Improved outcomes in paediatric intestinal failure with aggressive prevention of liver disease.
OBJECTIVE
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 microM/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 microM/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.
Sigalet
D
D
Alberta Children's Hospital/University of Calgary, Pediatric General Surgery, Calgary, Canada. sigalet@ucalgary.ca
Boctor
D
D
Robertson
M
M
Lam
V
V
Brindle
M
M
Sarkhosh
K
K
Driedger
L
L
Sajedi
M
M
D000465:Algorithms; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D001663:Bilirubin / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D013505:Digestive System Surgical Procedures / Q000379:methods; D004750:Enteral Nutrition / Q000379:methods; D005395:Fish Oils / Q000627:therapeutic use*; D005500:Follow-Up Studies; D020139:Gastroschisis / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007409:Intestinal Atresia / Q000601:surgery; D008107:Liver Diseases / Q000517:prevention & control*; D010288:Parenteral Nutrition / Q000009:adverse effects; D017410:Practice Guidelines as Topic; D011446:Prospective Studies; D012778:Short Bowel Syndrome / Q000097:blood / Q000150:complications / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents; D015415:Biomarkers; D005395:Fish Oils; D001663:Bilirubin