Age Based Therapy

Enteral Nutrition (EN): low-fat pediatric infant formula or fat free formula (adult product, tolerex, calorie modified). Formula prescription needs to provide adequate fat to prevent essential fatty acid deficiency by giving 0.5g/kg/day; or omega 6 fat as 2-4% of caloric intake and omega 3 as 1-2% of caloric intake. If the infant doesn’t tolerate fat at this intake level IV lipid may be necessary to prevent essential fatty deficiency.
Although some infants may require a fat free formula (Tolerex), they still need a small amount of appropriate fat to prevent an essential fatty acid deficiency (EFAD). Therefore, the formula prescription provides at least 0.5g fat/kg/day; or omega 6 fat as 2-4% of caloric intake and omega 3 as 1-2% of caloric intake. If the infant doesn’t tolerate fat at this intake level, IV lipid may be necessary to prevent essential fatty deficiency. Each child has different needs that should be determined in consultation with a pediatric dietitian.
Some centers have created fat free formula that can be made with local resources. The center at Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Ramat - Gan, Israel can be contacted directly for information.
Once ready to introduce solids, maintain a very low fat diet. Encourage fruits, vegetables, low fat whole grains, and proteins (see table of allowed foods). Fat to prevent EFA deficiency can be provided in the form of vegetable or nut oils, if tolerated enterally; parents are encouraged to use a variety of fat (see table consisting of oils rich in omega 3 and omega 6 essential fatty acids).
Most children with DGAT1 deficiency will tolerate very small quantities of oils. The following table contains information about the amounts of essential fatty acids in common oils, which should not be added to the diet without the advice of your child's dietitian and physician.

Oil

g EFA/tsp

Kcal EFA/tsp

4% EFA cal per 1000 cal (tsp)

Almond

0.9

7.8

Canola

1.5

13.3

Corn

2.7

24.3

1.7

Flaxseed

3.3

29.7

1.4

Olive

0.5

4.5

8.9

Soybean

2.9

1.5

Sunflower

3.3

29.6

1.4

Walnut

3.2

28.8

1.4

Reference: McCray S and Parrish CR. Nutritional Management of Chyle Leaks: An Update. Nutrition Issues in Gastroenterology, Series #94. Table 9. Practical Gastroenterology April 2011.

Supplements should include a daily multivitamin and additional calcium and vitamin D, as needed. Your dietitian can perform a nutrient analysis of your infant’s diet and provide individualized vitamin and mineral recommendations.
Some infants may require full or partial parenteral nutrition (PN) if unable to gain adequate weight with enteral nutrition alone. If the infant does not tolerate adequate enteral fat, an IV lipid emulsion should provide only enough fat to prevent essential fatty acid (EFA) deficiency.
Monitor the following nutrition-related lab values: fat soluble vitamins (A,D,E,K), calcium, zinc, iron panel, hepatic panel, cholesterol, triglycerides, electrolytes, fatty acid panel (triene:tetraene ratio) - (Team Note: doesn’t the T:T ratio reflect omega 6 status only) and albumin every 3-6 months.
Generally infants can start baby foods or thin purees between 4-6 months of age. Speak with your physician or dietitian regarding when to introduce solids to your infant. Select the Dietary Recommendations tab for a general guide to texture advancement for infants.

Advance concentration of formula gradually, as tolerated. If able to tolerate milk products, use fat free form.
Maintain well-balanced diet that is very low in fat. Supplement enough fat to prevent EFA deficiency in the form of vegetable or nut oils by giving 0.5g/kg/day or omega 6 fat as 2-4% of caloric intake and omega 3 as 1-2% of caloric intake. (see table below).