Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sickle cell disease is the most common hemoglobinopathy. Homozygous patients are prone to vaso-occlusive crises. A 19-year-old male patient with the homozygous sickle cell trait was admitted to the hospital due to a sickle cell crisis. During his admission he developed a left periorbital edema. The diagnosis of a subperiosteal orbital hematoma (SOH) was made by CT and MRI imaging. SOH is a rare complication of a VOC. The clinical course is mostly self-resolving, with some cases reporting the need for surgical decompression when orbital compression syndrome is clinically diagnosed. Differentiation between infection on imaging is necessary for further treatment.
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PMID:Subperiosteal Orbital Hematoma: Imaging Findings of a Rare Complication of Sickle Cell Disease: Subperiosteal orbital hematoma is a rare entity mainly seen in pediatric patients with sickle cell disease and occurs secondary to local vascular disturbances following facial bone infarction. 3127 94

An 18-month-old boy with sickle cell disease developed progressive left periorbital edema and temporalis fullness. Imaging revealed two inferotemporal subperiosteal fluid collections and adjacent temporalis muscle thickening. After undergoing an anterior orbitotomy with drainage and biopsy, the patient was diagnosed as having a vaso-occlusive sickle cell crisis involving the lateral orbit. [J Pediatr Ophthalmol Strabismus. 2020;57:e78-e81.].
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PMID:Vaso-occlusive Infarction of the Lateral Orbital Wall With Ipsilateral Temporalis Muscle Ischemia. 3309 Feb 31