Portacaval shunt in four patients with homozygous hypercholesterolaemia

Four patients with homozygous hyperbetalipoproteinaemia who had proved resistant to intensive medical therapy have undergone portacaval shunt. During 3 weeks of total parenteral alimentation before the operation, the serum-cholesterol decreased significantly in each of the four patients. During the fat-emulsion phase of hyperalimentation, one patient experienced a rise in cholesterol. Postoperatively, the size of xanthomas decreased in all cases; serum-cholesterol levels rose above those achieved during hyperalimentation, and this rise continued for as long as 3 months before a further reduction was recorded. In patients with angina, this improved considerably; and in most cases bruits decreased in itensity. The results at this stage do not, however, suggest portacaval shunt for all patients with homozygous hypercholesterolaemia.