Presentation
A 60-year-old woman presented with a cerebellar solid tumor manifesting as headache of 4 months, progressive vomiting, and ataxia of half a month. [ncbi.nlm.nih.gov]
Results: A 59-year-old woman presented with acute left-sided ophthalmoplegia. [scholars.northwestern.edu]
Clinical presentation is usually an acute onset of ocular symptoms and a pulsatile bruit is invariably present. [ajnr.org]
“Indirect” or “dural” carotid-cavernous fistulas typically present in elderly women with hypertension in a more subtle fashion. [medlink.com]
Entire Body System
- Disability
The final outcomes were nine good recovery, one moderately disabled, and one severely disabled by the Glasgow Outcome Scale. The causes of morbidity were early ischemia and subarachnoid hemorrhage from a newly formed aneurysm. [ncbi.nlm.nih.gov]
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In such circumstances, mainly concomitantly with disabling tinnitus, endovascular... [connection.ebscohost.com]
Annually, nearly 30,000 patients suffer a spontaneous subarachnoid hemorrhage, and most are dead or disabled as a result. With few exceptions, patients with a ruptured intracranial aneurysm warrant treatment. [oregon.providence.org]
- Severe Pain
This depends on several factors. [med.nyu.edu]
Case Description: A 53-year-old woman suffered severe left painful ophthalmoplegia. A magnetic resonance image (MRI) revealed thickness of the left cavernous sinus (CS). [surgicalneurologyint.com]
- Fatigue
Symptoms of cavernous sinus thrombosis may include: Severe headache often accompanied by tearing Swelling, redness, or irritation around one or both eyes Drooping eyelids Inability to move the eye High fever Pain or numbness around the face or eyes Fatigue [webmd.com]
Cardiovascular
- Vascular Disease
Dural cavernous sinus fistulas (DCSFs) are benign vascular diseases consisting in an arteriovenous shunt at the cavernous sinus that if misdiagnosed can lead to potentially serious ophthalmologic complications. [books.google.com]
Direct CCFs may result from: Trauma Rupture of a cavernous carotid aneurysm A tear in the wall of a weak cavernous internal carotid artery caused by congenital collagen vascular disease. [upmc.com]
Etiology Direct CCF may result from trauma, rupture of a cavernous carotid aneurysms, or from a tear in the wall of a congenitally weak cavernous ICA secondary to collagen vascular disease. Indirect CCF are usually spontaneous and idiopathic. [neurosurgery.pitt.edu]
Spontaneous fistulae usually result from a ruptured carotid aneurysm, in collagen vascular disease, atherosclerotic disease, hypertension, or at childbirth 3. 2. Flow: high or low flow 3. [sonoworld.com]
Eyes
- Diplopia
Cavernous sinus tumors Acute or slowly progressive ophthalmoplegia is the dominant presentation, with diplopia being the most common symptom. At times, painful diplopia is present. Usually the patient has a preceding history of cancer. [emedicine.medscape.com]
Seven patients complained of horizontal diplopia, whereas two had vertical diplopia and one had oblique diplopia. The nature of the diplopia in the remaining nine patients was not specified. [jnnp.bmj.com]
Also, we found a relation between the presence of fistulas and improvement of diplopia (βb=7.02; 95% CI, 1.71 to 12.32) and between increased age and improvement of diplopia (βb=3.68; 95% CI, 0.71 to 6.7). [stroke.ahajournals.org]
Patients experiencing diplopia can be treated with prism or occlusion therapy and IOP-lowering medications can be started if IOP is substantially elevated. [eyewiki.org]
Kim First published December 26, 2006, DOI: https://doi.org/10.1212/01.wnl.0000232727.26213.77 A 54-year-old man developed sudden left-sided headache and diplopia. [neurology.org]
- Chemosis
The left eye showed no chemosis, pulsation, or bruit. Ophthalmologic examination of the left eye revealed traumatic optic neuropathy. [nejm.org]
[…] aneurysm of the internal carotid artery bifurcation with a fistula to the cavernous sinus,which appeared following closed head trauma.A 39-year-old man suffered from a blunt head trauma in an automobile accident.Two weeks after the trauma,progressive chemosis [cmj.yiigle.com]
The patient demonstrated substantial clinical improvement with resolution of her chemosis and protruding eye. Citation: Yao CA, Tsou HK, Sheehan J and Pan HC. [austinpublishinggroup.com]
This results in acute onset of massive exophthalmos with orbital, ocular, and conjunctival chemosis, binocular diplopia, and visual loss. There are two types of C-C fistulas. Direct fistulas present with prominent acute symptoms. [emedicine.medscape.com]
Anterior drainage via the SOV can present with exophthalmos, chemosis, proptosis, and decreased visual acuity. Cranial nerve palsies, epitaxis, headache, bruit, and tinnitus may be present with either anterior or posterior drainage. [neurosurgery.pitt.edu]
- Painful Ophthalmoplegia
[…] field defects Primary intracranial tumors: Isolated or combined ophthalmoplegia and/or primary aberrant regeneration of the third cranial nerve Cavernous sinus aneurysms Isolated or combined ophthalmoplegia Painful ophthalmoplegia Decreased pain sensation [emedicine.medscape.com]
Case Description: A 53-year-old woman suffered severe left painful ophthalmoplegia. A magnetic resonance image (MRI) revealed thickness of the left cavernous sinus (CS). [surgicalneurologyint.com]
[…] is associated with pain. [1] Differential diagnosis Myasthenia Gravis Thyroid associated orbitopathy Internuclear ophthalmoplegia Chronic progressive external ophthalmoplegia Orbital pseudotumor Giant cell arteritis Acquired oculomotor nerve palsy evaluation [eyewiki.aao.org]
(A) Preoperative anteroposterior angiogram showing agiant aneurysm of the right intracavernous internal carotid artery (ICA) in a 76-year-old woman (Case 17) who presentedwith progressive ophthalmoplegia and orbital pain. [barrowneuro.org]
- Visual Impairment
Hence, if the patient has visual impairment or cranial nerve dysfunction, treatment should be done as soon as possible to decompress the compromised nerve. This way irreversible damage can be avoided. [jnnp.bmj.com]
She presented with intractable headache and acute worsening of double vision and visual acuity. [surgicalneurologyint.com]
- Eye Pain
Overview Snapshot A 24-year-old female presents with a progressivesly worsening headache and eye swelling. The pain is sharp and one-sided and affects the right forehead and eye. She has tried over-the-counter pain medication with no relief. [medbullets.com]
Pain is the symptom that patients often find the most difficult to tolerate. Patients usually present with sudden or insidious onset of redness in one eye, associated with progressive proptosis or bulging. [en.wikipedia.org]
Ears
- Tinnitus
Sigmoid sinus diverticulum and pulsatile tinnitus: analysis of CT scans from 15 cases. [connection.ebscohost.com]
Swelling of the membranes that cover the outside of the eyes and line the eyelids Forward displacement of the eyeballs Deteriorating vision Cranial nerve palsies Nosebleed Headache Bruit (a blowing sound in the vessel that can be heard with a stethoscope) Tinnitus [upmc.com]
Cranial nerve palsies, epitaxis, headache, bruit, and tinnitus may be present with either anterior or posterior drainage. Rare cases with subarachnoid hemorrhage. [neurosurgery.pitt.edu]
Those fistulas that involve the transverse and sigmoid sinuses, 2 of the most common locations for DAVFs, frequently cause pulsatile tinnitus. [ingentaconnect.com]
[…] diplopia, dysarthria, dysphagia, positional vertigo); or To evaluate members with signs/symptoms highly suggestive of leaking/ruptured ICA or AVM (i.e., blood in the cerebral spinal fluid, stiff neck, sudden explosive headache); or To evaluate pulsatile tinnitus [aetna.com]
- Pulsatile Tinnitus
Sigmoid sinus diverticulum and pulsatile tinnitus: analysis of CT scans from 15 cases. [connection.ebscohost.com]
tinnitus or lower cranial nerve dysfunction due to dissection-related mass effect. [neuroangio.org]
H93.A1 - H93.A9 Pulsatile tinnitus I60.00 - I60.9 Nontraumatic subarachnoid hemorrhage I67.0 - I67.9 Other cerebrovascular diseases I71.02 - I71.03 Dissection of abdominal or thoracoabdominal aorta I71.3 - I71.4 Abdominal aortic aneurysm, ruptured or [aetna.com]
Those fistulas that involve the transverse and sigmoid sinuses, 2 of the most common locations for DAVFs, frequently cause pulsatile tinnitus. [ingentaconnect.com]
Face, Head & Neck
- Epistaxis
Epistaxis related to internal carotid artery aneurysm is a rare cause of epistaxis. In this case we report a case of recurrent epistaxis for more than 7 months in a previously healthy Chinese man. [omicsonline.org]
No further epistaxis occurred, but the neurologic deficit in the left eye remained unchanged. [nejm.org]
[…] aneurysm as a cause of epistaxis. [jpma.org.pk]
Som, Cavernous carotid aneurysm presenting with epistaxis, Head & Neck, 2004, 26, 8, 741 Wiley Online Library 14 Ertugrul Çakir, Gökalp Karaarslan, Kayhan Kuzeyli, Haydar Usul, Özgür Ç. [onlinelibrary.wiley.com]
The natural evolution of carotid cavernous fistulae includes increasing proptosis, cranial nerve palsies, loss of vision (due to central retinal vein occlusion or glaucoma), epistaxis and intracranial or subarachnoid hemorrhage. [sonoworld.com]
Neurologic
- Papilledema
[…] their eyesight if they receive adequate medical care to treat the underlying cause of the papilledema. [connection.ebscohost.com]
Other symptoms include eyelid erythema, autonomic dysfunction, sensory changes in the ophthalmic and maxillary trigeminal nerve distributions, pupillary abnormalities, and papilledema (1, 3, 4, 6). [emdocs.net]
Progressive visual decline, papilledema, and refractory elevation of the intraocular pressure are all indications for emergent intervention.[ 1 ] The goal of CCF treatment is to completely occlude the fistula while simultaneously preserving normal blood [surgicalneurologyint.com]
[…] at risk (e.g., hyper-coagulable disorders, meningitis, oral contraceptive use, otitis media, sinusitis, underlying malignant process) or have signs or symptoms (e.g., drowsiness and confusion accompanying a headache, focal motor or sensory deficits, papilledema [aetna.com]
Workup
Differential diagnoses include orbital cellulitis, orbital apex syndrome, ICA aneurysm, malignancy of the CS, trauma, carotid-cavernous fistula (CCF), Tolosa-Hunt Syndrome, and ischemic stroke (6,14) Workup If CST is suspected, imaging should be ordered [emdocs.net]
Nevertheless, basic workup must be done. [eyewiki.aao.org]
The patient was admitted for further workup. A brain MRI demonstrated metastatic involvement of the cavernous sinus ( Figures 3, 4 ). [touchoncology.com]
Treatment
Device (PED) has been described for the treatment of brain aneurysms. [scholars.northwestern.edu]
Endovascular treatment of a giant internal carotid artery bifurcation aneurysm with drainage into cavernous sinus Chin Med J, 2012,125(03) : 539-542. [cmj.yiigle.com]
Treatment Twenty one of the 31 patients (68%) underwent treatment of their CCAs (table 3). [jnnp.bmj.com]
Diplopia, if present before treatment, improved in nearly all patients after treatment by coiling and in 81% of patients after treatment by means of balloon occlusion. [stroke.ahajournals.org]
Endovascular treatment with coils is effective in occluding the fistula. [ncbi.nlm.nih.gov]
Prognosis
prognosis for dural CCF is very good. [eyewiki.org]
Brain aneurysm: A cavernous aneurysm is a common finding that if truly cavernous, indicates that it is located outside of the brain and generally carries a benign prognosis. [healthtap.com]
Long-term prognosis in patients with vasculopathic sixth nerve palsy. Am J Ophthalmol 2002; 134 : 81–84. 13. Patel SV, Homes JM, Hodge DO, Burke JP. Diabetes and hypertension in isolated sixth nerve palsy. Ophthalmol 2005; 112 : 760–763. [nature.com]
The long term prognosis for treated cases is relatively good, with most complications occurring immediately after the procedure. [jnnp.bmj.com]
Etiology
CSS has a multitude of etiologies. [touchoncology.com]
After sections on anatomy and classification, etiology and pathogenesis of DCSFs, the symptomatology of the disease is described in detail. The role of modern imaging techniques in the diagnosis of DCSFs is then addressed. [books.google.com]
The most common known etiology is a posterior communicating artery aneurysm. This is a medical emergency. [eyewiki.aao.org]
The differential diagnosis of a cavernous sinus syndrome includes a wide array of vascular, infectious, inflammatory, and neoplastic etiologies. Fortunately, among vascular lesions, cavernous aneurysms are usually indolent and rarely rupture. [synchropublisher.com]
Etiology Direct CCF may result from trauma, rupture of a cavernous carotid aneurysms, or from a tear in the wall of a congenitally weak cavernous ICA secondary to collagen vascular disease. Indirect CCF are usually spontaneous and idiopathic. [neurosurgery.pitt.edu]
Epidemiology
Cavernous carotid artery aneurysms: Epidemiology, natural history, diagnostic and treatment. An experience of a single institution. Clin Neurol Neurosurg 2014;125:32-5. [ PUBMED ] 4. [neurologyindia.com]
Pathophysiology
We briefly reviewed the literature of similar cases and tried to analyze the possible pathophysiological relationship between these findings. [moh-it.pure.elsevier.com]
Dural carotid cavernous fistula Pathophysiology Direct carotid cavernous fistula – Arise most commonly secondary to a traumatic tear of internal carotid artery within the cavernous sinus. [eyewiki.org]
In some cases, the precise site of the lesion is clear, whereas in others, the location of the lesion is speculative. [1] Pathophysiology To understand the pathophysiology of the oculomotor nerve palsy it is essential to know its track. [eyewiki.aao.org]
Pathophysiology The two cavernous sinuses are located on both sides of the sella turcica. [emdocs.net]
Discussion Several classifications of CCF exist depending on anatomy, aetiology, and pathophysiology. One categorisation divides between traumatic and spontaneous fistulas. [jnnp.bmj.com]
Prevention
Page 344 - Primary prevention of ischemic stroke: A statement for healthcare professionals from the Stroke Council of the American Heart Association. [books.google.com]
To contain the infection, the body's immune system creates a clot to prevent bacteria or other pathogens from spreading. The clot increases pressure inside the brain. This pressure can damage the brain and may ultimately cause death. [webmd.com]
Although CCFs from ruptured cavernous sinus aneurysms may close spontaneously, as in 2 of our patients with low-flow CCFs, angiography and endovascular closure of the fistula should follow promptly to prevent possible hemorrhage from engorged cortical [ajnr.org]
Given the acutely worsening neuro-ophthalmologic symptoms as well as the robust direct CCF with the resultant engorged left SOV, urgent treatment was indicated to prevent devastating permanent neuro-ophthalmologic deficits. [surgicalneurologyint.com]