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

Abstract

Objectives and study: Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus 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. Despite the efficacy and safety of PIBD procedure, some patients do not respond to surgery and there is currently no possibility to predict pruritus response according to genotype or other clinical parameters. Our aim was to evaluate nasobiliary drainage (NBD) as a method to predict pruritus response to PIBD, in order to avoid unnecessary surgery. Methods: We present two PFIC 2 patients who underwent two and three transient endoscopic NBD prior to PIBD surgery, which was performed at the age of 25 and 28 years old respectively. Both patients suffered from invalidating pruritus refractory to medical therapy. Liver transplantation was not performed since both patients had normal liver function and normal liver histology (patient 1) or only very mild fibrosis (patient 2). Pruritus was assessed according to the following score: 0 = none, 1 = rubbing or mild scratching when undistracted, 2 = active scratching without evident skin abrasions, 3 = abrasions evident, 4 = cutaneous mutilation and scarring evident, impaired quality of life. Results: Both patients repeatedly responded to NBD (pruritus score 4 to 0-1) and had complete pruritus resolution after subsequent PIBD (score 4 to 0). Serum total biliary acids (TBA) decreased from 282 µmol/L and 314.4 µmol/L before PIBD to 40.7 µmol/L and 220.9 µmol/L after PIBD respectively (normal TBA levels < 10 µmol/L). Follow-up duration after surgery was seven years for patient 1 and one month for patient 2. Pruritus did not recur after PIBD in our patients despite important variations in TBA levels during follow-up. Mild post-endoscopic biological pancreatitis occurred in 1/5 NBD procedures, and resolved spontaneously. The only adverse effect observed within 7 years post-PIBD was very mild transient osmotic diarrhoea, easily treated with cholestyramine. Conclusion: Our data suggest that NBD is a safe and effective way to predict pruritus response before performing a permanent biliary diversion surgery in PFIC patients