Acute Soft Head Syndrome: A Case Report in Kuwait and a Clinical Framework for Management

TL;DR

A 15-year-old with sickle cell disease in Kuwait developed acute soft head syndrome, a rare condition causing painful scalp swellings, and recovered with conservative treatment.

Contribution

The paper presents a clinical framework for managing acute soft head syndrome in sickle cell disease patients based on a case report and limited literature.

Findings

The patient showed subgaleal hemorrhages consistent with acute soft head syndrome.

Conservative treatment led to clinical recovery without major interventions.

ASHS should be considered in the differential diagnosis for painful scalp swellings in SCD patients.

Abstract

Sickle cell disease (SCD) is a chronic, inherited hemoglobinopathy associated with multisystem complications, most commonly vaso-occlusive pain crises and stroke; however, rarer manifestations such as acute soft head syndrome (ASHS), characterized by painful scalp swellings from subgaleal hemorrhage, are often overlooked. We report the case of a 15-year-old Kuwaiti male with SCD who presented with a two-day history of headache, multiple scalp swellings, and low-grade fever, alongside a significant history of prior complications, including acute chest syndrome, stroke, splenectomy, and osteomyelitis. Laboratory investigations showed elevated inflammatory markers and leukocytosis, and neuroimaging revealed multiple subgaleal hemorrhages consistent with ASHS. The patient was managed conservatively with empirical antibiotics, hydration, and analgesia, with good clinical recovery. Based on this case and the limited existing literature, we propose a framework to aid early recognition and management of ASHS. This case highlights the importance of considering ASHS in the differential diagnosis of painful scalp swellings in patients with SCD, as timely recognition can support resolution with supportive care and prevent unnecessary interventions.

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