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Cavernous sinus syndrome

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Cavernous sinus syndromes refer to constellations of clinical signs and symptoms referable to lesions within or adjacent to the cavernous sinus.

Clinical presentation

Patients present with multiple unilateral cranial neuropathies involving any combination of the following:

ophthalmoplegia (cranial nerves III, IV, or VI), most commonly presenting as diplopia
facial sensory loss (cranial nerves V1 and V2)
Horner syndrome (oculosympathetic fibers)

Pain can occur, especially with inflammatory processes.

Additional symptoms may be vascular in origin:

chemosis
proptosis

Pathology

Causes are diverse ^1-4and may be organized by surgical sieve:

neoplastic
- secondary (direct invasion, perineural spread, or hematogenous spread)
  - head and neck tumors, e.g. nasopharyngeal carcinoma, adenoid cystic carcinoma, squamous cell carcinoma, juvenile angiofibroma
  - pituitary tumors, e.g. pituitary adenoma, craniopharyngioma
  - skull base tumors, e.g. chordoma, chondrosarcoma
  - distant metastases, e.g. lung cancer, breast cancer
  - hematolymphoid tumors, e.g. lymphoma, plasmacytoma, leukemia
  - histiocytoses, e.g. Langerhans cell histiocytosis, Rosai-Dorfman disease
- primary
  - meningeal tumors
    - meningioma
    - melanocytoma
  - peripheral nerve sheath tumors
autoinflammatory
- Tolosa-Hunt syndrome (idiopathic inflammation)
- inflammatory pseudotumor of the skull base
- granulomatosis with polyangiitis (Wegener's)
- sarcoidosis
- hypertrophic pachymeningitis
infection
- bacterial
  - meningitis
  - cavernous sinus thrombophlebitis (septic cavernous sinusitis)
  - actinomycosis
- fungal
  - mucormycosis
  - aspergillosis
- viral
  - herpes zoster ophthalmicus
vascular
- cavernous malformation (cavernous hemangioma)
- cavernous sinus thrombosis
- internal carotid artery aneurysm
- caroticocavernous fistula
- dural arteriovenous fistula
congenital/developmental
- epidermoid cyst
- dermoid cyst
traumatic

-<p><strong>Cavernous sinus syndromes</strong> refer to constellations of clinical signs and symptoms referable to lesions within or adjacent to the <a title="Cavernous sinus" href="/articles/cavernous-sinus">cavernous sinus</a>.</p><h4>Clinical presentation</h4><p>Patients present with multiple unilateral cranial neuropathies involving any combination of the following:</p><ul>
+<p><strong>Cavernous sinus syndromes</strong> refer to constellations of clinical signs and symptoms referable to lesions within or adjacent to the <a href="/articles/cavernous-sinus">cavernous sinus</a>.</p><h4>Clinical presentation</h4><p>Patients present with multiple unilateral cranial neuropathies involving any combination of the following:</p><ul>
~~-</ul><h4>Pathology</h4><p>Causes are diverse <sup>1-4 </sup>and may be organized by <a title="Surgical sieve (mnemonic)" href="/articles/surgical-sieve-mnemonic">surgical sieve</a>:</p><ul>~~
+</ul><h4>Pathology</h4><p>Causes are diverse <sup>1-4 </sup>and may be organized by <a href="/articles/surgical-sieve-mnemonic">surgical sieve</a>:</p><ul>
-<a title="WHO classification of head and neck tumors" href="/articles/who-classification-of-head-and-neck-tumors">head and neck tumors</a>, e.g. <a title="Nasopharyngeal carcinoma" href="/articles/nasopharyngeal-carcinoma">nasopharyngeal carcinoma</a>, <a title="Adenoid cystic carcinoma" href="/articles/adenoid-cystic-carcinoma">adenoid cystic carcinoma</a>, <a title="Squamous cell carcinoma (head and neck)" href="/articles/head-and-neck-squamous-cell-carcinoma-overview">squamous cell carcinoma</a>, <a title="Juvenile angiofibroma" href="/articles/juvenile-nasopharyngeal-angiofibroma">juvenile angiofibroma</a>
+<a href="/articles/who-classification-of-head-and-neck-tumors">head and neck tumors</a>, e.g. <a href="/articles/nasopharyngeal-carcinoma">nasopharyngeal carcinoma</a>, <a href="/articles/adenoid-cystic-carcinoma">adenoid cystic carcinoma</a>, <a href="/articles/head-and-neck-squamous-cell-carcinoma-overview">squamous cell carcinoma</a>, <a href="/articles/juvenile-nasopharyngeal-angiofibroma">juvenile angiofibroma</a>
-<a title="Pituitary tumors" href="/articles/pituitary-tumours">pituitary tumors</a>, e.g. <a href="/articles/pituitary-adenoma">pituitary adenoma</a>, <a title="Craniopharyngioma" href="/articles/craniopharyngioma">craniopharyngioma</a>
+<a href="/articles/pituitary-tumours">pituitary tumors</a>, e.g. <a href="/articles/pituitary-adenoma">pituitary adenoma</a>, <a href="/articles/craniopharyngioma">craniopharyngioma</a>
-<li>skull base tumors, e.g. <a title="Chordoma" href="/articles/chordoma">chordoma</a>, <a title="Chondrosarcoma of the skull base" href="/articles/chondrosarcoma-of-the-skull-base">chondrosarcoma</a>
+<li>skull base tumors, e.g. <a href="/articles/chordoma">chordoma</a>, <a href="/articles/chondrosarcoma-of-the-skull-base">chondrosarcoma</a>
~~-<li>distant metastases, e.g. <a title="Lung cancer" href="/articles/lung-cancer-3">lung cancer</a>, <a title="Breast cancer" href="/articles/breast-neoplasms">breast cancer</a>~~
+<li>distant metastases, e.g. <a href="/articles/lung-cancer-3">lung cancer</a>, <a href="/articles/breast-neoplasms">breast cancer</a>
-<a title="WHO classification of tumors of hematopoietic and lymphoid tissues" href="/articles/who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues-1">hematolymphoid tumors</a>, e.g. <a title="Lymphoma" href="/articles/lymphoma">lymphoma</a>, <a title="leukemia" href="/articles/leukemia">leukemia</a>
+<a href="/articles/who-classification-of-tumours-of-haematopoietic-and-lymphoid-tissues-1">hematolymphoid tumors</a>, e.g. <a href="/articles/lymphoma">lymphoma</a>, <a href="/articles/plasmacytoma">plasmacytoma</a>, <a href="/articles/leukemia">leukemia</a>
-<a title="2016 Histiocyte Society classification of histiocytoses" href="/articles/2016-histiocyte-society-classification-of-histiocytoses">histiocytoses</a>, e.g. <a title="Langerhans cell histiocytosis (CNS manifestations)" href="/articles/langerhans-cell-histiocytosis-cns-manifestations">Langerhans cell histiocytosis,</a> <a title="Rosai-Dorfman disease" href="/articles/rosai-dorfman-disease">Rosai-Dorfman disease</a>
+<a href="/articles/2016-histiocyte-society-classification-of-histiocytoses">histiocytoses</a>, e.g. <a href="/articles/langerhans-cell-histiocytosis-cns-manifestations">Langerhans cell histiocytosis,</a> <a href="/articles/rosai-dorfman-disease">Rosai-Dorfman disease</a>
~~-<li><a title="Meningioma" href="/articles/meningioma">meningioma</a></li>~~
~~-<li><a title="Meningeal melanocytoma" href="/articles/meningeal-melanocytoma">melanocytoma</a></li>~~
+<li><a href="/articles/meningioma">meningioma</a></li>
+<li><a href="/articles/meningeal-melanocytoma">melanocytoma</a></li>
~~-<a title="Peripheral nerve sheath tumors" href="/articles/peripheral-nerve-sheath-tumours">peripheral nerve sheath tumors</a><ul>~~
~~-<li><a title="Intracranial schwannoma" href="/articles/intracranial-schwannoma">schwannoma</a></li>~~
~~-<li><a title="Neurofibroma" href="/articles/neurofibroma">neurofibroma</a></li>~~
~~-<li><a title="Malignant peripheral nerve sheath tumour" href="/articles/malignant-peripheral-nerve-sheath-tumour">malignant peripheral nerve sheath tumor</a></li>~~
+<a href="/articles/peripheral-nerve-sheath-tumours">peripheral nerve sheath tumors</a><ul>
+<li><a href="/articles/intracranial-schwannoma">schwannoma</a></li>
+<li><a href="/articles/neurofibroma">neurofibroma</a></li>
+<li><a href="/articles/malignant-peripheral-nerve-sheath-tumour">malignant peripheral nerve sheath tumor</a></li>
~~-<a title="Tolosa-Hunt syndrome" href="/articles/tolosa-hunt-syndrome-3">Tolosa-Hunt syndrome</a> (idiopathic inflammation)</li>~~
~~-<li><a title="inflammatory pseudotumor of the skull base" href="/articles/inflammatory-pseudotumor-of-the-skull-base">inflammatory pseudotumor of the skull base</a></li>~~
~~-<li><a title="Granulomatosis with polyangiitis (CNS manifestations)" href="/articles/granulomatosis-with-polyangiitis-cns-manifestations-1">granulomatosis with polyangiitis (Wegener's)</a></li>~~
~~-<li><a title="Neurosarcoidosis" href="/articles/neurosarcoidosis">sarcoidosis</a></li>~~
~~-<li><a title="Hypertrophic pachymeningitis" href="/articles/hypertrophic-pachymeningitis">hypertrophic pachymeningitis</a></li>~~
+<a href="/articles/tolosa-hunt-syndrome-3">Tolosa-Hunt syndrome</a> (idiopathic inflammation)</li>
+<li><a href="/articles/inflammatory-pseudotumor-of-the-skull-base">inflammatory pseudotumor of the skull base</a></li>
+<li><a href="/articles/granulomatosis-with-polyangiitis-cns-manifestations-1">granulomatosis with polyangiitis (Wegener's)</a></li>
+<li><a href="/articles/neurosarcoidosis">sarcoidosis</a></li>
+<li><a href="/articles/hypertrophic-pachymeningitis">hypertrophic pachymeningitis</a></li>
~~-<li><a title="Meningitis" href="/articles/leptomeningitis">meningitis</a></li>~~
+<li><a href="/articles/leptomeningitis">meningitis</a></li>
~~-<a title="cavernous sinus thrombophlebitis" href="/articles/cavernous-sinus-thrombophlebitis">cavernous sinus thrombophlebitis</a> (septic cavernous sinusitis)</li>~~
+<a href="/articles/cavernous-sinus-thrombophlebitis">cavernous sinus thrombophlebitis</a> (septic cavernous sinusitis)</li>
~~-<li><a title="Angioinvasive central nervous system aspergillosis" href="/articles/cns-aspergillosis">aspergillosis</a>~~
+<li><a href="/articles/cns-aspergillosis">aspergillosis</a>
~~-<li>viral<ul><li><a title="herpes zoster ophthalmicus" href="/articles/herpes-zoster-ophthalmicus">herpes zoster ophthalmicus</a></li></ul>~~
+<li>viral<ul><li><a href="/articles/herpes-zoster-ophthalmicus">herpes zoster ophthalmicus</a></li></ul>
~~-<li><a title="Cavernous sinus thrombosis" href="/articles/cavernous-sinus-thrombosis">cavernous sinus thrombosis</a></li>~~
~~-<li>internal carotid artery <a title="Intracranial aneurysm" href="/articles/saccular-cerebral-aneurysm">aneurysm</a>~~
+<li><a href="/articles/cavernous-sinus-thrombosis">cavernous sinus thrombosis</a></li>
+<li>internal carotid artery <a href="/articles/saccular-cerebral-aneurysm">aneurysm</a>
~~-<li><a title="Dural arteriovenous fistula" href="/articles/dural-arteriovenous-fistula">dural arteriovenous fistula</a></li>~~
+<li><a href="/articles/dural-arteriovenous-fistula">dural arteriovenous fistula</a></li>
~~-<li><a title="Intracranial epidermoid cyst" href="/articles/intracranial-epidermoid-cyst">epidermoid cyst</a></li>~~
~~-<li><a title="Intracranial dermoid cyst" href="/articles/intracranial-dermoid-cyst-1">dermoid cyst</a></li>~~
+<li><a href="/articles/intracranial-epidermoid-cyst">epidermoid cyst</a></li>
+<li><a href="/articles/intracranial-dermoid-cyst-1">dermoid cyst</a></li>
~~-</ul><h4>See also</h4><ul><li><a title="Cavernous sinus mass" href="/articles/cavernous-sinus-mass">cavernous sinus mass</a></li></ul>~~
+</ul><h4>See also</h4><ul><li><a href="/articles/cavernous-sinus-mass">cavernous sinus mass</a></li></ul>

References changed:

1. Lee JH, Lee HK, Park JK, Choi CG, Suh DC. Cavernous sinus syndrome: clinical features and differential diagnosis with MR imaging. (2003) AJR. American journal of roentgenology. 181 (2): 583-90. <a href="https://doi.org/10.2214/ajr.181.2.1810583">doi:10.2214/ajr.181.2.1810583</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12876052">Pubmed</a> <span class="ref_v4"></span>
2. Keane JR. Cavernous sinus syndrome. Analysis of 151 cases. (1996) Archives of neurology. 53 (10): 967-71. <a href="https://www.ncbi.nlm.nih.gov/pubmed/8859057">Pubmed</a> <span class="ref_v4"></span>
3. Nadarajah J, Madhusudhan KS, Yadav AK, Chandrashekhara SH, Kumar A, Gupta AK. MR imaging of cavernous sinus lesions: Pictorial review. (2015) Journal of neuroradiology. Journal de neuroradiologie. 42 (6): 305-19. <a href="https://doi.org/10.1016/j.neurad.2015.04.010">doi:10.1016/j.neurad.2015.04.010</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26421483">Pubmed</a> <span class="ref_v4"></span>
4. Razek AA, Castillo M. Imaging lesions of the cavernous sinus. (2009) AJNR. American journal of neuroradiology. 30 (3): 444-52. <a href="https://doi.org/10.3174/ajnr.A1398">doi:10.3174/ajnr.A1398</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19095789">Pubmed</a> <span class="ref_v4"></span>

Sections changed:

Syndromes

Systems changed:

Central Nervous System

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Case 1: nasopharyngeal ~~carcinoma~~cancer

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Cavernous sinus syndrome

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