Monthly Key Publication Reviews

Publication: Norsa L, Goulet O, Alberti D, DeKooning B, Domellöf M, Haiden N, Hill S, Indrio F, Kӧglmeier J, Lapillonne A, Luque V, Moltu SJ, Saenz De Pipaon M, Savino F, Verduci E, & Bronsky J. Nutrition and Intestinal Rehabilitation of Children with Short Bowel Syndrome: A Position Paper of the ESPGHAN Committee on Nutrition. Part 1: From Intestinal Resection to Home Discharge. J Pediatr Gastroenterol Nutr. 2023 Aug 1;77(2):281-297. doi: 10.1097/MPG.0000000000003849. Epub 2023 May 31. PMID: 37256827.

Reviewer: Jeremiah Torrico, RND, MD, DPPS, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of the Philippines-Philippine General Hospital. Senior Lecturer, Department of Nutrition, College of Public Health, University of the Philippines-Manila

Why is This Paper Important: Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric age group1. Although parenteral nutrition (PN) is considered to be the first line of treatment, enteral nutrition (EN) plays a vital role in intestinal adaptation, cholestasis prevention, and maintenance of a healthy microbiota. However, SBS can lead to complications independent of the mode of nutrient administration, owing to the nature of the involved intestinal functional loss in the background of increasing nutritional demands.

There are limited randomized controlled trials investigating the effects of different modalities for SBS management in childhood. Their heterogenous results, together with differing expert opinions, make it difficult for clinicians to come up with best practices. There is a need to review current available evidence on SBS in order to guide clinical management, while preventing the associated complications, as well as improve physiological intestinal adaptation, eventually wean from PN, and support adequate growth. 

Summary: This is a position paper by the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) on nutrition and intestinal rehabilitation of children with SBS based on a systematic literature review. It aims to provide evidence based recommendations and practice points for pediatric patients with small intestinal resection who are at risk of developing SBS-IF requiring PN and eventual home parenteral nutrition (HPN) support. 205 articles were included for final selection and references were reviewed individually, with recommendations decided upon by voting in which a consensus of at least 90% was required. These were then rephrased until all authors agree with the final version for publication. 

After surgery, patients undergo major metabolic stress and the expected response differs depending on the postoperative phases. These can be differentiated into early acute (first 24–48 hours), late acute or intermediate postoperative (first week after surgery), and recovery or late postoperative (from first week until discharge from intensive care). During the early acute phase, fluid management and resuscitation are essential, with the need to calculate fluid and electrolyte requirements considering residual intestinal anatomy, presence of stoma, and levels of intestinal losses, while monitoring weight and urinary sodium sediment. PN should supply energy in the form of glucose under tight glycemic control, with the goal of exclusively maintaining physiologic state. In the intermediate postoperative phase, lipid as energy source should be supplied as part of PN, with composite intravenous lipid emulsions containing fish oil in patients for prolonged PN. Energy provision should be gradually increased considering enteral intake and intestinal malabsorption. Urine sodium levels should be monitored daily, maintaining the level at >20 mmol/L, while exceeding urinary potassium (NaU/KU >1) in patients who are not in diuretics. In the late postoperative phase, cycling PN should be initiated in all stable patients over 4 kg. Energy intake should be adjusted, taking into account cumulative deficits, tissue repair, and catch-up growth, while screening for micronutrient deficiency.

Feeding through the enteral route also plays a vital role and should be initiated as soon as possible, with oral feeding being the preferred route of administration. In cases where oral route is not possible, enteral tube feeding can be done, although there is a lack of evidence as to which is better between continuous and intermittent tube feeding. Human milk is the first line option and polymeric formula can be used in its absence. If both are not tolerated, extensively hydrolyzed formulas (EHF) are recommended as they are well tolerated and provide short peptides. The amount of feed to be given should be based on stoma output per day and surveillance of feeding tolerance should be assessed using multifactorial clinical evaluation of abdominal distension, vomiting, and stool output. Increasing the enteral feeds at 10–20 mL/kg/day may be attempted if stool output is <20–50 mL/kg/day or 6–10 stool/day. 

Since SBS and IF are chronic conditions, HPN should be considered in patients expected to require PN for more than 12 weeks as it has been demonstrated to be associated with less IF-related complications and better quality of life. Parents and/or caregivers should be trained on how to manage central venous catheters (CVC) and PN within a structured HPN program, including the use of antiseptic locks. Prior to discharge, it is essential that those who would undergo HPN should have a secure CVC, stable fluid and electrolyte needs, and should tolerate PN infusion breaks.

Commentary: This is the latest position paper summarizing key practice points and recommendations for the nutritional management of SBS based on current available evidence and expert opinion. The recommendations on PN are mainly divided into different phases in relation to expected physiologic changes. This is in conjunction with the division of treatment phases from time of surgery in critically ill neonates in which nutritional support is recommended to be adjusted based on the level of metabolic stress response and hepatic transition to anabolic protein metabolism when growth reoccurs, while avoiding overfeeding during the early catabolic phase of illness and underfeeding during recovery2. EN is likewise emphasized with immediate establishment of enteral feeding similar to ASPEN recommendations in pediatric SBS with some differences including the feed of choice3. ESPGHAN suggests polymeric formula as the second choice instead of EHF, noting that the former has higher concentration and higher long chain triglyceride content which improves intestinal adaptation. HPN was also noted as an integral part of management as it was shown to improve outcomes in those requiring prolonged PN support4. Although successful weaning from HPN is possible, the paper emphasizes caution to prevent HPN related complications such as central-catheter-related complications, IF-associated liver disease, and mortality5

Although this position paper establishes clear guidelines on the nutrition support in pediatric SBS, the quality of evidence is difficult to assess due to the differences in types of studies involved, most of which were retrospective cohort studies. There is need for more studies, particularly randomized controlled trials, to ascertain the quality of recommendations. Newer studies with longer follow-ups, including those involved in HPN, are necessary to determine the long term effects of these recommendations, as SBS and IF are considered to be chronic diseases which are at risk for more complications over time.

References:

  1. Merritt RJ, Cohran V, Raphael BP, Sentongo T, Volpert D, Warner, BW, Goday, PS, & Nutrition Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Intestinal Rehabilitation Programs in the Management of Pediatric Intestinal Failure and Short Bowel Syndrome. J Pediatr Gastroenterol Nutr, 65(5), 588–596. https://doi.org/10.1097/MPG.0000000000001722. PMID: 28837507. 
  2. Moltu SJ, Bronsky J, Embleton N, Gerasimidis K, Indrio F, Köglmeier J, de Koning B, Lapillonne A, Norsa L, Verduci E, Domellöf M, & ESPGHAN Committee on Nutrition. Nutritional Management of the Critically Ill Neonate: A Position Paper of the ESPGHAN Committee on Nutrition. J Pediatr Gastroenterol Nutr. 2021; 73(2), 274–289. doi: https://doi.org/10.1097/MPG.0000000000003076. PMID: 33605663.
  3. Channabasappa N, Girouard S, Nguyen V, & Piper H. Enteral Nutrition in Pediatric Short-Bowel Syndrome. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2020; 35(5), 848–854. https://doi.org/10.1002/ncp.10565. PMID: 32815247.
  4. Beath SV, Gowen H, Puntis JWL. Trends in paediatric home parenteral nutrition and implications for service development. Clin Nutr. 2011;30:499–502. doi:10.1016/j.clnu.2011.02.003. PMID: 21388723 
  5. Chen YC, Chou CM, Huang SY, Chen HC. Home Parenteral Nutrition for Children: What Are the Factors Indicating Dependence and Mortality?. Nutrients. 2023;15(3):706. Published 2023 Jan 30. doi:10.3390/nu15030706. PMID: 36771412.
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