Approach To Cavernous Sinus Syndrome.pptx

cavernous sinus syndrome, clinical approach, differential diagnosis and imaging

1. Approach To Cavernous Sinus Syndrome & Tolosa Hunt Syndrome DR. VIJAY PRAKASH HAWA SR NEUROLOGY GMC, KOTA

2. Cavernous sinus anatomy 2 cms long 1 cm wide Situated in middle cranial fossa on the sides of body of sphenoid Anterior – sup. Orbital fissure Posterior - Apex of petrous temp. bone Floor & medial wall – endosteal layer of dura Roof & lateral Wall - meningeal layer of dura

3. External relations 1. Above – Optic chiasma, backward and upward turn of carotid a. 2. Medial – Hypophysis cerebri 3. Lateral – Posterolateral part of cavum trigeminale containing ganglion 4. Below - Shenoid sinus

4. Structure passing through 1. Int. carotid A. surrounded by venous and sympathetic plexuses 2. Abducent N. (Inf. lat. to carotid art.) Structures in lateral wall Oculomotor N. Trcochlear N. Ophthalmic N. Maxillary N.

5. Tributaries 1. Sup. Ophthalmic vein 2. Branch of Inf. Ophthalmic vein 3. Central vein of retina 4. Superficial middle cererbal vein 5. Inferior cerebral veins 6. Sphenoparietal sinus 7. Anterior / Frontal trunk of middle meningeal vein

6. Communications 1. Transverse sinus via Sup. petrosal sinus 2. Int. Jugular vein through inf. petrosal sinus 3. Pterygoid venous plexus through emissary veins - foramen ovale , spinosum and lacerum 4. Facial vein through sup. Ophthalmic vein & angular vein or pterygoid venous plexus and deep facial vein 5. Opp. Cavernous sinus through ant / post intercavernous sinuses 6. Sup. Sag. Sinus through Middle cerebral vein

7. CAVERNOUS SINUS SYNDROME

8. Etiology of CSS RadioGraphics 2019; 39:795–819

9. Etiology of CSS in India

10. Wells CD, Moodley AA. HIV-associated cavernous sinus disease. S Afr J HIV Med. 2019;20(1), a862

11. When should impairment of the cavernous sinuses be suspected? Headache Ophthalmoplegia- Total (III, IV& VI) Or Partial Ptosis Exophthalmos Chemosis Horner Syndrome (Characterized By Partial Ptosis And Miosis, With Or Without Anhidrosis) Facial Pain Or Loss Of Sensation In The Trigeminal Nerve Distribution.

12. Cavernous sinus pathology: how to get clues?. Lesions are often difficult to distinguish from each other. There is no correlation between lesion size and clinical symptoms The first step is given by the clinical data: sudden and acute onset of the symptoms- vascular subacute and painful - inflammatory condition in young patients or lymphoma in older patients most common Progressive non-painful onset indicates slowly progressive disease

13. How to investigate a lesion of the cavernous sinuses MRI BRAIN Including coronal thin slices (2—3 mm) on T2 spin echo and T1 spin echo after contrast medium injection covering the cavernous sinuses and orbits, and axial thin slices on T1 spin echo with injection and fat saturation Vascular investigation by MRA TOF and dynamic MRA or arterial angioscan if vascular etiology is suspected

14. The second step depends on radiological findings The following questions need to be answered: Where is the initial location of the lesion? -In the cavernous sinus or nearby, as this lesion can be an extension of an extra cavernous lesion Which T2 signal does it show ? -intense T2 hyposignal or hypersignal? • How does it enhance after contrast injection? What are its extensions in the three spatial planes? What are its effects on the main structures involved (optic nerves, optic chiasm, etc.)?

15. MRI brain Approach to R/o etiology of CSS

16. Cavernous sinus meningioma Tubercular Cavernous Sinus Syndrome

17. 2. Orbital echo-Doppler- to explore vascular abnormalities detects indirect signs of fistula consisting of inversion and arterialisation of the flux in a dilated superior ophthalmic vein

18. 3. CT Head To Study The Skull Base. To Differentiate Identical Tissular Lesions, Due To Their Specific Bone Involvement i.e.; Hyperostosis In Case Of Meningioma, Lysis Caused By Metastases And Other Malignant Lesions, Microcalcifications (Chondrosarcoma), It Is Mandatory To Evaluate Facial Bones Or Base Of The Skull Impairment Due To The Lesion, And Presurgical Phase In Order To Better Identify Bone And Lesion Relationships.

19. TREATMENT Tumor-related : Surgical resection, stereotactic radiosurgery (e.g., Gamma Knife), or chemotherapy depending on the type of tumor. Infections : Urgent antibiotics or antifungal, along with drainage for abscesses or sinus involvement. Vascular Issues : Endovascular treatment such as embolization or coiling for aneurysms or fistulas. Symptomatic Treatment : Corticosteroids to reduce inflammation, pain management, and supportive care for eye symptoms (e.g., patching for diplopia).

20. Jpn J Ophthalmol Vol 45: 420–424, 2001

21. Tolosa Hunt Syndrome

22. severe and unilateral periorbital headache associated with painful and restricted eye movements Aka painful ophthalmoplegia/recurrent ophthalmoplegia/ ophthalmoplegia syndrome first described in the year 1954 by Dr. Eduardo  Tolosa and then Hunt et al. in 1961 Smith and Taxdal called it Tolosa Hunt Syndrome for the first time in 1966

23. Etiology Idiopathic non-specific inflammation in the region of the cavernous sinus and/or superior orbital fissure

24. Epidemiology worldwide annual estimated incidence is about one case per million per year. No geographical or racial preponderance. There is no male-female predisposition The average age of onset is 41 years according to the National Organization for Rare Disorder. case reports about bilateral involvement (approximately 5%).

25. Pathophysiology Dr. Eduardo Tolosa described the pathophysiology as "non-specific, chronic inflammation of the septa and wall of the cavernous sinus with the proliferation of fibroblasts an infiltration with lymphocytes and plasma cells.“ Hunt et al. added that "such inflammatory changes, in a tight connective tissue, may exert pressure upon the penetrating nerves.“ Further studies showed :- -the presence of granulomatous material deposits along with epitheloid cells and giant cells. -associated thickening of dura mater within the cavernous sinus -very rarely necrosis

26. rarely associated with intracranial inflammation, but systemic inflammation is not yet reported. does not seem to be any association with any infectious agent. Although there has not been a clear autoimmune etiology associated but could be a presenting complaint of these diseases

27. Clinical features: hallmark of this syndrome is PAIN - -sharp, shooting, stabbing, boring -severe, and intense -located in the periorbital region.  -presenting symptom and can precede ophthalmoplegia by up to 30 days

28. ASSOCIATED FEATURES – OPHTHALMOPLEGIA involvement of all three ocular motor nerves in different combinations Ophthalmic branch of the trigeminal nerve is commonly affected. Pupillary Abnormalities -sympathetic or parasympathetic (oculomotor) involvement Symptoms can last up to eight weeks if not treated and then resolve spontaneously. Have relapsing and remitting course Seldom any residual neurological deficits

29. oculomotor nerve -80% abducens nerve- 70% ophthalmic branch of trigeminal nerve - 30% trochlear nerve -29% Horner syndrome-20% Rarely involve V2,V3, VII CN and if extended in orbital apex can involve optic nerve  does not have any other neurological or systemic involvement  

30. Diagnosis : diagnosed through the clinical presentation, neuroimaging studies, and response to steroids. International Headache Society (IHS) Diagnostic Criteria:

31. Neuroimaging: inv. of choice MRI brain with contrast especially the coronal view show thickening of the cavernous sinus which is -isointense on T1, - iso or hypointense on T2, and -enhances with contrast. Other MRI findings include convexity of the lateral wall of the cavernous sinus, extension into the orbital apex rarely normal .

32. Other Tests: to rule out other causes of painful ophthalmoplegia Tolosa Hunt syndrome remains a clinical diagnosis of exclusion. Blood tests include Complete Blood Count Comprehensive Metabolic Panel HbA1C Erythrocyte Sedimentation Rate C Reactive Protein Angiotensin Converting Enzyme, Anti-nuclear Antibody, Anti-nuclear Cytoplasmic Antibody, Anti-dsDNA Antibody, Anti- sm Antibody, Lyme Panel, Serum Protein Electrophoresis, Fluorescent Treponemal Antibody Test.

33. CSF studies include glucose, protein, cell count and differential, cytology, culture and gram stain, angiotensin converting enzyme, syphilis and Lyme serology. Blood, as well as CSF studies, are expected to be normal in cases of Tolosa Hunt syndrome. If there are abnormalities found, another diagnosis should be considered

34. Diagnosis should be ruled out…… Anisocoria Benign Skull Tumors Brain Mets Cavernous Sinus Syndromes Cerebral Aneurysms Cerebral Venous Thrombosis CNS Whipple Disease Diabetic Neuropathy Epidural Hematoma Lyme Disease Meningioma Migraine Headache Neurosarcoidosis Pediatric Craniopharyngioma Polyarteritis Nodosa Primary CNS Lymphoma Primary Malignant Skull Tumors Systemic Lupus Erythematosus Tuberculous Meningitis Varicella Zoster

35. Trauma Vascular Intracavernous carotid artery aneurysm Posterior cerebral artery aneurysm Carotid-cavernous fistula Carotid-cavernous thrombosis Posterior communicating artery aneurysm Basilar artery aneurysm Internal carotid artery dissection Neoplasm Primary intracranial tumor Pituitary adenoma Meningioma Craniopharyngioma, others Primary cranial tumor Chordoma, others Local metastases Nasopharyngeal tumour Squamous cell carcinoma Distant metastases Lymphoma Multiple myeloma

36. Carcinomatous metastases Inflammation, infection Bacterial Contiguous sinusitis Mucocele (sphenoid sinus) Periostitis Abscess Viral Herpes zoster Fungal Mucormycosis , Actinomycosis Spirochetal Treponema pallidum Mycobacterial Mycobacterium tuberculosis Others Sarcoidosis Wegener's granulomatosis Eosinophilic granuloma Tolosa -Hunt syndrome Orbital pseudotumor Miscellaneous Diabetic ophthalmoplegia Ophthalmoplegic migraine Giant cell arteritis

37. Management: Glucocorticoids have been the mainstay of the treatment (no specific data to give recommendations about dose, duration, or route of administration) Symptom improvement, especially pain relief, is usually seen 24 to 72 hours after starting steroids. Cranial nerve palsies improve gradually and can take anywhere from two to eight weeks for recovery.  In routine use initially 1gm iv MPS for 3 days then 1mg/kg/day prednisone orally and gradually taper upto 8 weeks

38. Azathioprine, methotrexate, mycophenolate mofetil, cyclosporine, and infliximab have been used as second-line therapy Some case reports shows radiotherapy being used as second-line therapy for recurrent flare-ups leading to steroid dependence or as a first-line therapy in the presence of contraindications to steroids only in biopsy proven THS.

39. REFERENCES: The Ishikawa Classification of Cavernous Sinus Lesions by Clinico -anatomical Findings Mutsumi Yoshihara, Noriko Saito, Yoji Kashima and Hiroshi Ishikawa; Jpn J Ophthalmol 2001;45:420–424 Clinical and radiological profle of cavernous sinus syndrome: a study from eastern part of India Vinayak Narayan Rodge , Adreesh Mukherjee, Samar Biswas, Suchitra Majumdar and Goutam Gangopadhyay ; Egypt J Neurol Psychiatry Neurosurg (2023) 59:64 Imaging Spectrum of Cavernous Sinus Lesions with Histopathologic Correlation; Harsha Vardhan Mahalingam, MD Sunithi E. Mani, MD Bimal Patel, MD et al; RadioGraphics 2019; 39:795–819 Cavernous Sinus Syndrome: Clinical Features and Differential Diagnosis with MR Imaging Jeong Hyun Lee , Ho Kyu Lee , Ji Kang Park , Choong Gon Choi , Dae Chul Suh; AJR:181, August 2003 Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013 Jul;33(9):629-808. Amrutkar CV, Burton EV. Tolosa -Hunt Syndrome. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459225/

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