Case report: Acute appendicitis in appendix duplication.

INTRODUCTION: Duplication of the appendix is a very rare presentation. According to the Cave-Wallbridge classification, there are three types of duplicate appendix. PRESENTATION OF CASE: A 43 year old female presented with classical symptoms of acute appendicitis, with unremarkable inflammatory markers. The diagnosis was confirmed on pre-operative computer tomography (CT). During laparoscopy two tubular structures were identified: one arising from the tenia libera of the caecum adjacent to the terminal ileum and one retrocaecally at the confluence of the teniae. Both structures were excised using a laparoscopic linear stapler. Histopathological analysis demonstrated the accessory structure to be a microscopically unremarkable blind-ended tubular structure. The other specimen demonstrated acute gangrenous inflammation of the appendix. The patient had an uneventful recovery and was discharged home the following day. DISCUSSION: Appendix duplication is rare; however, failure to recognise it in a patient with acute appendicitis could result in a retained source of sepsis, requiring subsequent re-exploration of the abdomen. The case presented here represents a Type B2 according to the Cave-Wallbridge classification and is the most susceptible to inadvertent error due to having appendixes in both typical and atypical anatomical locations. This case also highlights the probability of this diagnosis being missed on pre-operative CT. CONCLUSION: This case report presents a unique opportunity for surgical trainees to review intra-operative laparoscopic images of a duplicate appendix, both to allow them to recognise this pathology if encountered in the future, and to embed the importance of ruling it out with thorough intra-operative examination.

Guideline for the diagnosis and treatment of Faecal Incontinence-A UEG/ESCP/ESNM/ESPCG collaboration.

INTRODUCTION: The goal of this project was to create an up-to-date joint European clinical practice guideline for the diagnosis and treatment of faecal incontinence (FI), using the best available evidence. These guidelines are intended to help guide all medical professionals treating adult patients with FI (e.g., general practitioners, surgeons, gastroenterologists, other healthcare workers) and any patients who are interested in information regarding the diagnosis and management of FI. METHODS: These guidelines have been created in cooperation with members from the United European Gastroenterology (UEG), European Society of Coloproctology (ESCP), European Society of Neurogastroenterology and Motility (ESNM) and the European Society for Primary Care Gastroenterology (ESPCG). These members made up the guideline development group (GDG). Additionally, a patient advisory board (PAB) was created to reflect and comment on the draft guidelines from a patient perspective. Relevant review questions were established by the GDG along with a set of outcomes most important for decision making. A systematic literature search was performed using these review questions and outcomes as a framework. For each predefined review question, the study or studies with the highest level of study design were included. If evidence of a higher-level study design was available, no lower level of evidence was sought or included. Data from the studies were extracted by two reviewers for each predefined important outcome within each review question. Where possible, forest plots were created. After summarising the results for each review question, a systematic quality assessment using the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach was performed. For each review question, we assessed the quality of evidence for every predetermined important outcome. After evidence review and quality assessment were completed, recommendations could be formulated. The wording used for each recommendation was dependent on the level of quality of evidence. Lower levels of evidence resulted in weaker recommendations and higher levels of evidence resulted in stronger recommendations. Recommendations were discussed within the GDG to reach consensus. RESULTS: These guidelines contain 45 recommendations on the classification, diagnosis and management of FI in adult patients. CONCLUSION: These multidisciplinary European guidelines provide an up-to-date comprehensive evidence-based framework with recommendations on the diagnosis and management of adult patients who suffer from FI.

Guideline for the diagnosis and treatment of faecal incontinence: a UEG/ESCP/ESNM/ESPCG collaboration [Pré-print]

The goal of this project was to create an up-to-date joint European clinical practice guideline for the diagnosis and treatment of faecal incontinence (FI), using the best available evidence. These guidelines are intended to help guide all medical professionals treating adult patients with FI (e.g., general practitioners, surgeons, gastroenterologists, other healthcare workers) and any patients who are interested in information regarding the diagnosis and management of FI. These guidelines have been created in cooperation with members from the United European Gastroenterology (UEG), European Society of Coloproctology (ESCP), European Society of Neurogastroenterology and Motility (ESNM) and the European Society for Primary Care Gastroenterology (ESPCG). These members made up the guideline development group (GDG). Additionally, a patient advisory board (PAB) was created to reflect and comment on the draft guidelines from a patient perspective. Relevant review questions were established by the GDG along with a set of outcomes most important for decision making. A systematic literature search was performed using these review questions and outcomes as a framework. For each predefined review question, the study or studies with the highest level of study design were included. If evidence of a higher-level study design was available, no lower level of evidence was sought or included. Data from the studies were extracted by two reviewers for each predefined important outcome within each review question. Where possible, forest plots were created. After summarising the results for each review question, a systematic quality assessment using the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach was performed. For each review question, we assessed the quality of evidence for every predetermined important outcome. After evidence review and quality assessment were completed, recommendations could be formulated. The wording used for each recommendation was dependent on the level of quality of evidence. Lower levels of evidence resulted in weaker recommendations and higher levels of evidence resulted in stronger recommendations. Recommendations were discussed within the GDG to reach consensus. These guidelines contain 45 recommendations on the classification, diagnosis and management of FI in adult patients. These multidisciplinary European guidelines provide an up-to-date comprehensive evidence-based framework with recommendations on the diagnosis and management of adult patients who suffer from FI.

Redundancy in the International Anorectal Physiology Working Group Manometry Protocol: A Diagnostic Accuracy Study in Fecal Incontinence.

BACKGROUND: Anorectal manometry (ARM) is essential for identifying sphincteric dysfunction. The International Anorectal Physiology Working Group (IAPWG) protocol and London Classification provide a standardized format for performing and interpreting ARM. However, there is scant evidence to support timing and number of constituent maneuvers. AIMS: To assess the impact of protocol modification on diagnostic accuracy in patients with fecal incontinence. METHODS: Retrospective analysis of high-resolution ARM recordings from consecutive patients based on the current IAPWG protocol and modifications thereof: (1) baseline rest period (60 vs. 30 vs. 10 s); (2) number of abnormal short squeezes (SS) out of 3 (SS1/SS2/SS3) based on maximal incremental squeeze pressures over 5 s; (3) resting anal pressures (reflecting recovery) at 25-30 versus 15-20 s after SS1. RESULTS: One hundred patients (86 F, median age 55 [IQR: 39-65]; median St. Mark's incontinence score 14 [10-17]) were studied. 26% and 8% had anal hypotonia and hypertonia, respectively. Compared with 60-s resting pressure, measurements had perfect correlation (κ = 1.0) over 30 s, and substantial correlation (κ = 0.85) over 10 s. After SS1, SS2, and SS3, 43%, 49%, and 46% had anal hypocontractility, respectively. Correlation was substantial between SS1 and SS2 (κ = 0.799) and almost perfect between SS2 and SS3 (κ = 0.9). Compared to resting pressure of 5 s before SS1, pressure recordings at 25-30 and 15-20 s after SS1 were significantly correlated. CONCLUSIONS: A 30-s resting anal pressure, analysis of 2 short-squeezes with a 20-s between-maneuver recovery optimizes study duration without compromising diagnostic accuracy. These findings indicate the IAPWG protocol has redundancy.

Mixed adenoneuroendocrine carcinoma (MANEC) of the lower gastrointestinal tract: A systematic review with Bayesian hierarchical survival analysis.

BACKGROUND: Mixed adeno-neuroendocrine carcinomas (MANEC) are a subgroup of mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) described as mixed neoplasms containing dual neuroendocrine and non-neuroendocrine components. The aim of this study was to appraise the prevalence of MANEC in the lower gastrointestinal (GI) tract and provide reliable estimates of survival. METHOD: A systematic review was undertaken in accordance with PRISMA guidelines using PubMed, Embase, Cochrane Library of Systematic Review, Web of Science, and Scopus databases, and a Bayesian hierarchical survival pooled analysis was performed. RESULTS: Of 182 unique records identified, 71 studies reporting on 752 patients met the inclusion criteria. Mean age was 64.2 ± 13.6, with a male-to-female ratio of 1.25. Overall, 60.3% of MANEC were located in the appendix, 29.3% in the colon, and 10.4% in the anorectum. More than a quarter (29%) of patients had stage IV disease at diagnosis, with higher prevalence in appendiceal than colonic and anorectal primaries. More than 80% had a high-grade (G3) endocrine component. Of the 152 patients followed up for a median of 20 months (interquartile range limits, 16.5-32), median overall survival was 12.3 months (95% credible interval [95%CrI], 11.3-13.7), with a 1.12 [95%CrI, 0.67-1.83] age-adjusted hazard ratio between metastatic and non-metastatic MANEC. Stage IV disease at diagnosis was more prognostically unfavorable in cases of colonic compared to anorectal origin. CONCLUSION: MANEC is a clinically aggressive pathological entity. The results of this study provide new insights for the understanding of tumor location within the lower GI tract and its prognosis in terms of overall survival.

Chronic constipation in adults: Contemporary perspectives and clinical challenges. 1: Epidemiology, diagnosis, clinical associations, pathophysiology and investigation.

BACKGROUND: Chronic constipation is a prevalent disorder that affects patients' quality of life and consumes resources in healthcare systems worldwide. In clinical practice, it is still considered a challenge as clinicians frequently are unsure as to which treatments to use and when. Over a decade ago, a Neurogastroenterology & Motility journal supplement devoted to the investigation and management of constipation was published (2009; 21 (Suppl.2)). This included seven articles, disseminating all themes covered during a preceding 2-day meeting held in London, entitled "Current perspectives in chronic constipation: a scientific and clinical symposium." In October 2018, the 3rd London Masterclass, entitled "Contemporary management of constipation" was held, again over 2 days. All faculty members were invited to author two new review articles, which represent a collective synthesis of talks presented and discussions held during this meeting. PURPOSE: This article represents the first of these reviews, addressing epidemiology, diagnosis, clinical associations, pathophysiology, and investigation. Clearly, not all aspects of the condition can be covered in adequate detail; hence, there is a focus on particular "hot topics" and themes that are of contemporary interest. The second review addresses management of chronic constipation, covering behavioral, conservative, medical, and surgical therapies.

Systematic evaluation of cough-anorectal pressure responses in health and in fecal incontinence: A high-resolution anorectal manometry study.

BACKGROUND: Anorectal manometry is the most commonly performed test of anorectal function. The cough-anorectal response is frequently assessed as part of a routine manometric investigation but has not previously been the subject of detailed analysis. This study systematically examined anorectal pressure responses to cough in health and evaluated the impact of parity and symptoms of fecal incontinence (FI) on measurements. METHODS: High-resolution anorectal manometry (HR-ARM) traces from nulliparous (n = 25) and parous (n = 25) healthy volunteers (HV: aged 41, range 18-64), and 57 parous patients with FI (age 47, range 28-72) were retrospectively reviewed. Cough-anorectal pressure responses were analyzed between groups by qualitative and quantitative approaches. KEY RESULTS: In health, traditional anal pressure measurements ("rest" and "squeeze") were similar between nulliparous and parous women. In contrast, incremental anal-rectal pressure difference during cough significantly differed: nulliparous 42 mm Hg (95% CI: 21-64) vs. parous 6 mm Hg (-14-25), P < 0.036). This measure also differed significantly between nulliparous HVs and patients with FI (-2 mm Hg (95% CI: -15-12), P < 0.001), but not between parous HVs and FI. Qualitatively, a color-contour trace resembling a "spear" in the upper anal canal was observed uniquely in FI. Of 25 patients with normal anal function by traditional measures, cough parameters were abnormal in 52%. CONCLUSIONS AND INFERENCES: Novel HR-ARM measures during coughing revealed differences in anal function between nulliparous and parous HV, and patients with FI, which were not detected by traditional measures. Cough-anorectal measurements may improve manometric yield, though clinical utility would require assessment by longitudinal studies.

The London Classification: Improving Characterization and Classification of Anorectal Function with Anorectal Manometry.

PURPOSE OF REVIEW: Objective measurement of anorectal sensorimotor function is a requisite component in the clinical evaluation of patients with intractable symptoms of anorectal dysfunction. Regrettably, the utility of the most established and widely employed investigations for such measurement (anorectal manometry (ARM), rectal sensory testing and the balloon expulsion test) has been limited by wide variations in clinical practice. RECENT FINDINGS: This article summarizes the recently published International Anorectal Physiology Working Group (IAPWG) consensus and London Classification of anorectal disorders, together with relevant allied literature, to provide guidance on the indications for, equipment, protocol, measurement definitions and results interpretation for ARM, rectal sensory testing and the balloon expulsion test. The London Classification is a standardized method and nomenclature for description of alterations in anorectal motor and sensory function using office-based investigations, adoption of which should bring much needed harmonization of practice.

The international anorectal physiology working group (IAPWG) recommendations: Standardized testing protocol and the London classification for disorders of anorectal function.

BACKGROUND: This manuscript summarizes consensus reached by the International Anorectal Physiology Working Group (IAPWG) for the performance, terminology used, and interpretation of anorectal function testing including anorectal manometry (focused on high-resolution manometry), the rectal sensory test, and the balloon expulsion test. Based on these measurements, a classification system for disorders of anorectal function is proposed. METHODS: Twenty-nine working group members (clinicians/academics in the field of gastroenterology, coloproctology, and gastrointestinal physiology) were invited to six face-to-face and three remote meetings to derive consensus between 2014 and 2018. KEY RECOMMENDATIONS: The IAPWG protocol for the performance of anorectal function testing recommends a standardized sequence of maneuvers to test rectoanal reflexes, anal tone and contractility, rectoanal coordination, and rectal sensation. Major findings not seen in healthy controls defined by the classification are as follows: rectoanal areflexia, anal hypotension and hypocontractility, rectal hyposensitivity, and hypersensitivity. Minor and inconclusive findings that can be present in health and require additional information prior to diagnosis include anal hypertension and dyssynergia. CONCLUSIONS AND INFERENCES: This framework introduces the IAPWG protocol and the London classification for disorders of anorectal function based on objective physiological measurement. The use of a common language to describe results of diagnostic tests, standard operating procedures, and a consensus classification system is designed to bring much-needed standardization to these techniques.

Functional anal canal length measurement using high-resolution anorectal manometry to investigate anal sphincter dysfunction in patients with fecal incontinence or constipation.

BACKGROUND: Using high-resolution anorectal manometry, we aimed to evaluate the: (a) optimal threshold to measure functional anal canal length (FACL) with intra- and inter-observer variability; (b) relationship between abnormal FACL and other phenotypic findings; and (c) utility of FACL measurement to discriminate between healthy volunteers (HV) and patients with fecal incontinence (FI) or chronic constipation (CC). METHODS: Consecutive patients referred for investigation of FI or CC in isolation were included. FACL was calculated at 5, 10, 20, 30, and 50 mm Hg above rectal pressure. 5th-95th percentile in HV defined limits of normality. KEY RESULTS: FACL was analyzed in 192 patients with FI (154 females), 204 patients with CC (175 females), and 134 HV (101 females). The optimal threshold for measurement of FACL was 20 mm Hg. Using this threshold, observer reproducibility was very high (ICC 0.99, P < 0.0001). FACL was shorter in females with FI and longer in females with CC compared to HV (2.3 vs 2.9 cm; and 3.4 cm vs 2.9 cm; both P < 0.0001). Twenty percent of females and 13% males with FI had a short FACL, associated with older age, anal hypotonia or hypocontractility, and a atrophic internal anal sphincter. However, abnormal FACL in isolation was only found in 1/190 FI (0.5%) and 2/201 CC (1%) patients; all other patients had other abnormal manometric findings. CONCLUSIONS AND INFERENCES: Considering overlap with other routinely evaluated manometric parameters, FACL measurement does not provide additional information in the diagnostic assessment of FI or CC.

High-resolution Anorectal Manometry Measures Are More Accurate Than Conventional Measures in Detecting Anal Hypocontractility in Women With Fecal Incontinence.

BACKGROUND & AIMS: Anorectal manometry is a common test of anal tone and contractility. However, existing measures are often criticized for their poor ability to detect functional differences between individuals with presumably normal sphincter function and individuals with fecal incontinence. We investigated whether new measures, derived from high-resolution anal manometry (HR-ARM), more accurately identify incontinent women with abnormal sphincter function than measures from conventional anal manometry (C-ARM). METHODS: We performed a prospective HR-ARM study of 85 healthy female volunteers (median age 41 years; range 18-68 years) and 403 women with fecal incontinence (median age 55; range 18-91 years) from June 2013 through December 2015. We compared the diagnostic accuracy of conventional measurements of contractility (C-ARM squeeze increment) and tone (C-ARM resting pressure) (the reference standards), with HR-ARM measures (HR-ARM squeeze increment, HR-ARM contractile integral, HR-ARM resting average, HR-ARM rest integral). We also investigated associations between symptom severity and structural sphincter integrity. RESULTS: We identified anal hypocontractility in 129 women with fecal incontinence using the C-ARM squeeze increment (32% sensitivity; likelihood ratio, 6.4), in 189 women using the HR-ARM squeeze increment (47% sensitivity; likelihood ratio, 9.4), and in 220 women using the HR-ARM contractile integral (55% sensitivity; likelihood ratio, 11.0). In contrast, HR-ARM measures of anal tone did not particularly outperform conventional measures (31% sensitivity for HR-ARM resting average vs 28% for C-ARM resting pressure). Ninety-eight of the 206 patients with fecal incontinence who were classified as having normal anal function, based on C-ARM measures, were reclassified as having abnormal function based on HR-ARM measures. CONCLUSION: In a prospective study of healthy women and women with fecal incontinence, we demonstrated that the newly developed HR-ARM contractile integral increased the sensitivity of detection of anal hypocontractility, from 32% to 55%, compared with conventional measurements of squeeze.

Expert consensus document: Advances in the evaluation of anorectal function.

Faecal incontinence and evacuation disorders are common, impair quality of life and incur substantial economic costs worldwide. As symptoms alone are poor predictors of underlying pathophysiology and aetiology, diagnostic tests of anorectal function could facilitate patient management in those cases that are refractory to conservative therapies. In the past decade, several major technological advances have improved our understanding of anorectal structure, coordination and sensorimotor function. This Consensus Statement provides the reader with an appraisal of the current indications, study performance characteristics, clinical utility, strengths and limitations of the most widely available tests of anorectal structure (ultrasonography and MRI) and function (anorectal manometry, neurophysiological investigations, rectal distension techniques and tests of evacuation, including defecography). Additionally, this article provides our consensus on the clinical relevance of these tests.

Colonic and anorectal motility testing in the high-resolution era.

PURPOSE OF REVIEW: The past few years have seen an increase in the number of research and clinical groups around the world using high-resolution manometry (HRM) to record contractile activity in the anorectum and colon. Yet despite the uptake and growing number of publications, the clinical utility and potential advantages over traditional manometry remain undetermined. RECENT FINDINGS: Nearly all of the publications in the field of anorectal and colonic HRM have been published within the last 3 years. These studies have included some data on normal ranges in healthy adults, and abnormalities in patient groups with constipation or fecal incontinence, anal fissure, perineal descent, rectal cancer, and Hirschsprung's disease. Most of the studies have been conducted on adults, with only three published studies in pediatric populations. Very few studies have attempted to show advantages of HRM over traditional manometry SUMMARY: High-resolution anorectal and colonic manometry provide a more comprehensive characterization of motility patterns and coordinated activity; this may help to improve our understanding of the normal physiology and pathophysiology in these regions. To date, however, no published study has conclusively demonstrated a clinical, diagnostic, or interventional advantage over conventional manometry.

Diagnostic accuracy study of anorectal manometry for diagnosis of dyssynergic defecation.

OBJECTIVE: The diagnostic accuracy of anorectal manometry (AM), which is necessary to diagnose functional defecatory disorders (FDD), is unknown. Using blinded analysis and standardised reporting of diagnostic accuracy, we evaluated whether AM could discriminate between asymptomatic controls and patients with functional constipation (FC). DESIGN: Derived line plots of anorectal pressure profiles during simulated defecation were independently analysed in random order by three expert observers blinded to health status in 85 women with FC and 85 age-matched asymptomatic healthy volunteers (HV). Using accepted criteria, these pressure profiles were characterised as normal (ie, increased rectal pressure coordinated with anal relaxation) or types I-IV dyssynergia. Interobserver agreement and diagnostic accuracy were determined. RESULTS: Blinded consensus-based assessment disclosed a normal pattern in 16/170 (9%) of all participants and only 11/85 (13%) HV. The combined frequency of dyssynergic patterns (I-IV) was very similar in FC (80/85 (94%)) and HV (74/85 (87%)). Type I dyssynergia ('paradoxical' contraction) was less prevalent in FC (17/85 (20%) than in HV (31/85 (36.5%), p=0.03). After statistical correction, only type IV dyssynergia was moderately useful for discriminating between FC (39/85 (46%)) and HV (17/85 (20%)) (p=0.001, positive predictive value=70.0%, positive likelihood ratio=2.3). Interobserver agreement was substantial or moderate for identifying a normal pattern, dyssynergia types I and IV, and FDD, and fair for types II and III. CONCLUSIONS: While the interpretation of AM patterns is reproducible, nearly 90% of HV have a pattern that is currently regarded as 'abnormal' by AM. Hence, AM is of limited utility for distinguishing between FC and HV.

Cost-effectiveness of laparoscopic versus open pyloromyotomy.

BACKGROUND: Infantile hypertrophic pyloric stenosis can be corrected by either open (OP) or laparoscopic pyloromyotomy (LP). LP may provide clinical benefits of reduced time to postoperative full feeds and reduced postoperative inpatient stay, but the cost effectiveness is not known. Our aim was to compare the cost effectiveness of laparoscopic and open pyloromyotomy. METHODS: OP and LP were compared in a multicenter randomized double-blind controlled trial, for which the primary outcomes were time to full feeds and time to discharge. In order to undertake a detailed cost analysis, we assigned costs, calculated on an individual patient basis, to laboratory costs, imaging, medical staff, medication, ward, operative, and outpatient appointments for 74 patients recruited from one of the participating centers. Data (mean ± SEM) were compared using linear regression analysis, adjusting for the minimization criteria used in the trial. RESULTS: Operation costs were similar between the two groups ($3,276 ± $244 LP versus $3,535 ± $152 OP). A shorter time to full feeds and shorter hospital stay in LP versus OP patients resulted in a highly significant difference in ward costs ($2,650 ± $126 LP versus $3,398 ± $126 OP; P = .001) and a small difference in other costs. Overall, LP patients were $1,263 (95% confidence interval $395-$2,130; P = .005) less expensive to treat than OP patients. Sensitivity analyses of laparoscopic hardware usage and of incomplete pyloromyotomy indicated that LP was consistently less expensive than OP. CONCLUSIONS: LP is a cost-effective alternative to OP as it delivers improved clinical outcome at a lower price.

Advancement anoplasty and sacral nerve stimulation: an effective combination for radiation-induced anal stenosis.

INTRODUCTION: Pelvic radiotherapy can cause anal stenosis. Patients can be left with severe rectal evacuatory difficulties, anal fissuring and resistant faecal incontinence. The management of such patients is difficult since surgical treatment can worsen faecal incontinence. CASE STUDY: We report a patient who was treated for recurrent fissuring and faecal incontinence secondary to severe anal stenosis caused by external beam radiotherapy to his prostate. A 74-year-old male patient underwent excision of the fissuring, fibrotic anal mucosa and internal sphincter and was then treated with a broad-based House advancement anoplasty. The patient's fissuring was successfully treated but he still suffered from faecal incontinence. The patient underwent sacral nerve stimulation with significant improvement in all faecal incontinence symptoms. CONCLUSION: The use of a novel combination of a House advancement anoplasty and sacral nerve stimulation is a safe and effective treatment rationale for treatment of radiation-induced anal stenosis.