Main page/Anterior skull base/Superior orbital fissure/Superior orbital fissure syndrome

Superior orbital fissure syndrome

General

  • Aka Rochen-Duvigneaud syndrome
  • A collection of symptoms caused by compression of structures just anterior to the orbital apex

Aetiology

  • Trauma
    • Most frequent cause of superior orbital fissure syndrome
    • 0.3-0.8% of patients
    • Often presents within 48 hours of a facial injury, but presentation can also be delayed by several days
  • Neoplasms (particularly lymphoma and rhabdomyosarcoma)
  • Infections (such as meningitis)
  • Syphilis
  • Sinusitis
  • Herpes zoster
  • Inflammation (such as Lupus, sarcoidosis, Tolosa-Hunt syndrome)
  • Vascular phenomena (such as carotid-cavernous fistulas, retro-orbital haematoma and carotid aneurysms)
  • Idiopathic

Risk factor

  • Pre-existing narrow superior orbital fissure

Clinical features

  • The optic nerve is spared which differentiates superior orbital fissure syndrome from orbital apex syndrome
  • Ophthalmoplegia:
    • Due to compression or damage to oculomotor, trochlear and abducens nerves
    • Anatomical structures within common tendinous ring (i.e. central sector) are the most vulnerable.
      • CN6 is most commonly damaged as it is within the central sector, lies close to the greater wing and has a relatively long intracranial course
      • CN4 is the least commonly involved as it exists above the common tendinous ring where it is well protected
  • Ptosis:
    • Due to loss of oculomotor motor supply to the levator palpebrae superioris and loss of sympathetic input (third order postganglionic) 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 (please note, this is different than relative afferent pupillary defect
  • 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 etiology include:
    • Chemosis and bruits in vascular causes:
      • Due to altered blood flow and vascular congestion
    • Facial trauma patients may concurrent subconjunctival hemorrhage, periorbital ecchymosis and soft tissue contusion
    • Extensive masses in the region of the superior orbital fissure may be marked by proptosis, eyelid swelling and chemosis

Investigation

  • Bloods
    • FBC
    • CRP/ESR
  • MRI/CT
  • CTA/MRA

Management

  • Treatment of cause
  • Conservative
    • Steroids

Outcomes

  • 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.

DDx

  • Orbital Apex Syndrome:
    • Distinguished by additional involvement of the optic nerve (CN II) alongside cranial nerves III, IV, V1, and VI.
  • Cavernous Sinus Syndrome:
    • Generally involves more extensive trigeminal nerve territory, may include Horner’s syndrome and pain, and can present with involvement of internal carotid artery structures.
  • Posttraumatic Carotid-Cavernous Fistula:
    • Suspect with signs like pulsatile exophthalmos, elevated intraocular pressure, “corkscrew” conjunctival vessels, systolic bruit, and a history of trauma.
  • Raeder’s Paratrigeminal Syndrome:
    • Presents with pain (ophthalmic ± maxillary nerve territory), oculosympathetic palsy (Horner’s), and lacks optic nerve involvement.
  • Inflammatory or Infectious Conditions:
    • Includes Tolosa-Hunt syndrome, idiopathic orbital inflammation, sarcoidosis, vasculitis, tuberculosis, Lyme, syphilis, bacterial or fungal sinusitis, and herpes zoster.
  • Neoplastic Disorders:
    • Lymphoma, metastatic tumors (breast, prostate, lung), sphenoid meningioma, sinonasal carcinoma infiltrating orbital fissure.
  • Vascular Lesions:
    • Cavernous sinus thrombosis, aneurysms, or pseudoaneurysms affecting nearby neurovascular bundles.
  • Other Neuromuscular Conditions:
    • Myasthenia gravis may occasionally mimic localized cranial nerve palsies.