Acute Soft Head Syndrome (Subgaleal Haematoma) with Periorbital Oedema as a Rare Presentation in Sickle Cell Disease.

Abstract

Sickle cell disease is a genetic condition frequently found in Africa and the Arabian Peninsula. Uncommon complications include subgaleal haematoma (soft head syndrome) and periorbital oedema. A 17-year-old male patient presented with body aches and progressive right parieto-temporal and frontal head swelling. Physical examination revealed puffiness of the right eye that progressed rapidly to reddish periorbital oedema sparing the extraocular muscle and pupil response to light. CT and MRI of the brain suggested multiple subgaleal haematomas (soft head syndrome) and right periorbital oedema. Subgaleal haematoma (soft head syndrome) and periorbital oedema are uncommon complications of sickle cell disease. Management is conservative rather than surgical. Subgaleal haematoma concurrently with periorbital oedema is a rare presentation of sickle cell disease.There are no guidelines on treatment, but the conditions in our patient resolved with conservative management.