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Published on: August 4, 2022
Inflammatory lesions of the orbit: a single paediatric rheumatology centre experience
Emily L Boulter1, Despina Eleftheriou, Neil J Sebire
1Rheumatology Department, Great Ormond Street Hospital, Great Ormond Street, Bloomsbury, London WC1N 3JH, UK. elboulter@gmail.com
Insights
Pediatric orbital inflammatory lesions are rare and have diverse causes. Early diagnosis through orbital biopsy is crucial for appropriate treatment and to rule out serious systemic conditions.
Area of Science:
- Pediatric Rheumatology
- Ophthalmology
- Pathology
Background:
- Orbital inflammatory lesions in children are uncommon.
- These lesions present a diagnostic challenge due to a wide differential.
- Prompt and accurate diagnosis is vital for effective management.
Purpose of the Study:
- To characterize clinical, laboratory, and histopathological findings in children with orbital inflammatory lesions.
- To determine the final diagnoses in this pediatric cohort.
- To highlight the importance of a multidisciplinary approach in managing these cases.
Main Methods:
- Retrospective descriptive case series.
- Study included children presenting to a tertiary pediatric rheumatology service (January 1999 - July 2010).
- Data collected on clinical presentation, imaging (MRI, CT), and orbital biopsy results.
Main Results:
- Ten patients identified, median age 11.5 years.
- Common final diagnoses included granulomatosis with polyangiitis (5/10), idiopathic orbital inflammation (3/10), atypical mycobacterial infection (1/10), and sarcoidosis (1/10).
- Orbital biopsy was performed in all cases, with 2 patients requiring repeat biopsies.
Conclusions:
- Inflammatory orbital masses in children have a broad differential diagnosis.
- Orbital biopsy and systemic evaluation are essential prior to immunosuppressive therapy.
- Early diagnosis prevents delays in treatment and manages potentially severe systemic manifestations.
Objectives:
To describe the clinical, laboratory, histopathological presentations and final diagnoses for children presenting to a tertiary paediatric rheumatology service with an inflammatory lesion of the orbit.
Methods:
This was a retrospective descriptive case series of children with an inflammatory lesion of the orbit presenting to a single paediatric rheumatology service between January 1999 and July 2010.
Results:
Ten patients, median age 11.5 (range 3.1-16.2) years at referral to the paediatric rheumatology department were identified; median duration of symptoms at referral was 9 (0.75-17) months. Imaging was performed in 9/10 cases: orbital MRI (n = 4), orbital CT scan (n = 1), both MRI and CT scan (n = 4). All 10 patients had an orbital biopsy; 2 patients had repeat biopsies. The final diagnoses were granulomatosis with polyangiitis (Wegener's) (n = 5; ANCA positive n = 4, ANCA negative n = 1), idiopathic orbital inflammation (n = 3), atypical mycobacterial infection (n = 1) and sarcoidosis (n = 1).
Conclusion:
Inflammatory mass lesion of the orbit is an unusual presentation in children. The differential diagnosis is wide and may evolve over time. Orbital biopsy and screening for systemic features is essential before treatment with CSs or other immunosuppressants to exclude malignancy, infection, vascular lesions, autoimmune conditions or other causes of orbital inflammation that can be associated with serious systemic manifestations.
