Autosomal dominant disorder due to defect in fibrillin (network for elastin) on chromosome 15

- tall, thin build with long extremities- arachnodactyly- ectopia lentis (typically upward and temporally)- mitral valve prolapse- dilation of the ascending aorta with cystic medial degeneration- hypermobile joints- pectus excavatum (inwardly depressed sternum), pectus carinatum