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Anterior decompression for progressive brain stem compression in the Klippel-Feil syndrome

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Abstract

In 1912 Klippel and Feil1 described a condition characterized by fusion and reduction in the number of cervical vertebrae. Since that time more than 200 cases have been reported. To our knowledge none have been treated by the transman-dibular translingual approach described by Hall et al.2 The importance of this malformation lies in the secondary effects produced on the nervous system. Symptoms include cord and brain stem compression, weakness, spasticity, staggering, dysmetria, nystagmus, sensory loss, and lower cranial nerve involvement.

We report a case of progressive brain stem compression treated by posterior decompressive cervical laminectomy followed by an anterior decompression.

Case report

An 18-year-old man was studied for progressive spasticity. He was the second of five siblings. The diagnosis of Klippel-Feil syndrome was suggested when the patient was 8 years old. At age 12 years he was more awkward than he had been the previous year. Clumsiness was noted particularly after he sat any length of time. He repeatedly dropped things and believed his grip was deteriorating. On one occasion while playing football he fell; his arms immediately became numb and he was unable to move for 45 minutes. This subsequently resolved completely. Since that time progressive quadrispasticity and bulbar symptoms have developed.

Family history. Both nonconsanguineous parents are alive and well. The oldest child, aged 19 years, had respiratory distress at birth requiring tracheostomy. Presently she has whistling respirations, mild spasticity, and no brevicollis. Cervical roentgenograms were abnormal. Her nonidentical twin is normal. A 14-year-old sister has no symptoms. . . .


 

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