ERCP-特发性纤维化胰腺炎

                               
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Here is a case you won’t see very often. A 13 year old girl was believed to have idiopathic fibrosing pancreatitis, a condition first described by Comfort in 1946 and since then, less than 100 cases have been described in the literature. Once bilio-enteric bypass surgery was considered to be gold standard treatment but nowadays temporary endoscopic drainage of the biliary tree is a less drastic alternative.

History:
3 months ago: on holiday developed diarrhoea and vomiting, persisted for 2 weeks.
Back hime, stool cultures showed Salmonella typhi, treated by GP with Ciprofloxacin. The diarrhoea settled.

3 weeks ago: admitted to local hospital with painless jaundice and severe pruritus. Investigations: Bilirubin 74 umol/l, ALT 270, Alk Phosp 300, PT 19sec; US showed 14mm CBD; CT scan: no pancreatic mass; MRCP: 3cm stricture in lower CBD, dilated CHD and IHD; ERCP: normal pancreatic duct, unable to access bile duct despite prolonged attempt. She was also treated with vitamin K, ursodeoxycholic acid and Tazocin. The jaundice and pruritus worsened, so...

Transferred to a specialist paediatric liver unit. Further investigations: Bilirubin 214 umol/L (peaked at 396), ALT 52, Alk Phosp 246, Alb 36, PT 11sec, Hb 10.6 g/dL, WBC 3.8, platelets 340, liver screen: -ve for EBV, CMV, adenovirus, Hep A, B, C and E, normal copper, caeuloplasmin, alpha-1 antitrypsin, liver autoantibodies (ANA, AMA, SMA, LKM) and amylase, IgG4 1.27 g/L (NR less than1.10).

The working diagnosis was idiopathic fibrosing pancreatitis (not biopsy proven) and it was felt that the best option was temporary biliary drainage…so further ERCP attempted (see video)...