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An arteriovenous malformation (AVM) consists of a tangle of dilated vessels that form an abnormal communication between the arterial and venous systems, really an arteriovenous stula It is a developmental abnormality representing persistence of an embryonic pattern of blood vessels and is not a neoplasm, but the constituent vessels may proliferate and enlarge with the passage of time Arteriovenous malformations have been designated by a number of other terms, such as angioma and arteriovenous aneurysm, but these are less appropriate; angioma suggests a tumor, and the term aneurysm is generally reserved for the lesions described in the preceding section Venous malformations, consisting purely of distended veins deep in the white matter, are a separate entity;

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they may be the cause of seizures and headaches but seldom of hemorrhage When a small hemorrhage occurs in relation to venous malformation, it is usually due to an associated cavernous malformation Vascular malformations vary in size from a small blemish a few millimeters in diameter lying in the cortex or white matter to a huge mass of tortuous channels constituting an AV shunt of suf cient magnitude, in rare instances, to raise cardiac output Hypertrophic dilated arterial feeders can be seen approaching the main lesion and to break up into a network of thin-walled blood vessels that connect directly with draining veins The latter often form greatly dilated, pulsating channels, carrying away arterial blood The tangled blood vessels interposed between arteries and veins are abnormally thin and do not have the structure of normal arteries or veins Arteriovenous malformations occur in all parts of the cerebrum, brainstem, and cerebellum (and spinal cord), but the larger ones are more frequently found in the central part of a cerebral hemisphere, commonly forming a wedge-shaped lesion extending from the cortex to the ventricle Some lie on the dural surface of the brain or spinal cord, but these most often turn out to be direct arteriovenous stulas, as discussed further on When hemorrhage occurs, blood may enter the subarachnoid space, producing a picture almost identical to that of ruptured saccular aneurysm, but generally less severe; since most AVMs lie within cerebral tissue, bleeding is more than likely to be intracerebral as well, causing a hemiparesis, hemiplegia, and so forth, or even death Arteriovenous malformations are about one-tenth as common as saccular aneurysms and about equally frequent in males and females The two lesions AVM and saccular aneurysm (on the main feeding artery of the AVM) are associated in about 5 percent of cases; the conjuction increases with the size of the AVM and the age of the patient (Miyasaka et al) Rarely, AVMs occur in more than one member of a family in the same generation or successive ones For a review of the embryologic theories of formation of AVMs, the reader is directed to the article by Fleetwood and Steinberg Clinical Features Bleeding or seizures are the main modes of presentation Most AVMs are clinically silent for a long time, but sooner or later they bleed The rst hemorrhage may be fatal, but in more than 90 percent of cases the bleeding stops and the patient survives The rate of hemorrhage in untreated patients is established to be 2 to 4 percent per year, far lower than for aneurysms The mortality rate in two major series (Crawford et al, Ondra et al) has been 1 to 2 percent per year but as high as 6 to 9 percent in the immediate year following a rst hemorrhage The matter of an increased risk of AVM rupture during pregnancy has been disputed The weight of evidence suggests that the risk is not raised by pregnancy alone, but as with saccular aneurysm that parturition and Valsalva activity is always a source of concern Before rupture, chronic, recurrent headache may be a complaint; usually the headache is of a nondescript type, but a classic migraine with or without neurologic accompaniment occurs in about 10 percent of patients probably with greater frequency than it does in the general population Most of the lesions associated with migraine-like headaches lie in the parieto-occipital region of one cerebral hemisphere, and about two-thirds of such patients have a family history of migraine Huge AVMs may produce a slowly progressive neurologic de cit because of compression of neighboring structures by the.

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