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May 1982

Klippel-Feil Syndrome With Epidural Fibroblastoma in the Area of Vertebral Fusion

Author Affiliations

From the Department of Neurosurgery, Puerta de Hierro Clinic, Autonomous University, Madrid.

Arch Neurol. 1982;39(5):318-319. doi:10.1001/archneur.1982.00510170060021

Klippel-Feil syndrome1 is a congenital malformation of the cervical spine that is manifested by a failure in the segmentation of the somatic mesoderm. Although diverse neurological anomalies occur with the syndrome, the association with intraspinal tumors is exceptional.2-5 This article reports an extradural cervical fibroblastoma in a case of Klippel-Feil syndrome. To our knowledge, this association has not been described previously.

REPORT OF A CASE  A 57-year-old man had a two-month history of left cervicobrachialgia and paraparesis. Neurological examination showed paresis of the left arm with no sensory disturbances. There were also considerable spasticity and paraparesis of the lower limbs. He had a very short neck and low implantation of the hairline.Roentgenograms of the cervical spine showed fusion of C-3 and C-4 and cervicoarthrosis (Fig 1). Iophendylate (Pantopaque) myelography through the lumbar route showed a halt of the contrast medium at the C-4 level that was thought