Superior orbital fissure syndrome (SOFS) (also known as Rochon–Duvigneaud syndrome) is a rare complication of craniofacial trauma with an orbital fracture that extends to the superior orbital fissure that results in injury to the cranial nerves III, IV, V (ophthalmic branch of the trigeminal nerve) and VI as they traverse the fissure into the orbit 1.
It also occurs due to other pathologies that may affect the superior orbital fissure:
- inflammatory e.g. Tolosa-Hunt syndrome
- malignancy e.g. head and neck, metastatic disease, lymphoma, perineural invasion by cutaneous malignancy
- infectious e.g. meningitis
- vascular lesions 2
Clinical presentation
- ophthalmoplegia or diplopia as a result of extraocular muscles paralysis
- ptosis due to paralysis of the levator palpebrae superioris, supplied by the third cranial nerve
- exophthalmos
- blindness or decreased vision represent additional injury to the optic nerve (II) that results in orbital apex syndrome 3
Treatment and prognosis
Conservative management is warranted unless there is a bony impingement of the neuronal structures and/or simultaneous compression of the optic nerve leading to blindness or diminished vision (orbital apex syndrome), which is a surgical emergency.
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