Intestinal rehabilitation in pediatric short bowel syndrome: expert strategies for autonomy

Young infants, babies, infants, and young children have tremendous gut growth potential, with the gut growing for the first several years of life.

Residual bowel should be discussed as a percentage of what is normal for a child of that age, not in absolute centimeters.

A term baby will have 160 centimeters of small bowel, and by the time the child is 5 years old, it almost triples to about 425 to 450 centimeters.

A patient can achieve enteral autonomy with as low as 10% expected gut length if they have the majority of their colon in continuity.

The more important factor is the presence or absence of the distal small bowel or ileum and right colon that can act as a reclamation of bile and support the enterohepatic circulation.

The distal ileum produces hormones and incretins like GLP-2, GLP-1, and PYY.

The importance is not the ileocecal valve itself, but rather the distal ileum and proximal colon where bile recirculation occurs and hormones that help intestinal growth and absorption are produced.

The strategy at the first operation should provide a pathway forward that allows early enteral feeding, and it is sometimes better to stage reconstruction under more controlled conditions.

In situations of overwhelming intestinal loss, the strategy is to provide proximal control that allows feeding to occur without the enteral stream going through, and to leave questionable bowel with the understanding that many segments deemed non-usable have potential to heal.

As long as proximal diversion is achieved and the baby is stable, questionable bowel can be left for potential use in later secondary reconstructive procedures.

The overarching principle is to deliver adequate nutrition to have normal growth within normal parameters, ideally enterally.

As much as possible, the goal is to establish normal feeding behavior, recognizing that enteral nutrition is more than just nutrition.

If a patient needs tube support from the beginning, bolus feeding should be tried rather than defaulting to continuous feeding.

When motility issues or delayed gastric emptying prevent tolerance of food into the stomach, the strategy is to feed beyond the stomach and decompress the stomach through an NG tube if the child fails both bolus and continuous gastric feeding.

Breast milk is the formula of choice, not only for its nutritional benefits but for all the other beneficial components within breast milk.

Formulas can be categorized as intact formulas or semi-elemental/elemental formulas, with protein and fat modules being particularly important.

Single amino acid level protein is the generalized preference, ensuring patients are not having high stool output, high fluid losses, wound breakdown, rashes, or emesis.

Long chain fat is the preferred fat module rather than MCT fat, even though there is a feeling that MCT fat is better tolerated in short bowel syndrome.

Long chain fat is a much stronger stimulus for GLP-2 release compared to MCT, which is important when trying to drive adaptation.

Not every child needs to be on SMOFlipid for parenteral nutrition.

For preterm babies, SMOFlipid is not the ideal lipid emulsion but is the best currently available option.

SMOFlipid does not have enough arachidonic acid, which is important for brain development.

There are two strategies to treat cholestasis: reduce the total amount of fat or change the composition to introduce SMOFlipid or omegaven.

Reducing lipid dose to 1 g per kg per day can reverse cholestasis but results in reduced calorie delivery that can impair growth and potentially impact neurocognitive development.

Omegaven is essentially pure fish oil with omega-3 and is dosed at 1 g/kg, so babies take a calorie deficit.

SMOFlipid is a better choice than omegaven as it is a more well-balanced emulsion with an omega-3 to omega-6 ratio of 2.5 to 1, is less inflammatory than intralipid, and promotes bile flow.

SMOFlipid should not be restricted and must be given at no less than 2 or 2.1 g per kg per day.