Results of portal decompression in patients with primary biliary cirrhosis.

Twenty-five patients with primary biliary cirrhosis undergoing portal decompression have been followed up for a mean of 51 months. Five patients with decompensated cirrhosis died postoperatively. Overall five year survival of 66% is comparable with that for other forms of cirrhosis but none of the long-term survivors, including three patients with a precirrhotic stage of primary biliary cirrhosis at the time of surgery, developed significant portal-systemic encephalopathy. The results suggest that portal decompression may be the therapy of choice for patients with well-compensated primary biliary cirrhosis who suffer recurrent variceal haemorrhage.