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A case of cervical diastematomyelia and syringohydromyelia in a 16-year-old female myelomeningocele patient is reported. Progressive weakness of the upper extremity led to an MR examination of the brain and spine, which revealed hydrocephalus, Chiari II malformation, cervical diastematomyelia with a syringohydromyelic cavity in each hemicord and a large dural sac in the lumbar region. Operative therapy consisted of detethering and shunting of the two syringes. Soon after surgery her symptoms improved. The need for early complete MR imaging of myelomeningocele patients presenting with new symptoms is emphasized.

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We report a case of a severe form of achondrogenesis type I. Prenatal ultrasonography showed a micromelic fetus; bony structures could not be identified. Postnatal radiographs revealed some foci of ossification in the ossa ilia, the clavicles, the upper and the lower jaw and the base of the skull. The long bones, the vertebral column and the ribs were not visible. The diagnosis was established by histologic examination of the growth plates.