Wegener's granulomatosis presenting as orbital pseudotumor in children.

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.