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Orbital vaso-occlusive crisis mimicking an abscess in pediatric sickle cell disease: A diagnostic pitfall
Daniel C Fong1, Wiktoria A Gocal2, Amal Isaiah1,3,4,5
1Department of Otorhinolaryngology-Head and Neck Surgery, University of Maryland School of Medicine, Baltimore, MD, USA.
Abstract:
Sickle cell disease (SCD) is characterized by vaso-occlusive crises (VOC) that typically affect bones with high marrow content. Orbital involvement is rare, observed more frequently in children due to their greater marrow space and rich vascularization. Orbital VOC can clinically and radiologically mimic orbital abscesses, posing significant diagnostic challenges. We present a case of a 6-year-old boy with SCD who developed an orbital VOC initially mimicking an orbital abscess. The patient presented with 4 days of progressive right eye swelling and systemic inflammatory signs. A contrast-enhanced computed tomography demonstrated findings suggestive of subperiosteal abscess, preseptal abscess, subdural empyema, and sinusitis. Subsequent magnetic resonance imaging showed features that could represent either an orbital hematoma or abscess formation. Given the constellation of clinical and radiologic findings suggesting infection with potential intracranial extension, the patient underwent urgent surgical intervention including right craniotomy, right orbitotomy, and nasal endoscopy. Intraoperative drainage revealed dark red-brown fluid without purulence. Surgical pathology demonstrated necrotic fibrovascular tissue with brisk neovascularization, consistent with an organizing hematoma. Cultures remained negative. The final diagnosis was orbital hematoma secondary to vaso-occlusive infarction of orbital bone marrow despite the initial suspicion of an abscess. This case illustrates how orbital VOC in children with SCD can radiologically and clinically mimic an orbital abscess, necessitating a multidisciplinary evaluation to resolve the complicated diagnostic picture. It emphasizes the importance of maintaining a broad differential diagnosis in pediatric SCD patients presenting with orbital findings, particularly when systemic inflammation confounds radiologic interpretation.
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