An atypical case of sickle cell disease related orbital roof infarction and review of literature
Author: Li Teng Kok
Base Hospital / Institution: Moorfields Eye Hospital
ePoster presentation
Abstract ID: 25-184
Purpose
Sickle cell disease (SCD) is known to cause painful vaso-occlusive crises in long bones with large marrows. Orbital infarction is a rare complication of sickle cell disease, and usually presents in children and adolescents with acute onset periocular swelling mimicking orbital cellulitis. We aim to describe an atypical case of orbital roof infarction in an adult with homozygous SCD presenting with an isolated unilateral ptosis. We also include a review of 44 cases of sickle cell related orbital infarctions reported in the literature to date.
Methods
Case report. Literature review conducted with search phrase ‘sickle cell’ and ‘orbital infarction’ on PubMed.
Results
We describe an atypical case of a 38 year old man with homozygous SCD who presented with an isolated, complete ptosis of his right eye with minimal swelling. He described brow ache on the same side, with no features of proptosis, ophthalmoplegia or compressive optic neuropathy. Due to the atypical presentation and age group of the patient, initial differential diagnoses were wide including an evolving third nerve palsy and osteomyelitis. Orbital imaging revealed infarction in the right frontal bone and orbital roof, with inflammatory soft tissue swelling. His condition resolved completely after four days with conservative medical therapy alone. Literature review of 44 cases revealed median presenting age of 12 years with a strong male predominance. Homozygous HbSS was the most frequently affected genotype. All cases presented with unilateral (66%) or bilateral (34%) eyelid swelling with or without pain. Previous vaso-occlusive crises was reported in 39%. 91% were treated conservatively with a combination of analgesia, fluids, and red cell exchange. 9% required surgical decompression due to the development of orbital compression syndrome.
Conclusion
Orbital infarction is a rare complication of SCD. It can present in adulthood and clinical presentation may vary. Most cases resolve with supportive management but few sight threatening cases require surgical decompression and early recognition is vital to prevent longstanding visual sequelae.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Mohsan | Malik | Moorfields Eye Hospital |
| Branka | Marjanovic | Moorfields Eye Hospital |
