The term superior orbital fissure syndrome (SOFS) also called as Rochon–Duvigneaud syndrome is applied to lesions located immediately anterior to the orbital apex, including the structures exiting the annulus of Zinn and often those external to the annulus.
This syndrome is characterised by Multiple cranial nerve palsies may be seen i
- Oculomotor nerve (III)
- Trochlear nerve (IV)
- Abducens nerve (VI)
- Ophthalmic branch of the trigeminal nerve (V1)
- In the absence of optic nerve pathology
The syndrome is characterised by retro-orbital paralysis of extraocular muscles impairment of the branches of the 1st division of the trigeminal nerve and frequently extension to involve the optic nerve.
Examination shows ophthalmoplegia, ptosis, decreased corneal sensation, and occasionally visual loss caused by mechanical optic nerve compression. The presence of proptosis, with swelling of eyelids and chemosis (swelling of ocular surface membranes), indicates significant mass extension within the orbit.
The clinical features are
- Pupillary dilation via alteration in cranial nerve III function in it's innervation of the pupillary constrictors.
- Ptosis - Cranial nerve III involvement causes paresis of the levator palpebrae superiorus muscle
- Pain
- Proptosis from engorgement of the ophthalmic vein and lymphatics.
- Extraocular Paresis of cranial nerves III, IV, and VI causing ophthalmoplegia.
- Parasthesia injury to the first division of cranial nerve V with hypesthesia of the supraorbital and supratrochlear nerves
- Loss of the corneal Reflex.
The orbital apex syndrome is characterised by all of the above plus optic nerve involvement, leading to changes in visual acuity
Potential causes of superior orbital fissure syndrome include
- Traumatic craniomaxillofacial injuries
- Tumours of the orbit (lymphoma or rhabdomyosarcoma) and adjacent structures
- Infection- Fungal infection of the orbit is an important though rare cause of an orbital apex syndrome and is usually secondary to sinus involvement.
- Inflammatory disorders, and vasculitic ischaemic diseases. Orbital inflammation may be isolated (non-specific orbital inflammation), or associated with systemic inflammation such as Wegener’s granulomatosis, polyarteritis nodosa, sarcoidosis, or, rarely, temporal arteritis.
Laboratory workup could include:
- Complete blood count (CBC)
- Erythrocyte sedimentation rate (ESR)
- C reactive protein
- Electrolytes
- Blood glucose
- Antinuclear antibody (ANA)
- Extractable nuclear antibodies (ENA)
- Antineutrophilic antibody -ANCA
- Serum protein electrophoresis
- Borrelia serology,
- Serum angiotensin converting enzyme (ACE).
- Cerebrospinal fluid (CSF) examination (infections, granulomatous disease, and malignancy) can be helpful.
- Biopsy may be necessary but is technically challenging and only considered after a trial of treatment with steroids with no initial response or following early relapse.