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Wegener's granulomatosis presenting as orbital pseudotumor in children
Insights
Wegener
Area of Science:
- Pediatric Rheumatology
- Ophthalmology
Background:
- Wegener's granulomatosis (WG) is a rare vasculitis that can affect children.
- Orbital pseudotumor is an idiopathic inflammatory condition that can mimic other orbital diseases.
Abstract:
The case of a nine-year-old white female who presented initially with idiopathic inflammatory pseudotumor and was ultimately diagnosed (22 months later) as having Wegener's granulomatosis is presented. A review of the literature reveals that, to date, there are 21 well-documented cases of Wegener's granulomatosis occurring in children under the age of 16. Of these 21 cases, eight patients (39%) manifested ocular or orbital signs during the course of the disease. Four of these cases (50%) presented initially with idiopathic inflammatory pseudotumor. The diagnosis of Wegener's granulomatosis should be considered in children with orbital pseudotumor. Long-term follow-up may be necessary to exclude this possibility.