Abstract
Hereditary spherocytosis is not a rare disease, having a prevalence of approximately 70,000 individuals in the United States. The major clinical manifestations of splenomegaly, hemolytic anemia, and jaundice are cured by splenectomy. Because of the heterogeneous clinical presentation of the disease, many individuals are not aware of their disease and are at a theoretical risk of traumatic rupture of the spleen from relatively minor abdominal trauma. Because splenectomy is curative for nearly all individuals with hereditary spherocytosis anti because biliary tract pathology is common, we recommend that all patients with confirmed hereditary spherocytosis and splenic injury undergo splenectomy. These patients should not be managed nonoperatively, as splenectomy is curative. It is important that trauma surgeons have a good understanding of the pathophysiology and treatment of this disease.