Outcomes of Children With Chronic Intestinal Failure

Abstract

Background and Aims: The aim of the study was to aid decisions on prognosis and transplantation; this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne. Methods: Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011. Results: A total of 51 children were included. Forty-two (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome, and 4 (8%) had congenital enteropathies. Median small bowel length for patients with SBS was 45 cm (interquartile range 30–80) or 23.9% of the expected length for age (interquartile range 17.0%–40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2–29.2) with intestinal failure–associated liver disease (IFALD) being the only predictor (P = 0.001). Out of 50 children 21 (42%) had IFALD. Children who were premature (P = 0.013), had SBS (P = 0.038), and/or frequent sepsis (P = 0.014) were more likely to develop IFALD. PN weaning occurred in 27 of 35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (P = 0.025), preservation of the ileocecal valve (P = 0.013) and colon (P = 0.011) being predictors. None of 5 (0%) patients with chronic intestinal pseudo-obstruction syndrome and 2 of 4 (50%) patients with congenital enteropathies weaned off PN. Overall sepsis rate was 7.3 episodes/1000 line days. Frequency of sepsis and longevity of central lines improved with time as patients grew older (both P < 0.001). Conclusions: Long-term PN with intestinal rehabilitation was effective in treating most children with intestinal failure. Children with severe refractory IFALD may have benefited from intestinal transplantation.

In neonatal‐onset surgical short bowel syndrome survival is high, and enteral autonomy is related to residual bowel length

“…This observation is consistent with what has been described in the literature 8,18 . However, unlike previous studies, we did not find any association of BSIs with mortality 3,27 . This is, once again, reflective of improved care that prevents mortality but fails to negate the effect of BSIs on EA.…”

“…It is also important to note that IFALD was not predictive of EA or survival in our cohort. This is different from the majority of previous publications that have found IFALD to be predictive of mortality 8,10,26,27 . In their meta‐analysis of 201 studies on pediatric patients with IF (the vast majority of which were prior to the advent of FOLEs), Pierret et al found that catheter‐related BSIs and IFALD were the only two factors predictive of mortality for their patients 8 .…”

Insuficiencia Intestinal Pediátrica, 10 años de experiencia en una Unidad de Cuidados Especializados

“…La eficacia del manejo entregado en UCE, se ve reflejada en 2 aspectos: El primero, y más importante, es el porcentaje de autonomía enteral al alta y, en segundo lugar, la disminución de la desnutrición al egreso. Respecto a la autonomía enteral, ésta se logró en un 66,7%, siendo comparable con el 56,9% de 15,20,22 . El grupo de pacientes que no lograron autonomía presentaban factores pronósticos desfavorables 20 : clasificación anatómica tipo I y II, ausencia de VIC, remanente intestinal < 40 cm y NM.…”

“…Respecto a la autonomía enteral, ésta se logró en un 66,7%, siendo comparable con el 56,9% de 15,20,22 . El grupo de pacientes que no lograron autonomía presentaban factores pronósticos desfavorables 20 : clasificación anatómica tipo I y II, ausencia de VIC, remanente intestinal < 40 cm y NM.…”

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

“…In the same work, the survival rate was 84% over 20 years in patients with chronic IF managed with long-term PN with reduced mortality related to the reduction in the volume of lipid administered and the use of FO-LEs. The authors found that IFALD was the main predictor of poor outcome, so that patients with severe and refractory IFALD represent the group in which intestinal transplantation may offer benefit ( 82 ). There is evidence about liver fibrosis regression after intestinal transplantation, probably due to the intestinal permeability and gut–liver axis improvement ( 29 ).…”