A rare and underrecognized complication of sickle cell disease in a pediatric patient: acute soft head syndrome.
TL;DR
The case of a 16-year-old male with hemoglobin SS (HbSS) SCD who presented to the emergency department with headache and progressive periorbital swelling during a vaso-occlusive crisis is reported.
Abstract
Acute soft head syndrome (ASHS) is an extremely rare and underrecognized complication of Sickle Cell Disease (SCD), and limited awareness can lead to misdiagnosis as infection or trauma. We report the case of a 16-year-old male with hemoglobin SS (HbSS) SCD who presented to the emergency department with headache and progressive periorbital swelling during a vaso-occlusive crisis. MRI demonstrated multifocal calvarial infarctions with overlying hemorrhagic subperiosteal collections and subgaleal edema without evidence of osteomyelitis or orbital cellulitis. Symptoms improved with conservative therapy, including analgesia and packed red blood cell transfusion (pRBC). This report emphasizes the need for a high index of suspicion and familiarity with characteristic MRI findings of calvarial bone infarcts and ASHS, as early recognition can prevent misdiagnosis and support timely optimization of sickle cell care.