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Clinical Neuroscience News, June 2013

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Case Study: Chiari Malformation

Harold L. Rekate, MD, FACS, FAAP and
Salvatore Insinga, DO

An eight-year-old girl with multiple birth defects related to monosomy of chromosome 18p, was brought to us from her home in another state for a neurosurgery consult. She is of extremely short stature, has hypothyroidism and asthma, and is moderately developmentally delayed with little indication of language facility; her medical history includes previous surgeries to address various physical limitations. We evaluated her to examine the causes of her extreme difficulty in swallowing; she was taking all her nourishment through a gastrostomy tube. She also had frequent bouts of uncontrolled coughing.

Our MRI scans showed a severe Chiari I malformation together with bony abnormalities of the anterior skull base, resulting in occipitocervical instability, anterior compression of the brainstem from a retroflexed odontoid and basilar invagination. We advised and performed a decompressive surgery, realignment of the occipital cervical region and fixation to reduce instability, as these are key to the patient’s swallowing issues and possibly other problems. Intraoperatively, we found her spinal cord sensory signals to be normal, but her motor signals extremely attenuated. Using ultrasound guidance, our team first performed a surgical reduction of her occipitocervical instability.

Next, under microscopic visualization, we did an extensive dissection of the scaring over the cerebellar tonsils and an autologous tissue graft was sewn in place to expand that area. Subsequently the patient’s head was gently repositioned in extension and distraction, followed by insertion of permanent fixation. This reduced pressure on the anterior brainstem, thus improving motor spinal cord signals dramatically.

By the fifth day, postoperatively she was ambulating under oral pain medication. By the eighth day, the patient showed a marked improvement in her ability to swallow and to eat ordinary solid food comfortably. She was also speaking more clearly, walking well and was in minimal pain. She returned home where she continues to improve.

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Case Study: Chiari Malformation

Preoperative sagittal cervical MRI showing basilar invagination. 

Case Study: Chiari Malformation

Postoperative MRI of the cervical spine showing reduction of odontoid (C2) process.