PEDIATRICS Vol. 39 No. 4 April 1967, pp. 555-562
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A POSTVERTEBRAL ENTODERMAL CYST ASSOCIATED WITH AXIAL DEFORMITIES: A CASE SHOWING THE "ENTODERMAL-ECTODERMAL ADHESION SYNDROME"

N. Prop Ph.D.1, E. L. Frensdorf M.D.2, and F. R. van de Stadt M.D.3

1 Department of Anatomy/Embryology, State University of Groningen, Netherlands
2 Department of Pathology, State University of Groningen, Netherlands
3 Department of Surgery, State University of Groningen, Netherlands

A postvertebral tissue mass excised from the lumbosacral region of a 10-week-old child contained a small coelomic cavity and a convoluted enteric cyst with a blind branch. Some cleft vertebrae occurred at the level of the tissue mass.

The ontogenetic development of this complex of anomalies is explained on the basis of an amended theory of Beardmore and Wiglesworth. The primary etiological factor is supposed to have been an adhesion among entodermal and ectodermal cells in the median plane of the embryo in a bilaminar stage. An adhesion of this type can be assumed to cause the following developmental disturbances: (1) a dorsal enteric diverticulum (or fistula), (2) a split (or diverted) notochord and a cleavage of vertebrae, (3) a cleft spinal cord. These defects can be modified by growth and processes of repair. With a reference to the primary anomaly produced, "the entodermal-ectodermal adhesion syndrome" has been proposed as a name for the final complex of malformations.

Submitted on July 28, 1966
Accepted on October 15, 1966