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It is characterized by multiple cerebral arteriovenous shunts leading to intracerebral bleeds and embolic phenomena. The possibility for cardiovascular instability and signs of congestive heart failure must be assessed.

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Bonnet-Dechaume-Blanc Syndrome; Cerebroretinal Arteriovenous Aneurysms.

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This syndrome was first described by P. Bonnet in 1937.

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Not probable.

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Multiple arteriovenous malformations exist; cause is unknown.

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Multiple malformations exist both intracerebrally and elsewhere. Retinal malformations are the most common (81% of cases). Neurological symptoms vary among central nervous system lesions based on location. Diagnosis is confirmed by fluorescein angiography, CT scan, and magnetic resonance imaging (MRI).

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The presence of intracerebral arteriovenous malformations may lead to intracerebral bleeds and embolic phenomena as the first presenting sign. Alternatively, ophthalmic arteriovenous malformations may cause sudden loss of vision as a result of bleeding. Hemorrhage caused by dental extraction overlying a lesion has been reported. Many of these lesions are now treated by embolization in the radiology department.

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History and examination must elucidate the location of the lesions.

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Intraoral lesions must be adequately protected at the time of anesthetic induction or intraoral instrumentation. Intracranial arteriovenous malformations are at risk of rupture if subjected to high swings in arterial blood pressure. Intraocular lesions may bleed for similar reasons. Gastrointestinal hemorrhage and hemoptysis have all been described. If very extensive, multiple arteriovenous malformations may lead to a high-output cardiac failure, requiring treatment before anesthesia and surgery. Central regional anesthesia is best avoided because of the risk of associated medullar angioma.

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Ketamine and Hypnomidate are best avoided because they can cause high blood pressure that is relatively contraindicated in arteriovenous malformations.

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Von Hippel Lindau Syndrome: Usually begins during young adulthood but may appear during childhood around the age of 8 years. Clinical features include headaches, dizziness and ataxia. Behavior problems may also be present. Cerebroretinal aneurysms may develop. Adrenal gland tumors have been reported.

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Sturge-Weber Syndrome: A hereditary disorder manifested by the presence of large facial port-wine stain angioma and intracranial arteriovenous malformations present at birth. Generalized seizures and an array of neurological symptoms usually occur at the age of 1 or 2 years. The vascular lesions in the brain usually involve the occipital or parieto-occipital regions.

Brodsky MC, Hoyt WF: Spontaneous involution of retinal and intracranial arteriovenous malformation in Bonnet-Dechaume-Blanc syndrome. Br J Ophthalmol 86(3):360, 2002.
Iizuka Y, Garcia-Monaco R, Alvarez H, et al: Multiple cerebral arteriovenous malformations in children. Childs Nerv Syst 8:437, 1992.  [PubMed: 1288852]

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