Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II

Eur J Pediatr. 2020 Oct;179(10):1547-1552. doi: 10.1007/s00431-020-03646-z. Epub 2020 Apr 14.


Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus that is refractory to medical therapy. Surgical biliary diversion techniques, including partial internal biliary diversion (PIBD), have been developed over the years to relieve pruritus without requiring liver transplantation. No clinical or genetic features can currently predict postoperative pruritus response. We present three PFIC type 2 (PIFC 2) patients who underwent transient endoscopic nasobiliary drainage (NBD) prior to PIBD surgery. Two patients repeatedly responded to NBD and presented with complete pruritus resolution after subsequent PIBD. NBD failed technically in the third patient, and PIBD was partially successful. Mild post-endoscopic biological pancreatitis occurred in 2/6 NBD procedures and resolved spontaneously. The only adverse effect observed within 7 years post-PIBD was very mild transient osmotic diarrhea.Conclusion: Our limited data suggest that NBD is a safe and effective way to predict pruritus response before performing permanent biliary diversion surgery in PFIC patients. What is Known: • Surgical biliary diversion techniques have been developed to relieve intractable pruritus in progressive familial intrahepatic cholestasis (PFIC). • No clinical or genetic features can currently predict pruritus response to surgery. What is New: • Our data suggest that nasobiliary drainage could be a safe and effective tool to predict pruritus response to biliary diversion and avoid unnecessary surgery in PFIC patients.

Keywords: Bile acids; Biliary diversion; Familial cholestasis; Nasobiliary drainage; Pruritus.

MeSH terms

  • Biliary Tract Surgical Procedures*
  • Cholestasis*
  • Cholestasis, Intrahepatic* / genetics
  • Cholestasis, Intrahepatic* / surgery
  • Drainage
  • Humans

Supplementary concepts

  • Cholestasis, progressive familial intrahepatic 1