By Pierre Lasjaunias M.D., Ph.D. (auth.)

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In children and young adults, proliferative and angioectatic diseases are frequently triggered by spontaneous or traumatic dissections, viral infections, immune phenomenon and other causes. Moyamoya disease, moyamoya-like syndromes, and proliferative angiopathy in children are the most typical disorders in this group (Figs. 9). Fig. 9a-j. A 15-year-old girl presenting with generalised seizures and referred to us with the diagnosis of large arteriovenous malformation. This corresponds to what we describe as proliferative angiopathy.

Usually bilateral and symmetrieal, it corresponds to a regional decrease in the cerebral blood flow caused by retrograde venous hyperpressure, leading to hydrovenous dysfunction. Arterial steal is not present or accessory in this syndrome. 19). These findings are never encountered in adults. It illustrates the role played by the subpial and medullary veins in the maintenance and development of the white matter. It may not be seen in lesions that open without restrietion into a subarachnoid venous outlet.

A young girl referred to us at the age of 5 years due to significant macrocrania already shunted. a Magnetic resonance imaging (MRI) demonstrated obvious tonsillar prolapse. Despite the spectacular picture, neurological and neurocognitive evaluation was normal. The child has a score of 4. b Following embolisation, there is almost complete occlusion of the vein of Galen aneurysmal malformation with shrinkage of the ectatic pouch. 18 a-c. A young adult male who had had a torcular dural arteriovenous lesion with macrocrania since infancy.

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Vascular Diseases in Neonates, Infants and Children: by Pierre Lasjaunias M.D., Ph.D. (auth.)
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