Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)

Ellis-van Creveld syndrome in identical twins.

“…Seven fingers on both hands have also been noted (Weller, 1951). Most did not show polydactyly of the feet (Ellis and van Creveld, 1940;MacGregor, 1954;Metrakos and Fraser, 1954;Weiss and Crosett, 1955;Chauss, 1955;Walls et al, 1959;Husson and Parkman, 1961;Ellis and Andrew, 1962;and others), but in some six toes were also noted (Caffey, 1952;Turner, 1956) as well as in our two cases. If the complete syndrome includes polydactyly of the hands and the feet then anything less than this would represent a formefruste of the disease.…”

Coalition of the carpal bones

“…Other related conditions include T u r n e r ' s syndrome and M a r f a n ' s syndrome [5]. Massive fusions of the carpal bones are often present in patients with the Ellis van Creveld syndrome [20]. Carpal coalition also occurs in patients with the handfoot-uterus syndrome [17], the oto-palato-digital syndrome [11], and in fibula a n d ulna duplication with absence of the tibia a n d radius [18].…”

“…Coalition of the carpal bones is rarely a symptomatic lesion except in patients with massive fusion and limitation of motion [20]. Fusions do not transmit stresses well and there is a tendency to fracture [5,13].…”