PEDIATRICS Vol. 21 No. 2 February 1958, pp. 298-307
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CHONDROECTODERMAL DYSPLASIA (ELLIS-VAN CREVELD SYNDROME)

Report of Two Cases

Harris L. Smith M.D.1 and Albert M. Hand M.D.1

1 Divisions of Pediatrics and Pathology and Microbiology, University of Tennessee College of Medicine and the Frank T. Tobey Memorial Children's Hospital, Memphis, Tennessee

Two cases of chondroectodermal dysplasia (Ellis-van Creveld syndrome) in infants are reported. The four major features of the syndrome (chondrodysplasia, ectodermal dysplasia, polydactylism, and congenital defect of the heart) were present in each of the infants. One of the cases (Case 1) is believed to be the first published instance of this syndrome in the Negro race. The clinical course of both infants was marked by progressive dyspnea and cyanosis, leading rapidly to death in the newborn period.

Detailed gross and microscopic pathologic findings are included. The cardiac malformation in one case was cor triloculare and in the other was cor biloculare with transposition of the great vessels. The thoracic cage of each infant was extremely narrowed anteriorly and flared inferiorly. Chondro-osseous junctions of ribs were in the anterior axillary line. Death was attributed to the congenital malformation of the heart, pulmonary hypoplasia, and diminished capacity of the thorax due to chondro-osseous dysplasia. The severe chest deformities together with serious congenital cardiac lesions distinguish these cases.

The literature is briefly reviewed, and the clinical and radiographic features of the syndrome are discussed.

Submitted on June 21, 1957
Accepted on August 2, 1957


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