A rare and underrecognized complication of sickle cell disease in a pediatric patient: acute soft head syndrome
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Updated: Aug 25, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Aanchal Gupta1, Blaise Jones2, Dan Pham3
1Department of Radiology, Division of Neuroradiology, University of Cincinnati Medical Center, Cincinnati, OH, USA. guptaac@ucmail.uc.edu.
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.
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