Acute Soft Head Syndrome, A Rare Complication of Sickle Cell Anemia: A Case Report

Acute soft head syndrome (ASHS) is a rare clinical complication of sickle cell disease (SCD). It presents as a progressive, non-traumatic, and tender swelling of one or more areas of the scalp. Aetiopathophysiology is still less understood but hypothesized to be a result of loss of cortical skull bone integrity from increased extramedullary hematopoiesis. Diagnosis is clinically made. However, imaging is very necessary to rule out an intracranial involvement, commonly an epidural hematoma. ASHS with no intracranial involvement is managed conservatively. We present the case of a 15-year-old adolescent living with SCD who presented with non-traumatic tender swellings of the scalp with no other form of SCD crises. A non-contrast head CT scan was done, which reported blood collection in the subgaleal space of the frontal and occipital regions with no associated bone fractures and intracranial bleeds. Both the patient and his parents were counseled on the condition and the need for admission. The patient received empirical antibiotics, adequate analgesia, hydration, and adequate bed rest for a total of 6 days on admission. There was marked resolution of the swelling on the day of discharge. ASHS is a rare clinical entity with limited literature and awareness. The aim of this case report is to impact knowledge and create awareness of ASHS.