This scoping review sought to summarize the current knowledge on the epidemiology, pathogenesis and clinical presentation of, and the investigations that may help characterize faecal incontinence (FI) in patients with SSc.

The planned scoping review was based on the methodological framework proposed by Arksey and O'Malley. Two databases were screened: PubMed (Medline), (Web of Science), and data extraction was performed using a predefined template.

A total of 454 abstracts were screened and 61 articles were finally included, comprising 32 original articles. The prevalence of FI was 0.4% to 77% in original articles that did not use FI among the mandatory inclusion criteria. Internal anal sphincter was reported as more impacted than external sphincter and vasculopathy of arterioles and extracellular matrix deposition with fibrous replacement of the internal sphincter were the key underlaying pathogenic events. The most represented patient-reported outcome in original articles was the Wexner FI score (22% of original articles) followed by the UCLA SCTC-GIT 2.0 (16% of original articles). Although there is no validated diagnostic approach for FI in SSc, 47% of original articles used anorectal manometry to assess rectal physiology in SSc patients. Conservative measures to treat either liquid or hard stool including anti-diarrhoeal medications and dietary adjustments were the first step of proposed FI management in included narrative reviews and guidelines.

This is the first scoping review exploring FI in SSc. We propose a new research agenda which may help improve treatment strategies and foster research focusing on a neglected manifestation of SSc.

Fecal incontinence is a common condition that can significantly impact patients' quality of life. Obstetric anal sphincter injury and anorectal surgeries are common etiologies. Endoanal ultrasound and anorectal manometry are important diagnostic tools for evaluating patients. There are various treatment options, including diet, lifestyle modifications, drugs, biofeedback therapy, tibial and sacral nerve neuromodulation therapy, and surgery. In this editorial, we will discuss current controversies and novel approaches to fecal incontinence. Screening for asymptomatic anal sphincter defects after obstetric anal sphincter injury and in patients with inflammatory bowel disease is not generally recommended, but may be helpful in selected patients. The Garg incontinence score is a new score that includes the assessment of solid, liquid, flatus, mucous, stress and urge fecal incontinence. Novel tests such as translumbosacral anorectal magnetic stimulation and novel therapies such as translumbosacral neuromodulation therapy are promising diagnostic and treatment options, for both fecal incontinence and neuropathy. Home biofeedback therapy can overcome some limitations of the office-based therapy. Skeletal muscle-derived cell implantation of the external anal sphincter has been further studied as a possible treatment option. Sacral neuromodulation may be useful in scleroderma, congenital fecal incontinence and inflammatory bowel disease but merits further study.

Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.

Treatment options were reviewed, and opinions were provided for most facets of jSSc including general management, some of which differs from adult systemic sclerosis, such as the use of corticosteroids, and specific organ involvement, such as skin, musculoskeletal, pulmonary, and gastroenterology.

We are suggesting the treat to target strategy to treat early to prevent cumulative disease damage in jSSc. Conclusions are derived from both expert opinion and available literature, which is mostly based on adult systemic sclerosis (aSSc), given shared pathophysiology, extrapolation of results from aSSc studies was judged reasonable.

Gastrointestinal tract involvement in systemic sclerosis concerns more than 90% of patients but is of heterogeneous clinical expression. It can involve the entire intestinal tract and be responsible for multifactorial malnutrition, which is frequent in this disease. It is a major source of deterioration in the quality of life and can even be life-threatening. Management is complex and multidisciplinary, ranging from simple hygienic and dietary measures, to specialized endoscopic or surgical interventional procedures, also including medical treatments, particularly proton pump inhibitors and prokinetics, with potential side effects. Ongoing research for new diagnostic and therapeutic tools promises to improve the management and prognosis of these patients.

Lower gastrointestinal (GI) symptoms are a frequently encountered problem for clinicians managing patients with systemic sclerosis. The current management practices are focused on the treatment of symptoms with little information available on how to use GI investigations in daily practice. This review demonstrates how to integrate the objective assessment of common lower GI symptoms into clinical care with the aim of guiding clinical decision making. Understanding the type of abnormal GI function that is affecting a patient and determining which parts of the gut are impacted can help clinicians to target therapy more precisely.

The gastrointestinal tract (GI) is the second most affected organ system in individuals suffering from systemic/localized scleroderma (SSc) or localized scleroderma. SSc can affect any part of the GI, between the oral cavity and anorectum. The annual incidence of SSc in the United States is estimated to be 19.3 cases per million adults, with the highest incidence reported in people aged 44 to 55. Females are 5 times more likely than males to suffer from SSc. Morbidity and mortality rates associated with SSc are predominantly elevated among patients with GI manifestations. Esophageal and intestinal manifestations impact 90% and 40% to 70% of patients with systemic scleroderma, respectively. SSc patients are known to suffer from small bowel hypomotility and small intestinal bacterial overgrowth, which cause malabsorption and malnutrition, ultimately contributing to the 50% mortality rate. Fecal incontinence is a common symptom of SSc that can lead to depression. SSc patients may suffer from gastrointestinal complications that can negatively impact their quality of life on a daily basis. Multidisciplinary approaches are necessary for systematically managing gastrointestinal complications associated with SSc. A prospective study should focus on developing targeted therapies to improve recovery patterns and prognosis in cases of SSc. This article summarizes the epidemiology, commonly reported clinical manifestations, complications, and available treatments for treating GI pathology in SSc patients.

Juvenile-onset systemic sclerosis (jSSc) is a complex multisystem inflammatory-driven disease of fibrosis, requiring multifaceted treatment including pharmacologic therapy, supportive care, and lifestyle modification. Most regimens are adapted from adult SSc treatment given the rarity of the disease. Landmark trials over the past decade in adult SSc have led to 2 Food and Drug Administration-approved therapies for SSc-associated interstitial lung disease, and several ongoing trials of other biological agents are underway. Resetting the immune system with autologous stem cell transplant to halt this disease earlier in its course, especially in pediatric onset where disease burden can accumulate, is on the horizon.

We recently read with interest the article "Diagnostic approach to faecal incontinence: What test and when to perform?". This is a comprehensive and practical review, which has particular significance for guiding clinicians to improve the examination strategy. Although we appreciate their work very much, based on the in-depth analysis of this research, we found some detailed problems in the article and will give our comments in this letter. If the author can further improve the relevant research, it will be valuable.

Systemic sclerosis is an autoimmune disease characterized by vascular disease, fibrosis of the skin, and internal organ dysfunction. Gastrointestinal involvement is the most frequent complication of internal organs, impacting up to 90% of patients. Gastrointestinal involvement can affect any region of the gastrointestinal tract from the mouth to the anus, with a predominance of disorders being observed at the level of the upper digestive tract. The gastrointestinal involvement primarily involves the esophagus, small bowel, and rectum. The severity of gastrointestinal involvement affects quality of life and is a marker of worse prognosis and mortality in these patients. In this review, we describe the current findings regarding gastrointestinal involvement by this entity.

Scleroderma is a chronic autoimmune disorder involving multiple organs and very commonly the gastrointestinal (GI) system; nevertheless, data on the involvement of the anal sphincter and consequent faecal incontinence (FI) are inadequate. FI in scleroderma was first reported in 1994 by Engel and colleagues, but its impact of added health care costs and declining quality of life (QoL) is poorly determined. Up to 40% of patients with GI involvement complain of FI, however, the quality of data available is poor owing to majority of the studies being retrospective and case reports or series of small study size. A direct involvement of internal anal sphincter muscularis propria has been demonstrated on anorectal ultrasound imaging suggesting a thin, atrophic or scarred internal sphincter. Treatment guidelines for incontinence in scleroderma are mainly symptomatic, with radical surgeries burdened by poor outcomes. Sacral neuromodulation is being used with good outcomes in a subgroup of patients, but larger, controlled studies are required to assess its efficacy on symptoms and prognosis.

The gastrointestinal (GI) tract is the most commonly affected internal organ system in systemic sclerosis (SSc). SSc may lead to impaired function in any region of the GI tract, from the esophagus to the anorectum, which causes significant morbidity as well as mortality in patient subsets. Given the low prevalence of SSc in the community, many rheumatologists may not have a systematic framework for diagnosing or treating the GI complaints in this disease. These practice recommendations aim to summarize and consolidate the current guidelines from the fields of gastroenterology and rheumatology and establish a symptom-based framework for diagnosis and management based on available evidence in the literature. Subject areas that are in need of additional research are also identified.

Numerous systemic diseases (vasculitis, connective tissue disease or sarcoidosis) can display an involvement of the perianal skin, the rectum and/or the anus. Such knowledge is important in order to treat these complications specifically when possible. Lesions of the anorectum arising from systemic diseases can sometimes cause perforations in the peritoneal cavity (if concerning the higher portion of the rectum) and/or fistulization to the anal margin. Differential diagnosis, mostly infectious or inflammatory (Crohn's disease) must be ruled out in every case. Other systemic diseases can display specific manifestations as this is the case in scleroderma which can lead to anal incontinence. Despite the relative rarity of these manifestations, their ignorance would forbid global management of these complex diseases. It should thus be detected in each consultation and a regular follow-up must be provided with a proctologist and/or a gastroenterologist when needed.

Systemic sclerosis is a chronic autoimmune disorder commonly involving the gastrointestinal tract, including the colon and anorectum. In this review, we summarize major clinical manifestations and highlight recent developments in physiology, diagnostics, and treatment.

The exact pathophysiology of systemic sclerosis is unclear and likely multifactorial. The role of the microbiome on gastrointestinal manifestations has led to a better understanding of potential pathogenic gut flora. Carbohydrate malabsorption is common. Evaluation using fecal calprotectin and high-resolution anorectal manometry may broaden our understanding of the etiologies of diarrhea and fecal incontinence and help with early recognition of pathology. Prucalopride, a high-affinity 5HT₄ agonist, and pyridostigmine, an acetylcholinesterase inhibitor, may help improve colonic transit in patients with constipation. Intravenous immunoglobulins have been used to target muscarinic receptor antibodies that are believed to contribute to gastrointestinal dysmotility. Colonic and anorectal manifestations of systemic sclerosis include constipation, diarrhea, and fecal incontinence, and can diminish quality of life for these patients. Recent studies regarding pathophysiology as well as diagnostic and treatment options are promising. Further targeted studies to facilitate early intervention and better management of refractory symptoms are still needed.

Although classification criteria for systemic sclerosis (SSc) do not incorporate gastrointestinal tract (GIT) manifestations often present in this disease, the GIT is the most common internal organ involved. Pathophysiology of GIT involvement is thought to be similar to other organs in SSc with fibroproliferative vascular lesions of small arteries and arterioles, increased production of profibrotic growth factors, and alterations of innate, humoral, and cellular immunity. These processes result in neuropathy progressing to myopathy with eventual fibrosis. Proper diagnostics and therapeutics for SSc-GIT involvement require the treating physician to have an understanding of an integrated approach and potential medication adverse effects.

Systemic sclerosis (SSc) is a chronic systemic disease characterized by microvasculopathy, autoantibodies, and extensive fibrosis. Intestinal involvement is frequent in SSc and represents a significant cause of morbidity. The pathogenesis of intestinal involvement includes vascular damage, nerve dysfunction, smooth muscle atrophy, and fibrosis, causing hypomotility, which leads to small intestinal bacterial overgrowth (SIBO), malabsorption, malnutrition, diarrhea, pseudo-obstruction, constipation, pneumatosis intestinalis, and fecal incontinence. Manifestations are often troublesome and reduce quality of life and life expectancy. Assessment of intestinal involvement includes screening for small intestine hypomotility, malnutrition, SIBO, and anorectal dysfunction. Current management of intestinal manifestations is largely inadequate. Patients with diarrhea are managed with low-fat diet, medium-chain triglycerides, avoidance of lactulose and fructose, and control of bacterial overgrowth with antibiotics for SIBO. In diarrhea/malabsorption, bile acid sequestrant and pancreatic enzyme supplementation may help, and nutritional support is needed. General measures are applied for constipation, and intestine rest plus antibiotics for pseudo-obstruction. Fecal incontinence is managed with measures for associated SIBO, or constipation, and with behavioral therapies. Pneumatosis intestinalis is usually an incidental finding that does not require any specific treatment. Immunomoduation should be considered early in intestinal involvement. Multidisciplinary approach of intestinal manifestations in SSc by gastroenterologists and rheumatologists is required for optimum management.

To determine whether anorectal biofeedback therapy can improve the symptoms of fecal incontinence (FI) in patients with scleroderma when compared to patients with functional FI, and also whether there is any effect on anorectal physiology or quality of life (QOL). FI in patients with scleroderma is highly prevalent and is associated with significant loss of QOL. Biofeedback has been proven to be an effective treatment for functional FI, but there are no data to support its use in scleroderma.

13 consecutive female patients (median age 59, IQR 47-65 years) with scleroderma, and 26 age- and parity-matched female patients with functional FI (disease controls, 2:1), underwent biofeedback therapy for management of FI. Fecal incontinence severity index (FISI), anorectal physiology, feeling of control and QOL were collected before and after 6 weeks of biofeedback therapy, with additional scoring repeated at 6-month follow-up.

After biofeedback treatment FISI, feeling of control and QOL significantly improved in both groups (p < 0.005). There was no difference in the degree in improvement in physiology, FISI or QOL between scleroderma patients and functional FI patients. Long-term improvement in FISI and control were seen in both groups and for QOL only in the scleroderma cohort (p < 0.05).

Patients with scleroderma benefit from biofeedback therapy to the same extent as that achieved in patients with functional FI. There are significant improvements in symptoms, physiology and QOL. Biofeedback is an effective, low-risk treatment option in this patient group.

To summarise current knowledge of Internal anal sphincter.

The internal anal sphincter (IAS) is the involuntary ring of smooth muscle in the anal canal and is the major contributor to the resting pressure in the anus. Structural injury or functional weakness of the muscle results in passive incontinence of faeces and flatus. With advent of new assessment and treatment modalities IAS has become an important topic for surgeons. This review was undertaken to summarise our current knowledge of internal anal sphincter and highlight the areas that need further research.

The PubMed database was used to identify relevant studies relating to internal anal sphincter.

The available evidence has been summarised and advantages and limitations highlighted for the different diagnostic and therapeutic techniques.

Our understanding of the physiology and pharmacology of IAS has increased greatly in the last three decades. Additionally, there has been a rise in diagnostic and therapeutic techniques specifically targeting the IAS. Although these are promising, future research is required before these can be incorporated into the management algorithm.

In patients with systemic sclerosis (SSc), gastrointestinal (GI) tract involvement is almost universal. Any segment of the GI tract from mouth to anus can be involved, and GI symptoms are a frequent cause of morbidity. In severe cases, GI tract involvement can progress to the point of malnutrition requiring parenteral nutrition. GI tract involvement in SSc contributes to disease-related mortality although mostly as a co-morbidity rather than direct cause of death. The review is intended to help address challenges in the assessment and treatment of GI tract involvement in SSc.

Systemic sclerosis is a multisystem autoimmune disorder that involves the gastrointestinal tract in more than 90% of patients. This involvement can extend from the mouth to the anus, with the oesophagus and anorectum most frequently affected. Gut complications result in a plethora of presentations that impair oral intake and faecal continence and, consequently, have an adverse effect on patient quality of life, resulting in referral to gastroenterologists. The cornerstones of gastrointestinal symptom management are to optimize symptom relief and monitor for complications, in particular anaemia and malabsorption. Early intervention in patients who develop these complications is critical to minimize disease progression and improve prognosis. In the future, enhanced therapeutic strategies should be developed, based on an ever-improving understanding of the intestinal pathophysiology of systemic sclerosis. This Review describes the most commonly occurring clinical scenarios of gastrointestinal involvement in patients with systemic sclerosis as they present to the gastroenterologist, with recommendations for the suggested assessment protocol and therapy in each situation.

Sacral nerve stimulation (SNS) has been recognised as an effective treatment for faecal incontinence. Many unresolved questions could be answered when comparing large data-series from different centres providing prospective data.

To present data, from an international two-centre SNS prospective database (SNSPD) on functional outcome and management of surgical complications in patients treated with SNS for faecal incontinence.

The SNSPD was designed in order to gather detailed pre- and perioperative information followed by a close follow-up in all patients undergoing SNS for bowel dysfunction. The SNSPD was open for inclusion of newly SNS implanted patients in May 2009, and closed on 31 December 2013. Two-centres Aarhus, Denmark, and Nantes, France, included and monitored all patients implanted due to bowel dysfunction according to database criteria.

In total, 164 faecal incontinent patients with a median follow-up of 22 (range 1-50) months were implanted. The Wexner incontinence score improved from 15 (range, 3-20) at baseline to 9 (range, 0-20) at latest follow-up (P < 0.001) and VAS impact on daily life improved from 85.5 (range, 3-100) to 20 (range, 0-100) (P < 0.001). Additional surgical intervention was required in 19.5 % during follow-up. Repositioning of the pacemaker due to pain or migration was the most common complication in 12.1 %. Infections leading to explantation occurred in 3.0 %.

Faecal incontinence may occur following rectal surgery and/or radiotherapy for rectal cancer. The aim of this paper was to review the evidence to support the use of sacral nerve stimulation (SNS) for patients with incontinence who had undergone rectal surgery or received rectal radiotherapy.

A search was performed of PubMed, Medline and Embase. All studies which reported the outcome of SNS in patients who had undergone a rectal resection or radiotherapy were reviewed.

The first report of SNS following rectal surgery was in 2002. Since then seven further studies have described its effect in patients who have undergone anterior resection or pelvic radiotherapy. The total number of patients was 57. All studies were single group series, which ranged in size from one to 15 patients. The follow-up ranged from 1 to 36 months. The success of peripheral nerve evaluation ranged from 47% to 100%. Permanent SNS improved the symptoms and in some studies this was reflected in improved quality of life. The wide variation of patient factors, operations performed, the dose of radiotherapy given and time from operation makes interpretation of the results difficult.

Larger studies with better patient selection are needed to investigate the effect of SNS on incontinence following radiotherapy or rectal surgery.

Systemic sclerosis (SSc) is a multisystem disorder of unknown aetiology leading to the deposition of excessive connective tissue in the skin, blood vessels and internal organs. Gastrointestinal involvement occurs in 90% of cases and the prevalence of faecal incontinence (FI) is 38%. This study comprises the largest case series assessing the efficacy of sacral nerve stimulation (SNS) treatment for incontinence in this patient group.

A retrospective analysis on prospectively collected data was performed on all SSc patients from our two centres who had undergone SNS for FI.

Ten female patients of mean age of 54 (37-72) years had temporary SNS performed. The mean duration of FI was 13 (2-25) years. All had passive FI. Each patient had preprocedure anorectal physiology and endoanal ultrasound examinations documenting internal sphincter atrophy/fragmentation or reduced anal resting pressure. Overall there was no statistically significant difference (P = 0.57) in the total Wexner incontinence scores before (mean 15.1 ± 2.6 SD) and during temporary SNS procedures (mean 13.1 ± 3.6 SD). Two patients with a significant improvement went on to have permanent SNS with only one achieving a favourable outcome at 1 year.

This study showed that SNS failed to reduce episodes of leakage in nine out of 10 patients with systemic sclerosis affected with incontinence.

The gastrointestinal tract, affecting more than 90% of patients, is the internal organ most frequently involved in systemic sclerosis. Any part of the gastrointestinal tract can be affected, from the mouth to the anus. Patients often experience reduced quality of life and impaired social life. Although only 8% have severe gastrointestinal involvement, mortality is high in those patients. Recent studies on the pathophysiology of the disease highlight new mechanisms to explain gastrointestinal dysmotility, but treatment remains symptomatic. This article reviews the pathophysiology of the gastrointestinal tract and discusses the investigation and management of the disease.

In Europe during the last decade sacral nerve stimulation (SNS) or sacral neuromodulation (SNM) has been used to treat faecal incontinence (FI) and constipation. Despite this, there is little consensus on baseline investigations, patient selection and operative technique. A modified Delphi process was conducted to seek consensus on the current practice of SNS/SNM for FI and constipation.

A systematic literature search of SNS for FI and constipation was conducted using PubMed. A set of questions derived from the search and expert opinion were answered on-line on two occasions by an international panel of specialists from Europe. A 1-day face-to-face meeting of the experts finalized the discussion.

Three hundred and ninety-three articles were identified from the literature search, of which 147 fulfilled the inclusion criteria. Twenty-two specialists in FI and constipation from Europe participated. Agreement was achieved on 43 (86%) of 50 domains including the set-up of service, patient selection, baseline investigations, operative technique and programming of the device. The median of agreement was 95% (35-100%).

Consensus was achieved on the majority of domains of SNS/SNM for FI and constipation. This should serve as a benchmark for safe and quality practice of SNS/SNM in Europe.

Gastrointestinal tract (GIT) commonly affects patients with systemic sclerosis (SSc). The GI involvement is quite heterogeneous varying from asymptomatic disease to significant dysmotility causing complications like malabsorption, weight loss and severe malnutrition. This review focuses on the management of GI involvement in SSc and has been categorized based on the segment of GIT involved. A brief discussion on the role of patient reported outcome measures in SSc-GI involvement has also been incorporated.

Fecal incontinence is common in systemic sclerosis (SSc), but the underlying mechanisms are not fully understood. The objectives of this study were to characterize anorectal physiological and morphological defects in SSc patients and to correlate the results with incontinence symptoms.

Twenty-five SSc patients underwent anorectal neurophysiological investigations, anal manometry, and ultrasound.

Eleven patients (44%) reported incontinence to solid or liquid feces, but no patient reported diarrhea. Increased fiber density (FD) was recorded in 78% of patients with and in 86% of patients without fecal incontinence not significant (NS). Incontinent patients had lower squeeze pressure (SP; median 49.5 mm Hg) in the high-pressure zone (HPZ) than continent patients (median 72 mm Hg; p = 0.01). In two of the incontinent patients, sonographic abnormalities of the internal anal sphincter (IAS) and the external anal sphincter (EAS) were present, whereas in another two patients isolated IAS abnormalities were seen. These four individuals had lower resting pressure at 1 cm and in the HPZ, and lower SP at 2 cm than patients with normal anorectal sonographic findings (p < 0.05).

Lower voluntary SP in incontinent patients and EAS sonographic abnormalities only in patients with incontinence suggest that the EAS is more important in maintaining fecal continence in SSc patients than has previously been reported. The finding of increased FD in most patients further supports involvement of the EAS function in SSc and could indicate previous nerve injury with consequent incomplete reinnervation.

Sacral nerve stimulation (SNS) is now well established as a treatment for fecal incontinence (FI) resistant to conservative measures and may also have utility in the management of chronic constipation; however, mechanism of action is not fully understood. End organ effects of SNS have been studied in both clinical and experimental settings, but interpretation is difficult due to the multitude of techniques used and heterogeneity of reported findings. The aim of this study was to systematically review available evidence on the mechanisms of SNS in the treatment of FI and constipation.

Two systematic reviews of the literature (performed in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses framework) were performed to identify manuscripts pertaining to (a) clinical and (b) physiological effects of SNS during the management of hindgut dysfunction.

The clinical literature search revealed 161 articles, of which 53 were deemed suitable for analysis. The experimental literature search revealed 43 articles, of which nine were deemed suitable for analysis. These studies reported results of investigative techniques examining changes in cortical, gastrointestinal, colonic, rectal, and anal function.

The initial hypothesis that the mechanism of SNS was primarily peripheral motor neurostimulation is not supported by the majority of recent studies. Due to the large body of evidence demonstrating effects outside of the anorectum, it appears likely that the influence of SNS on anorectal function occurs at a pelvic afferent or central level.

Faecal incontinence is common and significantly affects quality of life. Its treatment involves dietary manipulation, medical treatments, perineal rehabilitation or surgery. In this paper, the French National Society of Coloproctology offers recommendations based on the data in the current literature, including those on recently developed treatments. There is a lack of high quality data and most of the recommendations are therefore based either on grade of recommendation B or expert recommendation (Level 4). However, the literature supports the construction of an algorithm based on the available scientific evidence and expert recommendation which may be useful in clinical practice. The French National Society of Coloproctology proposes a decision-making algorithm that includes recent developments of treatment. The current recommendations support sacral nerve modulation as the key treatment for faecal incontinence. They do not support the use of sphincter substitutions except in certain circumstances. Transanal irrigation is a novel often successful treatment of faecal incontinence due to neurological disorders.

The gastrointestinal (GI) dysmotility of systemic sclerosis (SSc, scleroderma) patients requires careful evaluation and intervention. The lack of effective prokinetic drugs motivate researchers to search for alternative treatments.

We present an overview of the pathophysiology of SSc GI dysmotility and the advances in its management, with particular focus on acupuncture-related modalities and innovative therapies.

Original research articles were identified based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline methodology. We have searched the MEDLINE database using Medical Subject Heading (MeSH) for all English and non-English articles with an English abstract from 2005 to October 2012.

Only four original articles of various study designs were found studying Complementary and Alternative Medicine (CAM) therapies for SSc patients. Despite the small patient study numbers, CAM treatments, acupressure, and transcutaneous electroacupuncture, showed self-reported and physiologic evidence of improvement of GI functioning and/or symptoms in SSc patients.

CAM therapies include experimental modalities with the potential to offer relief of symptoms from GI dysmotility. Larger studies are needed to investigate their optimal use in patient subsets to tailor therapies to patient needs.

Gastrointestinal involvement (GI) is increasingly recognized as a major cause of both morbidity and mortality in systemic sclerosis (SSc). GI complications are common, second only to skin involvement, and affect up to 90% of patients. Although treatment modalities have changed little for upper gut symptoms such as GI reflux, there are emerging treatment modalities for the common lower gut symptoms (constipation and fecal incontinence), which will be reviewed. The important link between reflux and interstitial lung disease in SSc is also addressed. The aim of this review is to help the clinician understand and manage GI symptoms in SSc.

Sacral nerve modulation is a well accepted method for the treatment of defaecation disorders and voiding dysfunction. Results of sacral nerve modulation in patients with spinal cord lesions are not well assessed, but preliminary results look poor. Therefore, the purpose of this study was to assess the effectiveness of sacral nerve modulation for defaecation disorders and voiding dysfunction in patients with spina bifida.

Consecutive patients with spina bifida suffering from a myelomeningocele and combined faecal and urinary functional disorders that were eligible for peripheral nerve evaluation (PNE) were studied. A permanent sacral nerve modulation implantation was performed after successful PNE.

Ten patients (four female) were included in this study with a median age of 26.4 (range 11.1-41.0) years. In two the PNE was not possible. The median faecal incontinence days (6.0 vs 3.5) and episodes (8.5 vs 3.5) per 21 days decreased significantly during the 3-week period of PNE (P = 0.033). Only 3/10 (30%) patients had a more than 50% improvement and proceeded to a permanent sacral nerve modulation implantation. In one patient it was not possible to perform the permanent implant.

Preliminary results of sacral nerve modulation in a subgroup of spina bifida patients with combined faecal and urinary functional disorders look promising, but long-term results in larger patient groups need to be studied.

Sacral nerve stimulation has been shown to be an effective treatment for fecal incontinence and early studies reported success rates of 67% to 100%. However, "success" has been arbitrarily set at a 50% reduction in symptoms, and data are rarely reported with "intention to treat."

This study aimed to assess the true efficacy of sacral nerve stimulation for fecal incontinence compared with the published literature.

This prospective 5-year study was conducted to assess 50 patients with fecal incontinence treated with sacral nerve stimulation. All analyses were performed on an intention-to-treat basis.

This study took place in a single tertiary referral colorectal department.

Fifty consecutive patients with fecal incontinence refractory to conservative management were included in the study.

The interventions performed were temporary evaluation with or without permanent sacral nerve stimulation.

: Primary outcome measures were 1) attainment of continence, 2) reduction in fecal incontinence episodes, 3) improvement in Cleveland Clinic Fecal Incontinence scores, and 4) improvement in the ability to defer defecation.

Thirteen patients (26%) did not respond at the temporary evaluation stage or were dissatisfied with the result. Ten further patients (20%) did not achieve a 50% reduction in symptoms following permanent implantation. The median follow-up was 17 months (range, 2-55), at which time 27 patients (54%) experienced a 50% or more reduction in symptoms, including 13 (26%) who achieved apparent continence. Median fecal incontinence episodes per fortnight reduced from 14 (range, 0-53) to 2 (range, 0-20; P < .0001). Median Cleveland Clinic Fecal Incontinence scores reduced from 15 (range, 3-20) to 8 (range, 0-17; P < .0001). The ability to defer defecation improved significantly (P < .0001). These results compare favorably with the published literature.

Quality of life was not assessed.

This study demonstrates that sacral nerve stimulation can be an effective treatment for patients with fecal incontinence; however, when analyzed by intention to treat, the symptoms of fecal incontinence continue in the majority (74%) of patients.

The indications for sacral nerve stimulation are increasing, but the mechanism remains poorly understood.

This study aimed to examine the effect of sacral nerve stimulation on rectal compliance and rectal sensory function.

This was a prospective study.

This study took place at a university teaching hospital.

Twenty-three consecutive consenting patients (22 female; median age, 49 y) undergoing temporary sacral nerve stimulation for fecal incontinence were prospectively studied. Clinical response was assessed by the use of bowel diaries and Wexner scores.

Anal manometry, rectal compliance, volume and pressure thresholds to rectal distension (barostat), and rectal Doppler mucosal blood flow were measured before and at the end of stimulation.

Sixteen patients (70%) had a favorable clinical response. Median anal squeeze pressures increased with stimulation from 40 (range, 6-156) cmH2O to 64 (range, 16-243) cmH2O. Median rectal compliance did not significantly change with stimulation (prestimulation: 11.5 (range, 7.9-21.8) mL/mmHg, poststimulation: 12.4 (range, 6.2-22) mL/mmHg, P = .941). Rectal wall pressures associated with urge (baseline: 15.4 (range, 11-26.7) mmHg, poststimulation: 19 (range, 11.1-42.7) mmHg, P = .054) and maximal tolerated thresholds (baseline: 21.6 (8.5-31.9) mmHg, poststimulation: 27.1 (14.3-43.3) mmHg, P = .023) significantly increased after stimulation. Rectal Doppler mucosal blood flow did not significantly change with stimulation (baseline: 125.8 (69.9-346.8), poststimulation: 112.4 (50.2-404.1), P = .735). Changes in anal resting pressure and rectal wall pressures with stimulation were evident only in responders; however, changes in anal squeeze pressures were evident in both responders and nonresponders.

The study reports results following short-term stimulation in a small but homogenous group of patients. A larger long-term study will follow.

Temporary sacral nerve stimulation does not change rectal compliance, but is associated with significant changes to the pressure thresholds of rectal distension. This, together with the observation that outcome is not related to sphincter integrity, supports the hypothesis of an afferent-mediated mechanism of action.

SSc is a connective tissue, multisystem disorder of unknown aetiology. The gastrointestinal tract (GIT) is affected in up to 90% of patients. The exact pathophysiology of GIT involvement is not known, but it is related to both neurogenic and myogenic abnormalities as well as possible vascular and ischaemic changes. Thinning of the internal anal sphincter (IAS) has been demonstrated in SSc with faecal incontinence. We aimed to investigate anal sphincter structure in patients with SSc.

Forty-four SSc patients [24 symptomatic (Sx) and 20 asymptomatic (ASx)] and 20 incontinent controls (ICs) were studied. Patients underwent anorectal manometry and endoanal US.

In the ICs, external anal sphincter defects were more common, but the IAS was less atrophic, evident by both atrophy scores and IAS thickness. There was no significant difference in atrophy scores [Sx: 2 (1.5-3) vs ASx: 2 (1-2)] or IAS thickness [Sx: 1.85 (1.5-2.3) vs ASx: 1.8 (1.7-2.25)] between the Sx and ASx SSc patients.

Patients with SSc (both Sx and ASx) have thin and atrophic IAS, suggesting that IAS atrophy develops even in ASx patients and this may be amenable to treatment with sacral neuromodulation.

Sacral nerve stimulation (SNS) has become an established option in the management of defaecatory disorders. There are many data on the end-organ effects of SNS, but limited consensus on its mechanism of action. The objective of this review was to determine the effect of sacral nerve stimulation (SNS) on anorectal function.

Systematic literature review of the effect of sacral nerve stimulation (SNS) on anorectal function.

There was significant heterogeneity of data pertaining to the effects of SNS on anorectal function, with no consistent change in physiological measures identified. Most data supported a mixed mechanism of action on anal motor and rectal sensory functioning.

To date, conflicting data exist on the mechanism of action of SNS as determined by end-organ changes in anorectal physiology. Nevertheless, the data as they stand best support a mixed mechanism of action on anal motor and rectal sensory functions.

The aim of this article was to determine the effect of sacral nerve stimulation (SNS) on the treatment of faecal incontinence, constipation, irritable bowel syndrome, mixed urinary and bowel disorders, spinal injury and neurodegenerative disease, pain syndromes, and sexual dysfunction.

A Medline search was performed including the keywords and/or MeSH headings of 'sacral nerve stimulation', 'neuromodulation', 'artificial pacemaker', 'faecal incontinence', 'constipation' and 'anal pain'. Further studies were identified by cross-referencing from relevant articles and by appraisal of recent peer-reviewed conference abstracts and proceedings.

SNS has been used for the treatment of urinary, bowel and sexual dysfunction, as well as pain resulting from such disorders, and dysfunction arising from nerve injury and degenerative disease. There is a paucity of high quality evidence to support the use of SNS for the majority of novel indications at present.

Good quality prospective, cross-over studies are required to determine the true benefits of SNS. Further research into patient selection, operative technique and stimulation parameters for existing indications will ensure a place for SNS in the future treatment algorithm of functional pelvic floor disorders.

This study aimed to test the hypothesis that sacral nerve stimulation affects afferent vagal projections to the central nervous system associated with frontal cortex activation in patients with fecal incontinence.

Nine women and one man received temporary sacral nerve stimulation with permanent electrodes as a treatment for fecal incontinence.

We used positron emission tomography to record indices of regional cerebral blood flow before and after 30 minutes of continuous stimulation. We repeated this procedure after 2 weeks of continued stimulation, before and 30 minutes after arrest of the stimulation.

The initial stimulation activated a region of the contralateral frontal cortex that normally is active during focused attention. After 2 weeks of stimulation, this activation had been replaced by activity in parts of the ipsilateral caudate nucleus, a region of the brain thought to be specifically involved in learning and reward processing.

Sacral nerve stimulation induces changes in cerebral activity consistent with an effect on afferent projections of the vagus. The initial activation of the frontal cortex may reflect focused attention, whereas the subsequent activation of the caudate nucleus may reflect recruitment of mechanisms involved in learning and reward processing. These changes may contribute to the improved continence, which is an acquired result of the stimulation.

Sacral nerve stimulation (SNS) has recently been used in the management of faecal incontinence (FI). This study compared SNS to conservative management with regards to functional and quality of life outcomes.

Meta-analysis of studies published between 1995 and 2008 on SNS for FI was performed. Outcomes evaluated were functional, physiological and quality of life. A random-effects model was used and sensitivity analyses performed. Subgroup analyses were performed on age and sphincter status.

Thirty-four studies were included, reporting on 944 patients undergoing peripheral nerve evaluation; 665 underwent permanent SNS. Weekly incontinence episodes (weighted mean difference [WMD] -6.83; 95% confidence intervals [CI] -8.05, -5.60; p < 0.001) and incontinence scores (WMD -10.57; 95% CI -11.89, -9.24; p < 0.001) were significantly reduced with SNS; ability to defer defecation (WMD 7.99 min; 95% CI 5.93, 10.05; p < 0.001) was increased. Most SF-36 and FIQL domains improved following SNS, and mean anal pressures increased significantly (p < 0.001). Results remained consistent on sensitivity analysis. The under-56 years age group showed smaller functional but greater physiological and quality of life improvements. Results were similar between sphincter intact and impaired subgroups. The complication rate was 15% for permanent SNS, with 3% resulting in permanent explantation.

SNS results in significant improvements in objective and subjective measures for faecally incontinent patients.

Sacral nerve stimulation (SNS) is an effective but expensive treatment for faecal incontinence. About 50% of the patients are unresponsive for unknown reasons, hence knowledge of any factors predictive of success would be highly desirable. The aim of this study was to analyse the potential factors associated with a successful outcome of the temporary test of electrostimulation.

Eighty-five patients with faecal incontinence were tested for SNS. The cause was idiopathic in 45, iatrogenic or obstetric in 28, spinal lesion or neurological diseases in nine and anal malformation in three patients; 43 were tested with a unipolar electrode and 42 with a quadripolar electrode. The severity of faecal incontinence was evaluated using the American Medical System (AMS) score and Wexner's score.

A positive response was obtained in 45 patients (53%); 40 (47%) were implanted with a permanent pulse generator. Responders and nonresponders were comparable in age, duration of incontinence, anal manometry, pudendal nerve terminal motor latency and diabetes. Unipolar electrode test (PNE test) was able to elicit positive responses in 18 of 43 (42%) and the quadripolar in 27 of 42 patients (P < 0.001). Type of incontinence and gender did not affect the success rate. Patients with idiopathic incontinence had a significantly higher response rate (P =0.022). Multivariate regression analysis indicated use of a quadripolar electrode as the only independent variable predicting the success of SNS (OR = 5.58, P = 0.009).

Use of the quadripolar electrode is the only factor significantly related to the success of SNS.

Sacral nerve stimulation is offered to patients presenting with fecal incontinence of neurological or idiopathic etiology, when medical management has failed. The purpose of this study was to investigate the causes of surgical revision following sacral nerve stimulation in consecutive patients who had received implants.

From September 2001 to August 2009, 123 patients (105 women) of mean age 56 years were operated on for neurological (n = 104) or idiopathic (n = 19) fecal incontinence. The mean preoperative Cleveland Clinic score was 13/20 (range 6/20 to 19/20). Eighty-seven patients of 123 had a positive test and underwent stimulator implantation. Any stimulator dysfunction was prospectively studied.

Among the 87 patients, 36 had surgical revision of the device for the following reasons: device-related failure due to infection in 4 (successful reimplant in 4), electrode displacement in 2, electrode breakage in 2 (reimplantation of electrode in 4), and dysfunction owing to impedance increase of the system in 4; adverse stimulation with pain in 7 (stimulator repositioning in 4 and explantation in 3); battery depletion either spontaneously (n = 6) or owing to a MRI examination (n = 2); total or partial loss of clinical efficacy in 9 (removal of the generator and electrode).

Sacral nerve stimulation is a recognized treatment for fecal incontinence. The stimulator reoperation rate is high and is caused by stimulator dysfunction in 24% of cases.

The use of sacral nerve stimulation as a treatment for fecal incontinence for intact but functionally deficient sphincter and pelvic floor musculature, as well as for some sphincter injuries, is an attractive concept that is currently undergoing clinical trials in the United States. Electrical stimulation of the peripheral nerve supply to the striated anal sphincter muscles at the level of the sacral spinal nerves exploits the accessibility of the most distal common location of the dual peripheral nerve supply to these muscles. While the mechanism of sacral nerve stimulation's salutary effect remains conjectural at present and is likely multifactorial, current experimental data point toward both an enhancement in striated muscular activity as well as neuromodulation of sacral reflexes that regulate rectal sensitivity and contractility.