Radiology, Vol 151, 393-396, Copyright © 1984 by Radiological Society of North America
Polycarpaly and other abnormalities of the wrist in chondroectodermal dysplasia: the Ellis-van Creveld syndrome
GA Taylor, CE Jordan, SK Dorst and JP Dorst
Radiographs of 45 wrists of 23 patients who had chondroectodermal dysplasia
(CED) showed variable wrist malformations, which we grouped into eight
categories. In each patient, the wrists tended to be similar but seldom
identical; in six patients they were sufficiently dissimilar that we
classified the malformations in their two wrists into separate categories.
A ninth carpal bone was present in the wrists of all patients who were five
years old or older (42 of 45 wrists). It was located in the distal row,
medial to the hamate bone and proximal to the fifth and sixth metacarpals.
A tenth carpal bone was found in six wrists. Unlike the ninth carpal bone,
it varied in location. All of the carpal bones were deformed, and two or
three separate ossification centers of the hamate were found in some
individuals. Fusions between the capitate, the hamate, and the ninth carpal
bone were present by the last available examination in 71% of the 45 hands:
these included fusions between the capitate and hamate in 7%, between the
hamate and the ninth carpal bone in 47%, and between all three bones in
18%.
