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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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The most common signs/symptoms of DAVFs are:
1. Pulsatile tinnitus
2. Occipital bruit
3. Headache
4. Visual impairment
5. Papilledema
Pulsatile tinnitus is the most common symptom in patients, and it is associated with transverse-sigmoid sinus DAVFs. Carotid-cavernous DAVFs, on the other hand, are more closely associated with pulsatile exophthalmos. DAVFs may also be asymptomatic (e.g. cavernous sinus DAVFs).
CCF symptoms include bruit (a humming sound within the skull due to high blood flow through the arteriovenous fistula), progressive visual loss, and pulsatile proptosis or progressive bulging of the eye due to dilatation of the veins draining the eye. 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.
They may have a history of similar episodes in the past.
Carotid cavernous fistulae may form following closed or penetrating head trauma, surgical damage, rupture of an intracavernous aneurysm, or in association with connective tissue disorders, vascular diseases and dural fistulas.
Most commonly found adjacent to dural sinuses in the following locations:
1. Transverse (lateral) sinus, left-sided slightly more common than right
2. Intratentorial
3. From the posterior cavernous sinus, usually draining to the transverse or sigmoid sinuses
4. Vertebral artery (posterior meningeal branch)
Sinus pericranii typically present as soft palpable masses along midline skull, which may fluctuate in size depending on body positioning. Classically, these lesions are not associated with color change of the overlying skin, such as with other vascular lesions such as hemangioma.
Sinus pericranii (SP) is a rare disorder characterized by a congenital (or occasionally, acquired) epicranial venous malformation of the scalp. Sinus pericranii is an abnormal communication between the intracranial and extracranial venous drainage pathways. Treatment of this condition has mainly been recommended for aesthetic reasons and prevention of hemorrhage.
Vein of Galen aneurysmal malformations (VGAM) and Vein of Galen aneurysmal dilations (VGAD) are the most frequent arteriovenous malformations in infants and fetuses. VGAM consist 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.
Testing for a malformed vein of Galen is indicated when a patient has heart failure which has no obvious cause. Diagnosis is generally achieved by signs such as cranial bruits and symptoms such as expanded facial veins. The vein of Galen can be visualized using ultrasound or Doppler. A malformed Great Cerebral Vein will be noticeably enlarged. Ultrasound is a particularly useful tool for vein of Galen malformations because so many cases occur in infancy and ultrasound can make diagnoses prenatally. Many cases are diagnosed only during autopsy as congestive heart failure occurs very early.
A Cimino fistula, also Cimino-Brescia fistula, surgically created arteriovenous fistula and (less precisely) arteriovenous fistula (often abbreviated AV fistula or AVF), is a type of vascular access for hemodialysis. It is typically a surgically created connection between an artery and a vein in the arm, although there have been acquired arteriovenous fistulas which do not in fact demonstrate connection to an artery.
Surgically created AV fistulas work effectively because they:
- Have high volume flow rates (as blood takes the path of least resistance; it prefers the (low resistance) AV fistula over traversing (high resistance) capillary beds).
- Use native blood vessels, which, when compared to synthetic grafts, are less likely to develop stenoses and fail.
Vascular malformation is a blood vessel abnormality. There are many types, but the most common is arteriovenous malformation.
It may cause aesthetic problems as it has a growth cycle and can continue to grow throughout life. This is also known as Vascular giantism or lymphangiomas.
Just like berry aneurysm, an intracerebral arteriovenous fistula can rupture causing subarachnoid hemorrhage.
A developmental venous anomaly (DVA, formerly known as venous angioma) is a congenital variant of the cerebral venous drainage. On imaging it is seen as a number of small deep parenchymal veins converging toward a larger collecting vein.
Most people who develop SCSFLS feel the sudden onset of a severe and acute headache. It is a headache usually made worse by standing, typically becoming prominent throughout the day, with the pain becoming less severe when lying down. Orthostatic headaches can become chronic and disabling to the point of incapacitation. Some patients with SCSFLS will develop headaches that begin in the afternoon. This is known as "second-half-of-the-day headache". This may be an initial presentation of a spontaneous CSF leak or appear after treatment such as an epidural patch, and likely indicates a slow CSF leak.
Apart from headache, about 50% of patients experience neck pain or stiffness, nausea, and vomiting. Other symptoms include dizziness and vertigo, facial numbness or weakness, unusually blurry or double vision, neuralgia, fatigue, or a metallic taste in the mouth. Leaking CSF can sometimes be felt or observed as a discharge from the nose or ear.
Lack of CSF pressure and volume can allow the brain to sag and descend through the foramen magnum (large opening) of the occipital bone, at the base of the skull. The lower portion of the brain is believed to stretch or impact one or more cranial nerve complexes, thereby causing a variety of sensory symptoms. Nerves that can be affected and their related symptoms are detailed in the table at right.
Tarlov cysts are likely highly underdiagnosed as it was Isadore Tarlov's later research that led him to the understanding of their symptomology. Symptoms are based on the locations of the cysts along the spine, and follow general pathology of spinal injury:
- Pain
- Paresthesia
- Spasticity, Hypertonia
- Muscular Dysfunction or Weakness
- Radiculopathy
Although they are most frequently reported along sacral regions, they are rarely seen in other locations along the spine. Women are more likely to exhibit symptoms They can also appear in clusters or bilaterally along the spine, thus symptoms can be unilateral, bilateral, or with symptoms more dominant on one side. The cases of reported symptomatic Tarlov cysts ranges from 15% to 30% of the overall reported Tarlov cyst case, depending on the source of literature. Nevertheless, these cysts are important clinical entities because of their tendency to increase in size over time, potentially causing complications and eroding the surrounding bone tissue. Patients with symptomatic Tarlov cysts near the sacrum (and not other locations of the spine) can be divided into 4 categories, according to their experienced symptoms:
- Group 1 - Pain on tailbones that radiates to the legs with potential weakness;
- Group 2 - Pain on bones, legs, groin area, sexual dysfunctions, and dysfunctional bladder;
- Group 3 - Pain that radiate from the cyst site across hips to the lower abdomen;
- Group 4 - No pain, just sexual dysfunction and dysfunctional bladder.
DVA can be characterized by the Caput medusae sign of veins, which drains into a larger vein. The drains will either drain into a Dural venous sinuses or into a deep ependymal vein. It appears to look like a Palm tree.
The birth defect is diagnosed by the presence of a combination of these symptoms (often on approximately ¼ of the body, though some cases may present more or less affected tissue):
- One or more distinctive port-wine stains with sharp borders
- Varicose veins
- Hypertrophy of bony and soft tissues, that may lead to local gigantism or shrinking, most typically in the lower body/legs.
- An improperly developed lymph system
In some cases, port-wine stains (capillary port wine type) may be absent. Such cases are very rare and may be classified as "atypical Klippel–Trenaunay syndrome".
KTS can either affect blood vessels, lymph vessels, or both. The condition most commonly presents with a mixture of the two. Those with venous involvement experience increased pain and complications, such as venous ulceration in the lower extremities.
Those with large AVMs are at risk of formation of blood clots in the vascular lesion, which may migrate to the lungs (pulmonary embolism). If there is large-volume blood flow through the lesion, "high-output heart failure" may develop due to the inability of the heart to generate sufficient cardiac output.
Below are a list of commonly reported symptoms associated with sacral Tarlov cysts:
Back pain, perineal pain, secondary Sciatica, secondary piriformis muscle dysfunction with tertiary sciatica, Cauda equina syndrome, neurogenic claudication (pain caused by walking), neurogenic bladder, dysuria, urinary incontinence, coccygodynia, sacral radiculopathy, radicular pain, headaches, retrograde ejaculation, paresthesia, hypesthesia, secondary pelvic floor dysfunction, vaginismus, motor disorders in lower limbs and the genital, perineal, or lumbosacral areas, sacral or buttocks pain, vaginal or penile paraesthesia, Persistent Genital Arousal Disorder (PGAD) characterized by unwanted, unrelenting genital sensory awareness, itch or pain that can persist for days, months, even years), sensory changes over buttocks, perineal area, and lower extremity; difficulty walking; severe lower abdominal pain, bowel dysfunction, intestinal motility disorders like constipation or bowel incontinence.
All fast-flow malformations are malformations involving arteries. They constitute about 14% of all vascular malformations.
- Arterial malformation
- Arteriovenous fistula (AVF) : a lesion with a direct communication via fistulae between an artery and a vein.
- Arteriovenous malformation : a lesion with a direct connection between an artery and a vein, without an intervening capillary bed, but with an interposed nidus of dysplastic vascular channels in between.
Symptoms of subdural hemorrhage have a slower onset than those of epidural hemorrhages because the lower pressure veins bleed more slowly than arteries. Therefore, signs and symptoms may show up in minutes, if not immediately but can be delayed as much as 2 weeks. If the bleeds are large enough to put pressure on the brain, signs of increased ICP (intracranial pressure) or damage to part of the brain will be present.
Other signs and symptoms of subdural hematoma can include any combination of the following:
- A history of recent head injury
- Loss of consciousness or fluctuating levels of consciousness
- Irritability
- Seizures
- Pain
- Numbness
- Headache (either constant or fluctuating)
- Dizziness
- Disorientation
- Amnesia
- Weakness or lethargy
- Nausea or vomiting
- Loss of appetite
- Personality changes
- Inability to speak or slurred speech
- Ataxia, or difficulty walking
- Loss of muscle control
- Altered breathing patterns
- Hearing loss or hearing ringing (tinnitus)
- Blurred Vision
- Deviated gaze, or abnormal movement of the eyes.
An arteriovenous fistula is an abnormal connection or passageway between an artery and a vein. It may be congenital, surgically created for hemodialysis treatments, or acquired due to pathologic process, such as trauma or erosion of an arterial aneurysm.
Vascular malformation is a collective term for different disorders of the vasculature (errors in vascular development). It can be a disorder of the capillaries, arteries, veins and lymphatic vessels or a disorder of a combination of these (lesions are named based on the primary vessel that is malformed). A vascular malformation consists of a cluster of deformed vessels, due to an error in vascular development (dysmorphogenesis). However, endothelial turnover is stable in these defects. Congenital vascular malformations are always already present at birth, although they are not always visible. In contrast to vascular tumors, vascular malformations do not have a growth phase, nor an involution phase. Vascular malformations tend to grow proportionately with the child. Vascular malformations never regress, but persist throughout life.
Vascular malformations can be divided into slow-flow, fast-flow and complex-combined types.
Subdural hematomas are divided into acute, subacute, and chronic, depending on the speed of their onset. Acute subdural hematomas that are due to trauma are the most lethal of all head injuries and have a high mortality rate if they are not rapidly treated with surgical decompression.
Acute bleeds often develop after high speed acceleration or deceleration injuries and are increasingly severe with larger hematomas. They are most severe if associated with cerebral contusions. Though much faster than chronic subdural bleeds, acute subdural bleeding is usually venous and therefore slower than the typically arterial bleeding of an epidural hemorrhage. Acute subdural bleeds have a high mortality rate, higher even than epidural hematomas and diffuse brain injuries, because the force (acceleration/deceleration) required to cause them causes other severe injuries as well. The mortality rate associated with acute subdural hematoma is around 60 to 80%.
Chronic subdural bleeds develop over a period of days to weeks, often after minor head trauma, though such a cause is not identifiable in 50% of patients. They may not be discovered until they present clinically months or years after a head injury. The bleeding from a chronic bleed is slow, probably from repeated minor bleeds, and usually stops by itself. Since these bleeds progress slowly, they present the chance of being stopped before they cause significant damage. Small chronic subdural hematomas, those less than a centimeter wide, have much better outcomes than acute subdural bleeds: in one study, only 22% of patients with chronic subdural bleeds had outcomes worse than "good" or "complete recovery". Chronic subdural hematomas are common in the elderly.
Most subdural hygromas are small and clinically insignificant. Larger hygromas may cause secondary localized mass effects on the adjacent brain parenchyma, enough to cause a neurologic deficit or other symptoms. Acute subdural hygromas can be a potential neurosurgical emergency, requiring decompression. Acute hygromas are typically a result of head trauma—they are a relatively common posttraumatic lesion—but can also develop following neurosurgical procedures, and have also been associated with a variety of conditions, including dehydration in the elderly, lymphoma and connective tissue diseases.
SCSFLS is classified into two main types, cranial leaks and spinal leaks. The vast majority of leaks are spinal. Cranial leaks occur in the head. In some of these cases, CSF can be seen dripping out of the nose, or ear. Spinal leaks occur when one or more holes form in the dura along the spinal cord. Both cranial and spinal spontaneous CSF leaks cause neurological symptoms as well as spontaneous intracranial hypotension, diminished volume and pressure of the cranium. While referred to as "intracranial hypotension", the intracranial pressure may be normal, with the underlying issue instead being low-volume CSF. For this reason SCSFLS is referred to as "CSF hypovolemia" as opposed to "CSF hypotension".