The autosomal dominant syndrome named after Osler, Weber, and Rendu consists of two genotypes (types 1 and 2). Type 1 is associated with mucocutaneous telangiectasias, pulmonary arteriovenous fistulas, and arteriovenous shunts of the central nervous system. The associated spinal arteriovenous shunts are most often seen in the pediatric population and are always intradural (pial) arteriovenous fistulas, subtype C (ventral intradural AVF; or type IV, subtype C). The endothelial cells in this syndrome lack the molecule "endoglin" and form abnormal vessels, especially after injury.23
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