Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

BACKGROUND: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. METHODS: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. RESULTS: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. CONCLUSIONS: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. TYPE OF STUDY: Clinical consensus statement. LEVEL OF EVIDENCE: 3a.

Expression of intestinal drug transporter proteins and metabolic enzymes in neonatal and pediatric patients.

The development of pediatric oral drugs is hampered by a lack of predictive simulation tools. These tools, in turn, require data on the physiological variables that influence oral drug absorption, including the expression of drug transporter proteins (DTPs) and drug-metabolizing enzymes (DMEs) in the intestinal tract. The expression of hepatic DTPs and DMEs shows age-related changes, but there are few data on protein levels in the intestine of children. In this study, tissue was collected from different regions of the small and large intestine from neonates (i.e., surgically removed tissue) and from pediatric patients (i.e., gastroscopic duodenal biopsies). The protein expression of clinically relevant DTPs and DMEs was determined using a targeted mass spectrometry approach. The regional distribution of DTPs and DMEs was similar to adults. Most DTPs, with the exception of MRP3, MCT1, and OCT3, and all DMEs showed the highest protein expression in the proximal small intestine. Several proteins (i.e., P-gp, ASBT, CYP3A4, CYP3A5, CYP2C9, CYP2C19, and UGT1A1) showed an increase with age. Such increase appeared to be even more pronounced for DMEs. This exploratory study highlights the developmental changes in DTPs and DMEs in the intestinal tract of the pediatric population. Additional evaluation of protein function in this population would elucidate the implications of the presented changes in protein expression on absorption of orally administered drugs in neonates and pediatric patients.

European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis.

BACKGROUND: The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care. METHOD: A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations. RESULTS: The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection. RECOMMENDATIONS: The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi's approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.

Gallbladder torsion: a rare cause of acute abdomen in a 12-month old child.

Background: Gallbladder torsion is a rare cause of an acute abdomen, predominantly occurring in elderly women and less frequently diagnosed in the pediatric population. The diagnosis is difficult and rarely made preoperatively. However, suspicion needs to be raised in children with acute onset of abdominal pain. Ultrasound can demonstrate different signs putting forward the diagnosis but findings are often non-specific, therefore clinical suspicion should prompt a laparoscopic exploration.Case presentation: We report a case of a 12-month old girl consulting with progressive abdominal discomfort and vomiting. Ultrasound revealed an enlarged gallbladder with thickening of the wall but without demonstrable color Doppler flow and a more horizontal orientation outside its normal anatomic fossa. Gallbladder torsion was suspected. Emergency laparoscopic exploration confirmed the diagnosis and a laparoscopic cholecystectomy was performed. The postoperative course was uneventful.Conclusions: Gallbladder torsion, although rare, should be included in the differential diagnosis of an acute abdomen in children. Early recognition is necessary for a favorable outcome. The diagnosis might be supported by ultrasound but remains difficult, which is why laparoscopic exploration should be considered when the diagnosis remains unclear.

Incidence and risk factors of metachronous contralateral inguinal hernia development up to 25 years after unilateral inguinal hernia repair: a single-centre retrospective cohort study.

BACKGROUND: Patients undergoing unilateral inguinal hernia repair (IHR) are at risk of metachronous contralateral inguinal hernia (MCIH) development. We evaluated incidence and risk factors of MCIH development up to 25 years after unilateral IHR to determine possible indications for concomitant prophylactic surgery of the contralateral groin at the time of primary surgery. METHODS: Patients between 18 and 70 years of age undergoing elective unilateral IHR in the University Hospital of Leuven from 1995 to 1999 were studied retrospectively using the electronic health records and prospectively via phone calls. Study aims were MCIH incidence and risk factor determination. Kaplan-Meier curves were constructed and univariable and multivariable Cox regressions were performed. RESULTS: 758 patients were included (91% male, median age 53 years). Median follow-up time was 21.75 years. The incidence of operated MCIH after 5 years was 5.6%, after 15 years 16.1%, and after 25 years 24.7%. The incidence of both operated and non-operated MCIH after 5 years was 5.9%, after 15 years 16.7%, and after 25 years 29.0%. MCIH risk increased with older age and decreased in primary right-sided IHR and higher BMI at primary surgery. CONCLUSION: The overall incidence of MCIH after 25-year follow-up is 29.0%. Potential risk factors for the development of a MCIH are primary left-sided inguinal hernia repair, lower BMI, and older age. When considering prophylactic repair, we suggest a patient-specific approach taking into account these risk factors, the surgical approach and the risk factors for chronic postoperative inguinal pain.

Update of the international HerniaSurge guidelines for groin hernia management.

BACKGROUND: Groin hernia repair is one of the most common operations performed globally, with more than 20 million procedures per year. The last guidelines on groin hernia management were published in 2018 by the HerniaSurge Group. The aim of this project was to assess new evidence and update the guidelines. The guideline is intended for general and abdominal wall surgeons treating adult patients with groin hernias. METHOD: A working group of 30 international groin hernia experts and all involved stakeholders was formed and examined all new literature on groin hernia management, available until April 2022. Articles were screened for eligibility and assessed according to GRADE methodologies. New evidence was included, and chapters were rewritten. Statements and recommendations were updated or newly formulated as necessary. RESULTS: Ten chapters of the original HerniaSurge inguinal hernia guidelines were updated. In total, 39 new statements and 32 recommendations were formulated (16 strong recommendations). A modified Delphi method was used to reach consensus on all statements and recommendations among the groin hernia experts and at the European Hernia Society meeting in Manchester on October 21, 2022. CONCLUSION: The HerniaSurge Collaboration has updated the international guidelines for groin hernia management. The updated guidelines provide an overview of the best available evidence on groin hernia management and include evidence-based statements and recommendations for daily practice. Future guideline development will change according to emerging guideline methodology.

Rectal Biopsy for Hirschsprung's Disease: A Multicentre Study Involving Biopsy Technique, Pathology and Complications.

BACKGROUND: The heterogeneity of rectal biopsy techniques has encouraged us to search for a surgical and pathological standardisation of this diagnostic technique to exclude Hirschsprung's disease. The different amounts of information on the anatomopathology report prompted us to compile a template for the anatomopathology report for diagnostic rectal biopsies for surgical colleagues and pathologists working on Hirschsprung's disease. METHODS: We gathered the anonymous biopsy information and its pathology information from five hospitals for all patients in which rectal biopsies were taken to diagnose Hirschsprung's disease over two years (2020-2021). RESULTS: Of the 82 biopsies, 20 suction (24.4%), 31 punch (37.8%) and 31 open biopsies (37.8%) were taken. Of all biopsies, 69 were conclusive (84.2%), 13 were not (15.8%). In the suction biopsy group, 60% were conclusive and 40% were not; for punch biopsy, the values were 87% and 13%, respectively and for open biopsy, 97% and 3%. Inconclusive results were due to insufficient submucosa in 6/8 suction biopsies, 4/4 punch biopsies and 0/1 open biopsies. An insufficient amount of submucosa was the reason for an inconclusive result in 6/20 cases (30%) after suction biopsy, 4/31 (12.9%) cases after punch biopsy and 0 cases (0%) after open biopsy. We had one case with major postoperative bleeding post suction biopsy; there were no further adverse effects after biopsy. CONCLUSIONS: Diagnostic rectal biopsies in children are safe. Non-surgical biopsies are more likely to give inconclusive results due to smaller amounts of submucosa present in the specimen. Open biopsies are especially useful when previous non-surgical biopsies are inconclusive. An experienced pathologist is a key factor for the result. The anatomopathology report should specify the different layers present in the specimen, the presence of ganglion cells and hypertrophic nerve fibres, their description and a conclusion.

Effect of Degradation in Small Intestinal Fluids on Mechanical Properties of Polycaprolactone and Poly-l-lactide-co-caprolactone.

Polycaprolactone and poly-l-lactide-co-caprolactone are promising degradable biomaterials for many medical applications. Their mechanical properties, especially a low elastic modulus, make them particularly interesting for implantable devices and scaffolds that target soft tissues like the small intestine. However, the specific environment and mechanical loading in the intestinal lumen pose harsh boundary conditions on the design of these devices, and little is known about the degradation of those mechanical properties in small intestinal fluids. Here, we perform tensile tests on injection molded samples of both polymers during in vitro degradation of up to 70 days in human intestinal fluids. We report on yield stress, Young's modulus, elongation at break and viscoelastic parameters describing both materials at regular time steps during the degradation. These characteristics are bench-marked against degradation studies of the same materials in other media. As a result, we offer time dependent mechanical properties that can be readily used for the development of medical devices that operate in the small intestine.

Patient Satisfaction After Inguinal Hernia Surgery: Literature Review of an Overlooked Patient-Reported Outcome Measure.

INTRODUCTION: Patient satisfaction after inguinal hernia surgery is currently underappreciated and not as well studied as other patient-reported outcome measures (PROMs) on this topic. This study aims to review the literature and summarize available data. MATERIALS AND METHODS: A literature review was conducted using Medline with focus on patient-reported satisfaction after elective, inguinal hernia surgery in adults. All inguinal hernia repair techniques were considered. Small sample sizes and short follow-up periods were excluded. The methodology and results of the remaining articles were reviewed. Due to heterogeneity of reporting between articles, only a descriptive analysis was performed. RESULTS: The available data from patient-reported outcome measures regarding satisfaction yields considerable heterogeneity and lacks validation. We found that 53% of all included studies used an asymmetrical response questionnaire. Although there is an overwhelming positive patient satisfaction, wide ranges of satisfaction were seen (78-100% more than averagely satisfied, compared to 0-15% less than averagely satisfied). The number of patients not willing to undergo inguinal hernia repair again ranges from 1-16%. CONCLUSION: Our study demonstrates that patient-reported satisfaction after inguinal hernia surgery is not uniformly surveyed and remains unvalidated. Further research on patient-reported satisfaction would benefit from the reported raw data of a standardised, validated, and symmetrical five-point Likert or 11-point NRS scale on regular intervals pre- and postoperatively.

Prevention of Incisional Hernias by Prophylactic Mesh-augmented Reinforcement of Midline Laparotomies for Abdominal Aortic Aneurysm Treatment: Five-year Follow-up of a Randomized Controlled Trial.

INTRODUCTION: The incidence of incisional hernias (IHs) after open repair of an abdominal aortic aneurysm (AAA) is high. Several randomized controlled trials have reported favorable results with the use of prophylactic mesh to prevent IHs, without increasing complications. In this analysis, we report on the results of the 60-month follow-up of the PRIMAAT trial. METHODS: In a prospective, multicenter, open-label, randomized design, patients were randomized between prophylactic retrorectus mesh reinforcement (mesh group), and primary closure of their midline laparotomy after open AAA repair (no-mesh group). This article reports on the results of clinical follow-up after 60 months. If performed, ultrasonography or computed tomography were used for the diagnosis of IHs. RESULTS: Of the 120 randomized patients, 114 were included in the intention-to-treat analysis. Thirty-three patients in the no-mesh group (33/58-56.9%) and 34 patients in the mesh group (34/56-60.7%) were evaluated after 5 years. In each treatment arm, 10 patients died between the 24-month and 60-month follow-up. The cumulative incidence of IHs in the no-mesh group was 32.9% after 24 months and 49.2% after 60 months. No IHs were diagnosed in the mesh group. In the no-mesh group, 21.7% (5/23) underwent reoperation within 5 years due to an IH. CONCLUSIONS: Prophylactic retrorectus mesh reinforcement after midline laparotomy for the treatment of AAAs safely and effectively decreases the rate of IHs. The cumulative incidence of IHs after open AAA repair, when no mesh is used, continues to increase during the first 5 years after surgery, which leads to a substantial rate of hernia repairs.

Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. METHODS: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups 'poor' ≤ 11, and 'fair' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. RESULTS: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The 'below normal' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. CONCLUSIONS: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. LEVEL OF EVIDENCE: Level III.

Protocol to develop a core outcome set in incisional hernia surgery: the HarMoNY Project.

INTRODUCTION: Incisional hernia has an incidence of up to 20% following laparotomy and is associated with significant morbidity and impairment of quality of life. A variety of surgical strategies including techniques and mesh types are available to manage patients with incisional hernia. Previous works have reported significant heterogeneity in outcome reporting for abdominal wall herniae, including ventral and inguinal hernia. This is coupled with under-reporting of important clinical and patient-reported outcomes. The lack of standardisation in outcome reporting contributes to reporting bias, hinders evidence synthesis and adequate data comparison between studies. This project aims to develop a core outcome set (COS) of clinically important, patient-oriented outcomes to be used to guide reporting of future research in incisional hernia. METHODS: This project has been designed as an international, multicentre, mixed-methods project. Phase I will be a systematic review of current literature to examine the current clinical and patient-reported outcomes for incisional hernia and abdominal wall reconstruction. Phase II will identify the outcomes of importance to all key stakeholders through in depth qualitative interviews. Phase III will achieve consensus on outcomes of most importance and for inclusion into a COS through a Delphi process. Phase IV will achieve consensus on the outcomes that should be included in a final COS. ETHICS AND DISSEMINATION: The adoption of this COS into clinical and academic practice will be endorsed by the American, British and European Hernia Societies. Its utilisation in future clinical research will enable appropriate data synthesis and comparison and will enable better clinical interpretation and application of the current evidence base. This study has been registered with the Core Outcome Measures in Effectiveness Trials initiative. PROSPERO REGISTRATION NUMBER: CRD42018090084.

Allotransplantation of donor rectus fascia for abdominal wall closure in transplant patients: A systematic review.

BACKGROUND: Abdominal wall closure after intestinal, multivisceral or liver transplantation can be a major challenge. Different surgical techniques have been described to close complex abdominal wall defects, but results remain variable. Two promising transplant techniques have been developed using either non-vascularized or vascularized donor rectus fascia. This systematic review aimed to evaluate the feasibility, safety, and effectiveness of the two techniques. METHODS: A systematic review was performed in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Human studies published between January 2000 and April 2020 were included. Methodological quality appraisal was done using an adapted 10-item standardized checklist. RESULTS: The search resulted in 9 articles including 74 patients. Both techniques proved to be feasible and had similar results. After non-vascularized rectus fascia allotransplantation, there was a slightly higher rate of surgical site infections in the earlier reports. Overall, there were few complications, no fascial graft related rejections or deaths. The included articles scored low on quality appraisal, mostly due to the small number of cases and scarcely reported outcome parameters. CONCLUSIONS: This systematic literature review reports two emerging new techniques for complex abdominal wall closure in transplant patients, with promising results. Standardized data collection in a prospective manner could give us more detailed information about short- and long-term outcomes. Preclinical animal studies are necessary for a thorough investigation of the mechanisms of graft integration, the risk of hernia development and the alloimmune response against the graft.

Observational Cohort Study on the Use of a Slowly Fully Resorbable Synthetic Mesh (Phasix™) in the Treatment of Complex Abdominal Wall Pathology with Different Grades of Contamination.

PURPOSE: Abdominal wall hernia repair in contaminated cases remains a therapeutic challenge due to the high risk of post-operative surgical site occurrences (SSO). Slowly resorbable synthetic (biosynthetic) meshes have recently been introduced and may offer unique advantages when challenged with bacterial colonization during abdominal wall reconstruction. METHODS: A multicenter single-arm retrospective observational cohort study examined all consecutive patients in whom a poly-4-hydroxybutyrate mesh (Phasix™ or Phasix™ ST; Becton Dickinson, Franklin Lakes, NJ, USA) was used for the repair of an inguinal or ventral/incisional hernia, or to replace an infected synthetic mesh. Patient records were analyzed according to the level of contamination, using the classification score of the Centers for Disease Control and Prevention (CDC). The primary objective was to evaluate short-term postoperative morbidity by assessing SSOs and the need for reoperation or even mesh excision. RESULTS: A total of 47 patients were included. The median age was 68 years (30-87), the male/female ratio was 26/21, and the median BMI was 26.5 kg/m2 (16.4-46.8). There were 17 clean cases, 17 clean-contaminated, 6 contaminated and 7 dirty. Median follow-up time was 48 days. An SSO was seen in 4 clean (23.5%), 7 clean-contaminated (41.2%), 2 contaminated (33.3%) and 5 dirty cases (71.4%). A surgical site occurrence requiring procedural intervention (SSOPI) was seen in 2 clean (11.8%), 3 clean-contaminated (17.6%), 1 contaminated (16.7%) and 2 dirty cases (28.6%). Hernia recurrence was seen in 1 clean (5.9%), 2 clean-contaminated (11.8%) and 3 dirty cases (42.9%). Mesh excision had to be performed in only one case in the contaminated group. CONCLUSION: The Phasix™ mesh shows promising short-term results when used in contaminated hernia-related surgery. Even in contaminated or dirty conditions, with or without infection of the mesh, resection of the mesh only had to be performed once and patients could be managed either conservatively or by relatively minor reoperations. However, further research is needed to fully evaluate the safety and efficacy of these meshes.

Treatment of rectus diastasis: should the midline always be reinforced with mesh? A systematic review.

BACKGROUND: Two main trends are described for the treatment of diastasis recti: plication versus midline mesh reinforcement. Indications for these procedures have not been clearly described. This study reviewed the outcomes in the treatment of rectus diastasis with plication versus mesh by the assessment of durability, complications, and patient-reported outcomes. MATERIALS AND METHODS: A systematic review of literature on the treatment of diastasis recti was performed searching through PubMed, Embase, Web of Science, and Cochrane databases. This resulted in 53 eligible articles and predefined inclusion criteria led to the selection of 24 articles. Primary outcomes included recurrence and perioperative complications and secondary outcomes were defined as patient satisfaction, chronic pain, and quality of life. RESULTS: A total of 931 patients were surgically treated for rectus divarication (age range: 18 - 70 years). The most frequently noted comorbidity was obesity and 10.6 percent were smokers. Recurrence was reported in 5 percent of the patients. The most frequent complication was seroma (7 percent), followed by abdominal hypoesthesia (6 percent), and surgical site infection (2 percent). Chronic pain was reported in 4 percent of the patients. Satisfaction was assessed subjectively in the majority of patients and was generally rated as high. Follow-up period ranged from 3 weeks to 20 years. CONCLUSIONS: Durability, safety, and high patient satisfaction support surgical correction of rectus diastasis and could not favor a treatment method. Inter-rectus distance could not be identified as the indicator for technique, which emphasizes that other factors might add to the entity of abdominal wall protrusion more than previously thought.

Synthetic Versus Biological Mesh in Laparoscopic and Open Ventral Hernia Repair (LAPSIS): Results of a Multinational, Randomized, Controlled, and Double-blind Trial.

OBJECTIVE: The aim of this study was to investigate the approach (open or laparoscopic) and mesh type (synthetic or biological) in ventral hernias in a clean setting.Summary of Background Data: The level of evidence on the optimal surgical approach and type of mesh in ventral hernia repair is still low. METHODS: Patients with a ventral abdominal hernia (diameter 4-10 cm) were included in this double-blind randomized controlled trial across 17 hospitals in 10 European countries. According to a 2 × 2-factorial design, patients were allocated to 4 arms (open retromuscular or laparoscopic intraperitoneal, with synthetic or Surgisis Gold biological mesh). Patients and outcome assessors were blinded to mesh type used. Major postoperative complication rate (hernia recurrence, mesh infection, or reoperation) within 3 years after surgery, was the primary endpoint in the intention-to-treat population. RESULTS: Between September 1st, 2005, and August 7th, 2009, 253 patients were randomized and 13 excluded. Six of 61 patients (9.8%) in the open synthetic mesh arm, 15 of 66 patients (22.7%) in the open biological mesh arm, 7 of 64 patients (10.9%) in the laparoscopic synthetic mesh arm and 17 of 62 patients (27.4%) in the laparoscopic biological mesh arm had a major complication. The use of biological mesh resulted in significantly more complications (P = 0.013), also after adjusting for hernia type, body mass index, and study site. The trial was prematurely stopped due to an unacceptable high recurrence rate in the biological mesh arms. CONCLUSIONS: The use of Surgisis Gold biological mesh is not recommended for noncomplex ventral hernia repair. TRIAL REGISTRATION: This trial was registered at controlled-trials.com (ISRCTN34532248).