Orbital compression syndrome in sickle cell disease

Orbital compression syndrome is a rare but serious manifestation of sickle cell disease. The presented case describes a seven year old girl with sickle cell disease in a pain crisis. Despite conservative management the crisis proceeded. Besides pain and swelling of the left orbit the patient developed double vision with restriction of ocular motility. The CT scan showed the extensively protruded eyeball on the left side due to a retrobulbar haematoma. In the followings amaurosis and total loss of pupil reaction appeared, so that immediate surgical decompression was indicated to remove the subperiostal haematoma. Subsequent to haematoma release the pupil reflex was restored immediately with good prognosis for the visual function. The case demonstrates the fulminant sight-threatening history of an orbital haemorrhage due to sickle cell disease, where immediate recognition and early surgical treatment is required.