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Type presentatieE-poster
TitelUnilateral orbital mass as first presentation of eosinophilic granulomatosis with polyangiitis in a 7-year-old girl.
DoelTo describe a rare, pediatric case of eosinophilic granulomatosis with polyangiitis (EGPA) with isolated orbital involvement.
MethodesA 7-year-old girl presented with a fast-growing unilateral orbital mass.
ResultatenA 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.
ConclusieEGPA, 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.
BelangenverstrengelingNee
Auteurs 1
InitialenP
NaamBonny
InstituutDept of Oftalmology, GUH
StadGhent
Auteurs 2
InitialenR
NaamVan Paemel
InstituutDept of Internal Medicine and Pediatrics, GUH
StadGhent
Auteurs 3
InitialenE
NaamSchiettecatte
InstituutDept of Radiology, GUH
StadGhent
Auteurs 4
InitialenJ
NaamVan Dorpe
InstituutDept of Anatomic Pathology, GUH
StadGhent
Auteurs 5
InitialenT
NaamRenson
InstituutDept of Internal Medicine and Pediatrics, GUH
StadGhent
Auteurs 6
InitialenE
NaamKreps
InstituutDept of Oftalmology, GUH
StadGhent
Auteurs 7
InitialenV
NaamNinclaus
InstituutDept of Oftalmology, GUH
StadGhent
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