Idiopathic orbital inflammatory pseudotumor in Chinese children

Purpose: To study the clinical features of idiopathic orbital inflammatory pseudotumor (IOIP) in Chinese children.

Methods: Pediatric patients with IOIP seen between Jan. 1, 1978 and Dec. 31, 1999 in the Eye Hospital, Zhongshan Ophthalmic Center, Sun Yat-sen University were evaluated retrospectively. Their clinical features were compared with those of adult cases of IOIP.

Results: Of the 209 cases, 24 (11.5%) were equal to or less than 20 years old; 14 were female and 10 were male. There were 11 in the right eye, 9 in the left eye and 4 in both eyes. Palpable mass (58%), ocular motility restriction (46%), swollen eyelid (42%), proptosis (42%) and high orbital pressure (42%) were the five most common presenting signs in children with IOIP. Ptosis occurred more often in pediatric IOIP cases (38%) than in adult IOIP cases (9%) (P < 0.0003). However, compared with adult IOIP cases, pediatric patients showed less proptosis (69% vs. 42%, P = 0.0074). According to radiological and surgical findings, a local mass within the orbit was the most frequent subtype (50%), followed by dacryoadenitis (29%), myositis (8%), perineuritis (4%), eyelid pseudotumor (4%), and diffuse orbital inflammation (4%). The frequency of clinical subtypes in children was similar to that in adults. After systemic corticosteroids, surgical management and local radiotherapy the full recovery response rate was 29% and the total effective rate was 92%.

Conclusions: Pediatric IOIP accounted for 11.5% of all IOIP patients. Pediatric cases had more sign of ptosis and less sign of proptosis than in adult IOIP. The full recovery response rate was low in children with IOIP.