Short-bowel syndrome and intestinal failure — structured clinical encounter

Station 1 — recognition and classification

Asked for my first impression, I explain that a premature baby with a high-output jejunostomy after necrotising enterocolitis resection has intestinal failure from short-bowel syndrome. I would define short-bowel syndrome as the malabsorption following massive small-intestinal resection, and intestinal failure as the broader inability of the gut to sustain growth and hydration. The key prognostic feature here is the end-jejunostomy with the colon disconnected, which carries the poorest outlook of the three anatomical types, in contrast to a jejunoileal configuration with an intact colon and ileocaecal valve which would carry the best. [1]

Station 2 — the sodium principle and acute stabilisation

Asked how I would manage the stoma output of 60 mL/kg/day, I explain that this is a sodium-wasting state. The effluent is rich in sodium at roughly 90 to 100 mmol/L, so the child is in chronic negative sodium balance even with a normal serum sodium, and chronic sodium depletion suppresses growth independent of calories. I would measure the stoma output and its sodium content and replace the losses millilitre for millilitre with an appropriate sodium-containing solution, rather than restricting free water. I would secure central venous access, start parenteral nutrition, and begin trophic enteral feeding early to drive adaptation. [2]

Station 3 — adaptation and the trophic gut

Asked why I would feed the gut when most calories come from the line, I explain that enteral nutrition is trophic, not merely nutritive. Luminal nutrients stimulate villous lengthening and crypt hyperplasia and trigger the release of trophic hormones, especially glucagon-like peptide 2 from the L-cells of the distal ileum and proximal colon, which drives the structural and functional adaptation that can multiply absorptive capacity over months. I would advance enteral feeding, preferring continuous then bolus, with human milk where possible, and wean parenteral nutrition as output and growth allow. [1] [3]

Station 4 — IFALD prevention and the lipid strategy

Asked about the rising conjugated bilirubin, I recognise this as intestinal failure-associated liver disease and state that a rising conjugated bilirubin in an infant on parenteral nutrition is IFALD until proven otherwise. The two dominant modifiable drivers are the parenteral lipid load and recurrent sepsis. I would reduce or modify the lipid, using lipid restriction, a mixed emulsion such as SMOFlipid, or an omega-3 fish-oil-based emulsion, control any line infection with strict asepsis and antibiotics, and advance enteral feeding. I would also actively exclude biliary atresia, because a short-bowel cholestatic neonate needs the same work-up as any cholestatic baby. [5]

Station 5 — the older child and the rehabilitation ladder

Finally I describe how I would handle a 4-year-old with short bowel from volvulus, with a jejunal remnant joined to an intact colon, who is on home parenteral nutrition with a stalled weight and a dilated, dysfunctional segment on contrast study. I would measure a plasma citrulline to gauge functional enterocyte mass, with a low level reflecting reduced enterocyte mass and a rising trend paralleling adaptation. I would consider teduglutide at 0.05 mg/kg subcutaneously once daily, an analogue of glucagon-like peptide 2 resistant to breakdown, supported by the Chiba 2023 paediatric evidence. For the dilated segment I would discuss a serial transverse enteroplasty, which narrows and lengthens the loop to improve function, with the multicentre long-term data supporting parenteral nutrition reduction. I would reserve intestinal transplant for irreversible IFALD, loss of venous access, or recurrent life-threatening line sepsis. [3] [8] [10] [11]