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Idiopathic orbital inflammatory pseudotumor in Chinese children
Jianhua Yan1, Haijiang Qiu, Zhongyao Wu
1Zhongshan Ophthalmic Center, Section of Ocular Oncology and Orbital Disease, Sun Yat-sen University, Guangzhou, People's Republic of China. Yan2011@tom.com
Insights
Idiopathic orbital inflammatory pseudotumor (IOIP) in children presents with more ptosis and less proptosis than in adults. While effective treatments exist, the full recovery rate for pediatric IOIP remains low.
Area of Science:
- Ophthalmology
- Pediatric Medicine
- Inflammatory Diseases
Background:
- Idiopathic orbital inflammatory pseudotumor (IOIP) is a rare condition affecting the orbit.
- Understanding the specific clinical features in pediatric populations is crucial for diagnosis and management.
Purpose of the Study:
- To investigate the clinical characteristics of IOIP in Chinese children.
- To compare the features of pediatric IOIP with those observed in adult patients.
Main Methods:
- Retrospective analysis of pediatric IOIP cases from 1978-1999.
- Comparison of clinical signs, subtypes, and treatment outcomes between pediatric and adult IOIP cohorts.
Main Results:
- Pediatric IOIP constituted 11.5% of all cases, with common signs including palpable mass, restricted ocular motility, eyelid swelling, proptosis, and high orbital pressure.
- Children exhibited a higher incidence of ptosis (38% vs. 9%) and a lower incidence of proptosis (42% vs. 69%) compared to adults.
- Orbital mass was the most frequent subtype (50%) in children, similar to adults. The total effective treatment rate was 92%, but the full recovery rate was only 29%.
Conclusions:
- Pediatric IOIP exhibits distinct clinical features, notably increased ptosis and decreased proptosis compared to adult cases.
- Despite a high overall treatment effectiveness, achieving full recovery in children with IOIP is challenging.
- Further research into optimizing treatment strategies for pediatric IOIP is warranted.
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.
