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Study Design. Case report and clinical discussion.

Objective. To describe a rare case of intradural cervical disc herniation in a patient with Klippel-Feil syndrome (KFS).

Summary of Background Data. KFS is a congenital spinal malformation characterized by the failure in segmentation of 2 or more cervical vertebrae. The development of a cervical disc herniation in a nonfused segment is uncommon. Intradural disc herniation is rare, with only 21 cases reported in the cervical region.

Methods. We present a case of a 52-year-old woman with KFS (C5-C6 fusion) who developed acute radiculopathy secondary to an intradural cervical disc herniation. Neurologic examination revealed a mild (Grade 3/5) decrease in motor function of the fingers and difficulty in performing fine motor tasks with right hand.

Results. The patient underwent microsurgical removal of the herniated disc via an anterior approach followed by interbody fixation and anterior plating; exploration of the surgical field revealed 2 intradural disc fragments. After surgery, she experienced a complete remission of the symptoms.

Conclusion. To our knowledge, this is the first case of intradural cervical disc herniation in a KFS patient described in the literature.

(C) 2007 Lippincott Williams & Wilkins, Inc.