Idiopathic inflammatory orbital pseudotumor in childhood. I. Clinical characteristics
L. S. Mottow and F. A. Jakobiec
The cases of 29 patients aged 20 years or less with orbital inflammatory
pseudotumor were studied retrospectively. There was no sex predilection,
although the left orbit was involved twice as often as the right. Patients
typically had abrupt onset of periocular pain, early-morning swelling,
chemosis, conjunctival and extraocular muscle injection, proptosis, a
palpable mass, and extraocular motility disturbances. Visual acuity was
usually only mildly affected at onset. Forty-five percent of patients had
or subsequently developed bilateral orbital involvement in the absence of
notable systemic diseases. Papilledema and iritis were seen in 35% of
patients, respectively, particularly in bilateral cases. All cases
responded to steroids, but bilateral disease was the most apt to become
steroid dependent. Permanent functional impairments were seen most commonly
in patients who had alternating recurrent bilateral disease or who
underwent surgical exploration.
