Low-Flow Orbital Venous Malformation Masquerading as Rhabdomyosarcoma Academic Article uri icon


MeSH Major

  • Orbit
  • Orbital Neoplasms
  • Rhabdomyosarcoma
  • Vascular Malformations
  • Veins


  • A 15-month-old male was referred for biopsy of presumed rhabdomyosarcoma in the setting of rapidly progressing left-sided proptosis. Examination revealed left periorbital edema and left hypoglobus. MRI revealed a soft-tissue density mass within the left lateral retrobulbar space. Several days later, he developed acute periorbital ecchymosis and increasing edema. With high suspicion for a vascular lesion, a CT scan was performed with dynamic arterial and venous imaging. Central filling was noted in the lateral retrobulbar component with increased enhancement on delayed venous imaging in the middle cranial fossa component favoring the diagnosis of a low-flow orbital venous malformation. In the setting of spontaneous orbital hemorrhage and risk of future vision loss, the decision was made to proceed with a combined neurosurgical approach treating the intracranial component and debulking the orbital component. This case highlights the importance of thorough radiographic evaluation prior to proceeding with a surgical procedure in the appropriate clinical context.

publication date

  • November 2018



  • Academic Article



  • eng

Digital Object Identifier (DOI)

  • 10.1097/IOP.0000000000001236

PubMed ID

  • 30289825

Additional Document Info

start page

  • e186

end page

  • e189


  • 34


  • 6