Headache and the Eye

Ula Jurkunas, MD; John W. Gittinger, JR, MD


Compr Ophthalmol Update. 2004;5(4) 

In This Article

Multiple Cranial Nerves

Table 5 highlights processes causing either isolated or multiple cranial neuropathies. In the cavernous sinus, blockage of venous outflow causes proptosis and chemosis. Pain is usually present due to involvement of trigeminal nerve. Bilaterality also suggests cavernous sinus involvement.

High-flow carotid-cavernous fistulas manifest as severe proptosis, chemosis, ophthalmoplegia, loss of vision, and bruit. Nevertheless, posterior-draining low-flow dural AV fistulas may present with painful ocular motor nerve palsy without congested orbital features.[57] Cavernous sinus thrombosis is usually accompanied by the systemic manifestations of sepsis. Mucor mycosis and aspergillosis should be ruled out in these cases.[58]

A nonspecific noninfectious granulomatous inflammation of cavernous sinus and superior orbital fissure, Tolosa-Hunt syndrome, manifests as severe retro-orbital pain with ipsilateral ophthalmoplegia.[59] Imaging studies are usually nonspecific but occasionally show enlargement of extraocular muscle bellies and optic nerve, suggesting an etiologic continuum with idiopathic orbital inflammation, also called pseudotumor of the orbit (Figures 3-5).[60,64] A hallmark of Tolosa-Hunt syndrome is a rapid response to corticosteroids; nevertheless, steroid responsiveness is nonspecific as other conditions may improve transiently.

Right orbital pseudotumor in adult. Computed tomography of orbits shows right scleral thickening, stranding of orbital fat, and thickening of extraocular muscle tendons.

Computed tomography scan of bilateral orbital pseudotumor in 2-year-old child.

Tolosa-Hunt syndrome. Arrow shows signal enhancement at left orbital apex and cavernous sinus.

Similarly, idiopathic orbital inflammation has various presentations and may be a diagnosis of exclusion.[61,62] Patients experience acute retro-orbital pain with proptosis and soft tissue and orbital congestion. Radiologic findings and clinical course are usually suggestive of the diagnosis (Figure 3).[63] An unusual clinical presentation or course may require an orbital biopsy.[61,62,63]

Children have a higher prevalence of bilateral involvement and anterior chamber inflammation than adults (Figure 4).[63,65] Bilaterality in adults could be due to underlying systemic vasculitis. After infectious etiologies have been ruled out, the treatment of choice is oral prednisone.[61,64] Other causes of orbital pain are trauma, infection, cancer, or vasculitis ( Table 6 ).


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