducing continence not only by mechanical compression, but also by voluntary inhibition. The mechanism of defecation and rectal continence is described and four types of incontinence presented. Also, the mechanism of both the levator dysfunction syndrome and prolapse is demonstrated and a technique of repair is presented. The study defines two types of rectal anomalies: suprahiatal and infrahiatal. The role of the embryonic anorectal sinus, anorectal band, and epithelial debris in the genesis of perirectal suppuration, chronic anal fissure, pruritus ani, and hemorrhoids is described. The communicating veins, identified between the hemorrhoidal and vesical plexuses, offer an explanation for the vague pathologic aspects of recurrent bacteriuria, urethral discharge, cervicitis, and vaginitis, and provide a proper line for their treatment. They also serve to perform a new radiographic technique—anal cystography—and to administer drugs, including chemotherapeutics, in the treatment of pelvic malignancies.