To report the 10-year experience of two tertiary medical centers with children presenting with proptosis due to an intraorbital space-occupying lesion.
Patients were identified by file review. Data were collected on demographics, findings on ophthalmologic and imaging evaluations, etiology, treatment, and outcome.
Nineteen children (7 male) were included. Eleven patients had optic nerve glioma, including 9 with substantially decreased visual acuity. Treatment consisted of chemotherapy alone or with radiation, resection or anti-VEGF agents, MEK inhibitor, or observation only (n = 1). Visual and cosmetic outcomes were poor in all cases. Outcome for arteriovenous malformations was good following corticosteroid treatment (n = 1), but catheterization led to persistent proptosis and fluctuating visual acuity (n = 1). Compound capillary hemangioma (n = 1) was treated with laser and systemic beta blockers with satisfactory results. Rhabdomyosarcoma had a good prognosis in one patient treated with resection and radiation but was fatal in another even after chemotherapy. Juvenile xanthogranuloma, frontal bone osteoma, and localized hypertrophic neuropathy of the supraorbital nerve (n = 1 each) were treated by resection with good visual and cosmetic outcomes.
Proptosis accompanied by visual loss is an uncommon presentation in children and suggests an orbital tumor. We found that visual outcome was better when the nerve was not involved by tumor. Optic nerve glioma was the most common cause and failed to respond to various treatments. Catheterization for arteriovenous malformation did not prevent proptosis, and final visual acuity fluctuated. Surgery for rhabdomyosarcoma and xanthogranuloma led to remission with preservation of vision in 2 of 3 cases.
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This study was partially supported by the Zanvyl and Isabelle Krieger Fund, Baltimore, MD, USA. The funding organization had no role in the design or conduct of this research.
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No conflicts of interest.
All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the Rabin Medical Center and Bnai Zion Medical Center Institutional Ethics Committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Due to the retrospective nature of the study, informed consent was waived by the Rabin and Bnai Zion Medical Center Institutional Ethics Committee.
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Zahavi, A., Luckman, J., Ben-David, G.S. et al. Proptosis due to intraorbital space-occupying lesions in children. Graefes Arch Clin Exp Ophthalmol 258, 2541–2550 (2020). https://doi.org/10.1007/s00417-020-04840-3
- Optic nerve
- Optic nerve glioma