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.
Bonnet-Dechaume-Blanc Syndrome; Cerebroretinal
This syndrome was first described by P. Bonnet in
Multiple arteriovenous malformations exist; cause
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).
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.
History and examination must
elucidate the location of the lesions.
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.
Ketamine and Hypnomidate are best
avoided because they can cause high blood pressure that is relatively
contraindicated in arteriovenous malformations.
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.
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,
Iizuka Y, Garcia-Monaco R, Alvarez H, et al: Multiple cerebral arteriovenous
malformations in children. Childs Nerv Syst