Acquired Chiari malformations: incidence, diagnosis, and management

The acquired descent of the cerebellar tonsils radiographically indistinguishable from Chiari I malformations has been previously reported. The relationship between lumbar shunting procedures for hydrocephalus and symptomatic Chiari malformations has been established. We report the cases of 10 children with lumboperitoneal shunts in whom previous radiographic studies had confirmed a normal hindbrain configuration. Seven of the 10 patients acquired tonsillar descent into the foramen magnum, detected by magnetic resonance imaging, whereas the others remained normal. Four of seven patients were symptomatic; two underwent the removal of the lumboperitoneal shunt and conversion to a ventriculoperitoneal shunt, and two underwent posterior fossa decompression. Further magnetic resonance imaging revealed that one of the two patients who underwent conversion shows ascent of the cerebellar tonsils. All four patients became asymptomatic less than 6 months after treatment. In this article, we discuss seven cases of acquired Chiari malformations and the complete reversal of an acquired Chiari malformation after surgical treatment, as documented by magnetic resonance imaging. Cases of asymptomatic acquired Chiari malformations are reported, including those resulting from shunting for intracranial hypertension without hydrocephalus. We conclude that a craniospinal pressure gradient creates a potential for cerebellar tonsil descent and recommend that ventriculoperitoneal shunting be performed in children with communicating hydrocephalus to avoid this potential complication. We also recommend annual surveillance of the cervicomedullary junction in children with lumboperitoneal shunting. Finally, if symptomatic tonsillar descent occurs from lumbar shunting, a trial conversion to ventriculoperitoneal shunting may eliminate the need for posterior fossa decompression.