Cranial venous outflow obstruction | |
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Other names | Intracranial venous congestion |
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Main veins and sinuses of the human brain | |
Specialty | Neurology |
Diagnostic method | Medical imaging |
Cranial venous outflow obstruction, also referred to as impaired cranial venous outflow, impaired cerebral venous outflow, cerebral venous impairment is a vascular disorder that involves the impairment of venous drainage from the cerebral veins of the human brain. [1] [2]
The cause of cranial venous outflow obstruction is not fully understood. It is believed to be associated with various factors including anatomical abnormalities, thrombosis, posture, and increased intracranial pressure. [3]
The obstruction can occur in any part of the venous system involved in draining blood from the brain, like vertebral venous system (VVS) or cerebral venous sinus (CVS), but it is most commonly seen in the dural venous sinuses. [4]
Impaired cranial venous outflow can lead to increased venous pressure, decreased cerebrospinal fluid (CSF) absorption, brain cortex hypoperfusion, brain edema, blood–brain barrier (BBB) disruption, inflammatory reactions, hemorrhagic complications, and increased intracranial pressure. [1] Which can result in a variety of neurological symptoms, such as pseudotumor cerebri, chronic fatigue syndrome, different types of headaches, visual disturbances, pulsatile tinnitus and various neurodegenerative diseases. [5] [6] [7] [8] [9] [1] [10]
The pathophysiology of cranial venous outflow obstruction involves the disruption of normal venous drainage from the brain. Cerebral veins play a crucial role in draining brain interstitial fluid (ISF), and their significance has been linked in various neurological conditions. [1] It can be caused by extrinsic or intrinsic anomalies. [7]
Extrinsic anomalies are structural changes near the Internal Jugular Vein (IJV) that can cause venous outflow obstruction. These changes can be due to bone pressure, artery pressure, enlarged lymph nodes, or an enlarged thyroid. These factors can squeeze the vein wall and block the blood flow. This blockage can range from a local narrowing (stenosis) to a complete blockage (occlusion). [7]
Different parts of the IJV can be affected by different factors. The upper part of the IJV is often squeezed by the side parts of the neck bones at the vertebral C1 segment and by the styloid processes. The middle and lower parts of the IJV are more often squeezed by the nearby carotid artery, lymph nodes, and unusual muscles. Among all these factors, bone pressure is the most common, causing about 40% of extrinsic anomalies. A recent study showed that 41.9% of IJV narrowing in a Chinese group was due to external pressure. [7]
Intrinsic anomalies are changes within the IJV itself. These can include blood clots (thrombi), abnormalities in the vessel wall, and malformed venous valves. IJV thrombosis is rare and has only been mentioned in a few case reports. Defects inside the vein (flaps, webs, septa, membranes, and malformed valves) can hinder normal blood flow from the brain. This can result in changes in blood flow, including backflow (reflux), reduced flow, or no flow entirely. Doppler and intravascular ultrasound are effective tools for seeing malformed valves. There are many types of malformed valves, including fused, elongated, ectopic, accessory leaflet-containing, inverted, and double valves. [7]
Management of cranial venous outflow obstruction involves treating the underlying cause, if identifiable, and managing the symptoms. This can include medication to reduce intracranial pressure, anticoagulation therapy to prevent thrombosis, and in some cases, surgical intervention to restore normal venous drainage. [6] [11]
The epidemiology of cranial venous outflow obstruction is not well-studied, and the condition is likely underdiagnosed due to the nonspecific nature of its symptoms. However, it is known to affect individuals of all ages, with a higher prevalence in females and individuals with anatomical venous disorders (varicose veins), certain risk factors such as obesity and thrombophilic disorders and sedentary lifestyle. [12] [2]
Styloidogenic jugular venous compression syndrome (SJVCS) shares symptoms with idiopathic intracranial hypertension (IIH). Patients with SJVCS experience compression of venous outflow on both sides due to bone structures. It occurs due to compression of the internal jugular vein (IJV) between the C1 vertebrae transverse process and the temporal styloid process. Treatments like venous stenting and styloidectomy have shown positive results. [13] [14] [15] [16]
Diagnostics of this pathology might include advanced MR venography, direct microcatheter venography with manometry, 3D catheter angiographic studies, three-dimensional rotational venography (3D-RV), cone-beam rotational angiography (3D-RA), also CT venography or MRI scans with contrast with would potentially show obstruction. [5] [17]
Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri and benign intracranial hypertension, is a condition characterized by increased intracranial pressure without a detectable cause. The main symptoms are headache, vision problems, ringing in the ears, and shoulder pain. Complications may include vision loss.
Veins are blood vessels in the circulatory system of humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are those of the pulmonary and fetal circulations which carry oxygenated blood to the heart. In the systemic circulation, arteries carry oxygenated blood away from the heart, and veins return deoxygenated blood to the heart, in the deep veins.
Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare.
In medicine, a shunt is a hole or a small passage that moves, or allows movement of, fluid from one part of the body to another. The term may describe either congenital or acquired shunts; acquired shunts may be either biological or mechanical.
Cerebral edema is excess accumulation of fluid (edema) in the intracellular or extracellular spaces of the brain. This typically causes impaired nerve function, increased pressure within the skull, and can eventually lead to direct compression of brain tissue and blood vessels. Symptoms vary based on the location and extent of edema and generally include headaches, nausea, vomiting, seizures, drowsiness, visual disturbances, dizziness, and in severe cases, death.
Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury (mmHg) and at rest, is normally 7–15 mmHg for a supine adult. This equals to 9–20 cmH2O, which is a common scale used in lumbar punctures. - The body has various mechanisms by which it keeps the ICP stable, with CSF pressures varying by about 1 mmHg in normal adults through shifts in production and absorption of CSF.
The great cerebral vein is one of the large blood vessels in the skull draining the cerebrum of the brain. It is also known as the vein of Galen, named for its discoverer, the Greek physician Galen.
Intracranial hemorrhage (ICH), also known as intracranial bleed, is bleeding within the skull. Subtypes are intracerebral bleeds, subarachnoid bleeds, epidural bleeds, and subdural bleeds.
A thunderclap headache is a headache that is severe and has a sudden onset. It is defined as a severe headache that takes seconds to minutes to reach maximum intensity. Although approximately 75% are attributed to "primary" headaches—headache disorder, non-specific headache, idiopathic thunderclap headache, or uncertain headache disorder—the remainder are secondary to other causes, which can include some extremely dangerous acute conditions, as well as infections and other conditions. Usually, further investigations are performed to identify the underlying cause.
The emissary veins connect the extracranial venous system with the intracranial venous sinuses. They connect the veins outside the cranium to the venous sinuses inside the cranium. They drain from the scalp, through the skull, into the larger meningeal veins and dural venous sinuses. They may also connect to diploic veins within the skull.
Intraparenchymal hemorrhage (IPH) is one form of intracerebral bleeding in which there is bleeding within brain parenchyma. The other form is intraventricular hemorrhage (IVH).
The dural venous sinuses are venous sinuses (channels) found between the endosteal and meningeal layers of dura mater in the brain. They receive blood from the cerebral veins, and cerebrospinal fluid (CSF) from the subarachnoid space via arachnoid granulations. They mainly empty into the internal jugular vein. Cranial venous sinuses communicate with veins outside the skull through emissary veins. These communications help to keep the pressure of blood in the sinuses constant.
May–Thurner syndrome (MTS), also known as the iliac vein compression syndrome, is a condition in which compression of the common venous outflow tract of the left lower extremity may cause discomfort, swelling, pain or iliofemoral deep vein thrombosis.
A dural arteriovenous fistula (DAVF) or malformation is an abnormal direct connection (fistula) between a meningeal artery and a meningeal vein or dural venous sinus.
Cavernous sinus thrombosis (CST) is the formation of a blood clot within the cavernous sinus, a cavity at the base of the brain which drains deoxygenated blood from the brain back to the heart. This is a rare disorder and can be of two types–septic cavernous thrombosis and aseptic cavernous thrombosis. The most common form is septic cavernous sinus thrombosis. The cause is usually from a spreading infection in the nose, sinuses, ears, or teeth. Staphylococcus aureus and Streptococcus are often the associated bacteria.
Cerebral venous sinus thrombosis (CVST), cerebral venous and sinus thrombosis or cerebral venous thrombosis (CVT), is the presence of a blood clot in the dural venous sinuses, the cerebral veins, or both. Symptoms may include severe headache, visual symptoms, any of the symptoms of stroke such as weakness of the face and limbs on one side of the body, and seizures, which occur in around 40% of patients.
Chronic venous insufficiency (CVI) is a medical condition in which blood pools in the veins, straining the walls of the vein. The most common cause of CVI is superficial venous reflux which is a treatable condition. As functional venous valves are required to provide for efficient blood return from the lower extremities, this condition typically affects the legs. If the impaired vein function causes significant symptoms, such as swelling and ulcer formation, it is referred to as chronic venous disease. It is sometimes called chronic peripheral venous insufficiency and should not be confused with post-thrombotic syndrome in which the deep veins have been damaged by previous deep vein thrombosis.
Chronic cerebrospinal venous insufficiency is a term invented by Italian researcher Paolo Zamboni in 2008 to describe compromised flow of blood in the veins draining the central nervous system. Zamboni hypothesized that it might play a role in the cause or development of multiple sclerosis (MS). Zamboni also devised a surgical procedure which the media nicknamed a liberation procedure or liberation therapy, involving venoplasty or stenting of certain veins. Zamboni's ideas about CCSVI are very controversial, with significantly more detractors than supporters, and any treatments based on his ideas are considered experimental.
Vein of Galen aneurysmal malformations(VGAMs) and Vein of Galen aneurysmal dilations (VGADs) are the most frequent arteriovenous malformations in infants and fetuses. A VGAM consists of a tangled mass of dilated vessels supplied by an enlarged artery. The malformation increases greatly in size with age, although the mechanism of the increase is unknown. Dilation of the great cerebral vein of Galen is a secondary result of the force of arterial blood either directly from an artery via an arteriovenous fistula or by way of a tributary vein that receives the blood directly from an artery. There is usually a venous anomaly downstream from the draining vein that, together with the high blood flow into the great cerebral vein of Galen causes its dilation. The right sided cardiac chambers and pulmonary arteries also develop mild to severe dilation.
Spaceflight-associated neuro-ocular syndrome (SANS), previously called spaceflight-induced visual impairment, is hypothesized to be a result of increased intracranial pressure (ICP), although, experiments directly measuring ICP in parabolic flight have shown ICP to be in normal physiological ranges during acute weightless exposure. The study of visual changes and ICP in astronauts on long-duration flights is a relatively recent topic of interest to space medicine professionals. Although reported signs and symptoms have not appeared to be severe enough to cause blindness in the near term, long term consequences of chronically elevated intracranial pressure are unknown.