Twelve‐year outcomes of intestinal failure–associated liver disease in children with short‐bowel syndrome: 97% transplant‐free survival and 81% enteral autonomy

Abstract

Our aim was to analyze the outcomes in children with short‐bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure–associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007–2018. We retrospectively reviewed charts of 135 patients with SBS‐PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2–4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Of the 89 patients, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty‐eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. The median proportion of residual small bowel was 23% (interquartile range, 13%–38%) of the expected length for age and median, daily energy requirement by PN was 100%. Two received a transplant, three died (one posttransplant), and the remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy‐three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of eight of 89 patients with initial platelet count of <100,000/μl(median 50,500/μl) and median CB of 21 mg/dl, seven achieved CB normalization and had improved platelet count. Overall survival was 97% (censored 96.3%). We demonstrate high transplant‐free survival and enteral autonomy rates among children with SBS‐IFALD relying on low‐dose soybean lipid emulsion.

IFALD in children: What's new? A narrative review

“…An increased mortality is reported in patients with IFALD compared to those without it and in children with a more advanced disease. The 3-year survival rate reported is 86% even if Torres et al have recently reported an overall survival of 97% with 81% of patients achieving enteral autonomy in SBS children on low dose of SO-LEs ( 31 – 33 ).…”

Current Surgical Concepts and Indications in the Management of the Short Bowel State: A Call for the Use of Multidisciplinary Intestinal Rehabilitation Programs

“…In fact, a 360-degree multidisciplinary evaluation of the patient state is key to orienting management. Within an IRP, liver-sparing PN is commenced to lower the risk of the alarming IFALD [4][5][6]. Moreover, oral feeding is usually preferred since it more closely resembles the natural physiology, stimulating the bowel mucosa, enhancing adaptation and promoting brain learning.…”

“…This has mainly been due to improved overall care, new total parenteral nutrition (TPN) formulas, and improved surgical techniques [4,5]. However, TPN complications are still a challenge for rehabilitation teams and long-term parenteral nutrition (PN) may lead to intestinal failure-associated liver disease (IFALD) [6,7]. The final goal of treatment in these patients should be to achieve enteral autonomy (EA), minimizing complications.…”

“…The final goal of treatment in these patients should be to achieve enteral autonomy (EA), minimizing complications. Bowel lengthening procedures have increasingly been proposed for long-term TPN patients who fail to improve intestinal function with non-invasive strategies and, in selected groups of patients, bowel transplantation can be an option to consider [6,8,9]. The aim of non-transplant surgery is to restore normal (or close to normal) physiology by optimizing the absorptive surface area, improving peristalsis and decreasing transit time by reshaping morphology (i.e., narrowing and lengthening the bowel by taking advantage of pathologic dilatation).…”

Gastroschisis Outcomes: Presence of Histologic Chorioamnionitis and the Impact on Time to Full Enteral Feeds and Length of Hospital Stay

“…Some cases require extensive intestinal resection and possibly needing bowel and/or liver transplantation, though the latter is becoming less common with the emergence of specialized intestinal rehabilitation teams and newer therapies, such as bowel lengthening procedures, and treatment with fish oilbased lipid emulsions. [5][6][7][8] Gastroschisis induces a chronic inflammatory state in utero, confirmed by multiple analyses of amniotic fluid and fetal blood. These studies have shown the presence of inflammatory proteins, cells, and cytokines within the amniotic fluid, and the presence of systemic inflammation with elevated inflammatory markers in the fetal blood.…”