Understanding the clinical management of cavernous sinus syndrome (CCS) requires an extensive understanding of its anatomy. It is a small but complex, and it contains several important structures. The cavernous sinus (CS) is not a venous plexus, but it is a true dural venous sinus. It is bordered by the temporal bone of the skull and the sphenoid bone and lies lateral to the sella turcica. The inferior and lateral walls and the roof of CS are extensions of the dura mater. There may or may not be a thin layer of collagen at the medial wall. The blood-filled space contains the internal carotid artery (carotid siphon) surrounded by sympathetic fibers, as well as cranial nerves (CN). In particular, CN III (oculomotor nerve), IV (trochlear nerve), 2 branches of the V (trigeminal nerve): V1 (ophthalmic) and V2 (maxillary), all pass through this blood-filled space (Figure "Anatomy of the cavernous sinus"). Cavernous sinus syndrome is any disease process involving the CS. CCA is characterized by signs and symptoms resulting in ophthalmoplegia, chemosis, proptosis, Horner syndrome, or trigeminal sensory loss.
Determining the etiology of CCS remains difficult, despite the improvement of diagnostic techniques including improvements in imaging and analysis of blood and CSD. It is also difficult to make a diagnosis from CS tissue. Neoplastic (metastatic vs. primary), infectious, inflammatory, vascular, and traumatic processes are the principal causes of CCS. Jefferson first classified the disorder in 1938 into 3 types: anterior, middle, and posterior CCS. This classification is based on the extent of involvement of the trigeminal nerve. In 1996, Ishikawa emphasized the lack of a clinical-anatomical correlation in Jefferson's classification and proposed a new classification of CCS. CCS was classified into 3 separated part such as the anterior, middle, posterior by Ishikawa. They used the optic canal and the maxillary nerve as anatomical landmarks. The Ishikawa classification in a study with 162 patients with CS lesions was shown to be superior in identifying and classifying the localization of CS lesions. However, Ishikawa classification is not superior to Jefferson based on etiology, shown by a study at a tertiary care center in Northern India with 73 consecutive CCS patients.
Estimates of the incidence of CCS are not well documented in the literature. An analysis of 151 cases from Los Angeles in 1996 showed 89 (59%) were men. The average age was 39 years, with a range of 15 to 72 years. The tumor is the most common cause of CCS at 30%, followed by trauma at 24%. Other series excluded trauma as a cause of CCS. A series of 126 patients from Barcelona, Spain in 2007 showed that 42% were men. The average age was 55.5 with a range of 40 to 70 years. Tumor, again, being the most common cause of CCS with at 63%. According to this study, the second most common cause is of vascular etiology at 20%. In a 2017 study of 73 cases at tertiary care centers in Northern India showed that 64% were men. The average age was 44 years, with a range of 11 to 70 years. The tumor was still the most common cause of CCS at 30%, but unlike previously described series from the Western world, the prevalence of fungal infections was 24.6%, and the prevalence of Tolosa-Hunt syndrome (THS) was also much higher at 23.2%. Overall, CCS was not predominant in any gender. Patients may present at any age, ranging from 20 to 80 years of age.
Cavernous Sinus Tumors
Cavernous sinus vascular pathologies; vascular pathologies in the CS include carotid-cavernous fistulas (CCFs), carotid-cavernous aneurysms (CCAs), and cavernous sinus thrombosis (CST)
Complaints that occur in different etiologies of SSC share common symptoms such as a headache (up to 90%), diplopia (up to 90%), painless or painful ophthalmoplegia, ptosis, proptosis, chemosis, facial sensory loss, visual loss, fever, facial asymmetry, hearing loss, and seizure. Assessment should include a determination of whether symptoms are unilateral or bilateral, and if the onset of symptoms is acute, subacute, or chronic, and if conditions are painful or painless.
Physical exam findings depend on the etiology as well as the extent of CS involvement. The following sign(s) may be seen in patients with CCS:
Common symptoms and signs on presentation can usually accompany the specific etiologies below:
The diagnosis of CSS is challenging because of a wide variety of possible etiologies. Studies of the orbit, sella, and parasellar region play a major role in making a diagnosis. Precontrast and postcontrast scans are advisable. Contrast-enhanced computed tomography (CT) scans give a better visualization of bone and calcium. Magnetic resonance imaging (MRI) provides better detail of all soft tissues contained in the sinuses. CT angiography (CTA), CT venography (CTV) magnetic resonance angiography (MRA), MR venography (MRV) and conventional digital subtraction angiography helps to visualize all vascular structures with details. Routine, cytologic, infectious, and/or inflammatory examinations of serum and cerebral spinal fluid (CSF) may be helpful as well.
Cavernous Sinus Tumors
Cavernous Sinus Vascular
Cavernous Sinus Tumors
Cavernous Sinus Vascular
CSS are difficult to diagnose and manage. Because there are many causes of CSS, they are best managed by a multidisciplinary team that includes a specialty trained neurology nurse, neurologist, ophthalmologist, neurosurgeon, radiologist, internist, ENT surgeon, and an infectious disease specialist. The majority of these patients require monitoring by the ICU nurse. The treatment of SCC depends on the cause. Because of the vast number of structures that run in the cavernous sinus, some type of residual visual and neurological deficit may persist after treatment.
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