Ellis-Van Crevald syndrome.

A six year old child with symmetric polydactyly of both hands and genu valgum deformity was referred for radiographic assessment. On physical examination, there were six digits in both hands with dysplastic finger nails. The upper and lower extremies were shortened out of proportions to the trunk with genu valgum deformity. Radiograph of hands revealed bifid 5th metacarpals and complete duplication of the little fingers bilaterally with short distal and middle phalanges as compared to proximal ones (Fig. 1). Both knees showed hypoplasia and irregularity of lateral portion of upper tibial epiphyses with dome shaped metaphysis leading to valgus deformity. Chest radiograph revealed enlarged cardiac shadow, which on further assessment with 2D echocardiography revealed an atrial septal defect of ostium secondum type. The case was diagnosed as Ellis-Van Crevald syndrome.