SYNDACTYLY AND INTRACRANIAL ARTERIOVENOUS MALFORMATION - CASE-REPORT

A case of a 14-year-old boy with syndactyly of all limbs and intracran ial dural arteriovenous malformation (AVM) is presented. A failure of differentiation of cerebral vessels, possibly at 3 weeks gestational a ge, is discussed along with several alternative theories relating to t he pathogenesis of dural AVMs. The embryogenesis of syndactyly, occurr ing as an arrest of normal interdigital tissue regression at 5-7 weeks gestational age, is also reviewed. It is postulated that a single com mon intrauterine environmental insult of vascular origin occurring dur ing the second month of gestation, is the reason for their simultaneou s manifestation.