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Mammoo S, Alshahwani N, Mancha MA, Baghazal H, Ali M, Brisseau G. Intestinal autonomy in extreme premature infants with short bowel syndrome: national experience. Pediatr Surg Int 2025; 41:143. [PMID: 40411554 PMCID: PMC12103355 DOI: 10.1007/s00383-025-06036-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/03/2025] [Indexed: 05/26/2025]
Abstract
PURPOSE To investigate the outcomes of premature neonates with short bowel syndrome (SBS) managed under a BEAR (Bowel Elongation and Advanced Rehabilitation) protocol. METHODS This was a retrospective cohort study of preterm patients with SBS, treated at Sidra Medicine between January 2018 and February 2024. Data were extracted from electronic medical records, including patient demographics, clinical history, surgical interventions, parenteral nutrition (PN) duration, and long-term outcomes. The BEAR protocol incorporated a multidisciplinary approach with structured intestinal rehabilitation, hepato-protective PN strategies, and staged surgical interventions to promote enteral autonomy. RESULTS A total of 20 premature neonates with SBS were analyzed, with a median gestational age of 28 weeks and a median birth weight of 860g. Necrotizing enterocolitis was the primary cause of SBS in 90% of cases. Of the cohort, 80% successfully weaned off PN, achieving enteral autonomy at a median corrected age of 19.7 months. Seven patients underwent serial transverse enteroplasty (STEP), with 6/7 successfully transitioning to full enteral feeding. The study demonstrated favorable survival rates and reduced PN-associated complications. CONCLUSION The BEAR protocol provides a structured and effective approach to intestinal rehabilitation in premature neonates with SBS, facilitating early enteral autonomy and minimizing long-term PN dependence. These findings contribute valuable clinical insights into optimizing multidisciplinary management strategies for this high-risk population.
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Affiliation(s)
- Saleem Mammoo
- General and Thoracic Surgery Department, Sidra Medicine Doha, Doha, Qatar
| | - Noora Alshahwani
- General and Thoracic Surgery Department, Sidra Medicine Doha, Doha, Qatar.
| | | | - Hassan Baghazal
- General and Thoracic Surgery Department, Sidra Medicine Doha, Doha, Qatar
| | - Mansour Ali
- General and Thoracic Surgery Department, Sidra Medicine Doha, Doha, Qatar
| | - Guy Brisseau
- General and Thoracic Surgery Department, Sidra Medicine Doha, Doha, Qatar
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Lenti MV, Hammer HF, Tacheci I, Burgos R, Schneider S, Foteini A, Derovs A, Keller J, Broekaert I, Arvanitakis M, Dumitrascu DL, Segarra-Cantón O, Krznarić Ž, Pokrotnieks J, Nunes G, Hammer J, Pironi L, Sonyi M, Sabo CM, Mendive J, Nicolau A, Dolinsek J, Kyselova D, Laterza L, Gasbarrini A, Surdea-Blaga T, Fonseca J, Lionis C, Corazza GR, Di Sabatino A. European Consensus on Malabsorption-UEG & SIGE, LGA, SPG, SRGH, CGS, ESPCG, EAGEN, ESPEN, and ESPGHAN: Part 2: Screening, Special Populations, Nutritional Goals, Supportive Care, Primary Care Perspective. United European Gastroenterol J 2025. [PMID: 40088199 DOI: 10.1002/ueg2.70011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/27/2024] [Revised: 02/14/2025] [Accepted: 02/18/2025] [Indexed: 03/17/2025] Open
Abstract
Malabsorption is a complex and multifaceted condition characterised by the defective passage of nutrients into the blood and lymphatic streams. Several congenital or acquired disorders may cause either selective or global malabsorption in both children and adults, such as cystic fibrosis, exocrine pancreatic insufficiency (EPI), coeliac disease (CD) and other enteropathies, lactase deficiency, small intestinal bacterial overgrowth (SIBO), autoimmune atrophic gastritis, Crohn's disease, and gastric or small bowel resections. Early recognition of malabsorption is key for tailoring a proper diagnostic work-up for identifying the cause of malabsorption. Patient's medical and pharmacological history are essential for identifying risk factors. Several examinations like endoscopy with small intestinal biopsies, non-invasive functional tests, and radiologic imaging are useful in diagnosing malabsorption. Due to its high prevalence, CD should always be looked for in case of malabsorption with no other obvious explanations and in high-risk individuals. Nutritional support is key in management of patients with malabsorption; different options are available, including oral supplements, enteral or parenteral nutrition. In patients with short bowel syndrome, teduglutide proved effective in reducing the need for parenteral nutrition, thus improving the quality of life of these patients. Primary care physicians have a central role in early detection of malabsorption and should be involved into multidisciplinary teams for improving the overall management of these patients. In this European consensus, involving 10 scientific societies and several experts, we have dissected all the issues around malabsorption, including the definitions and diagnostic testing (Part 1), high-risk categories and special populations, nutritional assessment and management, and primary care perspective (Part 2).
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Affiliation(s)
- Marco Vincenzo Lenti
- Department of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy
- First Department of Internal Medicine, Fondazione IRCCS San Matteo, Pavia, Italy
| | - Heinz Florian Hammer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University, Graz, Austria
| | - Ilja Tacheci
- 2nd Department of Internal Medicine - Gastroenterology, University Hospital Hradec Králové, Charles University, Faculty of Medicine in Hradec Králové, Hradec Kralove, Czech Republic
| | - Rosa Burgos
- Endocrinology and Nutrition Department, Hospital Universitari Vall d'Hebron, Diabetes and Metabolism Research Unit, Vall d'Hebron Institut de Recerca (VHIR), Universitat Autonoma de Barcelona, Barcelona, Spain
| | - Stephane Schneider
- Gastroenterology and Nutrition, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur, Nice, France
| | - Anastasiou Foteini
- 4th Local Primary Care Team, Municipality Practice and Academic Practice of Heraklion, University of Crete, Crete, Greece
| | - Aleksejs Derovs
- Department of Internal Diseases, Rīga Stradiņš University, Riga, Latvia
| | - Jutta Keller
- Israelitic Hospital, Academic Hospital University of Hamburg, Hamburg, Germany
| | - Ilse Broekaert
- Department of Paediatrics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
| | - Marianna Arvanitakis
- Department of Gastroenterology, Digestive Oncology and Hepatopancreatology, HUB Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium
| | - Dan Lucian Dumitrascu
- 2nd Department of Internal Medicine, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
- 2nd Medical Department, Emergency Clinical County Hospital, Cluj-Napoca, Romania
| | - Oscar Segarra-Cantón
- Paediatric Gastroenterology and Clinical Nutrition Unit, Vall d'Hebron Hospital Universitari, Vall d'Hebron Barcelona Hospital Campus, Barcelona, Spain
| | - Željko Krznarić
- Department of Gastroenterology, Hepatology and Nutrition, University of Zagreb, Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | - Juris Pokrotnieks
- Department of Internal Diseases, Rīga Stradiņš University, Riga, Latvia
- Centre of Gastroenterology, Hepatology and Nutrition, Pauls Stradiņš Clinical University Hospital, Riga, Latvia
| | - Gonçalo Nunes
- Gastroenterology Department, Hospital Garcia de Orta, Almada, Portugal
- Egas Moniz Center for Interdisciplinary Research (CiiEM), Egas Moniz School of Health & Science, Almada, Portugal
| | - Johann Hammer
- Department of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria
| | - Loris Pironi
- Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
- Centre for Chronic Intestinal Failure, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - Marc Sonyi
- Clinic for General Medicine, Gastroenterology, and Infectious Diseases, Augustinerinnen Hospital, Cologne, Germany
| | - Cristina Maria Sabo
- 2nd Department of Internal Medicine, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
- 2nd Medical Department, Emergency Clinical County Hospital, Cluj-Napoca, Romania
| | - Juan Mendive
- La Mina Primary Health Care Academic Centre, Catalan Health Institute, University of Barcelona, Barcelona, Spain
| | - Adrien Nicolau
- Gastroenterology and Nutrition, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur, Nice, France
| | - Jernej Dolinsek
- Pediatric Gastroenterology, Hepatology and Nutrition Unit, Pediatric Department, University Medical Center Maribor, Maribor, Slovenia
- Faculty of Medicine, University of Maribor, Maribor, Slovenia
| | - Denisa Kyselova
- Department of Hepatogastroenterology, IKEM, Prague, Czech Republic
| | - Lucrezia Laterza
- Department of Translational Medicine and Surgery, Università Cattolica del Sacro Cuore, Rome, Italy
- CEMAD, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
| | - Antonio Gasbarrini
- Department of Translational Medicine and Surgery, Università Cattolica del Sacro Cuore, Rome, Italy
- CEMAD, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
| | - Teodora Surdea-Blaga
- 2nd Department of Internal Medicine, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
- 2nd Medical Department, Emergency Clinical County Hospital, Cluj-Napoca, Romania
| | - Jorge Fonseca
- Gastroenterology Department, Hospital Garcia de Orta, Almada, Portugal
- Egas Moniz Center for Interdisciplinary Research (CiiEM), Egas Moniz School of Health & Science, Almada, Portugal
| | - Christos Lionis
- Laboratory of Health and Society, School of Medicine, University of Crete, Heraklion, Greece
| | - Gino Roberto Corazza
- Department of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy
- First Department of Internal Medicine, Fondazione IRCCS San Matteo, Pavia, Italy
| | - Antonio Di Sabatino
- Department of Internal Medicine and Medical Therapeutics, University of Pavia, Pavia, Italy
- First Department of Internal Medicine, Fondazione IRCCS San Matteo, Pavia, Italy
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Dagorno C, Montalva L, Capito C, Lavrand F, Guinot A, De Napoli Cocci S, Gelas T, Dubois R, Dariel A, Dugelay E, Chardot C, Bonnard A. Serial Transverse Enteroplasty (STEP) for Short Bowel Syndrome (SBS) in Children: A Multicenter Study on Long-term Outcomes. J Pediatr Surg 2025; 60:161909. [PMID: 39368852 DOI: 10.1016/j.jpedsurg.2024.161909] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2024] [Revised: 08/31/2024] [Accepted: 09/04/2024] [Indexed: 10/07/2024]
Abstract
OBJECTIVES Short Bowel Syndrome (SBS), secondary to various underlying diseases, is one of the main causes of intestinal failure in children. Surgical management by serial transverse enteroplasty (STEP), is feasible in selected cases, but results on long-term follow-up are scarce. The aim of this study was to report long-term outcomes of the STEP procedure in children with SBS. METHODS We performed a multicenter national retrospective study reviewing medical charts of children who underwent a STEP technique between 2000 and 2022 in 6 university hospitals. Collected data included demographics, SBS history, surgical procedures, nutritional support (enteral or parenteral), STEP management, complications, and outcomes (nutritional support, digestive symptoms, growth). RESULTS STEP was performed in 36 SBS, resulting from 14 gastroschisis, 10 intestinal atresia, 8 necrotizing enterocolitis (NEC), 2 midgut volvulus, and 2 intestinal Hirschsprung disease. Median age at first STEP was 10.8 months [4.5; 63.8]. Bowel length significantly increased (47 vs 70 cm, p < 0.01), with a median gain of 16 cm [11; 25] or 34%. At surgery, 33 children had parenteral nutrition (PN). A second STEP was performed in 11 patients, at a median age of 4.2 years [2.8; 6.8]. One patient underwent a third STEP. Two children required intestinal transplantation. Median follow-up was 7 years [4.4;11.4]. Out of 33 children requiring PN before surgery, 14 children were weaned off PN. The remaining 19 children still required PN, but their dependency decreased by 19%. Out of 17 children presenting severe intestinal dilations and bacterial overgrowth, 12 became asymptomatic. Out of 20 with chronic obstructive symptoms, 8 patients still required supplementary PN whereas 12 achieved complete enteral autonomy, including 3 requiring supplementary enteral support. CONCLUSION STEP technique remains a surgical option in the management of these children, enabling a decrease in PN dependency, resulting in weaning off PN in some cases, as well as an improvement of clinical symptoms. TYPE OF STUDY Retrospective multicenter observational study. LEVEL OF EVIDENCE Level 3.
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Affiliation(s)
- Claire Dagorno
- Department of Pediatric Surgery, Robert-Debré Children's University Hospital, APHP, Paris, France; Department of General Pediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris, France
| | - Louise Montalva
- Department of Pediatric Surgery, Robert-Debré Children's University Hospital, APHP, Paris, France; Paris-Cité University, Paris, France; NeuroDiderot, INSERM UMR1141, Paris, France
| | - Carmen Capito
- Department of General Pediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris, France; Paris-Cité University, Paris, France
| | - Frederic Lavrand
- Department of General Pediatric Surgery, Children Hospital, CHU de Bordeaux, France
| | - Audrey Guinot
- Department of General Pediatric Surgery, Children and Mother Hospital, CHU de Nantes, France
| | - Stéphan De Napoli Cocci
- Department of General Pediatric Surgery, Children and Mother Hospital, CHU de Nantes, France
| | - Thomas Gelas
- Department of General Pediatric Surgery, Hospices Civils de Lyon, Children and Mother Hospital, CHU de Lyon, France
| | - Rémi Dubois
- Department of General Pediatric Surgery, Hospices Civils de Lyon, Children and Mother Hospital, CHU de Lyon, France
| | - Anne Dariel
- Department of General Pediatric Surgery, Timone Hospital, CHU de Marseille, France
| | - Emmanuelle Dugelay
- Department of Pediatric Gastro-enterology, Robert-Debré Children's University Hospital, APHP, Paris, France
| | - Christophe Chardot
- Department of General Pediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris, France; Paris-Cité University, Paris, France
| | - Arnaud Bonnard
- Department of Pediatric Surgery, Robert-Debré Children's University Hospital, APHP, Paris, France; Paris-Cité University, Paris, France; NeuroDiderot, INSERM UMR1141, Paris, France.
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Mlaver E, Smith SR, Matar AJ, Zhao VM, Leong R, Sharma J, Srinivasan JK, Galloway JR. Serial transverse enteroplasty in adults with parenteral nutrition dependence: A case series. JPEN J Parenter Enteral Nutr 2024; 48:965-973. [PMID: 39221785 DOI: 10.1002/jpen.2682] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/11/2024] [Revised: 07/07/2024] [Accepted: 08/10/2024] [Indexed: 09/04/2024]
Abstract
BACKGROUND Serial transverse enteroplasty is used to treat patients with chronic intestinal failure owing to short bowel syndrome. Current literature lacks discussion of its role for other etiologies of intestinal failure and its impact on adult patients' nutrition support needs and quality of life. METHODS We performed a case series on adults with parenteral nutrition (PN) dependence who underwent serial transverse enteroplasty at Emory University Hospital, a quaternary referral center between 2011 and 2022. Data collected included demographics, operative technique, and preoperative and postoperative PN requirements. A phone survey was administered to evaluate the impact of PN and the operation on quality of life. RESULTS Ten patients underwent the procedure of interest during the study period. Indications included short bowel syndrome following multiple abdominal operations or intra-abdominal catastrophe and chronic partial bowel obstruction with dysmotility. Bowel length increased by a median of 83%. All patients were discharged home after a median hospital stay of 21 days. At 1-year follow-up, survival was 100%, two (20%) patients fully weaned from PN, three others (30%) reduced PN frequency, and six (60%) decreased their daily parenteral energy requirement. Two additional patients fully weaned from PN by 18 months postoperatively. CONCLUSION This represents one of the largest case series of serial transverse enteroplasty in adults. Small intestinal length nearly doubled, and PN dependence was reduced in most patients. Given the low morbidity and good quality of life observed in this series, this procedure should be more widely investigated for patients with chronic intestinal failure.
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Affiliation(s)
- Eli Mlaver
- Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Savannah R Smith
- Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Abraham J Matar
- Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Vivian M Zhao
- Department of Pharmaceutical Services, Emory University Hospital, Atlanta, Georgia, USA
| | - Rachel Leong
- Department of Pharmaceutical Services, Emory University Hospital, Atlanta, Georgia, USA
| | - Jyotirmay Sharma
- Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Jahnavi K Srinivasan
- Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
| | - John R Galloway
- Department of Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
- Department of Pharmaceutical Services, Emory University Hospital, Atlanta, Georgia, USA
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Frau T, El Khatib M, De Dreuille B, Billiauws L, Nuzzo A, Joly F. Emerging drugs for the treatment of short bowel syndrome. Expert Opin Emerg Drugs 2024; 29:277-288. [PMID: 38761162 DOI: 10.1080/14728214.2024.2357567] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2024] [Accepted: 05/16/2024] [Indexed: 05/20/2024]
Abstract
INTRODUCTION SBS is a rare and disabling condition. The standard management is based on diet optimization with parenteral supplementation. In addition, glucagon-like peptide-2 (GLP-2)analogs, have shown promising results as disease-modifying therapies for SBS. AREAS COVERED Short bowel syndrome (SBS) is defined as a reduction in functional intestinal length to less than 200 cm, leading to intestinal failure (IF) leading to malnutrition and parenteral support dependency. This review discusses the current management of SBS-CIFpatients, the place of GLP-2 analog treatment in terms of efficacy, safety and availability, and the new perspectives opened by the use of enterohormones. EXPERT OPINION Clinical trials and real-world experience demonstrated that Teduglutide reduces dependence on parenteral support and has a place in the management of patients with SBS-CIF. The use of Teduglutide should be discussed in patients stabilized after resection and its introduction requires the advice of an expert center capable of assessing the benefit-risk ratio. The complex, individualized management of SBS-C IF requires theexpertise of a specialized IF center which a multidisciplinary approach. The arrival of new treatments will call for new therapeutic strategies, and the question of how to introduce and monitor them will represent a new therapeutic challenge.
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Affiliation(s)
- Tristan Frau
- Hopital Beaujon - Department of Gastroenterology and Nutrition Support APHP, University Paris VII, Clichy, France
| | - Myriam El Khatib
- Hopital Beaujon - Department of Gastroenterology and Nutrition Support APHP, University Paris VII, Clichy, France
| | - Brune De Dreuille
- Hopital Beaujon - Department of Gastroenterology and Nutrition Support APHP, University Paris VII, Clichy, France
- Gastrointestinal and Metabolic Dysfunctions in Nutritional Pathologies, Inserm UMR 1149, Centre de Recherche sur l'Inflammation Paris Montmartre, UFR de Médecine Paris Diderot Paris, France
| | - Lore Billiauws
- Hopital Beaujon - Department of Gastroenterology and Nutrition Support APHP, University Paris VII, Clichy, France
- Gastrointestinal and Metabolic Dysfunctions in Nutritional Pathologies, Inserm UMR 1149, Centre de Recherche sur l'Inflammation Paris Montmartre, UFR de Médecine Paris Diderot Paris, France
| | - Alexandre Nuzzo
- Hopital Beaujon - Department of Gastroenterology and Nutrition Support APHP, University Paris VII, Clichy, France
- Hôpital Bichat, Laboratory for Vascular Translational Science, Paris, France
| | - Francisca Joly
- Hopital Beaujon - Department of Gastroenterology and Nutrition Support APHP, University Paris VII, Clichy, France
- Gastrointestinal and Metabolic Dysfunctions in Nutritional Pathologies, Inserm UMR 1149, Centre de Recherche sur l'Inflammation Paris Montmartre, UFR de Médecine Paris Diderot Paris, France
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Cunningham AJ, Anderson T, Mueller C, Bruzoni M, Dunn JCY. Ileal lengthening through internal distraction: A novel procedure for ultrashort bowel syndrome. JOURNAL OF PEDIATRIC SURGERY OPEN 2024; 6:100124. [PMID: 39005758 PMCID: PMC11245380 DOI: 10.1016/j.yjpso.2024.100124] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 07/16/2024]
Abstract
Purpose Ultrashort bowel syndrome is a rare, but morbid surgical problem without effective treatment. Recent clinical analysis has demonstrated the critical influence of ileal length on ultimate enteral autonomy. Surgical techniques to increase ileal length in nondilated bowel do not exist. We describe a novel technique to lengthen ileum in children with ultrashort bowel syndrome. Methods Beginning in May 2021 prospective candidate children were identified. Candidacy for ileal tube lengthening included diagnosis of ultrashort bowel syndrome, intact ileocecal valve with remnant ileum, and proximal intestinal stoma or draining gastrostomy. Informed consent was obtained. Following laparoscopic lysis of adhesions, a balloon catheter was inserted through a left flank stab incision and into the lumen of the remnant ileum around a purse string suture. Cecopexy was performed in the right-lower quadrant. Clips were used to mark the cecum and the proximal extent of ileum. The catheter length was fixed externally at the completion of the procedure. Serial x-rays were used to measure distraction effect while increasing tension was applied to the catheter over the subsequent weeks. Ileal tube lengthening was performed until the end of the catheter was reached or the tube was dislodged. A contrast study was performed at the completion of lengthening. Intestinal length at time of restoration of continuity and clinical outcomes were recorded. Results Four infants were enrolled from May 2021-July 2023. Diagnoses leading to ultrashort bowel syndrome were mesenteric teratoma, necrotizing enterocolitis, and multiple intestinal atresia. At the time of restoration of intestinal continuity, a median of 1.75 cm (45 %) additional ileal length was achieved at a median of 25.5 days. There were no serious complications following ileal tube lengthening and no additional operative interventions were required. Conclusions Ileal lengthening through internal distraction is a feasible surgical intervention to salvage ileum for infants with ultrashort bowel syndrome. Ileal tube lengthening may result in distraction enterogenesis, providing a novel intervention to increase intestinal length. Level of evidence IV (Case series without comparison group).
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Affiliation(s)
- Aaron J Cunningham
- Division of Pediatric Surgery, Department of Surgery, Medical University of South Carolina Shawn Jenkins Children’s Hospital, 10 McClennan Banks Dr., MSC 918, Charleston, SC 29425, United States
| | - Taylor Anderson
- Division of Pediatric Surgery, Department of Surgery, Stanford University School of Medicine and Lucile Packard Children’s Hospital, Palo Alto, CA, United States
| | - Claudia Mueller
- Division of Pediatric Surgery, Department of Surgery, Stanford University School of Medicine and Lucile Packard Children’s Hospital, Palo Alto, CA, United States
| | - Matias Bruzoni
- Department of Pediatric Surgery, Texas Children’s Hospital, Austin, TX, United States
| | - James CY Dunn
- Division of Pediatric Surgery, Department of Surgery, Stanford University School of Medicine and Lucile Packard Children’s Hospital, Palo Alto, CA, United States
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Chatzidaki V, Wood R, Alegakis A, Lawson M, Fagbemi A. Parenteral support and micronutrient deficiencies in children with short bowel syndrome: A comprehensive retrospective study. Clin Nutr ESPEN 2023; 58:270-276. [PMID: 38057017 DOI: 10.1016/j.clnesp.2023.10.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2023] [Revised: 10/08/2023] [Accepted: 10/10/2023] [Indexed: 12/08/2023]
Abstract
BACKGROUND & AIMS Short bowel syndrome (SBS) is the leading cause of chronic intestinal failure. The duration of parenteral support (PS) and the long-term micronutrient needs in children with SBS vary, based on their clinical and anatomical characteristics. Our study aimed to review the clinical course and identify high risk patient groups for prolonged PS and long-term micronutrient supplementation. METHODS A retrospective review was conducted on electronic medical records of children with SBS and chronic intestinal failure who were enrolled in the multidisciplinary intestinal rehabilitation program at Manchester Children's Hospital, UK. Children were included in the review if they required PN for more than 60 days out of 74 consecutive days and had at least 3 years of follow-up. Statistical analysis was performed using IBM SPSS Statistics 24.0. RESULTS 40 children with SBS achieved enteral autonomy (EA) and 14 remained dependent on PS after 36 months of follow up. Necrotizing enterocolitis was the most common cause for intestinal resection (38.9%) followed by gastroschisis (22.2%), malrotation with volvulus (20.4%), segmental volvulus (9.3%) and long segment Hirschsprung disease (1.9%). Those who achieved EA had significantly longer intestinal length 27.5% (15.0-39.3) than those who remained on PS 6.0% (1.5-12.5) (p < 0.001). Type I SBS was only found in the PS cohort. Median PN dependence was 10.82 months [IQR 5.73-20.78]. Congenital diagnosis was associated with longer PN dependence (21.0 ± 20.0) than acquired (8.7 ± 7.8 months), (p = 0.02). The need for micronutrient supplementation was assessed after the transition to EA; 87.5% children had at least one micronutrient depletion, most commonly Vitamin D (64.1%), followed by iron (48.7%), Vitamin B12 (34.2%), and vitamin E (28.6%). Iron deficiency and vitamin A depletion were correlated with longer PS after multivariate analysis (OR: 1.103, 1.006-1.210, p = 0.037 and OR: 1.048, 0.998-1.102, p = 0.062 respectively). CONCLUSION In our cohort, small bowel length was the main predictor for EA. Children on longer PS, had more often a congenital cause of resection and were at risk for micronutrient deficiencies in EA.
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Affiliation(s)
- V Chatzidaki
- Department of Pediatric Gastroenterology, Royal Manchester Children's Hospital, NHS Foundation Trust, UK.
| | - R Wood
- Department of Pediatric Gastroenterology, Royal Manchester Children's Hospital, NHS Foundation Trust, UK
| | - A Alegakis
- Laboratory of Toxicology, University of Crete, Greece
| | - M Lawson
- Department of Pediatric Gastroenterology, Royal Manchester Children's Hospital, NHS Foundation Trust, UK
| | - A Fagbemi
- Department of Pediatric Gastroenterology, Royal Manchester Children's Hospital, NHS Foundation Trust, UK
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Pironi L, Cuerda C, Jeppesen PB, Joly F, Jonkers C, Krznarić Ž, Lal S, Lamprecht G, Lichota M, Mundi MS, Schneider SM, Szczepanek K, Van Gossum A, Wanten G, Wheatley C, Weimann A. ESPEN guideline on chronic intestinal failure in adults - Update 2023. Clin Nutr 2023; 42:1940-2021. [PMID: 37639741 DOI: 10.1016/j.clnu.2023.07.019] [Citation(s) in RCA: 71] [Impact Index Per Article: 35.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2023] [Accepted: 07/21/2023] [Indexed: 08/31/2023]
Abstract
BACKGROUND & AIMS In 2016, ESPEN published the guideline for Chronic Intestinal Failure (CIF) in adults. An updated version of ESPEN guidelines on CIF due to benign disease in adults was devised in order to incorporate new evidence since the publication of the previous ESPEN guidelines. METHODS The grading system of the Scottish Intercollegiate Guidelines Network (SIGN) was used to grade the literature. Recommendations were graded according to the levels of evidence available as A (strong), B (conditional), 0 (weak) and Good practice points (GPP). The recommendations of the 2016 guideline (graded using the GRADE system) which were still valid, because no studies supporting an update were retrieved, were reworded and re-graded accordingly. RESULTS The recommendations of the 2016 guideline were reviewed, particularly focusing on definitions, and new chapters were included to devise recommendations on IF centers, chronic enterocutaneous fistulas, costs of IF, caring for CIF patients during pregnancy, transition of patients from pediatric to adult centers. The new guideline consist of 149 recommendations and 16 statements which were voted for consensus by ESPEN members, online in July 2022 and at conference during the annual Congress in September 2022. The Grade of recommendation is GPP for 96 (64.4%) of the recommendations, 0 for 29 (19.5%), B for 19 (12.7%), and A for only five (3.4%). The grade of consensus is "strong consensus" for 148 (99.3%) and "consensus" for one (0.7%) recommendation. The grade of consensus for the statements is "strong consensus" for 14 (87.5%) and "consensus" for two (12.5%). CONCLUSIONS It is confirmed that CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for the underlying gastrointestinal disease and to provide HPN support. Most of the recommendations were graded as GPP, but almost all received a strong consensus.
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Affiliation(s)
- Loris Pironi
- Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy; Center for Chronic Intestinal Failure, IRCCS AOUBO, Bologna, Italy.
| | - Cristina Cuerda
- Nutrition Unit, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | | | - Francisca Joly
- Center for Intestinal Failure, Department of Gastroenterology and Nutritional Support, Hôpital Beaujon, Clichy, France
| | - Cora Jonkers
- Nutrition Support Team, Amsterdam University Medical Centers, Location AMC, Amsterdam, the Netherlands
| | - Željko Krznarić
- Center of Clinical Nutrition, Department of Medicine, University Hospital Center, Zagreb, Croatia
| | - Simon Lal
- Intestinal Failure Unit, Salford Royal Foundation Trust, Salford, United Kingdom
| | | | - Marek Lichota
- Intestinal Failure Patients Association "Appetite for Life", Cracow, Poland
| | - Manpreet S Mundi
- Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic College of Medicine, Rochester, MN, USA
| | | | - Kinga Szczepanek
- General and Oncology Surgery Unit, Stanley Dudrick's Memorial Hospital, Skawina, Poland
| | | | - Geert Wanten
- Intestinal Failure Unit, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Carolyn Wheatley
- Support and Advocacy Group for People on Home Artificial Nutrition (PINNT), United Kingdom
| | - Arved Weimann
- Department of General, Visceral and Oncological Surgery, St. George Hospital, Leipzig, Germany
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9
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Phelps HM, Warner BW. Intestinal adaptation and rehabilitation. Semin Pediatr Surg 2023; 32:151314. [PMID: 37276784 DOI: 10.1016/j.sempedsurg.2023.151314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/07/2023]
Abstract
Massive intestinal resection is a regrettably necessary but life-saving intervention for progressive or fulminant necrotizing enterocolitis (NEC). However, the resultant short bowel syndrome (SBS) poses its own array of challenges and complications. Within hours of such an abrupt loss of intestinal length, the intestine begins to adapt. Our ability to understand this process of intestinal adaptation has proven critical in our ability to clinically treat the challenging problem of short bowel syndrome. This review first highlights key data relating to intestinal adaptation including structural and functional changes, biochemical regulation, and other factors affecting the magnitude of intestinal adaptation responses. We then focus on intestinal rehabilitation as it relates to strategies to enhance intestinal adaptation while meeting nutritional needs and preventing complications of parenteral nutrition.
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Affiliation(s)
- Hannah M Phelps
- Division of Pediatric Surgery, Department of Surgery, Washington University in St. Louis School of Medicine, 9901 Wohl Hospital, Campus Box 8109, St. Louis, MO 63110, USA.
| | - Brad W Warner
- Division of Pediatric Surgery, Department of Surgery, Washington University in St. Louis School of Medicine, 9901 Wohl Hospital, Campus Box 8109, St. Louis, MO 63110, USA
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10
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Fujiki M, Osman M, Abu-Elmagd K. Growing experience of surgical gut rehabilitation: essential role in the management of gut failure in adult patients. Curr Opin Organ Transplant 2023; 28:228-236. [PMID: 37018744 DOI: 10.1097/mot.0000000000001070] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/07/2023]
Abstract
PURPOSE OF REVIEW With the inherent therapeutic limitations of gut transplantation, the concept of surgical gut rehabilitation was introduced to restore nutritional autonomy in pediatric patients. With favorable outcomes in these young patients, there has been increasing interest in the applicability of gut rehabilitative surgery to a growing population of adults with gut failure due to various etiologies. We aim to review the current status of surgical gut rehabilitation for adult gut failure patients in the era of multidisciplinary gut rehabilitation and transplantation. RECENT FINDINGS Indications for surgical gut rehabilitation have been gradually expanding, with gut failure after bariatric surgery recently added. Serial transverse enteroplasty (STEP) has been used with favorable outcomes in adult patients, including those with intrinsic intestinal disease. Autologous gut reconstruction (AGR) is the most frequently used surgical rehabilitative method; its outcome is further improved with conjunctive use of bowel lengthening and enterocyte growth factor as a part of comprehensive gut rehabilitation. SUMMARY Accumulated experiences have validated the efficacy of gut rehabilitation for survival, nutritional autonomy, and quality of life in adults with gut failure of various etiology. Further progress is expected with growing experience around the world.
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Affiliation(s)
- Masato Fujiki
- Transplant Center, Cleveland Clinic, Cleveland, Ohio, USA
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11
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Rampersad B, Dubay C. An extreme case of vanishing gastroschisis with in utero midgut strangulation. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2023. [DOI: 10.1016/j.epsc.2022.102487] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
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12
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Iyer K, DiBaise JK, Rubio-Tapia A. AGA Clinical Practice Update on Management of Short Bowel Syndrome: Expert Review. Clin Gastroenterol Hepatol 2022; 20:2185-2194.e2. [PMID: 35700884 DOI: 10.1016/j.cgh.2022.05.032] [Citation(s) in RCA: 19] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/02/2022] [Revised: 04/22/2022] [Accepted: 05/24/2022] [Indexed: 02/07/2023]
Affiliation(s)
- Kishore Iyer
- Recanati Miller Transplant Institute, Department of Surgery, Mount Sinai Hospital, New York, New York.
| | - John K DiBaise
- Division of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona.
| | - Alberto Rubio-Tapia
- Division of Gastroenterology, Hepatology, and Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio
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13
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Wendel D, Javid PJ. Medical and Surgical Aspects of Intestinal Failure in the Child. Surg Clin North Am 2022; 102:861-872. [DOI: 10.1016/j.suc.2022.07.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2022]
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14
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Sakarellos P, Papalois A, Gakiopoulou H, Zacharioudaki I, Katsimpoulas M, Belia M, Moris D, Aggelou K, Vagios I, Davakis S, Vailas M, Liakakos T, Diamantis T, Felekouras E, Kontos M. Growth of Intestinal Neomucosa on Pedicled Gastric Wall Flap, a Novel Technique in an Animal Model. J INVEST SURG 2022; 35:1329-1339. [PMID: 35196939 DOI: 10.1080/08941939.2022.2034196] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2021] [Revised: 01/13/2022] [Accepted: 01/21/2022] [Indexed: 10/19/2022]
Abstract
Background: Short bowel syndrome (SBS) remains an unsolved issue in modern medicine. Numerous experimental surgical techniques have been proposed in the attempt to increase the intestinal absorptive capacity.Materials and Methods: Ten female Landrace pigs, divided in two groups of 5 (A and B), were explored through a midline incision. A spindle-shaped vascularized full-thickness gastric wall flap (GWF) consisting of part of the major curvature with the gastroepiploic arch preserved was de-epithelialized and then placed as a "patch" to cover an antimesenteric border defect of either a nonfunctional blind intestinal loop (group A) or a functional intestinal loop of the gastrointestinal tract (group B). A spindle-shaped curved, rigid, low density polyethylene (LDPE) splint was sutured on the external surface of the patch in order to prevent shrinkage of GWF and collapse of the intestinal wall in group A.Results: There was a decrease of both dimensions of the patch. Microscopically a thin layer of columnar epithelial cells covered the center of the patch, evolving in shorter, blunt, poorly developed villi with increasing maturation laterally. The patch surface was covered by nearly 90%. In the three animals that died prematurely the coverage of GWF was negligent or suboptimal directly dependent on the length of survival.Conclusions: The hereby-described patching technique demonstrated the growth of intestinal neomucosa on the GWF. The capability of the stomach to provide large flaps and the advantages of the use of native tissues render this animal model valuable for the future research in the field.
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Affiliation(s)
- Panagiotis Sakarellos
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | | | - Harikleia Gakiopoulou
- First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | - Iro Zacharioudaki
- Experimental, Educational and Research Centre, ELPEN, Athens, Greece
| | - Michalis Katsimpoulas
- Experimental Surgery Unit, Center of Clinical, Experimental Surgery and Translational Research, Βιοmedical Research Foundation of the Academy of Athens, Athens, Greece
| | - Marina Belia
- Department of Haematology and Bone Marrow Transplantation, Laiko General Hospital, National and Kapodistrian University of Athens, Athens Greece
| | - Dimitrios Moris
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Kyveli Aggelou
- Department of Obstetrics and Gynecology, Alexandra Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Ilias Vagios
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Spiridon Davakis
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Michail Vailas
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Theodoros Liakakos
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Theodoros Diamantis
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Evangelos Felekouras
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Michael Kontos
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
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15
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Abstract
Due to recent advances, the mortality due to short bowel syndrome (SBS) has significantly decreased, but the morbidities are still high. Morbidities arising specifically due to dysmotility in SBS include feeding intolerance, prolonged dependence on parenteral nutrition, and associated complications such as intestinal failure associated liver disease, and bloodstream infections. The understanding of the pathogenesis of dysmotility in SBS has improved vastly. However, the tools to diagnose dysmotility in SBS in infants are restrictive, and the medical therapies to treat dysmotility are limited. Surgical techniques available for the treatment after failure of conservative management of dysmotility offer hope but carry their associated risks. The evidence to support either the medical therapies or the surgical techniques to treat dysmotility in SBS in children is scarce and weak. Development of newer therapies and efforts to build evidence to support currently available treatments in treating dysmotility in SBS is needed.
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Affiliation(s)
- Muralidhar H Premkumar
- Associate Professor, Division of Neonatology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, 6621 Fannin, Suite 6104, Houston, TX 77030, USA.
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16
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Boroni G, Parolini F, Stern MV, Moglia C, Alberti D. Autologous Intestinal Reconstruction Surgery in Short Bowel Syndrome: Which, When, and Why. Front Nutr 2022; 9:861093. [PMID: 35463997 PMCID: PMC9023091 DOI: 10.3389/fnut.2022.861093] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2022] [Accepted: 03/07/2022] [Indexed: 11/13/2022] Open
Abstract
Short bowel syndrome (SBS), secondary to any natural loss or after any extensive bowel resection for congenital malformations or acquired disease, is the most common cause of intestinal failure in children. Extensive introduction of parenteral nutrition (PN) has dramatically changed the outcome of these patients, allowing for long-term survival. The main goal in children with SBS remains to be increasing enteral tolerance and weaning from PN support. Post resection intestinal adaptation allows for achievement of enteral autonomy in a subset of these patients, but the inability to progress in enteral tolerance exposes others to long-term complications of PN. Autologous intestinal reconstruction surgery (AIRS) can facilitate the fulfilment of enteral autonomy, maximizing the absorptive potential of the remaining gut. All the different intestinal reconstruction techniques, from simple procedures like tapering, reversed segments, and colon interposition, to more complex lengthening procedures (LILT: longitudinal intestinal lengthening and tailoring, STEP: serial transverse enteroplasty, and SILT: spiral intestinal lengthening and tailoring) and techniques designed for peculiar problems like controlled intestinal tissue expansion or duodenal lengthening are presented. AIRS indications, clinical applications, and results reported in the literature are reviewed.
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Affiliation(s)
- Giovanni Boroni
- Department of Paediatric Surgery, ASST Spedali Civili di Brescia, Brescia, Italy
- *Correspondence: Giovanni Boroni,
| | - Filippo Parolini
- Department of Paediatric Surgery, ASST Spedali Civili di Brescia, Brescia, Italy
| | | | - Cristina Moglia
- Department of Paediatric Surgery, University of Brescia, Brescia, Italy
| | - Daniele Alberti
- Department of Paediatric Surgery, ASST Spedali Civili di Brescia, Brescia, Italy
- Department of Paediatric Surgery, University of Brescia, Brescia, Italy
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17
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Self-expanding intestinal expansion sleeves (IES) for short gut syndrome. Pediatr Surg Int 2022; 38:75-81. [PMID: 34709433 DOI: 10.1007/s00383-021-05024-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/01/2021] [Indexed: 10/20/2022]
Abstract
PURPOSE Many disease processes (necrotizing enterocolitis, caustic esophageal injury, malrotation with volvulus), can result in short-gut syndrome (SGS), where remnant intestinal segments may dilate axially, but rarely elongate longitudinally. Here we mechanically characterize a novel model of a self-expanding mesh prototype intestinal expanding sleeve (IES) for use in SGS. METHODS Gut lengthening was achieved using a proprietary cylindrical layered polyethylene terephthalate IES device with helicoid trusses with isometric ends. The IES is pre-contracted by diametric expansion, deployed into the gut and anchored with bioabsorbable sutures. IES expansion to its equilibrium dimension maintained longitudinal gut tension, which may permit remodeling, increased absorptive surface area while preserving vascular and nervous supplies. We performed mechanical testing to obtain the effective force-displacement characterization achieved on these prototypes and evaluated minimal numbers of sutures needed for its anchoring. Furthermore, we deployed these devices in small and large intestines of New Zealand White rabbits, measured IES length-tension relationships and measured post-implant gut expansion ex vivo. Histology of the gut before and after implantation was also evaluated. RESULTS Longitudinal tension using IES did not result in suture failure. Maximum IES suture mechanical loading was tested using 4-6 sutures; we found similar failure loads of 2.95 ± 0.64, 4 ± 1.9 and 3.16 ± 0.24 Newtons for 4, 6 and 8 sutures, respectively (n = 3, n.s). Pre-contracted IES tubes were deployed at 67 ± 4% of initial length (i.l.); in the large bowel these expanded significantly to 81.5 ± 3.7% of i.l. (p = 0.014, n = 4). In the small bowel, pre-contracted IES were 61 ± 3.8% of i.l.; these expanded significantly to 82.7 ± 7.4% of i.l. (p = 0.0009, n = 6). This resulted in an immediate 24 ± 7.8% and 36.2 ± 11% increase in gut length when deployed in large and small bowels, respectively, with maintained longitudinal tension. Maintained IES induced tension produced gut wall thinning; gut histopathological evaluation is currently under evaluation. CONCLUSION IES is a versatile platform for gaining length in SGS, which may be simply deployed via feeding tubes. Our results need further validation for biocompatibility and mechanical characterization to optimize use in gut expansion.
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18
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State-of-the-art colorectal disease: conservative surgical management of intestinal failure in adults. Int J Colorectal Dis 2021; 36:1597-1607. [PMID: 33629118 DOI: 10.1007/s00384-021-03863-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/26/2021] [Indexed: 02/04/2023]
Abstract
PURPOSE Intestinal failure (IF) is defined by the reduction of gut function under the minimum necessary for adequate absorption of macronutrients and/or electrolytes, requiring home parenteral nutrition (HPN) to maintain health and/or growth. Among the different causes of IF, short bowel syndrome (SBS) is one of the main causes of IF in adults. Management of IF and SBS is complex and requires a multidisciplinary approach. The aim of this study was to review the place and the modalities of conservative surgical procedures performed for IF in adults. RESULTS-CONCLUSION HPN has drastically modified the poor prognosis of these patients, leading to an overall survival around 70-75% at 5 years in patients without cancer. However, HPN is associated with life-threatening long-term complications, including liver failure and catheter-related complications, main causes of deaths for these patients. Surgery can be proposed, in order to try to reduce SBS consequences with either conservative or non-conservative procedures. Simple bowel continuity restoration should be performed as often as possible in order to wean or at least to reduce HPN. Lengthening procedures are proposed to increase the absorption surface of the remnant bowel. These procedures can be only performed on a dilated small bowel. The slowing transit time procedure is represented by segmental reversal of the small bowel. Intestinal transplantation is the last surgical option for patients with IF but is still today associated with high mortality and failure rates. Thus, conservative procedures have a major role in the surgical armentarium for patients with IF.
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19
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Lemoine C, Larkin K, Brennan K, Zoller-Thompson C, Cohran V, Superina R. Repeat serial transverse enteroplasty procedure (reSTEP): Is it worth it? J Pediatr Surg 2021; 56:951-960. [PMID: 33455806 DOI: 10.1016/j.jpedsurg.2020.12.026] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2020] [Revised: 12/10/2020] [Accepted: 12/24/2020] [Indexed: 11/19/2022]
Abstract
BACKGROUND The utility of repeat serial transverse enteroplasty (reSTEP) has been questioned after patients failed to achieve enteral autonomy. We compared the outcomes after reSTEP to one lifetime STEP (oneSTEP), and tried to identify patients at risk for reSTEP failure. METHODS All STEPs done at our institution were reviewed. Growth, enteral autonomy, bowel-derived bloodstream infection hospital admissions, complications, and need for bowel transplantation were evaluated (p<0.05 considered significant). RESULTS 24 patients underwent 32 STEP (16 oneSTEP, 8 reSTEP). reSTEP patients were younger at first surgery (1.01±1.05 vs. oneSTEP 3.06±4.73 years, p = 0.24). Median time to reSTEP was 1.1 year (0.6-5.7). Weight-for-length z-scores improved after reSTEP (0.02±1.40 to 0.22±1.42, p = 0.81). Bowel-derived bloodstream infections decreased after surgery (oneSTEP 1.50±2.25 to 0.94±1.73, p = 0.50; reSTEP 1.88±2.10 to 1.66±2.32, p = 0.52). 37.5% (9/24) patients achieved enteral autonomy at last follow-up: 7/16 oneSTEP, 2/8 reSTEP (p = 0.37). Two complications occurred after oneSTEP (staple line ulcer, leak), none following reSTEP. Three patients (oneSTEP 1/16, reSTEP 2/8, p = 0.19) underwent bowel transplantation (all gastroschisis). CONCLUSION Similar postoperative outcomes after reSTEP and single STEP (improved enteral tolerance, reduced rates of infections) support the use of reSTEP when clinically indicated, although reSTEP in young infants with a history of gastroschisis may need further evaluation.
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Affiliation(s)
- Caroline Lemoine
- Division of Transplant and Advanced Hepatobiliary Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Kevin Larkin
- Division of Transplant and Advanced Hepatobiliary Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Katherine Brennan
- Division of Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Carmyn Zoller-Thompson
- Division of Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Valeria Cohran
- Division of Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Riccardo Superina
- Division of Transplant and Advanced Hepatobiliary Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
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20
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Wang J, Micic D. Hepatobiliary Manifestations of Short Bowel Syndrome and Intestinal Failure-Associated Liver Disease. Clin Liver Dis (Hoboken) 2021; 17:297-300. [PMID: 33968392 PMCID: PMC8087921 DOI: 10.1002/cld.1053] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/04/2020] [Revised: 08/31/2020] [Accepted: 10/11/2020] [Indexed: 02/04/2023] Open
Affiliation(s)
- Jennifer Wang
- Department of Internal MedicineSection of Gastroenterology, Hepatology and NutritionUniversity of Chicago MedicineChicagoIL
| | - Dejan Micic
- Department of Internal MedicineSection of Gastroenterology, Hepatology and NutritionUniversity of Chicago MedicineChicagoIL
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21
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Double barrel enteroplasty for the management of short bowel syndrome in children. Pediatr Surg Int 2021; 37:169-177. [PMID: 33104840 DOI: 10.1007/s00383-020-04767-0] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/11/2020] [Indexed: 10/23/2022]
Abstract
BACKGROUND Currently, there are two well-established methods of bowel lengthening in patients with short bowel syndrome (SBS)-longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) [1-4]. Both procedures may carry a high reported morbidity and mortality of 30.2% and 14.4%, respectively [5]. We report the outcomes of a novel technique: double barrel enteroplasty (DBE) for autologous intestinal reconstruction. METHODS We performed a retrospective review of all ten patients who underwent DBE at our institution since 2011. All patients have SBS and were dependent on parenteral nutrition (PN) at the time of surgery. Etiologies were gastroschisis (n = 4), bowel atresia (n = 3), necrotising enterocolitis (n = 1), volvulus (n = 1), and near-total intestinal aganglionosis (n = 1). Patient survival, complications, and subsequent enteral autonomy were evaluated. RESULTS All patients are alive with normal liver function. Five children achieved enteral autonomy, while the remaining are on weaning PN. There was no bleeding, anastomotic leak, perforation, infective complications, or intestinal necrosis. No patient has required a liver and/or intestinal transplant. CONCLUSIONS Double barrel enteroplasty is technically feasible and safe. It has similar efficacy and may have fewer complications when compared with other methods of autologous intestinal reconstruction.
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22
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Choudhury RA, Yoeli D, Hoeltzel G, Moore HB, Prins K, Kovler M, Goldstein SD, Holland-Cunz SG, Adams M, Roach J, Nydam TL, Vuille-Dit-Bille RN. STEP improves long-term survival for pediatric short bowel syndrome patients: A Markov decision analysis. J Pediatr Surg 2020; 55:1802-1808. [PMID: 32345501 DOI: 10.1016/j.jpedsurg.2020.03.017] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2019] [Revised: 01/31/2020] [Accepted: 03/22/2020] [Indexed: 12/11/2022]
Abstract
INTRODUCTION Increasingly, for pediatric patients with short bowel syndrome (SBS), intestinal lengthening procedures such as serial transverse enteroplasty (STEP) are being offered with the hope of improving patients' chances for achieving enteral autonomy. However, it remains unclear to what extent STEP reduces the long-term need for intestinal transplant or improves survival. METHODS Based on existing literature, a decision analytic Markov state transition model was created to simulate the life of 1,000 pediatric SBS patients. Two simulations were modeled: 1) No STEP: patients were listed for transplant once medical management failed and 2) STEP: patients underwent STEP therapy and subsequent transplant listing if enteral autonomy was not achieved. Sensitivity analysis of small bowel length and anatomy was completed. Base case patients were defined as neonates with a small bowel length of 30cm. RESULTS For base case patients with an ostomy and a NEC SBS etiology, STEP was associated with increased rates of enteral autonomy after 10 years for patients with an ICV (53.9% [STEP] vs. 51.1% [No STEP]) and without an ICV (43.4% [STEP] vs. 36.3% [No STEP]). Transplantation rates were also reduced following STEP therapy for both ICV (17.5% [STEP] vs. 18.2% [No STEP]) and non-ICV patients (20.2% [STEP] vs. 22.1% [No STEP]). 10-year survival was the highest in the (+) STEP and (+) ICV group (85.4%) and lowest in the (-) STEP and (-) ICV group (83.3%). CONCLUSIONS For SBS patients, according to our model, STEP increases rates of enteral autonomy, reduces need for intestinal transplantation, and improves long-term survival. TYPE OF STUDY Economic/Decision Analysis or Modeling Studies LEVEL OF EVIDENCE: Level III.
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Affiliation(s)
- Rashikh A Choudhury
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO.
| | - Dor Yoeli
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Gerard Hoeltzel
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Hunter B Moore
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Kas Prins
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Mark Kovler
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Seth D Goldstein
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Stephan G Holland-Cunz
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Megan Adams
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Jonathan Roach
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Trevor L Nydam
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
| | - Raphael N Vuille-Dit-Bille
- University of Colorado Hospital, Department of Transplant Surgery, Aurora, CO; Johns Hopkins Hospital, Department of Pediatric Surgery, Baltimore, MD; Ann and Robert H. Lurie Children's Hospital of Chicago, Division of Pediatric Surgery, Chicago, IL; University Children's Hospital of Basel, Department of Pediatric Surgery, Basel, Switzerland; Colorado Children's Hospital, Department of Pediatric Surgery, Aurora, CO
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Soop M. Challenges in Crohn's disease: Intestinal failure and short bowel syndrome in Crohn's disease. SEMINARS IN COLON AND RECTAL SURGERY 2020. [DOI: 10.1016/j.scrs.2020.100749] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
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Abstract
Short bowel syndrome (SBS) is a malabsorptive state that may occur either after surgical bowel resection or as the result of congenital bowel anomalies. SBS can incur significant morbidity and mortality including intestinal failure, cholestasis, sepsis, and death. For patients with SBS, management involves a multidisciplinary approach that begins with neonatology, pediatric surgery, nutritionists, pharmacists, and nurses in the NICU and also includes the transition to an intestinal rehabilitation program. The aim of this review is to provide the neonatologist with an overview of the common causes of neonatal SBS, anticipated nutritional deficiencies, complications associated with SBS, and the surgical and medical management of SBS to assist in counseling affected families.
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Affiliation(s)
| | - Melissa E Danko
- Pediatric Surgery, Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, TN
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Management of Five Hundred Patients With Gut Failure at a Single Center: Surgical Innovation Versus Transplantation With a Novel Predictive Model. Ann Surg 2020; 270:656-674. [PMID: 31436550 DOI: 10.1097/sla.0000000000003523] [Citation(s) in RCA: 29] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
OBJECTIVE(S) To define the evolving role of integrative surgical management including transplantation for patients gut failure (GF). METHODS A total of 500 patients with total parenteral nutrition-dependent catastrophic and chronic GF were referred for surgical intervention particularly transplantation and comprised the study population. With a mean age of 45 ± 17 years, 477 (95%) were adults and 23 (5%) were children. Management strategy was guided by clinical status, splanchnic organ functions, anatomy of residual gut, and cause of GF. Surgery was performed in 462 (92%) patients and 38 (8%) continued medical treatment. Definitive autologous gut reconstruction (AGR) was achievable in 378 (82%), primary transplant in 42 (9%), and AGR followed by transplant in 42 (9%). The 84 transplant recipients received 94 allografts; 67 (71%) liver-free and 27 (29%) liver-contained. The 420 AGR patients received a total of 790 reconstructive and remodeling procedures including primary reconstruction, interposition alimentary-conduits, intestinal/colonic lengthening, and reductive/decompressive surgery. Glucagon-like peptide-2 was used in 17 patients. RESULTS Overall patient survival was 86% at 1-year and 68% at 5-years with restored nutritional autonomy (RNA) in 63% and 78%, respectively. Surgery achieved a 5-year survival of 70% with 82% RNA. AGR achieved better long-term survival and transplantation better (P = 0.03) re-established nutritional autonomy. Both AGR and transplant were cost effective and quality of life better improved after AGR. A model to predict RNA after AGR was developed computing anatomy of reconstructed gut, total parenteral nutrition requirements, cause of GF, and serum bilirubin. CONCLUSIONS Surgical integration is an effective management strategy for GF. Further progress is foreseen with the herein-described novel techniques and established RNA predictive model.
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Belza C, Wales PW. Multidisciplinary Management in Pediatric Ultrashort Bowel Syndrome. J Multidiscip Healthc 2020; 13:9-17. [PMID: 32021230 PMCID: PMC6957105 DOI: 10.2147/jmdh.s236130] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/28/2019] [Accepted: 12/27/2019] [Indexed: 12/28/2022] Open
Abstract
Pediatric intestinal failure (IF) remains a complex and devastating condition resulting in the inability of the gastrointestinal tract to absorb adequate fluids and nutrients to sustain life. The goal in the management of IF is to achieve enteral autonomy and when not possible to avoid and minimize the development of long-term complications. Survival rates for children with IF have continued to improve resulting in an increased population of children with more altered anatomy. While IF remains a rare disease, children with IF secondary to ultrashort bowel syndrome comprise an even smaller patient population. The goal of this article is to review the recent literature related to the impact of multidisciplinary intestinal rehabilitation programs (IRPs) on the management and outcomes of intestinal failure in children with ultrashort bowel syndrome and potential avenues to further improve the long-term outcomes of this patient population.
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Affiliation(s)
- Christina Belza
- Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada
| | - Paul W Wales
- Group for Improvement of Intestinal Function and Treatment (GIFT), The Hospital for Sick Children, University of Toronto, Toronto, Canada.,Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
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Abstract
Insufficient absorptive mucosal surface is the fundamental problem in the short bowel state. Intestinal adaptation has been well studied, and it is well recognized that it may lead to dilatation of the bowel with increased thickness of the bowel wall, resulting from both mucosal hypertrophy and hyperplasia. Autologous reconstructive surgery exploits bowel dilatation in short bowel syndrome and maximizes the absorptive potential of the available mucosal surface. Indeed, autologous gastrointestinal reconstructive procedures may be better viewed as optimizing bowel diameter rather than focusing on length, thus allowing better prograde peristalsis and improved contact between luminal nutrients and mucosa, ultimately enhancing absorption.
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Affiliation(s)
- Riccardo Coletta
- Department of Paediatric Surgery, Center for Intestinal Reconstruction and Rehabilitation, Meyer Children's Hospital, Viale Gaetanao Pieraccini. 24, Florence 50139, Italy; School of Environment and Life Science, University of Salford, Salford, UK
| | - Antonino Morabito
- Department of Paediatric Surgery, Center for Intestinal Reconstruction and Rehabilitation, Meyer Children's Hospital, Viale Gaetanao Pieraccini. 24, Florence 50139, Italy; School of Environment and Life Science, University of Salford, Salford, UK; Department of NeuroFarBa, University of Florence, Florence, Italy
| | - Kishore Iyer
- Intestinal Rehabilitation & Transplant Program, Icahn School of Medicine at Mount Sinai, Mount Sinai Hospital, One Gustave Levy Place, Box 1104, New York, NY 10029, USA.
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Abstract
Short bowel syndrome / intestinal failure (SBS/IF) is a rare and debilitating disease process that mandates a multidisciplinary approach in its management. Inflammatory bowel disease (IBD), in particular Crohn's disease (CD), predisposes patients to development of SBS/IF. This review discusses SBS/IF from the perspective of IBD, with an emphasis on prevention and treatment in the setting of CD. The aims of this review are to emphasize the unique treatment goals of the newly diagnosed SBS/IF patient, and highlight the role of both medical and surgical therapies in the management of IBD-related SBS/IF, including intestinal transplantation.
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Affiliation(s)
- Matthew A Fuglestad
- Department of Surgery, Division of General Surgery, University of Nebraska Medical Center, 983280 Nebraska Medical Center, Omaha, NE 68198-3280, USA
| | - Jon S Thompson
- Department of Surgery, Division of General Surgery, University of Nebraska Medical Center, 983280 Nebraska Medical Center, Omaha, NE 68198-3280, USA.
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Gondolesi GE, Doeyo M, Echevarria Lic C, Lobos F, Rubio S, Rumbo C, Ramisch D, Crivelli A, Schelotto PB, Solar H. Results of Surgical and Medical Rehabilitation for Adult Patients With Type III Intestinal Failure in a Comprehensive Unit Today: Building a New Model to Predict Parenteral Nutrition Independency. JPEN J Parenter Enteral Nutr 2019; 44:703-713. [DOI: 10.1002/jpen.1686] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2019] [Revised: 06/27/2019] [Accepted: 07/03/2019] [Indexed: 12/19/2022]
Affiliation(s)
- Gabriel E. Gondolesi
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Mariana Doeyo
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Constanza Echevarria Lic
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Fernando Lobos
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Santiago Rubio
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Carolina Rumbo
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Diego Ramisch
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Adriana Crivelli
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Pablo Barros Schelotto
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
| | - Hector Solar
- Intestinal Failure, Rehabilitation and Transplant Unit Favaloro Foundation University Hospital Fundacion Favaloro Buenos Aires Argentina
- Translational Medicine Transplantation and Bioengineering Research Institute (IMeTTyB) Favaloro University Buenos Aires Argentina
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Lauro A, Coletta R, Morabito A. Restoring gut physiology in short bowel patients: from bench to clinical application of autologous intestinal reconstructive procedures. Expert Rev Gastroenterol Hepatol 2019; 13:785-796. [PMID: 31282770 DOI: 10.1080/17474124.2019.1640600] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Introduction: Short bowel syndrome represents the leading etiology that causes intestinal failure both in children and adults. Total parenteral nutrition support has dramatically improved the prognosis for these patients but, if related irreversible complications occur, the alternative is represented by surgery and/or transplantation. Areas covered: Autologous gastrointestinal reconstructive procedures are a feasible, alternative approach with good long-term outcome data inexperienced surgical centers. Expert opinion: Ongoing innovative efforts have driven the surgical options for successful autologous reconstructive surgery: bowel elongation/tapering techniques (LILT, STEP, and the new SILT) together with the 'reversed bowel segment' procedure are now recognized procedures and all must be tailored to the individual patient needs to obtain the optimal result in terms of enteral autonomy. Background laboratory experimentation with new procedures e.g. options for bowel dilation techniques and distraction-induced enterogenesis, may provide additional management and treatment modalities.
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Affiliation(s)
- Augusto Lauro
- a Emergency General Surgery, St. Orsola University Hospital-Alma Mater Studiorum , Bologna , Italy
| | - R Coletta
- b Department of Pediatric Surgery, Pediatric Autologous Bowel Reconstruction, Rehabilitation & Regenerative Medicine Unit, Meyer Children's Hospital , Florence , Italy.,c Department of NEUROFABRA, University of Florence , Florence , Italy
| | - A Morabito
- b Department of Pediatric Surgery, Pediatric Autologous Bowel Reconstruction, Rehabilitation & Regenerative Medicine Unit, Meyer Children's Hospital , Florence , Italy.,c Department of NEUROFABRA, University of Florence , Florence , Italy
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Goulet O, Abi Nader E, Pigneur B, Lambe C. Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management. Pediatr Gastroenterol Hepatol Nutr 2019; 22:303-329. [PMID: 31338307 PMCID: PMC6629594 DOI: 10.5223/pghn.2019.22.4.303] [Citation(s) in RCA: 61] [Impact Index Per Article: 10.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/26/2019] [Accepted: 05/11/2019] [Indexed: 12/13/2022] Open
Abstract
Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.
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Affiliation(s)
- Olivier Goulet
- Department of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Sorbonne-Paris-Cité Paris Descartes Medical School, Paris, France
| | - Elie Abi Nader
- Department of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Sorbonne-Paris-Cité Paris Descartes Medical School, Paris, France
| | - Bénédicte Pigneur
- Department of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Sorbonne-Paris-Cité Paris Descartes Medical School, Paris, France
| | - Cécile Lambe
- Department of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Sorbonne-Paris-Cité Paris Descartes Medical School, Paris, France
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Modified serial transverse small-bowel enteroplasty procedure: A novel salvage technique for lengthening the gastric conduit for esophageal reconstruction. J Thorac Cardiovasc Surg 2019; 158:e77-e79. [PMID: 30992216 DOI: 10.1016/j.jtcvs.2019.03.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2019] [Revised: 02/22/2019] [Accepted: 03/02/2019] [Indexed: 11/20/2022]
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Coletta R, Morabito A. Non-transplant Surgical Management of Short Bowel Syndrome in Children: An Overview. Curr Pediatr Rev 2019; 15:106-110. [PMID: 30499416 DOI: 10.2174/1573396315666181129164112] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2018] [Revised: 10/12/2018] [Accepted: 11/24/2018] [Indexed: 01/02/2023]
Abstract
Management of severe Short Bowel Syndrome (SBS) is still one of the largest challenges of the medicine. Vast majority of the short bowel patients are children, the conditions that lead to this possible outcome most often are necrotizing enterocolitis (NEC), small intestinal volvulus as a result of intestinal malrotation, gastroschisis and the "apple peel" syndrome. Therefore, paediatricians and paediatric surgeons face this challenge most often. The nontransplant treatment appears to be effective using surgical procedure to increase absorptive surface and to reduce the transit time, but in some cases these procedures are enough to weaning of TPN. The aim of this review was to summarize the modern non-stransplant surgical management of short bowel syndrome.
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Affiliation(s)
- Riccardo Coletta
- Department of Paediatric Surgery, Meyer Children's Hospital, University of Florence, Florence, Italy
| | - Antonino Morabito
- Department of Paediatric Surgery, Meyer Children's Hospital, University of Florence, Florence, Italy
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Cheng W, Zhang S, Wang J, Zhou C, Li Y, Li J. Three-dimensional CT enterography versus barium follow-through examination in measurement of remnant small intestinal length in short bowel syndrome patients. Abdom Radiol (NY) 2018; 43:2955-2962. [PMID: 29700592 DOI: 10.1007/s00261-018-1597-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
PURPOSE To evaluate the feasibility and accuracy of remnant small intestinal length measured by barium follow-through (BaFT) examination and three-dimensional CT enterography (CTe). MATERIALS AND METHODS Twenty-nine consecutive short bowel syndrome patients (SBS) who underwent BaFT, CTe, and prior surgical measurements of small intestine were included. Measurements of total remnant small intestinal length on BaFT and CTe were compared to surgical measurements using Spearman's rank correlation coefficients, Bland-Altman plots, and paired t test. RESULTS The average remnant intestinal length was 73.1 ± 37.2 cm according to surgical measurement. There was a significant positive correlation between CTe and surgical measurement (r2 = 0.99; p < 0.0001), and a relatively weaker correlation between BaFT and surgical measurement (r2 = 0.71; p < 0.001). Compared with surgical measurement, the percentage differences of CTe and BaFT were 5.71 ± 6.71% and 27.14 ± 18.41% (mean ± SD), respectively. Furthermore, Bland-Altman plots showed good agreement between CTe and surgical measurement, whereas relatively poor agreement between BaFT and surgical measurement. However, significant difference was found among the three measurement methods by paired t test (p < 0.0001). CONCLUSIONS Assessment of remnant small intestinal length by CTe is accurate and acceptable for clinical application, whereas BaFT is less accurate although BaFT is more convenient and cheaper for clinical application. And CTe can provide a cost-effective and noninvasive determination of remnant small intestinal length in planning surgical and nutritional intervention in SBS patients.
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Affiliation(s)
- Wei Cheng
- Intestinal Rehabilitation and Transplant Center, Jinling Hospital, Medicine School of Nanjing University, Nanjing, 210002, China
| | - Shaoyi Zhang
- Intestinal Rehabilitation and Transplant Center, Jinling Hospital, Medicine School of Nanjing University, Nanjing, 210002, China
- Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Jian Wang
- Intestinal Rehabilitation and Transplant Center, Jinling Hospital, Medicine School of Nanjing University, Nanjing, 210002, China
| | - Changsheng Zhou
- Department of Medical Imaging, Jinling Hospital, Medicine School of Nanjing University, Nanjing, 210002, China
| | - Yousheng Li
- Intestinal Rehabilitation and Transplant Center, Jinling Hospital, Medicine School of Nanjing University, Nanjing, 210002, China.
- Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
| | - Jieshou Li
- Intestinal Rehabilitation and Transplant Center, Jinling Hospital, Medicine School of Nanjing University, Nanjing, 210002, China
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Bueno J, Burgos R, Redecillas S, López M, Balsells J. Duodenal lengthening in an adult with ultra-short bowel syndrome. A case report. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2018; 110:59-62. [PMID: 29106286 DOI: 10.17235/reed.2017.5187/2017] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND We have recently demonstrated the feasibility of lengthening the duodenum in children with short bowel syndrome and a dilated duodenum. This procedure gains additional intestinal length in a challenging area of autologous gut reconstruction. Herein, we report the successful application of this technique in an adult with ultra-short bowel syndrome. CASE REPORT A 25-year-old man with a history of mid-gut volvulus was referred to our center for intestinal transplant evaluation. Only a megaduodenum stump that reached as far as the third portion (30 cm of length) and the colon up to the hepatic flexure in the form of a mucous fistula was retained. A gastrostomy tube drained gastric and bilio-pancreatic secretions (output range: 2.5-4 liters/day). The time spent on parenteral nutrition (3 liters/day; 1500 calories/day) and I.V. fluid (1.5-2 liters/day) administration was 24 hours per day. The patient underwent duodenal lengthening and tapering with 7 sequential transverse applications (5 of 45 mm and 2 of 60 mm) of an endoscopic stapler on the anterior and posterior walls of the duodenum, respecting the pancreatic parenchyma and end-to-side duodeno-colonic anastomosis. The final duodenal length was 83 cm. The pre-lengthening citrulline level increased from 13.6 micromol/L to 21.6 micromol/L one year post-lengthening. After 24 month of follow-up, the time on a parenteral pump was shortened to 9 hours during the night. The volume and calorie requirements were also reduced by half. DISCUSSION Duodenal lengthening may be effective as part of the autologous intestinal reconstruction armamentarium in adults with short bowel syndrome.
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Affiliation(s)
- Javier Bueno
- Cirugía Pediátrica, Hospital Universitario Vall d´Hebron, España
| | - Rosa Burgos
- Unidad de Soporte Nutricional, Hospital universitario Vall d´Hebron, España
| | - Susana Redecillas
- Unidad de Soporte Nutricional, Hospital Universitario Vall d´Hebron, España
| | - Manuel López
- Cirugía Pediátrica, Hospital Universitario Vall d´Hebron, España
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Billiauws L, Maggiori L, Joly F, Panis Y. Medical and surgical management of short bowel syndrome. J Visc Surg 2018; 155:283-291. [DOI: 10.1016/j.jviscsurg.2017.12.012] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
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Vongbhavit K, Underwood MA. Predictive Value of the Aspartate Aminotransferase to Platelet Ratio Index for Parenteral Nutrition-Associated Cholestasis in Premature Infants With Intestinal Perforation. JPEN J Parenter Enteral Nutr 2017; 42:797-804. [PMID: 28792861 DOI: 10.1177/0148607117722755] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2017] [Accepted: 06/30/2017] [Indexed: 12/18/2022]
Abstract
BACKGROUND Parenteral nutrition-associated cholestasis (PNAC) is a major cause of morbidity and mortality in premature infants. Early predictors of PNAC would have clinical value. We sought to evaluate risk factors and liver function testing as predictors of PNAC in premature infants with intestinal perforation. METHODS Medical records of infants with a gestational age <34 weeks, birth weight <2000 g, and intestinal perforation due to either necrotizing enterocolitis or spontaneous intestinal perforation were reviewed. We analyzed clinical data and the maximum values of the aspartate aminotransferase (AST) to platelet ratio index (APRI), alanine aminotransferase (ALT), AST to ALT ratio, and total bilirubin (TB). RESULTS Sixty infants were identified, 17 infants with PNAC and 43 infants without PNAC. Sepsis, time to initiation of enteral feeds after perforation, and duration of PN were associated with PNAC. Within 2 weeks following intestinal perforation, APRI, ALT, and TB each differed significantly between infants who later developed PNAC and those that did not. The best APRI cut-point was 0.4775 within 2 weeks after perforation (area under the receiver operating characteristic curve, 0.90; positive predictive value, 85%; and negative predictive value, 87%); the cut-point for ALT was 13.5 (0.90, 85%, 84%), and the cut-point for TB was 3.55 (0.82, 69%, 83%), respectively, at 2 weeks after perforation. AST to ALT ratio did not differ between groups. CONCLUSIONS APRI and ALT had reasonable predictive value for PNAC in premature infants with intestinal perforation, with the APRI the best predictor within 2 weeks after perforation.
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Affiliation(s)
- Kannikar Vongbhavit
- Department of Pediatrics, Faculty of Medicine, Srinakharinwirot University, Nakhon-Nayok, Thailand.,Department of Pediatrics, University of California Davis School of Medicine, Sacramento, California, USA
| | - Mark A Underwood
- Department of Pediatrics, University of California Davis School of Medicine, Sacramento, California, USA
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Intestinal Rehabilitation Programs in the Management of Pediatric Intestinal Failure and Short Bowel Syndrome. J Pediatr Gastroenterol Nutr 2017; 65:588-596. [PMID: 28837507 DOI: 10.1097/mpg.0000000000001722] [Citation(s) in RCA: 127] [Impact Index Per Article: 15.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Intestinal failure is a rare, debilitating condition that presents both acute and chronic medical management challenges. The condition is incompatible with life in the absence of the safe application of specialized and individualized medical therapy that includes surgery, medical equipment, nutritional products, and standard nursing care. Intestinal rehabilitation programs are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation. These programs almost all include pediatric surgeons, pediatric gastroenterologists, specialized nurses, and dietitians; many also include a variety of other medical and allied medical specialists. Intestinal rehabilitation programs provide integrated interdisciplinary care, more discussion of patient management by involved specialists, continuity of care through various treatment interventions, close follow-up of outpatients, improved patient and family education, earlier treatment of complications, and learning from the accumulated patient databases. Quality assurance and research collaboration among centers are also goals of many of these programs. The combined and coordinated talents and skills of multiple types of health care practitioners have the potential to ameliorate the impact of intestinal failure and improve health outcomes and quality of life.
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Small Bowel Dilatation Predicts Prolonged Parenteral Nutrition and Decreased Survival in Pediatric Short Bowel Syndrome. Ann Surg 2017; 266:369-375. [PMID: 27433892 DOI: 10.1097/sla.0000000000001893] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
OBJECTIVE To analyze risk factors and prognostic significance of small bowel (SB) dilatation in children with short bowel syndrome (SBS). BACKGROUND In SBS, the remaining SB may dilate as part of intestinal adaptation. The impact of dilatation on parenteral nutrition (PN) dependence and survival has not been studied systematically. METHODS SB diameter of SBS children (n = 61) was measured in contrast SB series (n = 169, median age 0.94, range 0.32-2.7 years) during 2002 to 2015, and expressed as millimeters (SB width) and as ratio to L5 vertebra height (SB diameter ratio). Linear regression was used to examine risk factors for dilatation. PN weaning and survival were analyzed with Cox proportional hazards regression. RESULTS Maximal SB diameter ratio during follow-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight, age, PN duration, and remaining bowel length. Weaning off PN was 14-fold more likely in patients with maximal SB diameter ratio <2.00 compared with >3.00 (P = 0.005), and 5.4-fold more likely when maximal SB width was <20 mm compared with >30 mm (P = 0.023). After adjustment for age, remaining SB length, and the presence of ileocecal valve, both estimates of maximal SB dilatation remained significant independent predictors for weaning off PN. When all measurements were included, the cumulative survival was worse if SB diameter ratio exceeded 2.00 (P = 0.002-0.042). CONCLUSIONS SB dilatation predicts prolonged PN duration and decreased survival in SBS children. Measurement of maximal SB diameter standardized to L5 vertebra height may be a valuable objective tool for patient follow-up and assessment of prognosis.
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Hukkinen M, Kivisaari R, Koivusalo A, Pakarinen MP. Risk factors and outcomes of tapering surgery for small intestinal dilatation in pediatric short bowel syndrome. J Pediatr Surg 2017; 52:1121-1127. [PMID: 28185632 DOI: 10.1016/j.jpedsurg.2017.01.052] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/19/2016] [Revised: 11/20/2016] [Accepted: 01/24/2017] [Indexed: 12/12/2022]
Abstract
BACKGROUND In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. METHODS Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients (n=16) to those managed conservatively (n=44) with Cox proportional hazards regression. RESULTS SBS was caused by necrotizing enterocolitis (NEC) (n=31), SB atresia (SBA) (n=13), midgut volvulus (n=12), or gastroschisis (n=4). Patients with spontaneous symptomatic SB dilatation unable to wean parenteral nutrition (PN) underwent tapering surgery at median age of 1.04 (interquartile range 0.70-3.27) years. Missing ICV was related to an 8-fold (p=0.003) increased risk while SBA diagnosis was related to a 13-fold risk of tapering surgery (p<0.001). Increasing SB length and NEC diagnosis were protective of tapering (p=0.027-0.004). Of operated patients, 75% reached enteral autonomy during follow-up and their postoperative adjusted PN weaning rate was similar to nonoperated children (p=0.842). CONCLUSION SBS children with short remaining SB, missing ICV, and SBA etiology are more likely while NEC patients are less likely than others to necessitate tapering surgery. Postoperative PN weaning rates were comparable to patients who initially had more favorable intestinal anatomy and adapted without surgery.
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Affiliation(s)
- Maria Hukkinen
- Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland; Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland
| | - Reetta Kivisaari
- HUS Medical Imaging Center, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland
| | - Antti Koivusalo
- Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland; Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland
| | - Mikko P Pakarinen
- Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland; Section of Pediatric Surgery, Children's Hospital, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
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Abstract
PURPOSE OF REVIEW The goal of this review is to provide updates on the definition, pathophysiology, treatment, and prevention of intestinal failure-associated liver disease (IFALD) that are relevant to care of pediatric patients. RECENT FINDINGS Current literature emphasizes the multifactorial nature of IFALD. The pathogenesis is still largely unknown; however, molecular pathways have been identified. Key to these pathways are proinflammatory cytokines involved in hepatic inflammation and bile acids synthesis such as Toll-like receptor 4 and farnesoid X receptor, respectively. Research for prevention and treatment is aimed at alleviating risk factors associated with IFALD, principally those associated with parental nutrition. Multiple nutrients and amino acids are relevant to the development of IFALD, but lipid composition has been the primary focus. Lipid emulsions with a lower ratio of omega-6-to-omega-3 polyunsaturated fatty acids (FAs) appear to improve bile flow and decrease intrahepatic inflammation. Long-term consequences of these alternative lipid emulsions are yet to be determined. SUMMARY IFALD remains the greatest contributor of mortality in patients with intestinal failure. Many factors contribute to its development, namely, alterations in the gut microbiome, sepsis, and lack of enteral intake. Novel combinations of lipid formulations are promising alternatives to purely soy-based formulas to reduce cholestasis.
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Affiliation(s)
- Cathleen M Courtney
- aDivision of Pediatric Surgery, St. Louis Children's Hospital bDepartment of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA
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Gondolesi GE, Fernandez A, Burghardt KM, Nowakowski S, Kaufman SS, Pascher A, Florescu D, Ruiz P, Vianna R, Clarke S, Oltean M, Rumbo M, Mazariegos G, Sudan DL, Farmer DG. Meeting Report of the XIV International Small Bowel Transplant Symposium: Summary of Presentations, Workshops, and Debates From a Comprehensive Meeting on Intestinal Failure, Rehabilitation, and Transplantation, Buenos Aires, Argentina, June 10-13, 2015. JPEN J Parenter Enteral Nutr 2017; 42:477-489. [PMID: 28437160 DOI: 10.1177/0148607117701696] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2017] [Accepted: 02/23/2017] [Indexed: 11/16/2022]
Abstract
The 2015 meeting of the Intestinal Transplant Association was held in Buenos Aires, Argentina. This was the 14th International Small Bowel Transplant Symposium, and it was the first meeting organized as a joint venture of the Transplantation Society, the Intestinal Transplant Association, and the Argentinean Transplant Society (Sociedad Argentina de Trasplantes). Innovative aspects of the classic meeting format included workshops sessions, debates, and multicenter studies. This report highlights the most prominent scientific contributions and results of the first such symposium in a Latin American country.
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Affiliation(s)
| | | | | | | | | | - Andreas Pascher
- Campus Virchow and Mitte, Charité-Universitätsmedizin Berlin, Berlin, Germany
| | - Diana Florescu
- University of Nebraska Medical Center, Omaha, Nebraska, USA
| | - Phillip Ruiz
- University of Miami/Jackson Memorial Hospital, Miami, Florida, USA
| | - Rodrigo Vianna
- University of Miami/Jackson Memorial Hospital, Miami, Florida, USA
| | - Sara Clarke
- Birmingham Children's Hospital, Steelhouse Lane, Birmingham, UK
| | | | - Martin Rumbo
- Facultad de Ciencias Exactas, Universidad Nacional de La Plata, La Plata, Argentina
| | - George Mazariegos
- University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
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Abstract
Extensive loss of small bowel in all age groups has significant morbidity and mortality consequences. Despite the astonishing ability of the small bowel to compensate for an extensive loss, long-term parenteral nutrition and enteral nutrition, tailored to the need of the patients in relation to the missing intestinal regions is needed. Organ-preserving surgical intervention becomes necessary in patients with a very short intestinal transit time and in an other group of patients with impaired propulsive peristalsis. Intestinal transplantation is indicated in recurrent septical infections or if nearly all of the small bowel is missing. This review discusses indications and risks of the organ-preserving surgical therapies in children with short bowel syndrome.
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Affiliation(s)
- Michael E Höllwarth
- University Clinic for Pediatric and Adolescent Surgery, Medical University of Graz, Auenbruggerplatz 36, 8034, Graz, Austria.
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Barrett M, Demehri FR, Ives GC, Schaedig K, Arnold MA, Teitelbaum DH. Taking a STEP back: Assessing the outcomes of multiple STEP procedures. J Pediatr Surg 2017; 52:69-73. [PMID: 27865472 DOI: 10.1016/j.jpedsurg.2016.10.024] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2016] [Accepted: 10/20/2016] [Indexed: 12/12/2022]
Abstract
PURPOSE Short bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)-associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described. We investigated outcomes of initial STEP compared to ReSTEP procedures. METHODS This retrospective review of STEPs included 17 children and a total of 24 procedures. Demographics, complications, hospital readmission rates, postoperative costs, and PN weaning were analyzed. RESULTS Neither patient-specific data nor the etiology of SBS was predictive of requiring a ReSTEP. PN weaning was more likely in the year following a first STEP (18% wean rate vs. 0% for ReSTEP, p>.05). No ReSTEP patients reached enteral autonomy. Enteral nutrition (%EN) increases were greater after first STEP compared to ReSTEP (26.0% vs. 4.7%, p=0.03). This trend was true for bowel length as well, where first STEPs resulted in a 51% increase in bowel length compared to a 20% increase after in ReSTEP (p=0.02). CONCLUSIONS ReSTEPs failed to result in significant PN weaning, with no ReSTEP patients achieving enteral autonomy during follow-up. Given its higher costs, smaller bowel length gains, and limited ability to produce enteral autonomy, surgeons should carefully consider performing ReSTEP procedures. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Meredith Barrett
- Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan; Ann Arbor, MI.
| | - Farokh R Demehri
- Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan; Ann Arbor, MI
| | - Graham C Ives
- Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan; Ann Arbor, MI
| | - Kristen Schaedig
- University of Michigan Clinical Financial Planning and Analysis Center, University of Michigan Health System; Ann Arbor, MI
| | - Meghan A Arnold
- Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan; Ann Arbor, MI
| | - Daniel H Teitelbaum
- Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan; Ann Arbor, MI
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Parcours de soins du patient adulte souffrant de syndrome de grêle court avec insuffisance intestinale. NUTR CLIN METAB 2016. [DOI: 10.1016/j.nupar.2016.10.129] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
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Hashimoto K, Costa G, Khanna A, Fujiki M, Quintini C, Abu-Elmagd K. Recent Advances in Intestinal and Multivisceral Transplantation. Adv Surg 2016; 49:31-63. [PMID: 26299489 DOI: 10.1016/j.yasu.2015.04.003] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Key Words] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Affiliation(s)
- Koji Hashimoto
- Center for Gut Rehabilitation and Transplantation, Transplant Center, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Guilherme Costa
- Thomas E. Starzl Transplantation Institute, University of Pittsburgh Medical Center, 200 Lothrop Street, Pittsburgh, PA 15261, USA
| | - Ajai Khanna
- Center for Gut Rehabilitation and Transplantation, Transplant Center, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Masato Fujiki
- Center for Gut Rehabilitation and Transplantation, Transplant Center, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Cristiano Quintini
- Center for Gut Rehabilitation and Transplantation, Transplant Center, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Kareem Abu-Elmagd
- Center for Gut Rehabilitation and Transplantation, Transplant Center, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
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Ives GC, Demehri FR, Sanchez R, Barrett M, Gadepalli S, Teitelbaum DH. Small Bowel Diameter in Short Bowel Syndrome as a Predictive Factor for Achieving Enteral Autonomy. J Pediatr 2016; 178:275-277.e1. [PMID: 27587075 DOI: 10.1016/j.jpeds.2016.08.007] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2016] [Revised: 07/14/2016] [Accepted: 08/03/2016] [Indexed: 12/14/2022]
Abstract
Children with short bowel syndrome commonly have dilated small bowel. We found that the extent of dilation was associated with bowel length and that both were related to achieving enteral autonomy.
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Affiliation(s)
- Graham Chester Ives
- Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI
| | - Farokh R Demehri
- Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI
| | - Ramon Sanchez
- Section of Pediatric Radiology, Department of Radiology, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI
| | - Meredith Barrett
- Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI
| | - Samir Gadepalli
- Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI.
| | - Daniel H Teitelbaum
- Section of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI
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