Ellis-van Crevald (EvC) syndrome, also called Chondroectodermal dysplasia, is a form of short-limbed dwarfism.Ellis-van Creveld (EvC) syndrome is a skeletal dysplasia with an incidence of approximately 1 out of 1,50,000 live births.

Clinical features include postaxial polydactyly of hands which is seen in all the patients. Polydactyly may be just extra-soft tissue not adherent to the skeleton and devoid of bone, cartilage, joint or tendon, or the digit may show duplication with components like bifid metacarpal or there may be a complete digit formation with its own metacarpal and complete soft tissue.

There is also short-limbed disproportionate dwarfism, with the extremities shortened out of proportion to the trunk. The bone dysplasia is characterized by acromesomelia- relative shortening of the distal and middle segments as opposed to the proximal segments. These findings are well demonstrated in the hand, where the distal and middle phalangeal segments are proportionately shorter than the proximal segment.

Association- Congenital heart defects occur in about 50% of the patients. The most common heart defects are Atrial septal defects. Other less common ones are Ventricular septal defects, hypoplasia of the aorta and a single atrium.