Claire Josey, George Mazariegos, Elizabeth King, Pamela Holzer, Jeffrey Rudolph, Vikram Kalathur Raghu
{"title":"Real-world efficacy of long-term teduglutide use in pediatric patients with short bowel syndrome.","authors":"Claire Josey, George Mazariegos, Elizabeth King, Pamela Holzer, Jeffrey Rudolph, Vikram Kalathur Raghu","doi":"10.1016/j.intf.2025.100312","DOIUrl":"10.1016/j.intf.2025.100312","url":null,"abstract":"<p><strong>Background: </strong>In patients with short bowel syndrome (SBS), teduglutide reduces dependency on parenteral support (PS) by promoting intestinal growth and absorption. We aim to describe long-term outcomes using teduglutide in a large pediatric intestinal rehabilitation and transplant center.</p><p><strong>Material and methods: </strong>We performed a single-center, retrospective analysis of teduglutide use in patients with SBS ages 1-23 years. Sex, age, intestinal length, BMI, PS regimens, and isolated small bowel (ISB) transplant status are described. Subgroup analysis comparing younger and older patient cohorts was performed. Primary end point was a reduction in PS volume of ≥ 20 %.</p><p><strong>Results: </strong>27 patients (10 female; mean age 8.9 years) received subcutaneous teduglutide (0.05 mg/kg/d) once daily for mean 120.8 weeks. Mean bowel length was 56.9 (SD=52.1) cm. 5 were listed for ISB transplant, 2 were \"inactive,\" and 20 were not listed. 2 patients were post-enterectomy. A decrease in PS volume of ≥ 20 % was experienced by 67 % of patients, with 30 % reaching enteral autonomy at mean 71.57 (SD=56.37) weeks (Fig. 1). Significant decreases in PS volume, hours and days, nonprotein calories, parenteral nutrition dependency index (PNDI), and lipid dose were observed (Table 2). Of patients listed at baseline, 2 remained listed, 2 were removed, and 1 was reclassified as \"inactive.\" 1 was newly listed and 2 \"inactive\" patients were removed. 19 remained unlisted.The most frequent adverse event was pyrexia. 10 patients permanently discontinued treatment at mean 62.59 (SD=55.55) weeks, 4 due to related AEs.</p><p><strong>Conclusion: </strong>Pediatric SBS patients experienced significant decreases in PS dependency, providing real-world evidence of teduglutide efficacy.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"7 ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12805952/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146000405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Economic impact of intestinal failure from a patient perspective - A pilot study.","authors":"Maddison Breen, Quiney Lin, Liz Beyer, Michelle Cunich, Sharon Carey","doi":"10.1016/j.intf.2025.100070","DOIUrl":"10.1016/j.intf.2025.100070","url":null,"abstract":"<p><strong>Background: </strong>Intestinal Failure (IF) is a complex and life-long illness, requiring home parenteral and/or enteral nutrition, multiple medications, dietary changes and input from multiple healthcare specialists. The aim of this pilot study is to quantify the economic impact of IF from the patient perspective.</p><p><strong>Materials and methods: </strong>A tailored questionnaire was developed, piloted and administered to adult IF patients within a quaternary hospital in Sydney, Australia. The questionnaire collected health-related out-of-pocket expenses (OOPE) over the past 12-months. Data was analysed using descriptive statistics (median and range).</p><p><strong>Results: </strong>A total of 13 participants responded to the questionnaire. OOPE in a 12-month period was AUD $6099 (AUD $3170 - $45,744); this was 24.4 % of annual reported income. OOPE for participants in metropolitan regions were higher than those living in regional or rural regions (AUD $6538 versus AUD $6099, respectively). Participants with a motility disorder spent more on total OOPE than those without a motility disorder (AUD $7178 versus AUD $3905); with the majority of people living in metropolitan regions. OOPE for medications was the highest individual cost per year, AUD $2160 (AUD $820 - $6000). Average potential lost income was AUD $62,310 per annum.</p><p><strong>Conclusion: </strong>This pilot study shows the significant economic burden for people living with IF, including loss of income. Results warrant expansion to assess burden over a larger patient group. IF services need to be aware of the financial burden experienced by this patient group and ensure they are supported to access financial aid where eligible.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"6 ","pages":"100070"},"PeriodicalIF":0.0,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851378/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146129143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Colonic dilation is associated with increased central line associated blood stream infection for patients with intestinal failure.","authors":"Pamela Emengo, Shweta S Namjoshi, James C Y Dunn","doi":"10.1016/j.intf.2025.100064","DOIUrl":"10.1016/j.intf.2025.100064","url":null,"abstract":"<p><strong>Introduction: </strong>Management of intestinal failure involves supplemental nutrition and complication minimization to achieve enteral autonomy. Previous research to identify prognostic factors in IF focused on the small bowel, yet little work has been done to associate the colon with IF outcomes. We hypothesize that like small bowel dilation, colonic dilation is associated with intestinal failure morbidity. We also investigate whether a standardized colonic diameter ratio differs from the maximal colonic width in predicting outcomes in children with intestinal failure.</p><p><strong>Material and methods: </strong>We conducted a retrospective cohort study of all children with gastrointestinal contrast imaging and intestinal failure in our intestinal rehabilitation program between 2013 and 2023. Medical records were reviewed for patient information and imaging review. A colonic diameter ratio was calculated by dividing the maximal colonic diameter by the height of the fifth lumbar vertebra.</p><p><strong>Results: </strong>Thirty-two patients were assessed based on colonic width and colonic diameter ratio. The median age of patients was 7.0 years (IQR 3 - 11 years). Maximum colonic width correlated positively with age, catheter-associated bloodstream infection, and parenteral nutrition duration. Colonic diameter ratio, on the other hand, did not show a significant correlation with age or parenteral nutrition duration, but had significant positive correlations with catheter-associated bloodstream infection.</p><p><strong>Conclusion: </strong>Maximum colonic width was significantly associated with increased morbidity in patients with intestinal failure. Increased colonic dilation after normalization by the size of the patient is associated with increased central line associated bloodstream infections.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100064"},"PeriodicalIF":0.0,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851260/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146129132","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stephanie So, Catherine Patterson, Taru Palsa, Jemma Mears, Bianca C Bondi, Sydney Dempster, Christina Belza
{"title":"Neurodevelopmental, cognitive and motor function: Practice recommendations in pediatric intestinal failure and transplantation- position statement of the International Intestinal Rehabilitation and Transplant Association allied health committee.","authors":"Stephanie So, Catherine Patterson, Taru Palsa, Jemma Mears, Bianca C Bondi, Sydney Dempster, Christina Belza","doi":"10.1016/j.intf.2025.100062","DOIUrl":"10.1016/j.intf.2025.100062","url":null,"abstract":"<p><strong>Background: </strong>The objective of this position statement was to provide evidence-based and expert-informed recommendations for the assessment and treatment of neurodevelopment in children with intestinal failure and those undergoing intestinal transplant.</p><p><strong>Material and methods: </strong>A literature review was conducted to identify and review research articles from 2000 to 2023 that reported on neurodevelopmental, neurocognitive and motor outcomes in children (0-18 years) with intestinal failure or intestinal transplant. Key recommendations to address these outcomes were made based on scientific evidence and expert-informed opinion.</p><p><strong>Results: </strong>The impact of intestinal failure and transplant on developmental outcomes was established, along with key risk factors related to the disease that may result in poorer outcomes. The involvement of rehabilitation specialists was recommended as part of the multidisciplinary intestinal care team to provide assessment and treatment across all developmental domains from infancy onwards. Care teams should provide close follow-up which may include screening during out-patient clinic follow up, along with timely community referrals to address developmental delays. Children undergoing intestinal or multi-visceral transplant require assessment and treatment both pre and post-transplant to maximize developmental outcomes.</p><p><strong>Conclusions: </strong>This position paper reviews available literature related to developmental outcomes in intestinal failure and transplant, delineating risk factors which may contribute to poorer outcomes. It lays the foundation for tangible steps to assist intestinal care teams in advocating for the services of rehabilitation team members to provide ongoing assessment and treatment to optimize clinical outcomes.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100062"},"PeriodicalIF":0.0,"publicationDate":"2025-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851274/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146128750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J Azpiroz, J C Abate, A M Andres Moreno, J E Moreira, F Hernandez Oliveros, J C Raimondi, G Gondolesi, N R Lausada, P Stringa
{"title":"Standardizing training protocol of an intestinal transplantation model: A learning curve study.","authors":"J Azpiroz, J C Abate, A M Andres Moreno, J E Moreira, F Hernandez Oliveros, J C Raimondi, G Gondolesi, N R Lausada, P Stringa","doi":"10.1016/j.intf.2025.100060","DOIUrl":"10.1016/j.intf.2025.100060","url":null,"abstract":"<p><p>Rodent models of intestinal transplantation are crucial for advancing our understanding of various biological processes related to the procedure, including ischemia-reperfusion injury, acute cellular rejection and immunological tolerance, among others. These models also provide a platform for developing strategies and therapeutics aimed at improving long-term survival, with the ultimate goal of translating laboratory findings into clinical applications. With over twenty years of experience in intestinal transplantation in rodents, our team currently achieves a survival rate of 83 % in orthotopic transplants and 85 % in heterotopic transplants-success rates comparable to those reported by leading research groups in the field. This manuscript outlines the key steps involved in achieving successful results in rat intestinal transplantation models. It covers the entire process, from basic microsurgical training and graft procurement to implantation and postoperative care of the recipient. We hope this work will serve as a valuable guide for research groups seeking to establish these experimental transplantation models in their own laboratories.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100060"},"PeriodicalIF":0.0,"publicationDate":"2025-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851314/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146128985","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Karen Allsopp, Simon Lal, Anne Marie Sowerbutts, Debra Jones, Michael Taylor, Simon Harrison, Arun Abraham, Kirstine Farrer, Gavin Leahy, Antje Teubner, Sorrel Burden
{"title":"Evaluation of combined multi-chamber with compounded bags and multi-chamber bags only for British adults on home parenteral nutrition: A mixed methods pilot study.","authors":"Karen Allsopp, Simon Lal, Anne Marie Sowerbutts, Debra Jones, Michael Taylor, Simon Harrison, Arun Abraham, Kirstine Farrer, Gavin Leahy, Antje Teubner, Sorrel Burden","doi":"10.1016/j.intf.2025.100063","DOIUrl":"10.1016/j.intf.2025.100063","url":null,"abstract":"<p><strong>Background: </strong>People with chronic intestinal failure (IF) require home parenteral nutrition (HPN). Parenteral nutrition regimens can comprise of bespoke prescriptions compounded specifically for the individual, commercially produced multi-chamber bags (MCBs), or a combination of compounded and MCBs known as 'hybrid' regimens. This pilot study aimed to evaluate quality of life (QoL) and clinical outcomes with hybrid or MCBs-only parenteral nutrition regimens.</p><p><strong>Methods: </strong>This mixed-methods pilot study collected data from patients between June 2022 and January 2023. Data were gathered at baseline and 180 days post-discharge. Patients were interviewed about their lived experiences and completed a QoL questionnaire. Quantitative data were analysed using descriptive statistics while qualitative data analysis used framework analysis.</p><p><strong>Results: </strong>We recruited 28 patients, 16 on hybrid HPN regimens and 12 on MCBs. After 180 days, no catheter-related bloodstream infections were recorded. No differences were found between hybrid and MCBs-only regarding QoL. Patients on hybrid regimens reported no issues arising specifically from using two bag types, and some of those on MCBs-only appreciated extra flexibility in their lives.</p><p><strong>Conclusions: </strong>Both MCB and hybrid HPN regimens were acceptable to patients, with neither showing a substantial difference in QoL.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100063"},"PeriodicalIF":0.0,"publicationDate":"2025-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851116/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146128626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J M Ladowski, Mariya L Samoylova, Jeffrey Ord, Annette M Jackson, Debra L Sudan
{"title":"Small intestine transplant immunologic risk assessment: More data is needed.","authors":"J M Ladowski, Mariya L Samoylova, Jeffrey Ord, Annette M Jackson, Debra L Sudan","doi":"10.1016/j.intf.2025.100061","DOIUrl":"10.1016/j.intf.2025.100061","url":null,"abstract":"<p><strong>Background: </strong>Intestinal transplantation (IT) remains the only treatment modality for patients with irreversible intestinal failure who cannot be maintained on chronic parenteral nutrition. Multiple reports have demonstrated an association between donor-specific antibodies (DSA) in IT graft rejection, however these studies have largely been single-center analyses. The goal of this study was to examine the available immunological data from the International Intestinal Transplant Registry (IITR) and evaluate the association with rejection.</p><p><strong>Materials and methods: </strong>Demographic, outcomes, and serologic HLA data from donor/recipient pairs was obtained from the IITR. Outcomes including rejection, graft survival, and patient survival were analyzed based on age, DSA reporting, and molecular HLA mismatch. Eplet mismatch analysis was performed using HLAMatchmaker.</p><p><strong>Results: </strong>Our analysis revealed significant limitations in the granularity of data contained in the IITR.</p><p><strong>Conclusion: </strong>The IITR database is a promising option to perform a multicenter analysis of IT outcomes, but there are limitations in the data to allow a thorough immunologic examination.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100061"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851323/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146129038","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Conversion from SMOFlipid® to Omegaven® as a salvage therapy for intestinal failure associated liver disease.","authors":"Stella Sabbatini, Fernanda Takamatsu, Christina Belza, Glenda Courtney-Martin, Daniela Gattini, Yaron Avitzur","doi":"10.1016/j.intf.2025.100058","DOIUrl":"10.1016/j.intf.2025.100058","url":null,"abstract":"<p><strong>Background: </strong>Intestinal failure associated liver disease (IFALD) is a common complication of intestinal failure with limited treatment options. While Omegaven® can be used as a salvage therapy for IFALD in patients receiving intralipid®, its efficacy in IFALD associated with SMOFlipid® remains unclear. This study aims to assess Omegaven® as a salvage therapy in this setting.</p><p><strong>Methods: </strong>Retrospective study of children with intestinal failure who developed advanced IFALD (conjugated bilirubin over 50 µmol/L for at least 2 weeks) while receiving SMOFlipid® and converted to Omegaven® (1.0 g/kg/d). Patients were treated with Omegaven® for at least 7 weeks. The primary outcome was conjugated bilirubin (CB) < 34 μmol/l after Omegaven® Initiation<sup>.</sup></p><p><strong>Results: </strong>Out of 219 patients receiving SMOFlipid® between 2013 and 2024, 13 developed advanced IFALD and were converted to Omegaven®. Two patients did not meet inclusion criteria. All 11 remaining patients had a high parenteral nutrition dependency index (PNDI) at baseline. Following conversion to Omegaven®, 7 (63.6 %) patients achieved CB bellow 34 µmol/L, in a mean time of 12.5 weeks (SD 6.63). Non-responders had a higher incidence of gastrointestinal bleeding secondary to portal hypertension (75 % vs 0 %, p = 0.02), INR (1.8 vs 1.1, p = 0.03), and need for continuous glucose infusion (75 % vs 0 %, p = 0.02). Out of the 4 non-responders, one patient died of liver failure, 2 underwent multivisceral transplantation, and one remains listed for liver-intestine transplantation.</p><p><strong>Conclusions: </strong>Conversion to Omegaven® from SMOFlipid® reversed advanced IFALD in 63.6 % of patients. Response is less likely in children with synthetic liver dysfunction and clinically significant portal hypertension.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100058"},"PeriodicalIF":0.0,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851354/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146129068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M Ortega, A Crivelli, P Klin, G Gondolesi, H Solar
{"title":"Nutritional diagnosis in adult patients with Chronic Intestinal Failure: Glim criteria or Subjective Global Assessment.","authors":"M Ortega, A Crivelli, P Klin, G Gondolesi, H Solar","doi":"10.1016/j.intf.2025.100059","DOIUrl":"10.1016/j.intf.2025.100059","url":null,"abstract":"<p><strong>Background: </strong>Patients with Chronic Intestinal Failure (CIF) present increased risk for malnourishment. Nutritional assessment is commonly done through the Subjective Global Assessment (SGA). The Global Leadership Initiative on Malnutrition (GLIM) developed phenotypic and etiologic criteria for assessing and grading malnutrition. The aims of this study were to determine malnutrition in CIF patients comparing the GLIM criteria and SGA and determine the severity of malnutrition according to the GLIM phenotypic criteria.</p><p><strong>Material and methods: </strong>From June 2019 through December 2021 a retrospective analysis was conducted. All patients had a diagnosis of CIF. Malnutrition was evaluated using the SGA and the GLIM criteria, while the degree of malnutrition according to the phenotypic criteria. Observational, bivariate, multiple logistic regression and ROC analyses were performed.</p><p><strong>Results: </strong>Seventy patients with CIF were included. The mean age was 47.8 years, 51.4 % being female. The SGA showed 27 % of the patients were moderately malnourished and 33 % were severely malnourished. According to the GLIM criteria, 86 % of the patients presented at least one phenotypic criterion of malnutrition. Regarding the etiologic criteria all the patients had reduced food intake and/or impaired absorption or food assimilation. When grading malnutrition status 53 % presented moderate malnutrition and 33 %, severe malnutrition. The ROC analyses showed an area under the curve (AUC) of 0.92 for the SGA and 0.86 for the GLIM criteria (<i>p</i> = 0.98).</p><p><strong>Conclusion: </strong>In this study, a higher percentage of patients with malnutrition was diagnosed according to the GLIM criteria, although, when comparing ROC analyses, no statistical difference was found.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100059"},"PeriodicalIF":0.0,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851352/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146128829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Farhana Ali-Geiger, Michael Traynor, Lauren Baumann, Brian Bucher
{"title":"Case of neonate with total intestinal hirschsprung disease managed with a proximal jejunostomy.","authors":"Farhana Ali-Geiger, Michael Traynor, Lauren Baumann, Brian Bucher","doi":"10.1016/j.intf.2025.100052","DOIUrl":"10.1016/j.intf.2025.100052","url":null,"abstract":"<p><strong>Background: </strong>Hirschsprung disease (HD) is a congenital disorder of the enteric nervous system, and its management will differ clinically depending on the degree of aganglionosis. Total intestinal HD (TIHD) is a rare variant, accounting for < 1 % of all cases, and is defined as aganglionosis of nearly the entire intestine with less than 20 cm of ganglionated small bowel past the ligament of Treitz (LOT). TIHD can be challenging to manage with high risk for chronic intestinal failure.</p><p><strong>Case report: </strong>Ours is a neonate confirmed pathologically to have total intestinal aganglionosis, surgically managed with a jejunostomy formed at 40 cm distal to LOT.</p><p><strong>Conclusion: </strong>Case of TIHD that has benefitted from a proximal diverting jejunostomy.</p>","PeriodicalId":520517,"journal":{"name":"Intestinal Failure (New York, N.Y.)","volume":"5 ","pages":"100052"},"PeriodicalIF":0.0,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12851266/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146129158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}