07/04/2026
Superior orbital fissure syndrome (SOFS)
FOFS is also known as Rochon-Duvigneaud Syndrome, it is a type of orbital apex disorder.
The superior orbital fissure lies between the greater and lesser wings of the sphenoid bone. Multiple structures run through this area, and they can be categorized into three distinct regions based on their location relative to the common tendinous ring (also known as the annulus of Zinn).
1. The superolateral region outside the annulus of Zinn contains the trochlear, frontal, and lacrimal nerves (a branch of CN V1) along with the superior ophthalmic vein.
2. The central region within the annulus of Zinn transmits the superior and inferior divisions of the oculomotor nerve, the nasociliary nerve (another branch of CN V1), and the abducens nerve.
3. Inferiorly, outside the annulus of Zinn, one finds the inferior ophthalmic vein.
👉Medial to the superior orbital fissure and within the annulus of Zinn lies the optic canal. Within the optic canal, both the optic nerve and the ophthalmic artery traverse into orbit.
SOFS is a rare syndrome whose manifestations are based on compression of structures within the superior orbital fissure....Trauma is the most common underlying cause for SOFS, 0.3% of patients with skull or facial fractures develop this condition. Other studies have demonstrated similar incidences of approximately 0.8% following traumatic facial injury....Neoplasms, such as metastatic lesions, can cause a compressive mass effect within the superior orbital fissure. In women, breast cancer is the most common cause. ..Infections as meningitis, syphilis, and herpes zoster can lead to inflammation and compression of structures within the fissure. ...Vascular processes, as aneurysms and pseudoaneurysms, have also been identified causes. 👍👍👍Due to the often unpredictable nature of these aforementioned etiologies, the only reliable risk factor for the development of SOFS is an anatomically narrow superior orbital fissure. If the optic nerve is involved in patients with suspected SORS, the diagnosis changes and becomes known as orbital apex syndrome.
Signs/Symptoms
The clinical presentation for patients with SOFS can be directly correlated to the structures that are compromised.
1. Upper lid ptosis if the superior division of CN III is impacted, causing partial to complete paralysis of the levator palpebrae superioris muscle.
2. Partial or complete ophthalmoplegia is also a common finding and can develop with damage to CN III, IV, and/or VI.
3. Proptosis may occur due to decreased extraocular muscle tone or mass effect.
4. Anhidrosis can develop if sympathetic innervation to the eye is damaged as it runs along CN V1.
5. Anesthesia of the superior eyelid and lower forehead with loss of the corneal reflex leading to a neurotrophic cornea may also occur through the same process.
6. The pupil may appear dilated without the ability to constrict to light due to loss of parasympathetic input to the eye.
Work-up
The most common findings on CT for trauma patients include fractures to the zygomatic and orbital bones. CT is also beneficial for measuring the size of the superior orbital fissure.
For other causes of SOFS, as neoplastic, infectious, and inflammatory, MRI with and without contrast with fat suppressed images of the orbits is the modality of choice due to its superior visualization of soft tissue and nerve structures. Neoplastic lesions may reveal underlying metastasis or meningiomas, and infectious processes may show orbital cellulitis or subperiosteal abscesses.
In cases of suspected vascular abnormalities, both MRA and CTA are appropriate and would likely identify the underlying process.
If an infectious cause is suspected, laboratory evaluation with complete blood counts with differentials, basic metabolic panels, and blood cultures are standard. More targeted evaluation, such as a lumbar puncture for meningitis, will depend on the clinical presentation of the patient and the suspected infection.
