Synostosis (Subscribe)  

Brachydactyly ("short digits") is a general term that refers to disproportionately short fingers and toes, and forms part of the group of limb malformations characterized by bone dysostosis. The various types of isolated brachydactyly are rare, except for types A3 and D. Brachydactyly can occur either as an isolated malformation...

Alternative titles; symbols
CENANI-LENZ SYNDACTYLY
SYNDACTYLY, TYPE VII
Cenani and Lenz (1967) described 2 brothers with a form of syndactyly resembling that of Apert syndrome (101200). However, additional features were severe shortening of the ulna and radius with fusion, fusion of the metacarpals and 'disorganization' of phalangeal development.

Features oligodactyly (fewer digits), carpal and/or metacarpal synostosis and syndactyly

Characteristic chest deformities consist of pectus carinatum superiorly and pectus excavatum inferiorly. These sternal abnormalities are present in 70%–95% of cases. The thorax is broad and the internipple distance is large. About 15% of patients develop thoracic scoliosis. The shoulders are often rounded with scapula alata. Other common orthopaedic features...