Acute soft head syndrome in an adult with sickle cell anaemia in Abakaliki, south eastern Nigeria

Abstract

Acute soft head syndrome is a rare complication seen in patients with sickle cell anaemia (SCA). We report the case of a young male adult Nigerian and a known sickle cell patient who presented with complaints of headache, fever and multiple progressive swellings on his scalp, which were unrelated to any trauma events. Full clinical recovery, including complete resolution of the swellings on the scalp, occurred within a period of two weeks without any active intervention other than provision of analgesics and intravenous fluid for a few days at presentation. The pathophysiology of this syndrome is not fully understood but is thought to be related to vaso-occlusive crisis, leading to skull bone marrow infarction, and tearing of small vessels, with extravasation of blood into the subgaleal and epidural spaces. Acute soft head syndrome should be considered in the differential diagnosis of headache and scalp swellings in patients with sickle cell anemia.