Pediatric acute lymphoblastic leukemia presenting with periorbital edema

Objective: To describe a rare presentation of acute lymphoblastic leukemia (ALL) that is important to the field of pediatric otolaryngology. Methods: The case of a nine-year old female who presented with left periorbital edema as her initial symptom of ALL was reviewed along with pertinent literature. Results: At the time of patient's initial presentation, a computed tomography scan of the sinuses showed sphenoid sinus opacification and left lateral rectus muscle enlargement. She was started on antibiotics without improvement. Subsequently, magnetic resonance imaging of the brain demonstrated an infiltrative process involving the sphenoid sinus marrow space, lateral rectus muscles and dura. Her peripheral blood count revealed 40% blasts, and a bone marrow biopsy demonstrated B-cell ALL. She immediately underwent induction chemotherapy, and her post-induction bone marrow biopsy showed no evidence of residual disease. Conclusion: This case highlights the importance of understanding and considering rare, aggressive diseases that can masquerade as simple periorbital edema. Keywords: Acute lymphoblastic leukemia, ALL, Periorbital swelling, Periorbital edema, Proptosi