Orbital apex syndrome

Orbital apex syndrome, also known as Jacod syndrome, is a constellation of clinical findings, presenting as a result of several potential pathologies that compress or otherwise affect structures passing through the orbital apex.

Clinical presentation

Presentation is according to the structures affected ¹:

• cranial nerves III, IV, and VI: ophthalmoplegia, proptosis, and ptosis
• ophthalmic (V1) division of the trigeminal nerve: ipsilateral hypoesthesia of the forehead, upper eyelid, and cornea
• optic nerve (II): visual deficit that can lead to blindness which is the major finding in differentiating between this syndrome and superior orbital fissure syndrome and cavernous sinus syndrome.

Etiology

• trauma: usually fractures involving the orbital apex
• infection: orbital cellulitis, subperiosteal abscess of the orbit
• inflammatory: Tolosa-Hunt syndrome, sarcoidosis
• vascular lesions: ophthalmic artery aneurysm, carotid-cavernous fistula
• developmental: fibrous dysplasia and neurofibromatosis
• neoplastic: extension of intracranial tumors or intraorbital tumors, lymphoma, and metastasis
• endocrine: thyroid ophthalmopathy
• others: sino-nasal mucocele, foreign body, iatrogenic, intra-orbital dermoid, intra-orbital epidermoid

Treatment and prognosis

Prompt treatment is recommended to avoid serious complications, as optic nerve compression can result in optic nerve ischemia and atrophy, and subsequent vision loss. Treatment would be according to the etiology. For compressive disorders, decompressive surgery is usually needed ².

See also

• superior orbital fissure syndrome
• cavernous sinus syndrome