Proptosis due to intraorbital space-occupying lesions in children

Graefes Arch Clin Exp Ophthalmol. 2020 Nov;258(11):2541-2550. doi: 10.1007/s00417-020-04840-3. Epub 2020 Jul 16.


Purpose: To report the 10-year experience of two tertiary medical centers with children presenting with proptosis due to an intraorbital space-occupying lesion.

Methods: Patients were identified by file review. Data were collected on demographics, findings on ophthalmologic and imaging evaluations, etiology, treatment, and outcome.

Results: 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.

Conclusions: 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.

Keywords: Chemotherapy; Optic nerve; Optic nerve glioma; Surgery; Tumor.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Exophthalmos* / diagnosis
  • Exophthalmos* / etiology
  • Humans
  • Male
  • Optic Nerve Glioma*
  • Orbit / diagnostic imaging
  • Orbital Neoplasms* / complications
  • Orbital Neoplasms* / diagnosis
  • Orbital Neoplasms* / therapy
  • Retrospective Studies
  • Vision Disorders