Discontinuity of marginal artery at splenic flexure and rectosigmoid junction: A systematic review and meta-analysis.

AIM: The aim of this study was to provide comprehensive evidence-based assessment of the discontinuity of the marginal artery at the splenic flexure (SF) and the rectosigmoid junction (RSJ). METHOD: A systematic review was conducted of literature published to 26 December 2022 in the electronic databases PubMed, SCOPUS and Web of Science to identify studies eligible for inclusion. Data were extracted and pooled into a meta-analysis using the Metafor package in R. The primary outcomes were the pooled PPEs of the marginal artery at the SF and the RSJ. The secondary outcome was the size of vascular anastomoses. RESULTS: A total of 21 studies (n = 2,864 patients) were included. The marginal artery was present at the splenic flexure in 82% (95% CI: 62-95) of patients. Approximately 81% (95% CI: 63-94%) of patients had a large macroscopic anastomosis, while the remainder (19%) had small bridging ramifications forming the vessel. The marginal artery was present at the RSJ in 82% (95% CI: 70-91%) of patients. CONCLUSION: The marginal artery may be absent at the SF and the RSJ in up to 18% of individuals, which may confer a higher risk of ischaemic colitis. As a result of high interstudy heterogeneity noted in our analysis, further well-powered studies to clarify the prevalence of the marginal artery at the SF and the RSJ, as well as its relationship with other complementary colonic collaterals (intermediate and central mesenteric), are warranted.

Evaluation of the current guidelines for the management of haemorrhoidal disease using the Appraisal of Guidelines Research and Evaluation II instrument.

Background: Haemorrhoids are a very common disease and many professional societies have produced guidelines for their treatment. The aim of this study is to appraise the quality of the existing guidelines in the management of haemorrhoids. Methods: A systematic search of the literature was conducted in the EMBASE, Google Scholar, Cochrane library, and PubMed databases. The quality of guidelines was independently appraised using the Appraisal of Guidelines Research and Evaluation II (AGREE II) instrument by five of the authors. Results: Six guidelines of varying quality were identified and included in this study. The highest scoring guidelines were the SICCR (Società Italiana di Chirurgia Colorectale, which is Italian Society of Colorectal Surgery), ESCP (European Society of Coloproctology) and ASCRS (American Society of Colon and Rectal Surgeons) guidelines, scoring 86% each overall. There was considerable variability across not just the studies but across the different domains. The highest scoring domains were domain VI: editorial independence (median =95% across all studies) and domain I: Scope & Purpose (85%). The lowest scores were observed in domain V: Applicability (48%) and domain II: Stakeholder Involvement (41%). Only three of the six gained unanimous support for their use, whilst two of the guidelines were unanimously declared not suitable for clinical use. Conclusions: With the notable exception of three guidelines (SICCR, ESCP and ASCRS), the general quality of haemorrhoid guidelines is poor. Stakeholder (especially patient) involvement and instructions on how to implement recommendations is lacking from the majority of guidelines. This is an area that requires urgent attention if we are to improve guidelines in haemorrhoid management.

Systematic review and meta-analysis comparing perioperative outcomes of emergency appendectomy performed by trainee vs trained surgeon.

BACKGROUND: Appendectomy is a benchmark operation for trainee progression, but this should be weighed against patient safety and perioperative outcomes. METHODS: Systematic literature review and meta-analysis comparing outcomes of appendectomy performed by trainees versus trained surgeons. RESULTS: Of 2086 articles screened, 29 studies reporting on 135,358 participants were analyzed. There was no difference in mortality (Odds ratio [OR] 1.08, P = 0.830), overall complications (OR 0.93, P = 0.51), or major complications (OR 0.56, P = 0.16). There was no difference in conversion from laparoscopic to open surgery (OR 0.81, P = 0.12) and in intraoperative blood loss (Mean Difference [MD] 5.58 mL, P = 0.25). Trainees had longer operating time (MD 7.61 min, P < 0.0001). Appendectomy by trainees resulted in shorter duration of hospital stay (MD 0.16 days, P = 0.005) and decreased reoperation rate (OR 0.78, P = 0.05). CONCLUSIONS: Appendectomy performed by trainees does not compromise patient safety. Due to statistical heterogeneity, further randomized controlled trials, with standardized reported outcomes, are required.

Systematic review and meta-analysis comparing perioperative outcomes of pediatric emergency appendicectomy performed by trainee vs trained surgeon.

Appendicectomy is a common pediatric surgical procedure performed by trainees and surgeons with varying reported outcomes. It is a benchmark procedure for trainee progression and training benefits should be weighed against patient safety and perioperative outcomes. This systematic review and meta-analysis investigated any differential perioperative outcomes dependent on the grade of the operating surgeon. A systematic literature review and meta-analysis were performed comparing outcomes of pediatric appendicectomy performed by trainees versus trained surgeons. Of 2,086 articles screened, 5 retrospective non-randomized comparative studies reporting on 10,019 participants were analyzed. There was no difference in overall complications (OR 0.92; 95% CI 0.76, 1.12; P = 0.42), major complications [Clavien-Dindo (CD) III/IV] (OR 1.18; 95% CI 0.71, 1.97; P = 0.52), minor complications (CD I/II) (OR 1.13; 95% CI 0.57, 2.27; P = 0.72), post-op ileus (OR 0.74; 95% CI 0.10, 5.26; P = 0.76), wound infections (OR 0.87; 95% CI 0.62, 1.21; P = 0.41), abscess formation (OR 0.58; 95% CI 0.28, 1.22; P = 0.15), operation times [Mean Difference (MD) 2.31 min; 95% CI - 4.94, 9.56; P = 0.53] and reoperation rate (OR 1.22; 95% CI 0.23, 6.42; P = 0.81). Trainees had fewer conversions to open appendicectomy (OR 0.14; 95% CI 0.02, 0.88; P = 0.04). Appendicectomy performed on pediatric patients by trainees did not compromise patient safety. LEVEL OF EVIDENCE: III.

The WSES/SICG/ACOI/SICUT/AcEMC/SIFIPAC guidelines for diagnosis and treatment of acute left colonic diverticulitis in the elderly.

Acute left colonic diverticulitis (ALCD) in the elderly presents with unique epidemiological features when compared with younger patients. The clinical presentation is more nuanced in the elderly population, having higher in-hospital and postoperative mortality. Furthermore, geriatric comorbidities are a risk factor for complicated diverticulitis. Finally, elderly patients have a lower risk of recurrent episodes and, in case of recurrence, a lower probability of requiring urgent surgery than younger patients. The aim of the present work is to study age-related factors that may support a unique approach to the diagnosis and treatment of this problem in the elderly when compared with the WSES guidelines for the management of acute left-sided colonic diverticulitis. During the 1° Pisa Workshop of Acute Care & Trauma Surgery held in Pisa (Italy) in September 2019, with the collaboration of the World Society of Emergency Surgery (WSES), the Italian Society of Geriatric Surgery (SICG), the Italian Hospital Surgeons Association (ACOI), the Italian Emergency Surgery and Trauma Association (SICUT), the Academy of Emergency Medicine and Care (AcEMC) and the Italian Society of Surgical Pathophysiology (SIFIPAC), three panel members presented a number of statements developed for each of the four themes regarding the diagnosis and management of ALCD in older patients, formulated according to the GRADE approach, at a Consensus Conference where a panel of experts participated. The statements were subsequently debated, revised, and finally approved by the Consensus Conference attendees. The current paper is a summary report of the definitive guidelines statements on each of the following topics: diagnosis, management, surgical technique and antibiotic therapy.

A systematic review of the literature assessing operative blood loss and postoperative outcomes after colorectal surgery.

PURPOSE: There is no consensus in the literature regarding the association between operative blood loss and postoperative outcomes in colorectal surgery, despite evidence suggesting a link. Therefore, this systematic review assesses the association between operative blood loss, perioperative and long-term outcomes after colorectal surgery. METHODS: A literature search of MEDLINE, EMBASE, Science Citation Index Expanded and Cochrane was performed to identify studies reporting on operative blood loss in colorectal surgery. RESULTS: The review included forty-nine studies reporting on 61,312 participants, with a mean age ranging from 53.4 to 78.1 years. The included studies demonstrated that major operative blood loss was found to be a risk factor for mortality, anastomotic leak, presacral abscess, and postoperative ileus, leading to an increased duration of hospital stay. In the long term, the studies suggest that significant blood loss was an independent risk factor for future small bowel obstruction due to colorectal cancer recurrence and adhesions. Studies found that survival was significantly reduced, whilst the risk of colorectal cancer recurrence was increased. Reoperation and cancer-specific survival were not associated with major blood loss. CONCLUSION: The results of this systematic review suggest that major operative blood loss increases the risk of perioperative adverse events and has short and long-term repercussions on postoperative outcomes. Laparoscopic and robotic surgery, vessel ligation technology and anaesthetic considerations are essential for reducing blood loss and improving outcomes. This review highlights the need for further high quality, prospective, multicentre trials with a greater number of participants, and accurate and standardised methods of measuring operative blood loss.

Does the Choice of Extraction Site During Minimally Invasive Colorectal Surgery Change the Incidence of Incisional Hernia? Protocol for a Systematic Review and Network Meta-Analysis.

BACKGROUND: Various sites are used for specimen extraction in oncological minimally invasive colorectal surgery. The objective is to determine if the choice of extraction site modulates the incidence of incisional hernia (IH). METHODS/DESIGN: A systematic review will be performed in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. MEDLINE, Embase and CENTRAL will be searched to look for original studies reporting the incidence of IH after minimally invasive colorectal surgery. Studies will be excluded from the analysis if: 1) they do not report original data, 2) the outcome of interest (incidence of incisional hernia) is not clearly reported and does not allow to extrapolate and/or calculate the required data for network meta-analysis, 3) they include pediatric patients, 4) they include a patients' population with a conversion rate to laparotomy >10%, 5) they do not compare at least two different extraction sites for the operative specimen, 6) they report patients who underwent pure (and not hybrid) natural orifice transluminal endoscopic surgery (NOTES). Network meta-analysis will be performed to determine the incidence of IH per extraction site. DISCUSSION: By determining which specimen extraction site leads to reduced rate of IH, this systematic review and network meta-analysis will help colorectal surgeons to choose their extraction site and reduce the morbidity and costs associated with IH. REGISTRATION: The systematic review and meta-analysis protocol is registered in the International Prospective Register of Ongoing Systematic Reviews (PROSPERO) with number CRD42021272226. HIGHLIGHTS: Various sites are used for specimen extraction in oncological minimally invasive colorectal surgery, and the choice of the site may probably modulate the incidence of incisional hernia.The present protocol aims to design a systematic review which will identify original studies comparing two extraction sites during minimally invasive colorectal surgery in terms of incidence of incisional hernia.Network meta-analysis will be performed to determine the incidence of IH per extraction site.

A systematic review and meta-analysis of variants of the branches of the superior mesenteric artery: the Achilles heel of right hemicolectomy with complete mesocolic excision?

AIM: Dissection with subsequent ligation and resection of arteries at their origin (central vascular ligation) is essential for adequate oncological resection during right hemicolectomy with complete mesocolic excision. This technique is technically demanding due to the highly variable arterial pattern of the right colon. Therefore, this study aims to provide a comprehensive evidence-based assessment of the arterial vascular anatomy of the right colon. METHODS: A thorough systematic literature search through September 2020 was conducted on the electronic databases PubMed, Scopus and Web of Science to identify studies eligible for inclusion. Data were extracted and pooled into a meta-analysis using MetaXl software. RESULTS: A total of 41 studies (n = 4691 patients) were included. The ileocolic artery (ICA), right colic artery (RCA) and middle colic artery (MCA) were present in 99.7% (95% CI 99.4%-99.8%), 72.6% (95% CI 61.3%-82.5%) and 96.9% (95% CI 94.2%-98.8%) respectively of patients. Supernumerary RCA and MCA were observed in 3.2% and 11.4% respectively of all cases. The RCA shared a common trunk with the ICA and MCA in 13.2% and 17.7% respectively of patients. A retro-superior mesenteric vein course of the ICA and RCA was observed in 55.1% and 11.4% respectively of all cases. CONCLUSION: The vascular anatomy of the right colon displays several notable variations, namely the absence of some branches (RCA absent in 27.4% of cases), supernumerary branches, common trunks, and retro-superior mesenteric vein courses. These variations should be taken into consideration during right hemicolectomy with complete mesocolic excision to ensure adequate oncological resection while minimizing intra-operative complications.

Surgical anatomy of the accessory middle colic artery: a meta-analysis with implications for splenic flexure cancer surgery.

AIM: Surgical resection of splenic flexure cancers (SFCs) is technically demanding due to the complex regional anatomy, characterized by the presence of embryological adhesions, close proximity to the pancreas and spleen, and a highly heterogeneous arterial supply and lymphatic drainage. The accessory middle colic artery (AMCA) is increasingly being recognized as an important source of blood supply to the splenic flexure. The aim of this study is to determine the prevalence and anatomical features of the AMCA. METHOD: A systematic search of the scientific literature was conducted on PubMed and Embase from inception to November 2020 to identify potentially eligible studies. Data were extracted and prevalence was pooled into a meta-analysis using MetaXL and Meta-Analyst software. RESULTS: A total of 16 studies (n = 2203 patients) were included. The pooled prevalence (PP) of the AMCA was 25.4% (95% CI 18.1-33.4). Its prevalence was higher in patients without a left colic artery (LCA) (PP = 83.2%; 95% CI 70.4-93.1). The commonest origin for the AMCA was the superior mesenteric artery (PP = 87.9%; 95% CI 86.4-90.7). The AMCA shared a common trunk/gave rise to pancreatic branches in 23.1% of cases (95% CI 15.3-31.9). CONCLUSION: The AMCA contributes to the vascularization of the splenic flexure in approximately 25% of individuals, and may be an important feeder artery to SFCs, especially in the absence of a LCA. Preoperative identification of this artery is important to ensure optimal surgery for SFC and minimize complications.

Acute appendicitis management during the COVID-19 pandemic: A prospective cohort study from a large UK centre.

BACKGROUND: During the Covid-19 pandemic, non-operative management for acute appendicitis (AA) was implemented in the UK. The aim of this study was to determine the efficacy and outcomes of conservative versus surgical management of AA during the pandemic. MATERIALS & METHODS: We conducted an observational study in a tertiary referral centre. Data was collected from all patients (≥16 years) with a diagnosis of AA between November 1, 2019 to March 10, 2020 (pre-COVID period) and March 10, 2020 to July 5, 2020 (COVID period). RESULTS: A total of 116 patients in the pre-COVID period were included versus 91 in the COVID period. 43.1% (n = 50) of patients pre-COVID were classified as ASA 2 compared to 26.4% (n = 24) during the COVID period (p-value = 0.042). 72.5% (n = 66) of the patients during the COVID period scored as high risk using the Alvarado score compared to 24.1% (n = 28) in the pre-COVID period (p-value<0.001). We observed a significant increase in radiological evaluation, 69.8% versus 87.5% of patients had a CT in the pre-COVID and COVID periods respectively (p-value = 0.008). 94.9% of patients were managed operatively in the pre-COVID period compared to 60.4% in the COVID period (p-value<0.001). We observed more open appendicectomies (37.3% versus 0.9%; p-value<0.001) during the COVID period compared to the pre-COVID period. More abscess formation and free fluid were found intraoperatively in the COVID period (p-value = 0.021 and 0.023 respectively). Re-attendance rate due to appendicitis-related issues was significantly higher in the COVID period (p = 0.027). CONCLUSION: Radiological diagnosis of AA was more frequent during the COVID period. More conservative management for AA was employed during the COVID-19 pandemic, and for those managed operatively an open approach was preferred. Intra-operative findings were suggestive of delayed presentation during the COVID period without this affecting the length of hospital stay.

Surgical anatomy of sigmoid arteries: A systematic review and meta-analysis.

BACKGROUND: The purpose of this systematic review and meta-analysis was to determine the prevalence of the number of sigmoid arteries (SA) and variations in their origins. METHODS: A thorough systematic search of literature through February 2020 was conducted on major electronic databases to identify eligible studies. Data were extracted and pooled into a meta-analysis using Metafor package in R. The primary outcome was the variations in the SA origin (according to modified Zebrowski classification), and the secondary outcome was the prevalence of the number of SA. RESULTS: A total of 22 studies (n = 2653 patients) were included. Type 1 modified Zebrowski (separated origins or common trunk of the SA originating from descending recto-sigmoid trunk (DRST)) was the most common origin type of the SA (pooled prevalence estimate (PPE) = 49.67% (95% CI 32. 67- 66.71)), while type 3 (separated origins or common trunk of 1 or 2 SA originating from DRST or superior rectal artery (SRA) and 1 or 2 SA originating from DRST or SRA) was the least common (PPE = 0.18%; 95% CI 0.00-2.82)). Of the Type 1 variants, the not specified (N.S) variant was by far the most prevalent. The number of SA ranged from one to five, with three being the mode (PPE = 42.3%). CONCLUSION: This is the most comprehensive analysis of arterial vascular anatomy of the sigmoid colon. In light of the highly variable anatomical pattern displayed by the SA, thorough pre-operative knowledge of their anatomy can be crucial in minimizing incidences of iatrogenic injury.

Laparoscopic versus open complete mesocolic excision: a systematic review by updated meta-analysis.

Recent evidence has proven the non-inferiority of laparoscopic complete mesocolic excision (LCME) to open complete mesocolic excision (OCME) with regard to feasibility and oncological safety. However, the differences in survival benefits between the 2 procedures have not been assessed. The aim of this study was to evaluate whether or not one procedure was superior to the other using updated meta-analysis. A systematic search for relevant literature was performed in Pubmed, Embase, Cochrane library and Google scholar databases. This meta-analysis included retrospective studies and one randomised controlled trial comparing LCME to OSCME. LCME to OCME was evaluated using updated meta-analysis. The Newcastle-Ottawa scale was used to assess the methodologic quality of the studies. Fixed- and random-effects models were used, and survival outcomes were assessed using the inverse variance hazard ratio (HR) method. Operative time was significantly shorter in the OCME cohort than in the LCME cohort. Blood loss, wound infections, time to flatus, time to oral feeding, and length of hospital stay were significantly shorter in the LCME cohort than in the OCME cohort. The 1-, 3-, and 5-year overall survivals were better in the LCME cohort than in the OCME cohort ([HR = 0.37 (0.22, 0.65); p = 0.004], [HR = 0.48 (0.31, 0.74); p = 0.008], and [HR = 0.64 (0.45, 0.93); p = 0.02], respectively). No difference in the 1-year disease-free survival (DFS) between the 2 procedures was observed ([HR = 0.68 (0.44, 1.03); p = 0.07]). In contrast, the LCME cohort had better 3- and 5-year DFS rates than those of the OCME cohort ([HR = 0.63 (0.42, 0.97), p = 0.03] and [HR = 0.68 (0.56, 0.83), p = 0.001], respectively). The results of the present study must be interpreted cautiously because the included studies were retrospective from single centres. Therefore, selection, institutional and national bias may have influenced the results. LCME is associated with the faster postoperative recovery and some better potential survival benefits than OCME.

Robotic-assisted versus open pancreaticoduodenectomy for patients with benign and malignant periampullary disease: a systematic review and meta-analysis of short-term outcomes.

BACKGROUND: Although several non-randomized studies comparing robotic pancreaticoduodenectomy (RPD) and open pancreaticoduodenectomy (OPD) recently demonstrated that the two operative techniques could be equivalent in terms of safety outcomes and short-term oncologic efficacy, no definitive answer has arrived yet to the question as to whether robotic assistance can contribute to reducing the high rate of postoperative morbidity. METHODS: Systematic literature search was performed using MEDLINE, the Cochrane Central Register of Controlled Trials, and EMBASE databases. Prospective and retrospective studies comparing RPD and OPD as surgical treatment for periampullary benign and malignant lesions were included in the systematic review and meta-analysis with no limits of language or year of publication. RESULTS: 18 non-randomized studies were included for quantitative synthesis with 13,639 patients allocated to RPD (n = 1593) or OPD (n = 12,046). RPD and OPD showed equivalent results in terms of mortality (3.3% vs 2.8%; P = 0.84), morbidity (64.4% vs 68.1%; P = 0.12), pancreatic fistula (17.9% vs 15.9%; P = 0.81), delayed gastric emptying (16.8% vs 16.1%; P = 0.98), hemorrhage (11% vs 14.6%; P = 0.43), and bile leak (5.1% vs 3.5%; P = 0.35). Estimated intra-operative blood loss was significantly lower in the RPD group (352.1 ± 174.1 vs 588.4 ± 219.4; P = 0.0003), whereas operative time was significantly longer for RPD compared to OPD (461.1 ± 84 vs 384.2 ± 73.8; P = 0.0004). RPD and OPD showed equivalent results in terms of retrieved lymph nodes (19.1 ± 9.9 vs 17.3 ± 9.9; P = 0.22) and positive margin status (13.3% vs 16.1%; P = 0.32). CONCLUSIONS: RPD is safe and feasible as surgical treatment for malignant or benign disease of the pancreatic head and the periampullary region. Equivalency in terms of surgical radicality including R0 curative resection and number of harvested lymph nodes between the two groups confirmed the reliability of RPD from an oncologic point of view.

Prophylactic intra-abdominal drainage following colorectal anastomoses. A systematic review and meta-analysis of randomized controlled trials.

BACKGROUND: Clinically evident Anastomotic Leakage (AL) remains one of the most feared complications after colorectal resections with primary anastomosis. The primary aim of this systematic review and meta-analysis was to determine whether Prophylactic Drainage (PD) after colorectal anastomoses confers any advantage in the prevention and management of AL. METHODS: Systematic literature search was performed using MEDLINE, the Cochrane Central Register of Controlled Trials, and EMBASE databases for randomized studies comparing clinical outcomes of patients with Drained (D) or Undrained (UD) colorectal anastomoses performed for any cause. RESULTS: Four randomized controlled trials comparing D and UD patients undergoing colorectal resections with primary anastomosis were included for quantitative synthesis. In total, 1120 patients were allocated to group D (n = 566) or group UD (n = 554). The clinical AL rate was 8.5% in the D group and 7.6% in the UD group, with no statistically significant difference (P = 0.57). Rates of radiological AL (D: 4.2% versus UD: 5.6%; P = 0.42), mortality (D: 3.6% versus UD: 4.4%; P = 0.63), overall morbidity (D: 16.6% versus UD: 18.6%, P = 0.38), wound infection (D: 5.4% versus UD: 5.3%, P = 0.95), pelvic sepsis (D: 9.7% versus UD: 10.5%, P = 0.75), postoperative bowel obstruction (D: 9.9% versus UD: 6.9%, P = 0.07), and reintervention for abdominal complication (D: 9.1% versus UD: 7.9%, P = 0.48) were equivalent between the two groups. CONCLUSIONS: The present meta-analysis of randomized controlled trials investigating the value of PD following colorectal anastomoses does not support the routine use of prophylactic drains.

British Society of Gastroenterology consensus guidelines on the management of inflammatory bowel disease in adults.

Ulcerative colitis and Crohn's disease are the principal forms of inflammatory bowel disease. Both represent chronic inflammation of the gastrointestinal tract, which displays heterogeneity in inflammatory and symptomatic burden between patients and within individuals over time. Optimal management relies on understanding and tailoring evidence-based interventions by clinicians in partnership with patients. This guideline for management of inflammatory bowel disease in adults over 16 years of age was developed by Stakeholders representing UK physicians (British Society of Gastroenterology), surgeons (Association of Coloproctology of Great Britain and Ireland), specialist nurses (Royal College of Nursing), paediatricians (British Society of Paediatric Gastroenterology, Hepatology and Nutrition), dietitians (British Dietetic Association), radiologists (British Society of Gastrointestinal and Abdominal Radiology), general practitioners (Primary Care Society for Gastroenterology) and patients (Crohn's and Colitis UK). A systematic review of 88 247 publications and a Delphi consensus process involving 81 multidisciplinary clinicians and patients was undertaken to develop 168 evidence- and expert opinion-based recommendations for pharmacological, non-pharmacological and surgical interventions, as well as optimal service delivery in the management of both ulcerative colitis and Crohn's disease. Comprehensive up-to-date guidance is provided regarding indications for, initiation and monitoring of immunosuppressive therapies, nutrition interventions, pre-, peri- and postoperative management, as well as structure and function of the multidisciplinary team and integration between primary and secondary care. Twenty research priorities to inform future clinical management are presented, alongside objective measurement of priority importance, determined by 2379 electronic survey responses from individuals living with ulcerative colitis and Crohn's disease, including patients, their families and friends.

Proctalgia and constipation secondary to hypertrophic polyglucosan inclusion body myopathy of the internal anal sphincter: a case report.

BACKGROUND: Hereditary polyglucosan inclusion body myopathy of the internal anal sphincter is a rare cause of proctalgia fugax and constipation. Treatment options are explored. CASE PRESENTATION: A 61 year-old Caucasian woman presented with an 18-year history of severe anal pain and constipation. She had no response to medical treatment which included amitriptyline and topically administered diltiazem. Endoscopy revealed no abnormalities, whereas endoanal ultrasound showed an abnormally thick internal anal sphincter (> 5 mm) and anal manometry showed intermittent episodes of very high resting pressures in excess of 200 mmHg that resolved spontaneously after 2 minutes. She had no relief of her symptoms after receiving an injection of botulinum toxin to the internal anal sphincter. She subsequently underwent a lateral internal anal sphincterotomy which led to complete resolution of her symptoms. CONCLUSIONS: Hereditary polyglucosan inclusion body myopathy of the internal anal sphincter should be considered in the differential diagnosis of a patient presenting with severe anal pain and constipation in the absence of an anal fissure or sepsis. If medical therapy with calcium antagonists fails to provide symptom relief, lateral internal sphincterotomy should be considered rather than botulinum toxin injection.