Splenectomy in cryptogenetic splenomegaly

Abstract

An attempt which suffers from the defects of a “before and after” study has been made to determine the effects of splenectomy upon 175 patients with cryptogenetic splenomegaly. In 129 there was post-necrotic cirrhosis (group 1) whereas in the remaining 46 the hepatic condition was of less severe degree (group 2). Fourteen patients, 13 in group 1, died in the first three weeks after operation, a mortality of 8%. Observations suggest that the mortality can be reduced, for it would appear that the use of human fibrinogen and of cortisone may prevent respectively “fibrinolytic” Intraperitoneal bleeding and thrombosis of the portal vein, the two major causes of death. Contact with 34 of the 161 patients surviving operation was lost within one to eight months. The late effects of splenectomy have been assessed on the remaining 127. Twenty-six died from 2 months to 8 years (mean 2.1 + 0.4 years) after operation, and the causes of death are presented. Only one of these deaths, from an unrelated disease, occurred in group 2. Of the remaining 25 deaths all save one were related either directly or indirectly to cirrhosis of the liver or to splenectomy. It is concluded that the presence of established cirrhosis of the liver is the most important factor in prognosis. The surviving 101 patients have been observed for from 2 to 9 (mean 4.3 + 0.2) years. After splenectomy the incidence of gastro-intestinal haemorrhage was reduced and its form altered. Bleeding from other mucous surfaces ceased. Presumably these effects were due to the correction of a vascular fragility which occurs in this condition. It is concluded that it is unlikely that the operation influenced bleeding from oesophago-gastric varices. Thrombosis of the portal vein, which has a high incidence in this syndrome, was not encountered after splenectomy. The operation did not result in any permanent effect upon oesophago-gastric varices or upon portal venous pressure. A remarkable beneficial effect was exerted upon ascites, but an explanation of this effect is lacking. After operation there was a significant elevation of the venous haematocrit, the consequence of correction of excessive haemolysis and of reduction in the previously expanded plasma volume, and leucopenia and thrombocytopenia were also corrected. The only significant change in tests of liver function was a reduction in the serum copper oxidase activity, and this was a direct consequence of removal of the spleen. Although splenectomy did not precipitate the occurrence of hepatic encephalopathy, this complication developed for the first time in a number of patients during the period of observation after operation. Chronic ulcers of the leg healed rapidly after operation and no further ulcers developed. In a majority of instances in which they occurred dwarfism, failure in development of secondary sex characters, and amenorrhoea were corrected. Evidence is presented indicating that the operation was followed by an increased incidence of pneumococcal and streptococcal infection and this increase was not restricted to the younger age-group. © 1963, British Medical Journal Publishing Group. All rights reserved.