Retinal arteriovenous malformations (AVMs) are rare congenital vascular anomalies.
Initially named as “racemous haemangiomas” or “cirsoidal aneurisms”, these anomalies are defined by aberrant arterio-venous shunts [112]. Possible associations with cerebral arteriovenous malformation have been described, i.e. Bonnet-Dechaume-Blanc syndrome (1937) or Wyburn-Mason syndrome (1943) [113]. Arteriovenous malformations (AVM) are typically unilateral, non hereditary and asymptomatic.
These vascular malformations usually involve a single or multiple quadrants of the fundus, being, above all, found at the level of the papillomacular fascicle and the superotemporal quadrant [112-114].
Archer first classified in 1973 [114] these congenital malformations into
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- arteriovenous malformation group 2: defined by direct arteriovenous shunts between a branch of the retinal artery and vein, without any capillary plexus interposition; medium caliber vessels are typically involved (Figure 8). - arteriovenous malformation group 3: it accounts for a diffuse, marked dilatation of the retinal vessels, with many large caliber anastamosing channels. Visual impairment could be associated (Figure 9).
The most frequent ocular complications reported for all the AVM groups, are: vascular occlusions, aneurysm formation, intraretinal haemorrhage, exudation and cystoid macular oedema. Furthermore, neovascular glaucoma and open angle glaucoma have been described as a result of a retinal central vein occlusion [1].
Concerning groups 2 and 3 AVM, the occurrence of multiple arteriovenous malformations, in different extraocular sites, including central nervous system, orbit, eyelid, maxillofacial region, has been described
Type II: a greater amount of tissue extends into the spinal canal compared to Chiari malformation type I.
Correspondingly, there are two pathways that transports blood to the brain called internal carotid artery and vertebral artery. The internal carotid artery has three layers call the tunica adventitia, tunica media, and tunica intima. Tunica intima is made up of smooth muscle cells and elastin. The basilar artery forms and it branches out to the posterior cerebral arteries. The posterior cerebral arteries form the internal carotid arteries and when they connect they make cerebral arterial circle ( circle of willis). The middle cerebral arteries branch out two separate arteries called the anterior cerebral arteries. Each of these arteries are the force that direct the blood flow to the brain. There are three tiny vascular systems that work together to profuse the deep brain. Which are the pial, subependymal, and lenticulostriate arteries. The small area of white matter that depends on blood flow is called the subcortical “shed water” area. The subcortical is more prone than other areas of the brain to have ischemia. The leading cause of ischemia is the fibrin builds up and this cause a narrowing of the lumen. Which does not allow the flow of red blood cells and deprives the white matter of tissue of oxygen. The tissue then losses density and produces white matter lesions. The neurons become demyelinated which leads to loss of cognitive ability.
The Arnold-Chiari malformation refers to the second type of malformations associated with the Chiari group, where the irregular formation of the brainstem and cerebellum sees them
Recurrent epistaxis is one of the diagnostic criteria for Hereditary Hemorrhagic Telangiectasia (HHT). HHT is an autosomal-dominant disorder that is also depicted by skin and mucosal telangiectasias. Feared complications of HHT include rupture of pulmonary or cerebral arteriovenous malformations (AVM). The etiology of HHT is most often due to genetic mutations that impair normal angiogenesis. We report a case of suspected HHT in a 49-year-old female, with a first-degree relative with HHT, and a history of recurrent epistaxis status post coiling of left and right internal maxillary arteries. Of special note, her initial hemoglobin level was 1.7, but she was alert and walking at triage. Her presentation was consistent with multiple prior admissions in the past three years. Patients with suspected hereditary telangiectasia should receive a comprehensive work up, including serum studies, imaging, and possibly genetic testing. Treatment should focus on both acute management of the bleed and prevention of future complications.
An arteriovenous malformation (AVM) is an abnormal collection of blood vessels in which arterial blood flows directly into the draining vein without the normal interposed capillaries. AVM are tangled anastomosis of blood vessels of varying calibre in which arteriovenous shunting occurs in a central nidus in which the area towards in which multiple feeding arteries converge and from which enlarged vein drains.1 They have a higher rate of bleeding than normal vessels. AVMs can occur anywhere in the body. Brain AVMs are of special concern because of the damage they cause when they bleed. The incidence of AVM is about 1 in 100,000 per year in unselected
Malpositioned simply means that the aorta opens inside the heart in the wrong place. In a normal, healthy heart, the aorta is connected to the left ventricle, allowing oxygen rich blood to be pumped throughout the body. In patients suffering from tetralogy of Fallot, the aorta is slightly shifted to the right. This slight shift places the artery directly over the ventricular septum defect, which is typically located at the tip of the ventricles. As previously stated, the VSD allows oxygen rich and oxygen poor blood to mix together. With the aorta being directly over the VSD, this allows the mixture of blood to be pumped through the rest of the body, again contributing to the cyanotic presentation of the
The human body is a highly complex system of organs that operates efficiently at a cellular level to ensure proper functionality and longevity of the human race. However, even the slightest changes to its operation can lead to complications. In order to better understand the human body and how it works; an understanding of the diseases it is susceptible to is necessary. The cardiovascular system, in particular, is vulnerable to debilitating disease such as aneurysms, which is a ballooning of a blood vessel, or even an embolism (when any foreign body enters the blood stream). Blood vessels include arteries, capillaries and veins and therefore these events can occur anywhere in the body (i.e. the heart or the brain). There are different types of aneurysms including saccular, fusiform and pseudo-aneurysms. Saccular aneurysms only develop on part of the vessel wall and are spherical in shape. Fusiform aneurysms are ovoid in shape and develop over the entire vessel cross section. Pseudo-aneurysms are not true aneurysms but they occur as a result of a tear in the vessel wall thus collecting the blood pooled by extravascular tissue (Group). The exact cause of an aneurysm is not known but is correlated with certain previous existing conditions. Existing conditions that may have an effect on the development of an aneurysm are inherited predispositions to this disease, lifestyles, and high blood pressure. Some individuals are predisposed to developing aneurysms if their blood
Arteriovenous Malformation Resectionis a type of procedure which is performed when there is an abnormal tangle of vessels in the brain or spinal cord which can occur with one or more arteries which can be directly connected to one or more veins people are born with this although the it is not found to be hereditary. The arteries which carry oxegen aged blood to the heart and to the tissues and veins take blood back from the tissues to the heart n an Arteriovenous Malformation Resection the direct connection between one or more arteries and veins gives rise to many problems. The most serious problem that you have to deal with is when that the veins are usually thinned walled and may not be able to accept high blood pressure flow for a long period of time. The result of this is that
While the finding of a prepapillary vascular loop may be incidental, the unusual karyotype in this child makes the possibility of a causal relationship viable due to the fact, 8% of all congenital ocular abnormalities are associated with an abnormal karyotype (ORIGINAL CITATION, secondary source- Traboulsi E text). To the best of our knowledge, this is the first report of congenital prepapillary loop in a child with a chromosome abnormality. Congenital prepapillary loops have not yet been associated with an underlying genetic cause and of the reported pediatric cases, none were identified as having an underlying genetic or chromosome condition that may have contributed to the development of this vascular anomaly.5, 6, 7 Moreover, the youngest
Hereditary hemorrhagic telangiectasia, is also known as the Osler-Weber-Rendu Syndrome, Hereditary hemorrhagic telangiectasia effects approximately 1 in 5,000 people. It effects males and females from all racial ethnic groups. Dr. Rendu first described Hereditary hemorrhagic telangiectasia as a hereditary disorder involving nosebleeds and characteristic red spots that was distinctly different from hemophilia. HHT is a hereditary disorder that is passed down through generations, Its characterized by abnormal blood vessels. Blood vessels are the tubes that carry blood around our bodies. There are two types of blood vessels; arteries and veins. Arteries carry blood under high pressure out to all areas of the body after being pumped by the heart. Veins carry blood that should be under low pressure, back to the heart. An artery does not usually connect directly to a vein. Usually there are very small vessels called capillaries that connect an artery to a vein. A human with HHT has a tendency to form blood vessels that lack normal capillaries between an artery and vein. This means the arterial blood under high pressure flows directly into a vein without first having to squeeze through very small
This syndrome is diagnosed because of a few different factors. It is diagnosed when one has multiple tumors in the brain, spinal cord, or eye. Even if one
Sturge-Weber syndrome is also called "encephalotrigeminal angiomatosis." It is a congenital disorder. It causes a vascular birthmark and neurological abnormalities.
DAVFs are predominately idiopathic, however, a small percentage of patients had a history of trauma, prior craniotomy or pre-existing cerebral venous sinus thrombosis. Pathological arteriovenous fistula results in increased intracranial dural and cortical venous blood flow which impairs normal cerebral venous drainage. So, DAVFs can be classified based on the pattern of venous drainage which the most commonly used is the Cognard’s classification scheme. This classification divides the DAVFs into five types (type I-V) depending on direction of dural sinus drainage, the presence of cortical venous drainage (CVD) and venous outflow architecture(6). Type I lesions drain into the dural sinus, an antegrade flow direction, and lack of CVD. Type II
Arteriovenous malformations (AVMs) are currently diagnosed and treatment plan is formulated by using digital subtraction angiography (DSA) (Machet, Portefaix, Kadziolka, Robin, Lanoix & Pierot, 2012). There are some drawbacks to DSA, amongst them is the cost, patient discomfort, contrast injection and radiation exposure (Dautry, Edjlali, Roca, Rabrait, Wu, Johnson, Wieben, Trystram, Rodriguez-Regent, Alshareef, Turski, Meder, Naggara & Oppenheim, 2015). While DSA is considered to be the “Gold Standard” of care for AVMs there are studies underway to determine if brain MRA studies can obtain images
Vascular malformations involve invariably every organ of the body and are grouped into following four types on the basis of their gross and histopathologic characteristics: