Akademik Veri Yönetim Sistemi
50th Annual Meeting European Paediatric Ophthalmology Society, Leiden, Hollanda, 9 - 11 Ekim 2025, ss.20, (Özet Bildiri)
O-112 - Multidisciplinary Management of Orbital Langerhans Cell Histiocytosis: Insights from a Pediatric Case Series
Introduction: Langerhans Cell Histiocytosis is a rare disorder characterized by clonal proliferation of Langerhans cells, with potential multi-system involvement. Ocular manifestations, although uncommon, can be the initial presentation. This study aimed to evaluate the clinical characteristics, diagnostic processes and outcomes of pediatric LCH patients with orbital involvement.
Methods: We retrospectively reviewed the records of 10 pediatric patients diagnosed with LCH and orbital involvement between January 1999 and December 2022 at a tertiary referral center. Data collected included demographic features, ophthalmologic findings, systemic involvement, imaging studies, histopathological confirmation, treatment modalities, and clinical outcomes. Patient management was coordinated by a multidisciplinary team comprising pediatric oncologists and ophthalmologists.
Results: The mean age at diagnosis was 34.9 ± 26.4 months (range: 7–75), with a male predominance of 70%. Left eye involvement was seen in 70% of patients, and right eye involvement in 30%. Most patients (70%) presented initially with ocular symptoms, predominantly proptosis and periorbital swelling. Osseous lesions were identified in all patients, whereas bone erosion was detected in 90% of cases, and 60% had multisystem involvement. Diabetes insipidus was observed as a complication in 20% of the patients. Systemic chemotherapy was the main treatment, with radiotherapy or surgery in selected cases. The mean follow-up was 84.1 ± 58.1 months (range: 9– 190), with disease recurrence observed in 5 patients.
Conclusion: Ocular manifestations in pediatric LCH can be a crucial early sign. Multidisciplinary collaboration significantly enhances diagnostic accuracy and therapeutic outcomes, underscoring its importance in managing such complex cases.