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Ocular System

Orbital varix

Vascular hamartoma typified by a plexus of low pressure, low flow, thin walled and distensible vessels that intermingle with the normal orbital vessels.

Vascular hamartoma typified by a plexus of low pressure, low flow, thin walled and distensible vessels that intermingle with the normal orbital vessels.

  • Orbital venous varices are the M/C cause of spontaneous intraorbital hemorrhage
  • M/C cause of reducible & intermittent proptosis

Aetiology

Primary orbital varices:

Idiopathic and confined to the orbit

Secondary orbital varices:

Acquired causes
  • Carotid-cavernous fistula
  • Dural arteriovenous fistulas
  • Intracranial arteriovenous malformations

Pathophysiology

Orbital varices typically result from a congenital weakness in the postcapillary venous wall. This can lead to the proliferation and dramatic dilation of the valveless orbital veins. These varices distend during maneuvers that increase venous pressure depending on the extent of communication with the venous system.

Clinical features

  • Unilateral stress proptosis (non-pulsatile, intermittent and reducible):
    • Manifests with activities that increase venous pressure (coughing, crying, bending, straining, breath holding, or Valsalva maneuver)
  • Intermittent diplopia
  • Intermittent periorbital pain
A photograph showing subtle proptosis of the right eye, characterised by loss of the levator recession in the upper eyelid and the visible rim of conjunctiva superior to the limbus of the iris. | Wade, R. G., Maddock, T. B., & Ananth, S. (2013). Orbital varix thrombosis: a rare cause of unilateral proptosis. BMJ case reports, 2013, bcr2012007935. https://doi.org/10.1136/bcr-2012-007935

Complications

Can lead to visual loss
  • Spontaneous intraorbital hemorrhage
  • Acute thrombosis (acute onset of retro-orbital pain, proptosis, and decreased visual acuity)
  • Lacrimal mass (larger lesions involving the superior ophthalmic vein)

Diagnosis

Imaging:

During imaging, dynamic maneuvers such as Valsalva, in conjunction with specific positioning, can aid in the visualization of varix
  • Ultrasound (USG)
  • Colour Doppler imaging
  • Computerized tomography (CT)
  • MRI
  • Magnetic resonance venography (MRV)
Axial and coronal CT slices with intravenous contrast showing a serpiginous soft tissue mass within the superior portions of the right orbit, consistent with a thrombosed right superior orbital varix. The cavernous sinus remains intact. | Wade, R. G., Maddock, T. B., & Ananth, S. (2013). Orbital varix thrombosis: a rare cause of unilateral proptosis. BMJ case reports, 2013, bcr2012007935. https://doi.org/10.1136/bcr-2012-007935

Differential diagnosis:

Other orbital venous malformations
  • Varicocele
  • Venous angioma
  • Lymphangioma

Management

Most orbital varices may be managed conservatively and only warrant surgery in the presence of recurrent thrombosis, disfiguring proptosis or acute visual loss.

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