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Vein of Galen Aneurysm

Vein of Galen Arteriovenous Malformations


Presentation

  • Patients with neonatal vein of Galen aneurysms present with parenchymal loss on CT which is related to vascular steal and may be progressive.[ncbi.nlm.nih.gov]
  • Thus choroidal types and those presenting in the neonatal period do poorly.[radiopaedia.org]
Dysostosis
  • The authors report a case of thrombosed vein of Galen aneurysm in a 4.5-year-old girl with craniofacial dysostosis (Crouzon's disease). Six previously reported cases appear in the literature.[ncbi.nlm.nih.gov]
Fishing
  • Embryos were collected by natural spawning, and raised in fish water with methylene blue at 28.5 C.[doi.org]
Progressive Dementia
  • Although the number of patients is small, the risk for progressive dementia in this group is higher than in patients with other dural AVMs.[neupsykey.com]
Weight Gain
  • One of the most important goals of medical treatment is to permit normal oral feeding, which ensures weight gain and allows the child to go home and to return at 5 months of age for embolization.[radiologykey.com]
Feeding Difficulties
  • Mild symptoms include feeding difficulties, lack of weight gain, and tachycardia. Chest radiographs show cardiomegaly, especially involving the right side. If the cardiac failure is mild, medical treatment generally suffices.[radiologykey.com]
Left Ventricular Dysfunction
  • Theoretically, when left ventricular dysfunction owing to chronic volume overload occurs, digoxin should be beneficial. Dobutamine and dopamine may be used in severe situations to improve cardiac output.[radiologykey.com]
Muscle Weakness
  • The neurological examination revealed a total absence of interaction with either the environment or the examiner, failure to emit sounds, severe axial hypotonia, muscle weakness, signs of spasticity as well as pyramidal signs.[austinpublishinggroup.com]
Loss of Initiative
  • Six had a large degree of vascular steal demonstrated by cerebral angiography and all had significant parenchymal loss on initial CT.[ncbi.nlm.nih.gov]
Headache
  • Other features may include increased head circumference resulting from hydrocephalus, unusually prominent veins on the face and scalp, developmental delay, persistent headache, and other neurological findings. [1] [2] [3] Vein of Galen aneurysm is often[rarediseases.info.nih.gov]
  • The initial symptom typically falls into one of four categories: intracranial hemorrhage, seizure, headache and focal neurologic problems. Children are more likely to present with hemorrhage than adults.[childrenshospital.vanderbilt.org]
  • The child developed normally over the next six years, but then a sudden headache occurred, followed quickly by coma. CT revealed an intraventricular hemorrhage from the malformation and evidence of hydrocephalus.[nejm.org]
  • Case 2 An 8-year-old boy presented with 7 months history of recurrent headaches that were worse in the morning and associated with blurring of vision. The headaches were preceded by a 1-week history of irrational speech and behavior.[wajradiology.org]
  • Sometimes they can cause seizures or headaches. AVMs are rare. The cause is not known, but they seem to develop during pregnancy or soon after birth. Doctors use imaging tests to detect them. Medicines can help with the symptoms from AVMs.[icdlist.com]
Recurrent Headache
  • Case 2 An 8-year-old boy presented with 7 months history of recurrent headaches that were worse in the morning and associated with blurring of vision. The headaches were preceded by a 1-week history of irrational speech and behavior.[wajradiology.org]
Language Delays
  • The other patient had focal posterior cerebral artery territory infarcts on pretreatment MR imaging because of hypotension in the neonatal period and has mild motor and language delay.[neupsykey.com]

Workup

White Matter Lesions
  • MR imaging ( Fig. 12 ) may show focal white matter lesions or diffuse destruction of the brain (the melting brain), which is generally associated with severe cardiac insufficiency.[radiologykey.com]

Treatment

  • Surgery is not indicated as a treatment for VGAM, except in very rare cases. Patients undergoing VGAM treatment should have regular programmed clinical and morphological examinations.[orpha.net]
  • Endovascular treatments involve delivering drugs, balloons, or coils to the site of the malformation through blood vessels via catheters. These treatments work by limiting blood flow through the vein.[en.wikipedia.org]
  • Figure 4 summarizes this treatment algorithm. Figure 4 Treatment algorithm Cardiology assessment of cardiac function and superior vena cava flow should proceed right away.[surgicalneurologyint.com]
  • If the treatment goal cannot be achieved in one session, the patient will undergo a second session of treatment another day.[neupsykey.com]
  • Three of the seven children with mural lesions died shortly after treatment; the other four achieved normal or near-normal development after treatment.[ajnr.org]

Prognosis

  • Prognosis The neurological prognosis for VGAM with early treatment is excellent; however, the prognosis is poorer for patients with severe neonatal malformations. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • The value of antenatal sonography and magnetic resonance imaging in the prediction of prognosis is discussed.[ncbi.nlm.nih.gov]
  • Treatment and prognosis Prior to endovascular intervention, the prognosis was dismal, with 100% mortality without treatment and 90% mortality following surgical attempts 5 .[radiopaedia.org]
  • The prognosis of the disease has been notably dark, with death rates reaching 100% in the past.[nature.com]

Etiology

  • Clinical and laboratory findings are described in the case of a patient with a vein of Galen aneurysm who presented with recurrent aseptic meningitis for which no etiology could be identified.[ncbi.nlm.nih.gov]
  • The etiology of this outflow obstruction is unknown but may be related to underdevelopment of the jugular bulb, abnormal skull base maturation, and high-flow angiopathy of the venous system causing kinking or thrombosis at the tentorial or dural edge[clinicalgate.com]
  • We are not aware of any experimental studies that have investigated the etiology of progressive sinus occlusion.[neupsykey.com]
  • (Etiology) The exact cause of Vein of Galen Malformation is not known. It is believed that genetic changes in the developing fetus in the first trimester can lead to this abnormality.[dovemed.com]

Epidemiology

  • Summary Epidemiology The lesion is rare, with less than 800 cases (representing less than 10% of all cerebral arteriovenous malformations) reported so far. Vein of Galen aneurysmal malformation (VGAM) is more frequent in males than in females.[orpha.net]
  • Sero-epidemiological survey of human cytomegalo virus infection among expectant mothers in Bida, Nigeria. Internet J Infect Dis 2008;6. 17. Cutts FT, Robertson SE, Diaz-Ortega JL Samuel R.[wajradiology.org]
  • […] severe intracranial AVMs, including vein of Galen aneurysmal malformation, which was symptomatic at birth or during infancy, extracranial AVM of the face and extremities, and Parkes Weber syndrome, previously considered sporadic and nongenetic. [3] Epidemiology[emedicine.medscape.com]
  • Recinos PF, Rahmathulla G, Pearl M, Recinos VR, Jallo GI, et al. (2012) Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am 23(1): 165-177.[medcraveonline.com]
  • Sander Connolly, Epidemiology and natural history of arteriovenous malformations, Neurosurgical Focus, 11, 5, (1), (2001).[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • CONCLUSION: Our data demonstrate that most treated children survive and undergo normal neurological development; an understanding of the clinical, anatomical, and pathophysiological features of VGAM has, therefore, reversed the former poor prognosis.[ncbi.nlm.nih.gov]
  • Color-flow imaging and pulsed Doppler ultrasonography provided anatomical and pathophysiological information regarding cardiac hemodynamics and intracranial blood flow; with the patient's clinical status, these methods provided a reliable, noninvasive[en.wikipedia.org]
  • Pathophysiology The cardiac manifestations are due to an increase in blood flow through the cerebral arteriovenous shunt.[jnaccjournal.org]

Prevention

  • […] of preeclampsia Prediction and prevention of preterm birth Prediction and prevention of stillbirth Prediction and management of fetal growth restriction Problems of multiple pregnancies In 2016/2017, the Fetal Medicine Foundation made a donation of 22[fetalmedicine.org]
  • (10 - 16 June) and Paediatric expert and Consultant Paediatric Endocrinologist Rakesh Amin at Great Ormond Street Hospital (GOSH), is urging parents to learn the key steps on how to lower their children’s risk of developing type 2 diabetes, and even prevent[gosh.nhs.uk]
  • The high pressure in the veins can prevent the infant’s brain from draining adequately. This can lead to widespread brain injury and sometimes causes severe loss of tissue in the brain.[childrenshospital.org]
  • In a child who has not presented with cardiac failure, the aim of endovascular therapy is to prevent consequences of chronic cerebral venous hypertension and to promote normal cerebral development. Please contact our office for more information.[neurosurgery.ufl.edu]
  • This reduces the flow of blood and eases vascular overload, thus improving the heart condition How can Vein of Galen Malformation be Prevented? Presently, there exist no guidelines or methods to prevent Vein of Galen Malformation from developing.[dovemed.com]

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