Cavernous sinus syndrome pdf

Tumors also may arise from either a pituitary tumor or by locally spreading, such as nasopharyngeal carcinoma with intracranial extension more in Southeast Asia. CCAs do not carry a major risk of subarachnoid hemorrhage.

CCFs can be classified as indirect, low-flow, or dural CCFs an indirect communication between the cavernous sinus and branches of the internal or external carotid artery within the adjacent dura.

They can also be classified as being direct or high-flow CCFs a direct connection between the intracavernous carotid artery and the cavernous sinus. The causes of CST are aseptic or infectious. Aseptic causes typically occur after surgery or trauma. The most common causative microorganism is Staphylococcus aureus, followed by milleri group streptococci which include Streptococcus constellatus , Streptococcus intermedius , and Streptococcus anginosus.

Septic cavernous sinus thrombosis is a rare but serious complication of cavernous sinus infections. Bacterial infection sources are explained above. One patient with tuberculosis is also reported. Out of 18 fungal infections, Aspergillosis accounted for An inflammatory process of unknown etiology causes Tolosa-Hunt syndrome. Inflammation causes swelling, pressure and then dysfunction of the structures, particularly of the CN.

Cavernous sinus tumors may cause isolated or combined ophthalmoplegia, painful ophthalmoplegia, anesthesia in CNIII, unitemporal or bitemporal visual field defects, acromegaly, and galactorrhea. Herpes zoster also may present with acute zoster ophthalmicus, typical skin lesion, and keratitis. Evaluation The diagnosis of CSS is challenging because of a wide variety of possible etiologies.

Cavernous Sinus Tumors To find out a source of mass. Is it primary, metastasis or local spread? A biopsy of the cavernous sinus tumors is rarely needed for diagnosis of primary tumors if no tumors are spreading from another primary source. Conventional digital subtraction angiography is the gold-standard test for diagnosis of CCF. CCFs can be seen on an orbital or trans-cranial ultrasound as well.

An aseptic thrombosis may be associated with hypercoagulable states as well as lymphoproliferative disorders. The patient may need further hematological workup. Order workup with protein, glucose, cell counts, cytology, Lyme and syphilis serology in cerebrospinal CSF. Investigate additional systemic inflammatory and granulomatous processes after other common causes have been excluded such as those from a tumor or vascular etiology.

Workup should include an erythrocyte sedimentation rate, C-reactive protein, angiotensin converting enzyme, antinuclear antibody, anti-dsDNA antibody, anti-Sm antibody, anti-neutrophil cytoplasmic antibody, serum protein electrophoresis with immunotyping from serum, CSF protein, glucose, cell counts, cytology, and angiotensin-converting enzyme.

Radiotherapy is important in providing excellent tumor control and avoiding the risk of surgery. About patients with cavernous sinus meningiomas treated with stereotactic radiosurgery in three large series from a dose of 12 to 14 Gy resulted in a five-year tumor control rate ranging from 94 to 98 percent.

Transsphenoidal surgery is the preferred treatment for most kinds of pituitary adenomas. Gamma knife surgery GKS is a common treatment for recurrent or residual pituitary adenomas. Carotid-cavernous aneurysms have the lowest rates of rupture is shown for unruptured cerebral aneurysms in a Japanese cohort.

Unruptured intracranial aneurysms treatment has been evaluated by a ISUIA performing cost-effectiveness analyzes that showed treatment was ineffective or not cost-effective for large aneurysms greater than 25 mm and located in the cavernous carotid artery for year-old patients. The management of CCFs depends on the classification of CCFs, the onset of symptoms, and the risk of long-term neurological impairment.

Endovascular treatment with trans-arterial or transvenous embolization is the preferred approach for closure of CCFs. Surgery can be considered when endovascular treatment is not possible or is unsuccessful. Surgery includes placement of packing, sealing with fascia and glue, suturing or clipping the fistula. Stereotactic radiosurgery is indicated when an endovascular approach is not feasible, and surgical intervention is difficult or carries a risk of significant morbidity. The management of increased intraocular pressure with topical agents, beta-blockers, acetazolamide, and intravenous corticosteroids may give some relief.

If vision loss is threatened or intraocular pressure remains elevated, closure of the fistula required for favorable pressure control. Patient with CST should be treated for 3 to 4 weeks with intravenous antibiotic s or somites 6 to 7 weeks on the clinical picture.

Treatment should be broad-spectrum with vancomycin vs. Retrospective analysis suggests that treatment with heparin may reduce mortality in carefully selected cases of septic cavernous-sinus thrombosis. Steroids in the acute phase of cerebral venous thrombosis CVT were not useful and were detrimental in patients without parenchymal cerebral lesions.

Glucocorticoids have been used for the management of THS since the s. Close clinical follow-up with repeat MRI every 1 to 2 months is necessary to be sure followed by a gradual taper for at least 4 to 6 weeks up to several months. Close clinical follow-up with repeat MRI, 1 to 2 months, is necessary to be sure the glucocorticoid treatment remains effective, and no evidence of another etiology develops, as seen in a case report showing the progression of THS to a CCF.

Radiotherapy and immunosuppressive medications like cyclosporine, azathioprine, methotrexate, mycophenolate mofetil, and infliximab have been tried in many case reports with benefits.

Other inflammatory CCS may respond to treatment of the specific systemic inflammation or vasculitis etiology. Differential Diagnosis Carotid-cavernous aneurysms. Review Questions Access free multiple choice questions on this topic.

Comment on this article. Figure Anatomy of the cavernous sinus. References 1. Microsurgical anatomy and approaches to the cavernous sinus.

No more fear of the cavernous sinuses! Diagn Interv Imaging. The Ishikawa classification of cavernous sinus lesions by clinico-anatomical findings.

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MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy. Eur J Radiol. Single-fraction radiosurgery for presumed intracranial meningiomas: efficacy and complications from a year experience. Stereotactic radiosurgery providing long-term tumor control of cavernous sinus meningiomas. Radiosurgical treatment of cavernous sinus meningiomas: experience with treated patients. J Neurooncol. Gamma Knife surgery for pituitary adenomas: factors related to radiological and endocrine outcomes.

The natural course of unruptured cerebral aneurysms in a Japanese cohort. N Engl J Med. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity.

Intracranial dural arteriovenous malformations: results of stereotactic radiosurgery in 17 patients. Clin Oncol R Coll Radiol. Desa V, Green R. Cavernous sinus thrombosis: current therapy. Thrombophilia is a significant risk factor for cavernous sinus thrombosis. Women who are pregnant, post-partum, or receiving oral contraceptives or hormone replacement therapy may be at increased risk. A variety of thrombophilic genetic disorders may lead to cavernous sinus thromboses.

Acquired disorders such as antiphospholipid antibody syndrome, hyperhomocysteinemia, heparin-induced thrombocytopenia, and obesity could also contribute to cavernous sinus thrombosis risk. Other risk factors for thrombosis include severe dehydration, such as in the hyperosmolar non-ketotic state, nephrotic syndrome, and sickle cell disease.

Cavernous sinus thrombosis is so rare that incidence data is difficult to estimate. A male or female predominance in cavernous sinus thrombosis is uncertain. Despite a female predominance in cerebral venous thrombosis, this may not be the case for cavernous sinus thrombosis. Weerasinghe reported a male to female ratio in 88 septic adult cases.

Similarly, Thatai et al. Historically, cerebral venous thrombosis has been reported to be more common in children and neonates than in adults, although the effects of routine vaccination and frequent use of antibiotics such as for otitis on this relationship are uncertain. The incidence and mortality may be decreasing likely due to the availability and use of antibiotics. The cavernous sinuses one on each side of the sella turcica, above and lateral to the sphenoid sinuses, anteriorly superior orbital fissure and posteriorly petrous part of the temporal lobe are trabeculated cavernous spaces created by the layers of dura mater and filled with venous blood.

This blood drains the superior and inferior ophthalmic veins and superficial cortical veins anteriorly and then drains into the basilar plexus posteriorly via the superior and inferior petrosal sinuses. Within the lumen of the cavernous sinuses pass the horizontal segment of the internal carotid artery, the sympathetic plexus and cranial nerve VI abducens medially.

Whereas the outer layers of the lateral wall of the carotid sinus are traversed by cranial nerves III oculomotor , V the first and second branches, ophthalmic and maxillary, of the trigeminal and IV trochlear. Embolization of bacteria and other infectious organisms which trigger thrombosis which then can trap infection within the cavernous sinus. Cavernous sinus thrombosis leads to decreased drainage from the facial vein and superior and inferior ophthalmic veins resulting in facial and periorbital edema, ptosis, proptosis, chemosis, discomfort and pain with eye muscle movement, papilledema, retinal venous distention, and loss of vision.

Lack of valves in the dural sinus system allows flow through the emissary veins into and out of the cavernous sinus and thrombus can propagate into the dural system.

Also, communication between the right and left cavernous sinuses via the intercavernous sinuses, anterior and posterior to the sella, allows the spread of thrombus and infection from one side to the other. Local compression and inflammation of cranial nerves can lead to several partial or complete cranial neuropathies including:.

Septic cavernous venous thrombosis can result in the central nervous system or infectious pulmonary complications. Because the dural venous and cavernous system is valveless, this venous blood can communicate with the dural sinuses and cerebral and emissary veins leading to meningitis, dural empyema or brain abscess.

Infection can spread via the jugular vein to the pulmonary vasculature resulting in septic emboli or abscess, pneumonia or empyema. Stroke can occur following carotid artery narrowing, vasculitis, or hemorrhagic infarction following progression to cortical vein thrombosis.

Symptoms may be present at onset or progress subacutely over days. Usually, it starts with one eye and then progresses to another eye. Less common symptoms may include rigors, stiff neck, facial numbness, confusion, seizures, stroke symptoms, or coma. Neurologic findings such as altered mentation, lethargy, or obtundation, are not unusual.

Seizures or stroke syndromes such as hemiparesis are rare. Individually, a sixth cranial neuropathy is the most common neuropathy, resulting in partial ophthalmoplegia with limited eye abduction. Most cases, however, progress rapidly to complete external ophthalmoplegia from third, fourth and sixth cranial neuropathy.

Internal ophthalmoplegia results in a nonreactive pupil, from paralysis of the iris and ciliary body, either constricted miosis from loss of sympathetic fibers from the short ciliary nerves or dilated mydriasis from loss of parasympathetic fibers from cranial nerve III.

The sensory exam might reveal diminished sensation to face due to compression of the ophthalmic and maxillary branches of the fifth cranial nerve and an impaired corneal reflex. The optimal diagnostic test is neuroimaging with either contrast-enhanced computed tomography CT or magnetic resonance imaging MRI. Contrast-enhanced MRI brain shows bulging of the cavernous sinus, increased dural enhancement, and absent flow void is seen.

CTV and enhanced-MRV can detect dilation of the cavernous sinus, enhancement, and convexity of the lateral wall which is normally concave on coronal views, heterogeneous and asymmetric filling defects after contrast, increased density of orbital fat, thrombosis in the superior ophthalmic vein or veins and tributaries leading to the cavernous sinus.

Additionally, carotid artery narrowing, carotid arterial wall enhancement, cerebral infarcts, intraparenchymal hemorrhages, empyema, meningitis, cerebritis or abscess may be noted. Blood cultures should be obtained routinely and are frequently positive. Lumbar puncture is important to exclude meningitis and may show elevated opening pressure and pleocytosis even in culture-negative samples.

Screening for thrombophilia may give false results during anticoagulation therapy and should be delayed until after treatment is completed. Because of the rarity of diagnosis, no randomized controlled trials are available, and expert opinion guides treatment. In general, antimicrobial and antithrombotic therapies are primary considerations.

Antimicrobial therapy includes an anti-staphylococcal agent vancomycin if methicillin resistance is high, or nafcillin , a third-generation cephalosporin, and metronidazole for anaerobic coverage as well as antifungal therapy with amphotericin B.

A prolonged duration of parenteral therapy, typically three to four weeks or at least two weeks beyond clinical resolution is suggested. Most experts recommend anticoagulation, in the absence of strong contraindications, with either unfractionated heparin UFH or low molecular weight heparin LMWH for several weeks to several months. There does remain controversy with anticoagulation. The advantage would be to halt the progression of thrombosis, prevent clot propagation, and possibly allow penetration of antibiotics, whereas the risk would be systemic or intracranial bleeding or even dissemination of septic emboli.

Although cavernous sinus thrombosis differs from cerebral venous and sinus thrombosis, the Cochrane Collaboration Coutinho suggests that anticoagulation for cerebral venous and sinus thrombosis appears safe, even in the presence of intracranial hemorrhage, and demonstrates a potentially important mortality reduction though not statistically significant.

The European Federation of Neurological Societies EFNS recommends three months of anticoagulation in secondary cerebral venous and sinus thrombosis with a transient risk factor, six to 12 months for idiopathic cerebral venous and sinus thrombosis and those with mild thrombophilia and indefinitely if subsequent cerebral venous and sinus thrombosis or severe thrombophilia.

As in cerebral venous and sinus thrombosis, there is inadequate evidence to support thrombolysis in cavernous sinus thrombosis. Corticosteroids are often given but without demonstrated efficacy. The potential benefit would be decreased inflammation and vasogenic edema surrounding cranial nerves and orbital structures. Steroids are necessary, however, for cases of hypopituitarism. No surgical interventions are recommended for the cavernous sinuses themselves.

However, some patients might require sphenoidectomy, ethmoidectomy, maxillary antrostomy, mastoidectomy, abscess drainage, craniotomy subdural empyema , orbital decompression, or ventricular shunt placement.

The differential diagnosis includes other causes of cavernous sinus syndrome and painful ophthalmoplegia. The disorder is best managed by an interprofessional team that includes a primary care provider nurse practitioner, physician assistant, family physician ophthalmologist, neurosurgeon, neurologist, infectious disease expert, hematologist, and an emergency department physician. Other complications can include sepsis, meningitis, subdural empyema, brain abscess, blindness, panhypopituitarism, intracranial hypertension, infectious arteritis or mycotic aneurysm of the internal carotid artery, vasospasm, septic emboli, stroke from carotid narrowing, cortical vein thrombosis or hemorrhagic infarction, coma, and death.

This book is distributed under the terms of the Creative Commons Attribution 4. Turn recording back on. National Center for Biotechnology Information , U. StatPearls [Internet]. Search term. Cavernous Sinus Thrombosis Michael C. Author Information Authors Michael C.

Continuing Education Activity Cavernous sinus thrombosis CST is a rare, life-threatening disorder that can complicate facial infection, sinusitis, orbital cellulitis, pharyngitis, or otitis or following traumatic injury or surgery, especially in the setting of a thrombophilic disorder. Introduction Cavernous sinus thrombosis CST is a rare, life-threatening disorder that can complicate facial infection, sinusitis, orbital cellulitis, pharyngitis, or otitis or following traumatic injury or surgery, especially in the setting of a thrombophilic disorder.

Etiology Cavernous sinus thrombosis is usually septic, but can also be aseptic. Epidemiology Cavernous sinus thrombosis is so rare that incidence data is difficult to estimate. Pathophysiology The cavernous sinuses one on each side of the sella turcica, above and lateral to the sphenoid sinuses, anteriorly superior orbital fissure and posteriorly petrous part of the temporal lobe are trabeculated cavernous spaces created by the layers of dura mater and filled with venous blood.

Septic cavernous sinus thrombosis occurs from the following: Local spread, often from valveless facial and ophthalmic veins. Adjacent infections, such as sinusitis possibly the most common cause , especially sphenoiditis and ethmoiditis Press.

Facial cellulitis or abscess especially within the danger triangle of the face, formed by the corners of mouth and bridge of the nose. Diplopia from partial or complete external ophthalmoplegia due to compression of the sixth abducens , third oculomotor and fourth trochlear nerves. Limited eye abduction from abducens palsy is most common early finding, often progressing to inability to move the eye in any direction when III, IV and VI are involved.

Numbness or paresthesias around the eyes, nose, forehead and loss of corneal blink reflex from the ophthalmic nerve, a branch of the trigeminal nerve V. Facial pain, paresthesias or numbness from compression of the maxillary branch of the trigeminal nerve.