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BOOK EXCERPT
Neuro-Ophthalmology Review Manual, Sixth Edition
Lanning B. Kline MD; Frank J. Bajandas MD

Chapter 7
Cavernous Sinus Syndrome
Lanning B. Kline, MD

I. General considerations

  1. The ocular motor cranial nerves lie in proximity within the cavernous sinus and superior orbital fissure
  2. Since the cavernous sinus contains structures that continue through the superior orbital fissure, it is often impossible to state whether a lesion is in the sinus or in the fissure. More general designation is parasellar syndrome
  3. Typically, patients present with periorbital or hemicranial pain, combined with ipsilateral ocular motor cranial nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic (V1) and occasionally maxillary (V2) division of the trigeminal nerve. Clinically, various combinations of these cranial nerve palsies occur
  4. The “orbital apex syndrome” should be reserved for multiple ocular motor cranial nerve palsies plus optic nerve dysfunction

Figure 7-1. Coronal view of the left cavernous sinus and its contents
Figure 7-1. Coronal view of the left cavernous sinus and its contents.

Figure 7-2. Lateral schematic view of the left cavernous sinus
Figure 7-2. Lateral schematic view of the left cavernous sinus; cranial nerves that traverse the sinus are highlighted in black.

II. Anatomy (Figures 7-1 and 7-2)

  1. Traditionally, the cavernous sinus was thought to be an unbroken, trabeculated structure, but recent studies demonstrate that it is a plexus of various-sized veins that divide and coalesce
  2. Major constituents:
    1. III nerve
    2. IV nerve
    3. VI nerve
    4. Ophthalmic nerve (V1)
    5. Sympathetic carotid plexus
    6. Intracavernous carotid artery
  3. The III, IV, V1 nerves all lie in a lateral wall of the cavernous sinus. The VI nerve lies freely within the sinus, just lateral to the intracavernous carotid

III. Causes of cavernous sinus syndrome producing painful ophthalmoplegia

  1. Trauma
  2. Vascular
    1. Intracavernous carotid artery aneurysm
    2. Posterior cerebral artery aneurysm
    3. Carotid-cavernous fistula
    4. Carotid-cavernous sinus thrombosis
  3. Neoplasm
    1. Primary intracranial tumor
      1. Pituitary adenoma
      2. Meningioma
      3. Craniopharyngioma
      4. Sarcoma
      5. Neurofibroma
      6. Gasserian ganglion neuroma
      7. Epidermoid
      8. Hemangioma/hemangiopericytoma
      9. Eosinophilic granuloma
    2. Primary cranial tumor
      1. Chordoma
      2. Chondroma
      3. Giant cell tumor
    3. Local metastases
      1. Nasopharyngeal tumor
      2. Cylindroma
      3. Adamantinoma
      4. Squamous cell carcinoma
    4. Distant metastases
      1. Lymphoma
      2. Multiple myeloma
      3. Carcinomatous metastases
  4. Inflammation
    1. Bacterial: sinusitis, mucocele, periostitis
    2. Viral: herpes zoster
    3. Fungal: mucormycosis
    4. Spirochetal: Treponema pallidum
    5. Mycobacterial: Mycobacterium tuberculosis
    6. Unknown cause: sarcoidosis, Wegener’s granulomatosis, Tolosa-Hunt syndrome

IV. Of the more common causes of cavernous sinus syndrome, these points deserve emphasis:

  1. Intracavernous carotid artery aneurysm
    1. Typically produces slowly progressive, unilateral ophthalmoplegia
    2. May become painful
    3. Rarely rupture, but this occurrence produces a carotid-cavernous fistula
  2. Carotid-cavernous fistula
    1. Due to direct communication between intracavernous carotid artery and cavernous sinus
    2. High-flow, high-pressure fistula
    3. Most common cause is head trauma
    4. Clinical picture: chemosis, proptosis, ocular motor nerve palsies, bruit, retinopathy, increased intraocular pressure
  3. Dural-cavernous fistula
    1. Due to communication of dural branches of internal or external carotid arteries and cavernous sinus or vessels in region of sinus
    2. Low-flow, low-pressure fistula
    3. Most commonly occur spontaneously
    4. More subtle clinical picture: don’t be fooled into treating these patients for “red eye”
    5. A minority of patients may develop cortical venous drainage of their fistula, with increased risk of intracerebral hemorrhage or cerebral venous infarction. Suggestive clinical signs are bilateral orbital congestion and postauricular bruit. Workup: magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), magnetic resonance venography (MRV), cerebral angiography
    6. On occasion, fistula flow is directed posteriorly, causing chronic ocular motor cranial nerve palsies without orbital congestive signs (“white-eyed shunt”)
  4. Nasopharyngeal carcinoma
    1. Two to three times more common in males
    2. Predilection for Asian patients
    3. Varied clinical presentation
      1. Nasal obstruction
      2. Rhinorrhea
      3. Epistaxis
      4. Otitis media
      5. Proptosis
      6. Ipsilateral dry eye
    4. Ninety-five percent of patients with nasopharyngeal carcinoma have VI nerve paresis at some time during clinical course
    5. Radiologic study of choice: cranial magnetic resonance scanning with attention to subcranial soft tissue in region of nasopharynx
    6. Pharyngoscopy and biopsies of the nasopharynx if clinical suspicion high
  5. Two aspects of neoplastic involvement of the parasellar region require particular attention
    1. Mode of onset and clinical course do not prognosticate the type of lesion (ie, neoplastic disease may have an acute clinical presentation as well as an expected insidious course)
    2. High-dose corticosteroid therapy may initially improve signs and symptoms due to neoplasm
  6. Tolosa-Hunt syndrome
    1. Painful ophthalmoplegia due to granulomatous inflammation occurring in the cavernous sinus
    2. Spontaneous remissions may occur after days or weeks
    3. Recurring attacks may occur at intervals of months or years
    4. Systemic steroids usually lead to marked improvement of signs and symptoms within 48 hours
    5. Categorically, diagnosis of exclusion and patients with this diagnosis require careful follow-up

V. Imitators of cavernous sinus syndrome

  1. Myasthenia
  2. Ocular dysthyroidism
  3. Orbital disease: inflammation, infection, neoplasm, trauma
  4. Diabetic ophthalmoplegia
    1. Typically acute, often painful, mononeuropathy with full recovery within 3 months
    2. Less frequent occurrence of simultaneous paralysis of multiple ocular motor nerves. Often painful, recurrent, and not responsive to steroid therapy
  5. Giant cell arteritis
    1. Single or multiple ocular motor nerve palsies
    2. Produces ischemic necrosis of extraocular muscles
  6. Botulism
    1. Occurs in six forms: food-borne, wound, infantile, infant, hidden, inadvertent
    2. Ophthalmologic findings include dilated, poorly reactive pupils, ptosis, and ophthalmoplegia
    3. Affected individuals have nausea, vomiting, associated with facial, pharyngeal, and generalized proximal weakness, and no sensory deficits
    4. Botulinum toxin is the most potent poison known
    5. Causes cholinergic blockage by preventing release of acetylcholine at neuromuscular junction
  7. Miller Fisher syndrome
    1. Bulbar variant of Guillain-Barré syndrome, characterized by triad of ataxia, areflexia, ophthalmoplegia
    2. In evolution, this cranial polyneuropathy may mimic unilateral or bilateral ocular motor cranial nerve palsies, but usually progresses to a virtually total ophthalmoplegia with involvement of pupils and accommodation
    3. Patients may also have facial diplegia, respiratory and swallowing difficulties, and confusion
    4. Often follows gastroenteritis from Campylobacter jejuni infection
    5. Over 90% of patients have antibodies to the ganglioside GQ1b, which cross-reacts with ganglioside structure in wall of C. jejuni
    6. Anti GQ1b antibodies have been shown to damage the motor nerve terminal by a complement-mediated mechanism, possibly targeting neuronal membrane of presynaptic Schwann cells
    7. Bickerstaff’s brainstem encephalitis is a closely related condition with alterations in consciousness and long tract signs seen in addition to ophthalmoplegia and ataxia
    8. Typically benign and self-limited
    9. If needed, treatment includes plasmapheresis and IV immunoglobulin (Ig)

VI. Ocular neuromyotonia

  1. Patient reports episodic diplopia or oscillopsia
  2. Failure of extraocular muscles to “relax” following sustained, eccentric gaze
  3. Previous history of invasive pituitary adenoma or other intracranial tumors treated with radiation therapy
  4. Due to episodic, involuntary discharge of ocular motor nerves producing sustained and inappropriate contraction of their respective ocular muscles
  5. Treatment: carbamazepine, phenytoin

Bibliography

Chapters

Parkinson D. Anatomy of the cavernous sinus. In: Smith JL, ed. Neuro-Ophthalmology. Vol 6. St Louis, Mo: CV Mosby; 1972:73-101.

Schatz NJ, Farmer P. Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia. In: Smith JL, ed. Neuro-Ophthalmology. Vol 6. St Louis, Mo: CV Mosby; 1972:102-112.

Articles

Acieino M, Trobe JD, Cornbluth WT, Gebarski SS. Painful oculomotor palsy caused by posterior-draining dural carotid cavernous fistulas. Arch Ophthalmol. 1995;113:1045-1049.

Barricks ME, Traviesa DB, Glaser JS, et al. Ophthalmoplegia in giant cell arteries. Brain. 1977;100:209-221.

Cherrington M: Botulism: update and review. Sem Neurol. 2004;24:155-163.

Chiba A, Kusonoki S, Obata H, Machinumi R, Kanazana I. Serum anti-GQ1b antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology. 1993;43:1911-1917.

Fisher CM. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia, areflexia). N Engl J Med. 1956;255:57-65.

Harris FS, Rhoton AL. Anatomy of the cavernous sinus. A microsurgical study. J Neurosurg. 1976;45:169-180.

Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71:577-582.

Newton TH, Hoyt WF. Dural arteriovenous shunts in the region of the cavernous sinus. Neuroradiology. 1970;1:71-81.

Overell JR, Willison HJ. Recent developments in Miller Fisher syndrome and related disorders. Curr Opin Neurol. 2005;18:562-566.

Plant GT. Putting ocular neuromyotonia in context. J Neuroophthalmol. 2006;26:241-243.

Sanders MD, Hoyt WF. Hypoxic ocular sequelae of carotid-cavernous fistulae. Br J Ophthalmol. 1969;53:82-97.

Sergott RC, Grossman RI, Savino PJ, Bosley TM, Schatz NJ. The syndrome of paradoxical worsening of dural-cavernous sinus arteriovenous malformations. Ophthalmology. 1987;94:205-221.

Shults WT, Hoyt WF, Behrens MM, et al. Ocular neuromyotonia. Arch Ophthalmol. 1986;104:1028-1034.

Smith JL, Taxdal DSR. Painful ophthalmoplegia: the Tolosa-Hunt syndrome. Am J Ophthalmol. 1966;61:1466-1472.

Stiebel-Kalish H, Setton A, Berenstein A, et al. Bilateral orbital signs predict cortical venous drainage in cavernous sinus dural AVMs. Neurology. 2002;58:1521-1524.


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