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Chiara II Malformation

Many children with spina bifida will have a deformity of the brain called Chiari II malformation. About one in every three children with spina bifida will exhibit mild symptoms of Chiari syndrome, usually in the form of feeding and swallowing problems. About one in twenty children with spina bifida will exhibit severe symptoms. You should become familiar with the signs and symptoms of Chiari malformation before your child leaves the hospital:

  • hypersensitivity to objects in the mouth (ie lumps in food, toothbrush)

  • gagging

  • choking

  • vomiting

  • stridor (high pitched "croupy" sounds)

  • tightness and/or weakness of arms

  • arching of head backwards

  • weak suck when bottle/breast feeding

Children may outgrow the difficulties of choking and swallowing, and difficulties with eating and drinking may diminish as they grow. Some children with hypersensitivity in or around the mouth may be helped by an occupational therapist or a speech language pathologist. A physiotherapist may provide suggestions for positioning to reduce neck arching and tightness of the arms.

Decompression Surgery

If symptoms persist or are severe, surgery may be required to reduce the pressure in the brain stem area. One option is the decompression of the brainstem by removing the back of the cervical vertebrae and the lowest part of the skull. This serves to unroof the cervical spine, thereby relieving pressure on the low-lying brainstem. You and your neurosurgeon should discuss the options.

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