Acute soft head syndrome in a sickle cell disease patient

Abstract

Acute soft head syndrome is an extremely rare complication in children with sickle cell anemia. We present a 16-year old male patient known to have sickle cell anemia who presented to our emergency department with low grade fever, headache, skull pain and swelling. Magnetic Resonance of the brain was done demonstrating subgaleal collections overlying calvarial signal abnormalities. The combination of clinical and radiological findings were indicative of acute soft head syndrome. Acute soft head syndrome is a rare complication in children with sickle cell anemia and the pathophysiology is related to osteonecrosis and bone infarcts of the calvarium as well as secondary reactive sub-galeal collections. Treatment includes conservative management with intravenous fluids and analgesics, in addition, our patient was treated with antibiotics since differentiation clinically and by imaging can be challenging. Acute soft head syndrome should be considered in the differential diagnosis of headache and skull swelling in children with sickle cell anemia.