Pancreatitis and pancreatic disorders — branching viva

Station opening

Examiner: "Define paediatric pancreatitis and outline your approach to a child presenting with suspected acute disease." [1]

Strong candidate (must-hit)

• Defines paediatric pancreatitis by the NASPGHAN two-of-three criteria: at least two of characteristic abdominal pain, a serum lipase or amylase at or above three times the upper limit of normal, and imaging consistent with pancreatitis; frames the approach around recognising the clinical picture, sending the lipase early, confirming with ultrasound, and delivering early aggressive hydration, adequate analgesia and early enteral feeding, while identifying and treating the underlying cause; states that the commonest paediatric causes are medications, trauma and genetic variants rather than gallstones and alcohol. [1]

Weak candidate

• "Pancreatitis is inflammation of the pancreas, and I would give fluids, antibiotics and keep the child nil by mouth." [2]

Branch A — The eight-year-old on valproate

Examiner: "An eight-year-old boy on valproate for epilepsy presents with severe epigastric pain radiating to the back, vomiting and a lipase six times the upper limit of normal. Outline your management and the action regarding the drug." [9]

Strong

• Diagnoses acute pancreatitis from the two-of-three criteria (pain, lipase above three times the upper limit of normal) and identifies valproate as the likely trigger; starts early aggressive isotonic crystalloid hydration at one and a half to two times maintenance for the first twenty-four to forty-eight hours; provides adequate analgesia with age-appropriate opioids; plans early enteral feeding within forty-eight to seventy-two hours as the child improves; stops valproate and engages the neurology team for an alternative antiseizure medication; does not give prophylactic antibiotics. [2] [9]

Weak

• "I would continue the valproate and add antibiotics, and keep him fasting until the lipase normalises." [2]

Branch B — The ten-year-old with three episodes and a family history

Examiner: "A ten-year-old girl has had three episodes of pancreatitis with full recovery between them. Her father had childhood recurrent abdominal pain and pancreatic cancer at forty-five. What is your investigation plan?" [5]

Strong

• Confirms the INSPPIRE definition of acute recurrent pancreatitis (two or more discrete episodes with full inter-episode recovery); identifies the likely hereditary pancreatitis from the autosomal dominant family history and the early-onset cancer; sends a PRSS1 gene analysis as the first-line genetic test, with a broader panel covering SPINK1, CFTR, CTRC and CASR; arranges a magnetic resonance cholangiopancreatography for ductal anatomy; sends a faecal pancreatic elastase for early exocrine insufficiency; and plans genetic counselling and cancer surveillance in adulthood, because PRSS1 mutations carry a high lifetime risk of pancreatic cancer. [5]

Weak

• "I would just monitor her and treat each episode as it comes." [5]

Branch C — The twelve-year-old with chronic pancreatitis and steatorrhoea

Examiner: "A twelve-year-old with known chronic pancreatitis now has steatorrhoea and poor weight gain. Her faecal elastase is one hundred and twenty. How do you manage her exocrine insufficiency?" [4]

Strong

• Confirms exocrine pancreatic insufficiency from the faecal elastase below two hundred micrograms per gram; starts pancreatic enzyme replacement at approximately five hundred to one thousand lipase units per kilogram per meal with about half that per snack, taken with all meals and snacks containing fat, titrated to symptom control; states the daily maximum of ten thousand lipase units per kilogram per day to avoid fibrosing colonopathy; adds fat-soluble vitamin supplementation and dietitian support; screens for glucose intolerance as the endocrine reserve may also be failing; and coordinates care with paediatric gastroenterology, dietetics and endocrinology. [4]

Weak

• "I would tell her to eat a high-fat diet and take a multivitamin." [4]

Branch D — The fourteen-year-old being considered for total pancreatectomy

Examiner: "A fourteen-year-old with intractable chronic pancreatitis is on high-dose opioids and has been referred for total pancreatectomy with islet autotransplantation. Explain the rationale and the consequences of the procedure." [12]

Strong

• Explains that total pancreatectomy with islet autotransplantation is a last-resort option for the child whose pain is refractory to medical and endoscopic management; the pancreas is removed to eliminate the source of pain, and the islets are isolated and infused into the liver via the portal vein to preserve some endogenous insulin secretion; the evidence shows it reduces opioid use and improves quality of life in carefully selected children; the consequence is insulin-dependent diabetes in most patients, because islet survival is incomplete; the decision is made by a multidisciplinary team including paediatric gastroenterology, surgery, endocrinology, psychology and pain services; and it is performed only at specialist centres. [12] [5]

Weak

• "She should just have a pancreatectomy to cure the pain." [12]

Close

Examiner: "Summarise your approach to the child with suspected pancreatitis in one sentence." [1]

Strong

• "Paediatric pancreatitis is defined by the NASPGHAN two-of-three criteria, managed with early aggressive hydration, adequate analgesia and early enteral feeding within forty-eight to seventy-two hours without prophylactic antibiotics, and driven by finding the cause, because the child with a PRSS1 mutation or a valproate trigger needs cause-specific management to prevent the progression to chronic disease, exocrine insufficiency and cancer." [1] [2]