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October 1961

Klippel-Feil Syndrome: Syringomyelia, Diastematomyelia, and Myelomeningocele—One Disease?

Author Affiliations

From the Department of Neurological Surgery, The Cleveland Clinic Foundation and The Frank E. Bunts Educational Institute.

Arch Surg. 1961;83(4):638-644. doi:10.1001/archsurg.1961.01300160150019

The skeletal anomalies of the Klippel-Feil syndrome are present also in the adult with syringomyelia,1 in the adolescent with diastematomyelia,2 and in the infant with myelomeningocele.1 These anomalies, believed due to improper segmentation of the mesodermal somites in embryonal life,2 consist of varying degrees and combinations of dilatation, bifid states, fusion, shortening, and curvature of the craniovertebral column, with associated deformities of the rib cage and extremities. Within the lumen of the distended craniovertebral column, the central nervous system also exhibits varying degrees of distention of its lumen (hydrocephalus and hydromyelia).1,3

Hypothesis  This occurrence of a distended tube of neural tissue within a distended tube of bone suggests cause and effect, namely, that overdistention of the neural tube (hydrocephalus and hydromyelia or "hydrocephalomyelia") in embryonal life has resulted in distortion of the encompassing sclerotomes, which then give rise to distorted bones—that in the individual case,

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