A Taiwanese infant with clinically apparent oto-palato-digital syndrome type II had Arnold-Chiari I malformation. Arnold-Chiari I malformation has not been reported previously to occur in association with oto-palato-digital type II syndrome. The pathogenesis of both conditions has remain unclear although the Arnold-Cliari I malformation is most likely due to a developmental abnormality of improperly times or incomplete closure of the neural tube. We propose the physician who care for children with OPD type II must be aware of one more condition Copyright (C) 1999 Elsevier Science B.V.
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