Abstract
Chiari malformation is commonly considered a congenital condition. To our knowledge,
reports of progressively symptomatic Chiari Ι malformation with craniometaphyseal
dysplasia are rare. The authors present a case of progressively symptomatic Chiari
Ι malformation occurring in an 11-month-old infant with craniometaphyseal dysplasia.
The patient presented with a typical facial appearance and radiological skeletal survey.
Posterior fossa decompression was performed. In the meantime, the patient was given
Rocaltrol® (calcitriol) and adopted a low calcium diet. His neurological symptoms
were markedly improved after surgery. The clinical presentations, radiographical features
and prognosis of the patient are discussed with reference to the literature.
Key words
Chiari malformation - bone dysplasia - craniometaphyseal dysplasia - posterior fossa
decompression
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Prof. Changhong Shen
Department of Neurosurgery
General Hospital of Tianjin Medical University
No. 54, Anshan Road, Heping District
300052 Tianjin
China
Email: tjpns@yahoo.com.cn
