Presentation type | E-poster |
Title | Unilateral orbital mass as first presentation of eosinophilic granulomatosis with polyangiitis in a 7-year-old girl. |
Purpose | To describe a rare, pediatric case of eosinophilic granulomatosis with polyangiitis (EGPA) with isolated orbital involvement. |
Methods | A 7-year-old girl presented with a fast-growing unilateral orbital mass. |
Results | A 7-year-old girl was referred for preseptal cellulitis, not responding to amoxicillin. She presented with a mobile, tender superotemporal orbital mass palpable through the upper eyelid, associated with pain and restricted upgaze. Other ophthalmological parameters were normal. MRI showed an intra-orbital lesion involving the superior rectus muscle and lacrimal gland, iso-intense on T1, hyperintense on T2, with marked contrast enhancement and no diffusion restriction. Biopsy revealed dense eosinophilic infiltration with granulomas and perivascular inflammation. Serology was positive for P-antineutrophil cytoplasmic antibodies (ANCA) with elevated myeloperoxidase (MPO) supporting EGPA, an ANCA associated vasculitis. Systemic work-up showed no other inflammatory lesions. Surgical debulking followed by high-dose corticosteroids and rituximab achieved sustained clinical and biochemical remission. |
Conclusion | EGPA, rare in children, is an exceptionally rare cause of pediatric orbital masses. Early biopsy and immunologic testing are essential for diagnosis, differentiation with other vasculitides and orbital tumors, and prompt treatment. |
Conflict of interest | No |
Initials | P |
Last name | Bonny |
Department | Dept of Oftalmology, GUH |
City | Ghent |
Initials | R |
Last name | Van Paemel |
Department | Dept of Internal Medicine and Pediatrics, GUH |
City | Ghent |
Initials | E |
Last name | Schiettecatte |
Department | Dept of Radiology, GUH |
City | Ghent |
Initials | J |
Last name | Van Dorpe |
Department | Dept of Anatomic Pathology, GUH |
City | Ghent |
Initials | T |
Last name | Renson |
Department | Dept of Internal Medicine and Pediatrics, GUH |
City | Ghent |
Initials | E |
Last name | Kreps |
Department | Dept of Oftalmology, GUH |
City | Ghent |
Initials | V |
Last name | Ninclaus |
Department | Dept of Oftalmology, GUH |
City | Ghent |