Vein of Galen malformations

Developmental Malformations

Updated 09.22.2024
Released 01.31.2000
Expires For CME 09.22.2027

Vein of Galen malformations

Authors: John Tucker Allred MD, Diana Tzeng MD

See Contributor Disclosures

Editor: Michael V Johnston MD†

Vein of Galen malformations

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Introduction

Overview

Vein of Galen malformations are a rare and congenital vascular abnormality consisting of one or more arteriovenous shunts draining into the median vein of the prosencephalon, the embryonic precursor of the vein of Galen. It is a rare condition, and the vast majority of cases are seen in the pediatric population. Management is best performed in large centers by a multidisciplinary team familiar with the entity. Advances in endovascular technique as well as critical care have helped improve the treatment and outcomes in what was once a condition with a uniformly poor prognosis. This article provides an overview of the condition and will be of interest to diverse medical fields, including neonatology, pediatrics, obstetrics, neurology, neurosurgery, and both diagnostic and interventional neurology.

Key points

	• The term vein of Galen malformation is actually a misnomer as the actual abnormality consists of one or more arteriovenous shunts that drain into the median prosencephalic vein of Markowski, an embryonic precursor to the vein of Galen that normally involutes.
	• Neonates commonly present with high output heart failure due to arteriovenous shunting. Children more commonly present with macrocephaly, hydrocephalus, and seizures due to venous hypertension.
	• The decision of when and if to treat patients through endovascular embolization is complex; factors to be considered include age of the patient, the presence of established cerebral damage, cardiorespiratory function, hepatic function, and renal function.
	• If possible, endovascular treatment of neonates is delayed until at least 5 to 6 months of age. During this time, patients are treated medically for heart failure, but there are cases of severe heart failure or other end organ dysfunction that necessitate early endovascular intervention.
	• Endovascular treatment may be staged and is most commonly performed via embolization with cyanoacrylate glue. Transvenous approaches may be used in combination or when an arterial route is not feasible or successful. Open neurosurgical treatment has little role in the modern day aside from ventricular shunting for hydrocephalus or, rarely, evacuation of intraparenchymal hemorrhage.
	• Infants and neonates presenting with severe cardiorespiratory function have the worst prognosis, and untreated malformations are almost always fatal. In appropriately selected patients without significant brain damage, the majority have good outcome and normal neurologic function.

Historical note and terminology

Many of the entities reported in past literature may not have been true vein of Galen malformations due to the complexity of the lesion and later developments in understanding embryology and pertinent anatomy. The first mention of a possible vein of Galen malformation by Steinhel in 1895, cited by the eminent neurosurgeon Walter Dandy, was in fact an arteriovenous malformation draining into an already formed vein of Galen, a distinct entity with greater risk of hemorrhage (07). There are later papers describing treatment via arterial ligation of arteriovenous communications into a vein of Galen that encounter the same problem and may or may not have been true vein of Galen malformations (04). Landmark work by Raybaud and others provided an improved understanding of cerebral venous anatomy and embryology and laid the groundwork for the current understanding of vein of Galen malformations. It was recognized that the abnormally dilated vein is actually a persistent embryonic precursor to the vein of Galen called the median prosencephalic vein, also called the median prosencephalic vein of Markowski (23).

Clinical manifestations

Presentation and course

	• Vein of Galen malformation may be detected on ultrasound as a cystic, midline brain lesion.
	• The large amount of arteriovenous shunting that occurs in a vein of Galen malformation commonly leads to high output heart failure
	• Patients most commonly present in the neonatal period with symptoms due to high output heart failure or as infants with hydrocephalus, seizures, developmental delay, or failure to thrive.
	• Older children or very rarely young adults may present with headaches, seizures, or intracranial hemorrhage.

The presentation of vein of Galen malformations is best understood by knowing the usual presentations by patient age group and by understanding the patterns of organ system derangement. Patients may present in the antenatal, neonatal, infantile, pediatric, or, very rarely, young adult age groups.

Vein of Galen malformations may be detected on antenatal ultrasound at the earliest by 25 weeks gestation as a cystic, midline brain abnormality posterior to the third ventricle (03). Color flow doppler technique may aid further in characterizing the lesion as a vascular abnormality (27; 22). Other important features that may be evaluated on antenatal ultrasound are presence of hydrocephalus and cardiac dysfunction (12). Fetal MRI aids in assessment of cardiac dysfunction and brain atrophy, both of which have prognostic significance (09).

More often, patients are diagnosed after birth; heart failure or multiorgan system failure is a common presentation in neonates. The first 24 hours after birth are often normal. In utero, the low resistance of the placenta competes as an alternative route for blood with the vein of Galen malformation; thus, flow through it is reduced. After birth and subsequent removal of the placenta, the flow through the vein of Galen malformation greatly increases, and cerebral blood flow may constitute as much as 80% of cardiac output (16; 24). Arteriovenous shunting leads to a significant amount of blood returning to the right side of the heart, which is not prepared to receive or pump such a large volume. High output heart failure may ensue, and severe pulmonary hypertension may be an additional complication (09; 24). Lastly, large arteriovenous shunts may reduce diastolic pressure in the aorta and can lead to myocardial ischemia. Heart failure may range from failure to thrive or mild volume overload to cardiorespiratory failure requiring intubation and multiorgan system failure in severe cases. Cyanosis may prompt a search for congenital heart disease (18).

Patients presenting in infancy generally present with symptoms attributable to cerebral venous hypertension rather than serious heart failure. Macrocephaly, hydrocephalus, and seizures are most common. Patients presenting with cardiac dysfunction later than the neonatal period usually have milder dysfunction amenable to medical therapy, which allows for appropriate delay in endovascular treatment to a later time (03; 24). Venous hypertension may lead to increased head circumference due to hydrocephalus. The hydrocephalus may be obstructive due to compression of the cerebral aqueduct or communicating due to decreased resorption of CSF as a result of venous hypertension. Other indications of venous hypertension may include dilated scalp veins, proptosis, cranial bruit, or recurrent epistaxis (09). Severe cases are characterized by calcifications, developmental delay, and seizures, though endovascular treatment has been observed to lead to improvement in cerebral calcifications (02). Rarely these calcifications may be associated with movement disorders. The most severe cases show irreversible brain damage and rapid atrophy called melting brain (01; 15). Patients who have patent venous sinuses do not experience melting brain syndrome because of the absence of pial and subpial venous reflux, though calcifications and a slow and permanent insult still occurs without early treatment (19).

Older children and, very rarely, young adults may present with headaches or seizures attributable to subarachnoid or intraparenchymal hemorrhage (12; 09; 01; 24). Microaneurysms may occur in the blood vessel network supplying the vein of Galen malformation, which creates an additional risk of hemorrhage (13).

Prognosis and complications

Beginning in utero, the use of ultrasound and fetal MRI can establish those patients who have a poor prognosis. However, the mere presence of a vein of Galen malformation is not necessarily an indication for termination of the pregnancy. Patients who show antenatal heart failure have 80% mortality (25). Similarly, the observation of brain atrophy or cardiac insufficiency on fetal MRI is associated with a poor prognosis (06). The poor outcomes of these patients are not improved with successful radiologic obliteration of the lesion; thus, heart failure or severe cerebral damage observed on antenatal imaging are potential indications for termination of pregnancy or conservative treatment on birth (10; 19).

After birth, neonates who have severe heart failure have the worst prognosis, though the development of endovascular methods to treat the arteriovenous shunting in a staged manner has greatly improved these patients compared to the past (03; 24). Before the advent of endovascular embolization, mortality rates for affected patients neared 100% (13).

There remains a significant amount of mortality in patients who present with early heart failure or multiorgan failure as well as neurologic disability among survivors. Multiple sources indicate a mortality rate ranging between 10% and 30%, with neonates at greater risk. Among patients treated with endovascular methods, between 50% and 74% have good outcome and normal neurocognitive function (19; 24; 11; 20). In a study of the long-term outcomes of children with vein of Galen malformations 6 to 11 years after treatment, authors found that 37% of patients had poor outcome and 13% of patients died despite or because of treatment complications (26).

Biological basis

	• The median prosencephalic vein of Markowski drains the fetal choroid plexus and is present from 6 to 11 weeks of gestational age; it normally involutes except at its most caudal end where it receives the internal cerebral veins and persists as the great vein of Galen.
	• The vein of Galen malformation is actually a misnomer and results from the development of abnormal arteriovenous shunts into the median prosencephalic vein of Markowski, which normally involutes but instead becomes hugely dilated due to arteriovenous shunting.
	• Vein of Galen malformations may be classified as choroidal or mural according to the classification scheme by Lasjaunias, or as types I to IV by Yasargil with only types I to III as true vein of Galen malformations.

A discussion of the ultimate cause for vein of Galen malformations must start with a basic understanding of development of cerebral vasculature and, more specifically, the venous system. During fetal development of the nervous system, closure of the anterior and posterior neuropores occurs between gestational weeks 6 and 10. During this time, the telencephalon blood supply is from multiple choroidal arteries arising from the fetal choroid plexus. Venous drainage of the choroid plexus is provided by paired internal cerebral veins that terminate on the caudal portion of the median prosencephalic vein of Markowski. The median prosencephalic vein of Markowski appears at gestational week 6, and it begins to involute in a rostral to caudal manner. By gestational week 11, the median vein regresses such that only the caudal portion where the internal cerebral veins join it remains, now as the great vein of Galen (09; 24; 17). For unknown reasons, it is during this period between gestational weeks 6 and 11 that arteriovenous shunts develop (23). The high blood flow and pressure lead to progressive dilation of the median prosencephalic vein, and instead of regressing, the vein enlarges in an aneurysmal fashion. This large dilation is possible because the vessel lies unencumbered in the subarachnoid space within a cistern called the velum interposition. Thus, though the caudal portion of the median prosencephalic vein normally persists in a small portion as the vein of Galen, a vein of Galen aneurysmal formation is a misnomer because the vessel of interest is actually an abnormally dilated and persistent median prosencephalic vein.

The arterial supply to the median prosencephalic vein varies and has led to a classification scheme developed by Lasjaunias to describe the two basic types of angioarchitecture of these lesions. The first type, termed choroidal, represents a developmentally more primitive arrangement of vessels in which choroidal arteries, including the anterior choroidal, posterior choroidal, and pericallosal arteries, contribute and form a complex arterial network before connecting with the vein. The choroidal type generally leads to a more severe clinical presentation and course. The second mural type of arrangement is less severe clinically and consists of a direct arteriovenous fistulous connection of one or more arteries with the vein (09; 19). Yasargil developed a similar classification system based on arterial feeder patterns, with types I to III representing true vein of Galen malformations and type IV representing an arteriovenous malformation producing a dilated vein of Galen proper (28).

Etiology and pathogenesis

The reason for development of arteriovenous shunts into the median vein of the prosencephalon remains unknown. It is postulated that an insult to the cerebrum of the fetus occurs between gestational weeks 6 and 11, the time during which the median prosencephalic vein is present. It is during this time that the arteriovenous shunt develops and the median prosencephalic vein persists as a large, aneurysmally dilated vessel. Genetic data and rare reports indicate that the vein of Galen malformation may occur in some cases due to RASA1 or EPHB4 genes (17).

Diagnostic workup

	• Evaluation of a patient should include history and physical examination, laboratory tests of hepatic and renal function, transfontanellar ultrasound, transthoracic echocardiogram, and magnetic resonance imaging of the brain.
	• Catheter-based cerebral angiography should not be performed purely as a diagnostic test but only if the patient should undergo endovascular treatment.

Evaluation of the pediatric patient suspected to have a vein of Galen malformation should begin with a complete history and physical including weight and head circumference. There should be special attention to neurologic function and development along with cardiorespiratory function and any clinical signs of renal or hepatic impairment. Laboratory tests of kidney and liver function are essential to assess for evidence of end organ dysfunction. A transthoracic echocardiogram should be obtained to assess for presence of and extent of any cardiac dysfunction. A transfontanellar ultrasound should be obtained to assess for encephalomalacia and may further aid in visualizing and characterizing any vascular malformations. Magnetic resonance imaging of the brain is essential to assess for brain atrophy, status of myelination, and morphology of the lesion. Noncontrast computed tomography may detect the vein of Galen malformation as a mass; however, in younger patients, magnetic resonance imaging of the brain is preferred as it spares the patient radiation. Commuted tomography with angiography has excellent resolution for the assessment of vascular architecture but does involve radiation exposure. Catheter-based cerebral angiography is the gold standard imaging modality for evaluation of the vascular lesion. However, it should not be obtained unless it has been deemed appropriate for the patient to receive endovascular embolization for treatment.

Management

	• The decision of when and if to treat patients through endovascular embolization is complex and factors to be considered include age of the patient, the presence of established cerebral damage, cardiorespiratory function, hepatic function, and renal function.
	• If possible, endovascular treatment of neonates is delayed until at least 5 to 6 months of age. During this time, patients are treated medically for heart failure as necessary. There are cases of severe heart failure or other end organ dysfunction that necessitate early endovascular intervention.
	• Endovascular treatment may be staged and is most commonly performed via embolization with cyanoacrylate glue. Transvenous approaches may be used in combination or when an arterial route is not feasible or successful. Open neurosurgical treatment has little role in modern-day treatment aside from ventricular shunting for hydrocephalus or, rarely, evacuation of intraparenchymal hemorrhage.

Management of patients with vein of Galen malformations is complex and best suited to a multidisciplinary team in a large center familiar with the condition. The management may be divided into medical, endovascular, and surgical approaches. The specific approach and decision-making process is influenced by the patient’s age at presentation and severity of disease.

Seizures should be managed with appropriate antiseizure medications based on the patient’s age and medical comorbidities. For patients in an intensive care unit setting or in whom there is suspicion of status epilepticus or frequent seizures, continuous EEG monitoring should be used.

An important part of medical management consists of treating any heart failure. Ideally, this is done until the patient is at least 5 to 6 months of age before endovascular treatment is pursued. There is a wide spectrum of severity of symptoms of cardiac insufficiency. Mild cases may manifest with feeding difficulties, tachycardia, minimal volume overload, and changes in chest radiography. Such cases may do well on only diuretics until definitive treatment with endovascular methods can be achieved. More severe cases may manifest with failure to thrive, severe volume overload, and respiratory failure. The most severe cases manifest with respiratory failure requiring mechanical ventilation, cardiogenic shock, and multiorgan failure, which may necessitate emergent endovascular embolization to help improve cardiac function. Until such measures can be undertaken, patients may require inotropes, diuretics, vasodilators, and mechanical ventilation (08; 24). Serial ultrasound may aid in assessing response to medical therapy.

The decision-making process to pursue endovascular treatment of a vein of Galen malformation must take into account many factors. First, angiography is technically challenging and high risk in neonates and becomes easier by 5 to 6 months of age (03). The ideal management of a neonate or infant with a vein of Galen malformation is medical treatment until this age. There are cases in which patients have heart failure refractory to medical therapy who require endovascular treatment on a more emergent basis before the age of 5 to 6 months. There is a set of patients in whom the presence of established severe brain damage or severe multiorgan failure are appropriate indications for conservative management or a comfort-focused model of care. Even if such patients have successful radiographic embolization, they are destined for extremely poor neurologic outcome (09; 19). Making matters more difficult is the observation that some patients have extremely poor neurologic outcomes despite normal brain imaging before successful emergency embolization.

Such complexity led Lasjaunias and colleagues to develop a 21-point score, called the Bicetre Neonatal Evaluation Score, with 5 domains describing cerebral, cardiac, respiratory, renal, and hepatic function to guide the complex decision-making process in neonates. A score of less than 8 indicates severe multiorgan dysfunction and generally leads to a decision not to pursue endovascular treatment. A score between 8 and 12 generally leads to emergency endovascular treatment, whereas scores above 12 lead to a decision for medical management and delaying of endovascular therapy until the age of 5 to 6 months (19). It should be kept in mind that such a scoring system cannot predict outcomes perfectly and that there are exceptions to the expected course of both low and high scores. For example, a patient with initial score of 6 underwent endovascular therapy and was neurologically intact after more than 2.5 years of follow up, whereas other patients with high scores of 21 had sudden declines and death before endovascular treatment (21; 24). Thus, patient care and decisions must be individualized.

When endovascular treatment is pursued, it may be taken in a staged manner with multiple treatments over the course of several months, as complete obliteration of the lesion is not necessary for cardiac function to improve. Besides improvement of cardiac function, the goals of endovascular treatment are to prevent complications of cerebral venous hypertension and ensure normal neurologic development. Arterial access may be performed through the umbilical artery in the first 3 days of life or through a femoral approach. Treatment involves the use of a liquid embolic agent, commonly cyanoacrylate glue, to occlude one or more vessels leading to a decrease in size of the aneurysmal vein and improvement in cardiac function and venous hypertension. An arterial approach is generally preferred in review of the literature. However, a transvenous approach through femoral or jugular veins, or even direct puncture of the torcula, may be used in cases in which arterial treatment is not feasible or successful. A combination of arterial and venous treatment approaches may also be used (09; 13; 24).

When hydrocephalus is present, the need for a ventricular shunt must be reviewed extremely carefully. Hydrocephalus may be well tolerated in neonates and infants in whom the fontanelles remain open. The hydrocephalus seen in patients with vein of Galen malformations is generally a secondary phenomenon from venous hypertension induced by the primary lesion. Thus, treatment of the vein of Galen malformation will address the hydrocephalus. Furthermore, there is evidence that ventricular shunting may cause harm in the form of hemorrhage due to venous congestion and an underdeveloped venous system (19; 13; 24). Ventricular shunting may be necessary in emergency situations where patients have clinical signs of elevated intracranial pressure due to hydrocephalus. Endoscopic ventriculostomy is also a viable option in these patients.

Traditional surgery has largely been supplanted by endovascular approaches in the treatment of vein of Galen malformations. It may be necessary in cases of intraparenchymal hemorrhage and hematoma evacuation, the treatment of hydrocephalus as mentioned above, or in cases where endovascular attempts at embolization have failed (13).

Outcomes

Patients who present in the neonatal period generally have a higher mortality than those presenting later as this generally indicates a large shunt with early and severe effects on cardiac function. A good outcome is expected in patients who do not have signs of significant brain damage, and reviews have indicated normal neurologic function in approximately 50% to 74% of appropriately selected patients who underwent endovascular embolization (03; 19; 24; 11; 20). A meta-analysis of 27 studies and 578 patients found that all-cause mortality was 14% and rate of good neurologic outcome was 62%. Additional findings of interest were that neonates and patients presenting with congestive heart failure showed significantly decreased likelihood of good outcome. Lastly, studies that used the Bicetre score from Lasjaunias as a selection criteria for treatment showed greater rates of good neurologic outcome than those who did not (05).

References

01: Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, Lasjaunias P. Vein of Galen aneurysmal malformations. Neuroimaging Clin N Am 2007;17(2):189-206. Erratum in: Neuroimaging Clin N Am 2007;17(4):xi. PMID 17645970
02: Bansal A, Gailloud P, Jordan L, Ruíz DS. Regression of cerebral calcifications after endovascular treatment in a case of vein of Galen arteriovenous malformation. J Neurosurg Pediatr 2009;4(1):17-20. PMID 19569904
03: Bhattacharya JJ, Thammaroj J. Vein of galen malformations. J Neurol Neurosurg Psychiatry 2003;74 Suppl 1(Suppl 1):i42-4. PMID 12611934
04: Boldrey E, Miller ER. Arteriovenous fistula (aneurysm) of the great cerebral vein (of Galen) and the circle of Willis; report on two patients treated by ligation. Arch Neurol Psychiatry 1949;62(6):778-83. PMID 15396364
05: Brinjikji W, Krings T, Murad MH, Rouchaud A, Meila D. Endovascular treatment of vein of galen malformations: a systematic review and meta-analysis. AJNR Am J Neuroradiol 2017;38(12):2308-14. PMID 28982789
06: Brunelle F. Brain vascular malformations in the fetus: diagnosis and prognosis. Childs Nerv Syst 2003;19(7-8):524-8. PMID 12920542
07: Dandy W. Arteriovenous aneurysm of the brain. Arch Surg 1928;17:190.
08: Frawley GP, Dargaville PA, Mitchell PJ, Tress BM, Loughnan P. Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation. Arch Dis Child Fetal Neonatal Ed 2002;87(2):F144-9. PMID 12193525
09: Gailloud P, O'Riordan DP, Burger I, et al. Diagnosis and management of vein of galen aneurysmal malformations. J Perinatol 2005;25(8):542-51. PMID 16015373
10: Garcia-Monaco R, De Victor D, Mann C, Hannedouche A, Terbrugge K, Lasjaunias P. Congestive cardiac manifestations from cerebrocranial arteriovenous shunts. Endovascular management in 30 children. Childs Nerv Syst 1991;7(1):48-52. PMID 2054809
11: Gopalan V, Rennie A, Robertson F, et al. Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series. Dev Med Child Neurol 2018;60(4):424-429. PMID 29359331
12: Gupta AK, Varma DR. Vein of Galen malformations: review. Neurol India 2004;52(1):43-53. PMID 15069238
13: Hoang S, Choudhri O, Edwards M, Guzman R. Vein of Galen malformation. Neurosurg Focus 2009;27(5):E8. PMID 19877798
14: Jayaprasad N. Heart failure in children. Heart Views 2016;17(3):92-9. PMID 27867456
15: Khullar D, Andeejani AM, Bulsara KR. Evolution of treatment options for vein of Galen malformations. J Neurosurg Pediatr 2010;6(5):444-51. PMID 21039167
16: King WA, Wackym PA, Viñuela F, Peacock WJ. Management of vein of Galen aneurysms. Combined surgical and endovascular approach. Childs Nerv Syst 1989;5(4):208-11. PMID 2790832
17: Komiyama M. The median vein of prosencephalon of Markowski: from morphology to genetics. Interv Neuroradiol 2020;26(6):752-6. PMID 33283591
18: Kothari SS, Naik N, Juneja R, Saxena A. Aneurysm of the vein of galen in neonates: report of four cases. Indian Heart J 2001;53(4):499-502. PMID 11759944
19: Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery 2006;59(5 Suppl 3):S184-94; discussion S3-13. PMID 17053602
20: Lecce F, Robertson F, Rennie A, et al. Cross-sectional study of a United Kingdom cohort of neonatal vein of galen malformation. Ann Neurol 2018;84(4):547-55. PMID 30155909
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22: Nuutila M, Saisto T. Prenatal diagnosis of vein of Galen malformation: a multidisciplinary challenge. Am J Perinatol 2008;25(4):225-7. PMID 18548395
23: Raybaud CA, Strother CM, Hald JK. Aneurysms of the vein of Galen: embryonic considerations and anatomical features relating to the pathogenesis of the malformation. Neuroradiology 1989;31(2):109-28. PMID 2664553
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27: Vintzileos AM, Eisenfeld LI, Campbell WA, Herson VC, DiLeo PE, Chameides L. Prenatal ultrasonic diagnosis of arteriovenous malformation of the vein of Galen. Am J Perinatol 1986;3(3):209-11. PMID 3521637
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Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Authors

John Tucker Allred MD

Dr. Allred of Thomas Jefferson University has no relevant financial relationships to disclose.
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Diana Tzeng MD

Dr. Tzeng of Thomas Jefferson University received consulting fees from Abbott and Astra Zeneca.
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Editor

Michael V Johnston MD†

Dr. Johnston of Johns Hopkins University School of Medicine had no relevant financial relationships to disclose.
See Profile

Former Authors

Imad S Khan MD
Robert J Singer MD

Patient Profile

Age range of presentation: 0 month to 18 years
Sex preponderance: Male:Female ratio of 3:1
Heredity: There are rare reports of vein of Galen malformations believed to be caused by RASA1 or EPHB4 genes; it is unknown what percentage of vein of Galen malformations are caused by these genes.
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: None selectively affected

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Vein of Galen malformations

Associated Disorders

Capillary malformation-arteriovenous malformation syndromes 1 and 2

Differential Diagnosis

Arteriovenous malformation into vein of Galen
Cerebral aneurysm
Congenital heart disease
Valvular disease
Thiamine deficiency

Also Relevant

Cerebral venous thrombosis
Cerebral venous thrombosis in infants and children
Endovascular management of vein of Galen malformations

Vein of Galen malformations

Vein of Galen malformations

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Differential diagnosis

Diagnostic workup

Management

Outcomes

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview.
Start a Free Account
to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

X-linked hydrocephalus (L1 syndrome)

Breath-holding spells

Cerebro-oculo-facio-skeletal syndrome

Ischemic stroke

Cerebral embolism

Hormonal contraception and stroke

Acute cerebellar ataxia in children

Vein of Galen malformations

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Differential diagnosis

Diagnostic workup

Management

Outcomes

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

X-linked hydrocephalus (L1 syndrome)

Breath-holding spells

Cerebro-oculo-facio-skeletal syndrome

Ischemic stroke

Cerebral embolism

Hormonal contraception and stroke

Acute cerebellar ataxia in children

This is an article preview.
Start a Free Account
to access the full version.