Presentation
Known case of sickle cell disease, presented in emergency department with unilateral (left) painful progressive proptosis and complete ophthalmoplegia for two hours. No history of head trauma.
Patient Data
A well-defined heterogeneous mixed density extra-conal lesion measuring approximately 25 x 30 x 30 mm, displacing the eyeball anteriorly (proptosis) is seen in the left superolateral orbital cavity. A small mildly hyperdense extra-conal lesion measuring 5 x 14 x 15 mm, without any significant mass effect is seen in the right superolateral orbital cavity. No intra-orbital abnormality is seen. No orbital or skull bone fracture is seen.
Re-accumulation of left retrobulbar hemorrhage (history of emergent surgical decompression of left orbital hematoma one day ago). Mild thickening of the left peri-orbital soft tissues is noted which is likely related to the recent surgery. An interval increase is noted in the size of right retrobulbar hemorrhage. Interval development of mixed density left frontal epidural hematoma associated with mass effect over the left frontal lobe, left lateral ventricle & mild right-sided mid-line shift of about 7 mm. Small left subdural hematoma is also noted.
Status post left frontal craniotomy with almost complete evacuation of the extradural hematoma and resolution of the mass effect. Small residual left subdural hematoma. Stable bilateral retro-bulbar hemorrhages.
Minimal residual left subdural hematoma. Complete interval resolution of right retro-bulbar hemorrhage whereas significant interval improvement is seen in the left retro-bulbar hemorrhage.
The patient underwent emergent evacuation of the left retrobulbar hematoma due to acute orbital compression syndrome. Follow-up scan next day showed bilateral retrobulbar hematomas which were managed conservatively and interval development of left epidural hematoma which was managed surgically.
Case Discussion
Vaso-occlusion of the orbital bone marrow leading to orbital wall infarction is a rare clinical presentation of sickle cell disease1. It is commonly seen in young children as compared to the adults due to abundance of marrow space in the orbital bone in children1. In sickle cell disease, a classical feature of orbital wall infarction is hemorrhage which may be epidural (intracranial) or subperiosteal (orbital)1. Extravasation of blood from the diseased/necrotic vessels, underlying bleeding disorder and minor injury are the few proposed mechanisms of subperiosteal hemorrhage. Subperiosteal hemorrhage without neurologic manifestations can be managed conservatively whereas large hematomas or hematomas with orbital compression syndrome need emergent surgical decompression to preserve the ocular function1.