Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis

BackgroundPatients with progressive familial intrahepatic cholestasis (PFIC) often require liver transplantation to survive. An alternative approach is surgical diversion of bile, that is, partial external biliary diversion (PEBD). The aim of the study was to describe 13 patients with PFIC who have undergone PEBD.MethodsClinical and laboratory workups including growth data and histology specimens were analyzed to evaluate the short-term effects of PEBD. Follow-up, including liver biopsies, was performed 11 to 21 (median, 14) months post-PEBD.ResultsAll patients showed typical features of PFIC. Eight out of 13 presented with variable signs of coagulopathy, and one patient presented with hypocalcemic seizures. The surgery was uneventful in all, but 4 patients were readmitted because of dehydration and electrolyte imbalance caused by excessive stomal losses. One month post-PEBD, 7 patients were apruritic. One patient had stomal dysfunction, showed no improvement on cholestasis after surgery, and had to undergo liver transplantation 2 months post-PEBD. At follow-up, significant biochemical improvement and gains in growth were seen in most of the patients.ConclusionsMost of the patients with PFIC presented with signs of coagulopathy. Partial external biliary diversion had a dramatic effect on cholestasis and growth, although not all patients benefited from the surgery. Episodes of dehydration post-PEBD must be considered.