Idiopathic orbital inflammatory pseudotumor in Chinese children.
OBJECTIVE
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.
Yan
Jianhua
J
Zhongshan Ophthalmic Center, Section of Ocular Oncology and Orbital Disease, Sun Yat-sen University, Guangzhou, People's Republic of China. Yan2011@tom.com
Qiu
Haijiang
H
Wu
Zhongyao
Z
Li
Yongping
Y
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017677:Age Distribution; D001707:Biopsy, Needle; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D016727:Orbital Pseudotumor / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D015995:Prevalence; D011336:Probability; D011379:Prognosis; D011878:Radiotherapy / Q000379:methods; D012720:Severity of Illness Index; D017678:Sex Distribution; D013514:Surgical Procedures, Operative; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones