BASICS

DESCRIPTION

• Orbital apex syndrome: A constellation of findings including combined motor and sensory deficit of the eye and orbit secondary to multiple etiologies: Pathology involving the oculomotor nerve (III), trochlear nerve (IV), abducens nerve, (VI), and the first division of the trigeminal nerve (V1) in association with decreased optic nerve (II) function (1)[B]

• Cavernous sinus syndrome: orbital apex syndrome plus second branch of trigeminal nerve (V2) without optic nerve involvement

• Superior orbital fissure syndrome: cavernous sinus syndrome minus optic neuropathy

EPIDEMIOLOGY

Incidence

• Rare

• Positive correlation between the number of cranial nerves involved and presence of cavernous sinus syndrome (2)[B]

RISK FACTORS

• Diabetes

• Immunosuppression

• Cancer

• Trauma

• Sinus surgery

PATHOPHYSIOLOGY

Mass, inflammation, or vascular lesions in the region of the cavernous sinus, superior orbital fissure, or orbital apex causing dysfunction of the nerves in that area by direct compression, inflammation, or ischemia.

ETIOLOGY

• Inflammatory

• Infectious

• Neoplastic

• Traumatic/iatrogenic

• Vascular

COMMONLY ASSOCIATED CONDITIONS

• Infectious

• Fungal: aspergillosis, mucormycosis

• Bacterial: Staph, Strep, mycobacterium TB

• Inflammatory: sarcoidosis, systemic lupus erythematosus (SLE), giant cell arteritis, Wegener’s granulomatosis, idiopathic orbital pseudotumor (Tolosa–Hunt syndrome)

• Local neoplastic: meningioma, nasopharyngeal carcinoma, pituitary adenoma, squamous cell Ca

• Metastatic neoplastic: lung, breast, renal cell, malignant melanoma

• Vascular: carotid cavernous aneurysm or fistula, cavernous sinus thrombosis (septic or aseptic)

• Iatrogenic: endoscopic sinus surgery

• Trauma: penetrating injury, orbital fracture, foreign body

DIAGNOSIS

HISTORY

• Binocular double vision

• Reduced vision

• ±Pain

• ±Proptosis

PHYSICAL EXAM

• Best corrected visual acuity

• Decreased pupil reactivity with afferent pupillary defect

• Color vision/brightness

• Cranial nerve examination

• Decreased extraocular motility (multiple patterns)

• Oculomotor and abducens nerve most frequently involved

• Decreased sensation face, periorbital skin, cornea

• Orbital examination: lid position, resistance keratopathy associated with neurotrophic cornea (severe cases may require tarsorrhaphy.)

TREATMENT

ADDITIONAL TREATMENT

Issues for Referral

• Infectious disease: referral for inpatient internal medicine and infectious disease management

• Intraorbital mass with intracranial pathology refer to neurosurgery or oculoplastic surgery for biopsy, endocrinology for glucocorticoid management/replacement, infectious disease as necessary

• Inflammatory referral to rheumatology/immunology for treatment and ongoing care

Additional Therapies

Possible radiation therapy (RT), stereotactic RT.

COMPLEMENTARY & ALTERNATIVE THERAPIES

Immunosuppression for inflammatory lesions including steroids, methotrexate, azathioprine (Imuran), tumor necrosis factor alpha therapies (3)[B], (4)[C].

SURGERY/OTHER PROCEDURES

• Surgical biopsy may be required for definitive diagnosis of underlying etiology (5).

• Aggressive surgical debridement required for treatment of orbital mucormycosis

• Exenteration (surgical removal of all orbital contents) may be required if patient is at risk for cavernous sinus thrombosis secondary to greater than 50% risk of death associated with septic cavernous sinus thrombosis.

IN-PATIENT CONSIDERATIONS

Initial Stabilization

• Diabetes: mucormycosis—intensive care medical stabilization of blood sugar and ketoacidosis, and initial antifungal therapy prior to any surgical management

• Septic cavernous thrombosis requires emergent inpatient management

Admission Criteria

• Evidence of sinus disease in diabetic patient consistent with fungal sinusitis

• Signs of sepsis, or infection, ketoacidosis, hyperosmolar states, hyperglycemia

Discharge Criteria

Ability to treat with outpatient therapy, oral antibiotic, or antifungal therapies

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• In patients with infectious disease within 1 week of discharge with infectious disease and orbital surgeon or neuro-ophthalmology.

• In patients with inflammatory lesions 1 week with rheumatology, orbital surgeon or neuro-ophthalmology

Patient Monitoring

Monitor vision, corneal sensation, proptosis, and pattern of extraocular motility dysfunction

DIET

As per medical consultants

PATIENT EDUCATION

Patients with reduced vision and decreased corneal sensation must be educated to be aware of increased redness, swelling, and discharge as signs and symptoms of corneal decompensation, secondary to exposure, and possible infection with the ultimate risk of perforation and permanent visual loss or in extreme cases loss of eye.

PROGNOSIS

Related to underlying etiology

COMPLICATIONS

• Visual loss secondary to optic atrophy

• Orbital scarring with restrictive extraocular muscle dysfunction and diplopia.

• Retinal (macular) ischemia secondary to steal phenomenon in carotid–cavernous (CC) fistula

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