Which of the following statements or descriptions typically characterizes the syndrome of overwhelming postsplenectomy sepsis?

D. A syndrome of rapidly appearing septic shock unresponsive to antibiotic therapy, with an average mortality of 50%.

DISCUSSION: In 1952 King and Schumaker suggested that children who had undergone splenectomy were at risk for the development of bacterial infections, and the syndrome of overwhelming postsplenectomy sepsis (OPSS) was suggested by Diamond in 1969. The syndrome is unlike fulminating bacteremias and septicemia in individuals with normal splenic function. The onset is sudden, with nausea, vomiting, headache, and confusion leading to coma. The new infecting organism is a gram-positive organism in over half the cases, primarily Streptoccoccus pneumoniae. Blood cultures may occasionally demonstrate up to as many as 10 6 bacterial organisms per cu. mm. circulating in the bloodstream. Disseminated intravascular coagulation is common along with hypoglycemia, electrolyte imbalance, and shock unresponsive to antibiotics and fluid or pharmacologic support. Mortality has generally been reported as high as 50% and even up to 80% for pneumococcal infections. The true incidence of overwhelming postsplenectomy sepsis following a splenectomy from trauma is not well defined. Green and colleagues suggested that the risk of OPSS is 166 times the rate expected for the general population. Eraklis and Filler suggested that the incident rate of mortality from sepsis and OPSS is 78 times greater than that expected for the general population. Despite this increased frequency, overwhelming postsplenectomy sepsis remains a rare event. Singer's large review of 688 children who had undergone splenectomy for trauma demonstrated only a 1.45% incidence of postsplenectomy sepsis, but a 40% mortality. The occurrence of OPSS appears to be less following splenectomy for trauma when compared with splenectomy for congenital hematologic disorders. Nonetheless, the recognition of the severe nature of this entity has prompted many trauma surgeons to more aggressively attempt splenic salvage. Animal laboratory evidence suggests that at least 50% of the splenic tissue mass must be preserved to prevent overwhelming postsplenectomy sepsis. The immunologic function of the spleen that appears to be most beneficial in preventing OPSS is the spleen's capacity for clearance of blood-borne particles and the provision of circulating opsins, which assist in cell-mediated immunologic functions.