Orbital Pseudotumor After Kawasaki Disease: A Case Report

Introduction: Orbital pseudotumor is an inflammatory mass of orbital tissue of unknown etiology that occurs infrequently in childhood. Clinical presentation includes acute orbital pain exacerbated by eye movement, proptosis, conjunctival or lid edema, and decreased ocular motility. Here we describe a case of orbital pseudotumor in a patient with recent history of Kawasaki disease.

Case Description: A 2-year-old male presented with a 2.5-week history of intermittent bilateral eyelid erythema and edema. Two months prior he was treated for Kawasaki disease with IVIG and infliximab, and remained on daily aspirin. Initially his pediatrician gave oral antibiotics (one week of keflex then one week of amoxicillin) and benadryl. He was referred to Ophthalmology for persistent symptoms and treated for a stye with topical tobradex. After 3 days without improvement, he was referred to our emergency department for imaging. Mother denied limited or painful extra-ocular movements, eye exudate, conjunctival injection, fevers, eye trauma, insect bites, rash, joint pain, or preceding viral symptoms. On exam he was afebrile with mild right supraorbital edema and erythema and right proptosis. His extra-ocular movements were intact and visual acuity was normal. Laboratory studies were not consistent with an infectious or autoimmune process, and a repeat echo showed improved coronary dilation. MRI orbits demonstrated right orbital inflammation with associated proptosis consistent with orbital pseudotumor (Figure 1). He was admitted to the general pediatric floor and received IV solumedrol with significant improvement in eye exam within 24 hours. Brain MRI noted bilateral frontal lobe chronic ischemic insults; thus a head/neck CTA (unremarkable) was obtained to evaluate for vasculitis. He was discharged on a 6-week oral corticosteroid taper. Follow-up after completion of taper revealed normal eye findings without recurrence.

Discussion: Orbital pseudotumor is an idiopathic orbital inflammation requiring differentiation from orbital cellulitis and malignancies, diagnosed by clinical and radiologic findings. It is unresponsive to antibiotics and very responsive to systemic corticosteroids as seen with our patient. The course is considered self-limiting, though late treatment risks permanent restriction of ocular motility. Orbital pseudotumor is associated with autoimmune SLE, Crohn disease, Lyme disease, myasthenia gravis and paraneoplastic syndromes. It has been, though rarely, associated with Kawasaki disease, which was suspected in this patient. His findings of frontal lobe ischemic insults prompted evaluation for systemic vasculitis, though given normal workup this was likely from vasculitic changes from Kawasaki disease.

Conclusion: Patients with periorbital edema, proptosis and limited ocular motility should be evaluated for orbital cellulitis and orbital neoplasm, with consideration for orbital pseudotumor. Though this is rare in children, it may occur in relation to Kawasaki disease or other autoimmune processes. CT or MRI with contrast-enhanced sequences allow for diagnosis to facilitate treatment with systemic corticosteroids, which is essential for management.