Superior orbital fissure syndrome
Superior orbital fissure syndrome (also known as Rochen-Duvigneaud syndrome)
Superior orbital fissure syndrome (also known as Rochen-Duvigneaud syndrome) is a collection of symptoms caused by compression of structures just anterior to the orbital apex (1). The complex and crowded anatomy of the superior orbital fissure produces a characteristic pattern of cranial nerve, pupillary and extraocular findings. Importantly the optic nerve is spared which differentiates superior orbital fissure syndrome from orbital apex syndrome (2). This constellation of findings was first described by Hirschfeld in 1858 and officially named by Rochen-Duvigneaud in 1896 (3).
Superior orbital fissure syndrome is rare with the majority of the literature being case reports or series. The most frequent cause is trauma where superior orbital fissure syndrome is seen in 0.3-0.8% of patients (3, 4) and common associations are motorcycle accidents, zygomatic fractures and orbital fractures (5). Other causes include neoplasms (particularly lymphoma and rhabdomyosarcoma)(6), infections (such as meningitis (7), syphilis (8), sinusitis (9), herpes zoster (10)), inflammation (such as SLE, sarcoidosis, Tolosa-Hunt syndrome)(2) and vascular phenomena (such as carotid-cavernous fistulas, retro-orbital haematoma and carotid aneurysms)(4, 11).
The only reported risk factor for superior orbital fissure syndrome is a pre-existing narrow superior orbital fissure (4).
The superior orbital fissure is a bony cleft found at the orbital apex between the roof and lateral wall. It is a communication between the orbital cavity and middle cranial fossa and is bounded by the greater wing, lesser wing and body of sphenoid (12). Its dimensions are 22mm in length and 2-8mm in width (narrowest and widest parts) and it is pear-shaped with a wide base nasally on the body of sphenoid and an apex directed superotemporally (4).
Anteriorly the superior orbital fissure is related to the annulus of Zinn (common tendinous ring) on which the four rectus muscles attach. The annulus splits the superior orbital fissure into three sectors: lateral, central (oculomotor foramen) and inferior (see Image 1). The lateral sector contains the lacrimal, frontal and trochlear nerves and superior ophthalmic vein. The central sector, which is surrounded by the annulus of Zinn, contains the superior and inferior branches of oculomotor nerve, nasociliary and abducens nerves. The inferior sector contains the inferior ophthalmic vein (12-14).
Compression of structures in superior orbital fissure syndrome can be attributed to a bony fragment (such as with facial fractures) or mass effect. Since the orbital muscle cone is of relatively fixed volume, being bounded by the intermuscular membrane and Tenon’s capsule, any oedema or bleeding or masses in this area compromises delicate neural structure (15).
Anatomical structures within common tendinous ring (i.e. central sector) are the most vulnerable. In particular the abducens nerve is most commonly damaged (3) as it is within the central sector, lies close to greater wing and has a relatively long intracranial course RIA. The trochlear nerve is the least commonly involved (3) as it exists above the common tendinous ring where it is well protected (4). The anatomical proximity of the superior orbital fissure to the cavernous sinus and internal carotid artery permits vascular pathology such as carotid aneurysms and carotid-cavernous fistulas to cause compression (3).
The pathophysiology underlying clinical findings is discussed below.
The common clinical findings in superior orbital fissure syndrome include the following and their individual anatomical basis are explained (4, 16)
- Ophthalmoplegia: due to compression or damage to oculomotor, trochlear and abducens nerves
- Ptosis: due to loss of oculomotor motor supply to the levator palpebrae superioris and loss of sympathetic to Muller’s muscle
- Proptosis: due to decreased tension in the extraocular muscles with loss of innervation
- Fixed dilated pupil: due to loss of parasympathetic supply to the pupil by the oculomotor nerve
- Lacrimal hyposecretion and eyelid or forehead anaesthesia: due to damage to branches of the ophthalmic division of the trigeminal nerve
- Loss of corneal reflex: due to loss of afferent input from the ophthalmic division of the trigeminal nerve.
Other findings which may give insight into underlying aetiology include:
- Chemosis and bruits in vascular causes: due to altered blood flow and vascular congestion (3)
- Facial trauma patients may concurrent subconjunctival haemorrhage, periorbital ecchymosis and soft tissue contusion (3)
- Extensive masses in the region of the superior orbital fissure may be marked by proptosis, eyelid swelling and chemosis (6)
It should also be noted a phenomena of partial superior orbital fissure syndrome has also been described where involvement is strictly confined to the central sector thus associated with isolated oculomotor, abducens and nasocilairy involvement (4)
The diagnosis of superior orbital fissure syndrome is suspected on clinical grounds. Imaging studies play in role in confirming the diagnosis and, more importantly, give insight into underlying aetiology. Early use of plain film with superior orbital fissure views (20-25° head tilt) has largely been replaced by widespread availability of CT and MRI. The most common imaging modality is currently CT with 2mm fine cuts looking for bony fragments or compressive masses. Where a vascular cause is suspected angiography is recommended to define any carotid-cavernous fistula or carotid aneurysm (4).
In cases managed conservatively or medically there is little consensus on need for follow-up imaging but Shama et al. (2012) suggests a repeat MRI at 6 months (1).
Important differentials to distinguish from superior orbital fissure syndrome are:
- Orbital apex syndrome: where the main differentiator is optic nerve involvement (2).
- Cavernous sinus syndrome: which can be classed into three subtypes. The posterior cavernous sinus syndrome includes whole trigeminal involvement. The middle cavernous sinus syndrome involves the first and second divisions of the trigeminal nerve (ophthalmic and maxillary). The anterior cavernous sinus syndrome involves ophthalmic nerve involvement only (15). Furthermore cavernous sinus syndrome may be associated with Horner’s syndrome and pain which is in contrast to superior orbital fissure syndrome (17).
The management of superior orbital fissure syndrome is not standardised and there exist medical and surgical options. Indications for urgent intervention are vascular causes (requiring embolization) and bony fractures with displaced fragments or severe foraminal narrowing (3).
Conservative medical treatment involves mega dose steroids and is suitable in patients not meeting criteria for urgent surgical intervention as discussed above. The commonest regime mirrors that of spinal cord/nerve injury protocols and involves intravenous IV methylprednisolone as a 30mg/kg bolus followed by a 5.4mg/kg 48 hour infusion and eventual per oral prednisone taper over 2 weeks (4, 18).
Surgical management in the non-urgent setting is performed at an average of 10.7 days due to concerns that early intervention is associated with increased swelling and ocular pressure (3). Both orbital and cranial extradural approaches have been described including extranasal intraorbital to access the lateral wall, extranasal transethomoidal to access the medial wall, a modified extranasal intraorbital route, transtemporal route in causes of infectious collections and combined orbital and cranial for deep decompressions (19, 20).
Complications and prognosis
No adverse effects from medical therapy with high dose steroids in this setting have been described (18). Complete recovery of all nerves has been reported in 24-40% of patients receiving steroid treatment compared to 21.4% in those without. Recovery is usually extended over a period of months with progress plateauing at 6 months. The abducens nerve which is most commonly damaged shows the best recovery.
Possible sequelae from superior orbital fissure syndrome may include remaining deficit requiring further strabismus or ptosis surgery (3, 4)
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