Work

A rare and underrecognized complication of sickle cell disease in a pediatric patient: acute soft head syndrome

Year: 2026

Type: article

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.

Source: Emergency Radiology

DOI: 10.1007/s10140-026-02531-w

Authors: Aanchal Gupta, Blaise Jones, Dan Pham, Omar Niss, Marguerite Caré

Institutions: University of Cincinnati, University of Cincinnati Medical Center, Cincinnati Children's Hospital Medical Center

Language: English

Topic: Hemoglobinopathies and Related Disorders

Subfield: Genetics

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $3,990