Isolated Unilateral Orbital Compression Syndrome in A 19-Year-Old Male With Homozygous Sickle Cell Disease

Abstract

This study aimed to report a rare case of a rapidly progressive isolated unilateral orbital compression syndrome in a male with homozygous sickle cell disease, who presented with proptosis and optic nerve dysfunction. He neither had long bone pain crisis nor fever at the time of presentation that was managed surgically to preserve vision.Rapidly progressive left orbital swelling is observed in a 19-year-old homozygous sickle cell disease patient associated with severe pain, headache, and impaired vision. Computed tomography of the orbit confirmed the presence of a unilateral large superior sub-periosteal cystic mass. Surgical exploration via anterior orbitotomy revealed a large sub-periosteal hematoma occupying the superior orbit which was evacuated. The patient completely recovered within 14 days post-surgery and regained his vision.Orbital involvement in sickle cell disease is rare, however, it can occur as a sequela of vaso-occlusive crisis and bone marrow infarctions leading to bleeding and sub-periosteal hematomas in the orbit. Prompt diagnosis and management of orbital compression syndrome are crucial to prevent permanent optic nerve damage. Hence, cautious evaluation and close monitoring are important, especially in cases where surgical evacuation is indicated for quick recovery and prevention of visual loss.