• Other disorders that may resemble CSS include orbital cellulitis, sphenoid sinusitis, thyroid eye disease, cavernous carotid aneurysm, orbital apex tumor, orbital pseudotumor, ocular migraine, and ocular trauma.
• CSS may be due to septic or aseptic venous thrombosis, neoplasms, carotid-cavernous fistulas, or idiopathic inflammation.
• First priority is to rule out septic cavernous sinus thrombosis, a life-threatening complication of infections of the face, sinuses, middle ear, teeth, and orbit.
• Infectious agents include Staphylococcus aureus, Streptococcus pneumoniae, gram-negative rods, and anaerobes.
• Mucormycosis and Aspergillus may be found in immunocompromised patients.
• Aseptic venous thrombosis has been associated with sickle cell anemia, trauma, dehydration, vasculitis, pregnancy, oral contraceptive use, congenital heart disease, inflammatory bowel disease, and hypercoagulable states.
• Neoplasms involving the cavernous sinus include pituitary adenomas, meningiomas, craniopharyngiomas, lymphomas, neuromas, chordomas, chondrosarcomas, and nasopharyngeal carcinomas. Neoplasms may present with diplopia, visual-effect deficits, headache, or isolated cranial nerve deficits. The lateral extension of pituitary neoplasms into the cavernous sinus usually affects the third cranial nerve, with the fourth and sixth nerves less commonly involved. Rupture of a cystic craniopharyngioma may appear as acute CSS.
• Carotid-cavernous fistulas, which often have a more chornic course, are direct high-flow shunts between the internal carotid artery and the cavernous sinus. Almost always sequelae of trauma, they may present with a history of ocular motility deficits, arterialization of conjunctival vessels, and a bruit usually heard best over the orbit.
• Non-specific and idiopathic inflammation of the cavernous sinus, also called idiopathic cavernous sinusitis or Tolosa-Hunt syndrome, has been reported in patients as young as 3½ years. This is a diagnosis of exclusion.

Question: History of a recent local infection?
Significance: Facial furuncle or cellulitis, sinusitis, dental infection, otitis, or orbital cellulitis may predispose to CSS.

Question: Are local or systemic symptoms present?
Significance: Fever, headache, eye pain, diplopia, and facial parasthesias may be present.

Finding: Conjunctival injection with lid swelling and proptosis.
Significance: Signs of cavernous sinus venous congestion

Finding: Ptosis, anisocoria, ophthalmoparesis, and facial sensory changes
Significance: Signs of cranial nerve involvement

Finding: Horner syndrome.
Significance: Sympathetic nerve fibers traveling with V1 may be affected

Finding: Begin unilaterally
Significance: May rapidly spread bilaterally.

Finding: Optic nerve and visual acuity are spared early in CSS.
Significance: Can be affected as it progresses.

Finding: Funduscopic findings include venous dilatation and hemorrhages.
Significance: Papilledema is rare in acute cavernous sinus disease.

Finding: Resistance to retropulsion
Significance: May be very painful to elicit, but is a helpful sign.

Finding: Ocular bruit
Significance: May be heard in any acute CSS, but especially in carotid-cavernous fistula.

Finding: Signs of meningitis and systemic toxicity
Significance: These rapidly evolve if infections are untreated.

Test: CBC, ESR, PT/PTT, blood culture
Significance: Basic studies in any child with suspected acute CSS. A lumbar puncture should be performed if there is no contraindication and infection is suspected.

Test: MRI or CT
Significance: Any child with proptosis, cranial-nerve findings, or an ocular bruit should have an urgent MRI or CT. MRI, with and without gadolinium, with special attention to the cavernous sinus and parasellar region, is the imaging study of choice. Magnetic resonance venography (MRV) may be helpful.

Test: Angiography
Significance: Suspected carotid-cavernous fistulas require angiography.

Test: Nasopharyngeal biopsy and culture
Significance: Helpful if Mucormycosis or Aspergillus is suspected.

Test: Blood cultures
Significance: Positive in 70% of cases of septic venous sinus thrombosis.

• For septic cavernous sinus thrombosis, broad-spectrum antibiotics, including anaerobe coverage, are begun immediately. Duration of therapy is usually 2 to 4 weeks beyond the resolution of symptoms.
• Amphotericin B is given if Mucormycosis or Aspergillus is suspected.
• Surgical drainage of the primary infection (i.e., sinusitis) may be indicated (avoiding surgical manipulation of the cavernous sinus itself).
• Anticoagulation is controversial, but one study in adults found heparin reduced morbidity from septic cavernous sinus thrombosis.
• Post-traumatic carotid-cavernous fistulas rarely close spontaneously and have been treated with endoarterial balloon embolization.
• Consultation with a neuro-oncologist and a neurosurgeon are important for suspected neoplasms or surgical lesions.
• Idiopathic cavernous sinusitus, a diagnosis of exclusion, responds to corticosteroids. Treatment should not be started until neoplasm and infection have been ruled out.

• Septic cavernous sinus thrombosis may relapse, or embolic abscesses may develop, 2 to 6 weeks after therapy has been stopped. Mortality remains 13% to 30%, and less than 40% of patients recover fully from cranial-nerve deficits.
• Patients with carotid-cavernous fistulas frequently have persistent cranial-nerve deficits even after embolization.
• Idiopathic cavernous sinusitis responds to steroids, but relapses can be problematic. Clinical follow-up and serial MRI scans are indicated to rule out a low-grade neoplasm or fungal infection.

• Local bacterial infections—sinusitis, facial cellulitis, dental abscesses, otitis, pre-septal cellulitis and orbital cellulitis—should be treated promptly.
• Patients with suspected hypercoagulable states should be evaluated fully; dehydration must be avoided in such patients and anti-coagulation may be necessary.

• Ophthalmoplegic migraine must be distinguished from CSS by neuroimaging studies and history. Proptosis does not occur in migraine. This is a diagnosis of exclusion, especially on first presentation.
• Acute infection and hemorrhage of the pituitary gland—pituitary apoplexy—may present with acute bilateral ophthalmoplegia and signs of acute pituitary insufficiency. This most commonly occurs with pituitary neoplasms, but may also occur in pregnant women at the time of delivery. Chronic granulomatous disorders and tuberculosis may underlie CSS.

Q: Will my child’s eye movements return to normal?
A: In most cases, oculomotor nerves regain function as other signs improve, though they may take the longest to recover.

Q: Can more pain medicine be given?
A: There is often an attempt to balance side effects of sedation and hypoventilation against the need for pain control, especially when intracranial pressure is a concern.

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