Long-term outcomes of six patients after partial internal biliary diversion for progressive familial intrahepatic cholestasis

Background

Partial internal biliary diversion (PIBD) is an alternative approach for the treatment of devastating pruritus in patients with progressive familial intrahepatic cholestasis (PFIC). In these patients quality of life can be improved and progression of liver disease can be delayed while waiting for liver transplantation. The aim of our study was to evaluate six patients with PFIC who have undergone PIBD in long-term follow-up.

Methods

Retrospective review of the records of six patients who underwent PIBD for PFIC between 2008 and 2010 was conducted to evaluate age, growth, clinical and laboratory studies for long-term outcome.

Results

Serum postoperative bile acid levels were reduced from a mean 340.1 μmol/L (range 851–105) preoperatively to a mean of 96.3 μmol/L at postoperative fifth year. The difference between pre- and postoperative bile acid levels was statistically significant (p = 0.018). AST decreased from 79.1 U/L (range 43–150 U/L) to 64.6 U/L (range 18–172 U/L), ALT decreased from 102.8 U/L (range 35-270 U/L) to 84.6 U/L and total bilirubin decreased from 2.9 μmol/L (range 0.35–6.4 μmol/L) to 1.53 μmol/L (range 0.3–2.4). Again, the decrease in total bilirubin levels was significant (p = 0.043). Pruritus was diminished from a mean of + 4 (range 4–4) preoperatively to a mean of + 2 (4–0). One patient who underwent liver transplantation owing to relapsing pruritus died from postoperative sepsis in the early postoperative period at the fifth year after PBID. Five symptom-free patients have not required liver transplantation at a mean period of 6.1 ± 0.83 years (5.1–7.0 years) follow-up.

Conclusion

PBID is an effective surgical procedure in the long-term and can delay the need for liver transplantation in children with PFIC by reducing jaundice and pruritus.