Short-bowel syndrome and intestinal failure — formative SAQs

SAQ 1 — The premature neonate with a high-output jejunostomy (10 marks, 15 minutes)

A baby born at 27 weeks gestation develops necrotising enterocolitis and undergoes a massive small-bowel resection, leaving a 35-centimetre jejunal remnant ending in a jejunostomy with the colon disconnected. On day 14 the stoma output is 60 mL/kg/day, the weight has fallen, and the conjugated bilirubin is rising on parenteral nutrition. [1]

a) Define short-bowel syndrome and intestinal failure, and explain why this child's end-jejunostomy configuration carries a particularly poor prognosis for enteral autonomy. (2 marks) [1]

b) Explain the sodium principle as it applies to this high-output stoma, and state how you would manage the stoma losses acutely. (3 marks) [2]

c) Outline your strategy to prevent intestinal failure-associated liver disease, naming the two dominant modifiable drivers and the lipid approaches used regionally. (3 marks) [5]

d) Describe the staged definitive plan for this child, including the role of enteral feeding, the timing of establishing colon continuity, and the place of teduglutide and transplant. (2 marks) [1] [3]

SAQ 2 — The older child on home parenteral nutrition (10 marks, 15 minutes)

A 4-year-old with short-bowel syndrome from mid-gut volvulus remains on home parenteral nutrition at night despite daytime oral feeding. She has a short jejunal remnant joined to an intact colon. Over six months her weight has stalled and a contrast study shows a dilated, dysfunctional segment of small bowel. [8]

a) What is the role of plasma citrulline in assessing this child, and what does a low or rising level indicate? (2 marks) [11]

b) Give the dose, route and frequency of teduglutide and the mechanism by which it drives adaptation; cite the paediatric evidence. (3 marks) [3]

c) Explain the rationale for a serial transverse enteroplasty in this child, what the procedure does, and what the long-term outcome data show. (3 marks) [8]

d) State the three classical indications for intestinal transplantation, and explain how the decision between continued rehabilitation and transplant is made. (2 marks) [10]