Improved Outcomes in Paediatric Intestinal Failure with Aggressive Prevention of Liver Disease

Abstract

Background/Purpose: A protocol-driven care algorithm for the care of intestinal failure (IF) centred on therapies to prevent Parenteral Nutrition Associated Cholestasis (PNAC) was instituted in 2006. We report our results from 2006–2009, and compare them to the outcomes of our previous cohort of patients (1998–2006).

Methods: With regional ethics board approval, we have been prospectively gathering data on patient with IF cared for by our regional surgical unit. IF was defined as a residual bowel length of <40 cm or a requirement for PN for greater than 60 days. With the development of a multidisciplinary care team, a protocol-driven strategy to prevent PNAC was instituted in 2006, with aggressive introduction of enteral feeds, use of prophylactic antibiotics to prevent bacterial overgrowth, lipid reduction and use of a fish oil-derived lipid preparation for cholestasis and Serial Transverse Enteroplasty (STEP) if bowel dilation occurred.

Results: In the era from 1998–2006, 33 patients were identified, with a 72% survival; the direct bilirubin averaged 112±34 μM/L after 3 months of PN. 8/33 (27%) of patients received prophylactic antibiotics, and none received fish oil-based lipids. The most common causes of IF were gastroschisis (30%) and atresia (21%); 31 of 33 patients were infants. Average time to intestinal rehabilitation/death was 4.5±3 months. All deaths were related to sepsis or PN/liver failure. In the era from 2006–2009, 22 patients have been followed, with 100% survival*. Average bilirubin after 3 months of PN was 8±2.2 μM/L*, 20/22 (90%)* received prophylactic antibiotics, and 6/22(27%)* received fish oil-based lipid PN. The common causes of IF were gastroschisis 15/22 (68%) and atresia (27%). 18/22 are weaned from PN, and the average time to intestinal rehabilitation was 2.7±1.3 months, 4 patients underwent STEP procedures. (*p<0.05 by Fischer's exact or Student's t-test, data mean±SD).

Conclusions: The institution of an aggressive protocol of advancing enteric feeds, oral antibiotic prophylaxis for bacterial overgrowth, fish oil-based lipid use, and the STEP procedure for dilated bowel has resulted in an apparent increase in survival and a remarkable improvement in liver function in a paediatric IF population. Further studies to define the relative importance of these therapies are recommended.

Key words

TPN - Omegevan - short bowel syndrome - STEP procedure - bacterial - overgrowth

References

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