Partial posterior split cervical spinal cord with Klippel-Feil syndrome.

A 50 year-old woman was known with Klippel-Feil syndrome (KFS), consisting of a clinical triad of a short neck, low posterior hairline and restricted neck motion. She was complaining of new onset neck pain radiating down to midscapular region. Physical examination revealed moderate weakness of both biceps and polex muscles. She had diminished sensation of pinprick and light touch between C3-6 dermatoms. Radiographs revealed multiple contiguous fusion between C1 to C6 vertebrae (Fig. A). No sign of instability was detected. Magnetic resonance imaging (MRI) confirmed bony fusion of the vertebral bodies. There is complete fusion between first cervical vertebra to the fourth cervical vertebra and incomplete fusion between fifth and sixth servical vertabrae. The seventh cervical vertebra is normal (Fig. B). Additionally posterior partial split cervical spinal cord from foramen magnum to third cervical vertebra was evident (Fig. C). Based on the clinical presentation and typical imaging findings, the diagnosis of partial split cervical spinal cord and cervical upper vertebrae multiple noncontigiuous fusions were made. MRI of the thoracolumbar spine was unremarkable. The patient has been neurologically stable for 2 years after the initial presentation. Her functional deficit remains mild and did not require any operation.