Chiari III malformation treated with CSF diversion and delayed surgical closure

Chiari III malformations are extremely rare hindbrain malformations that ar e associated with a high early mortality rate, or severe neurologic deficit s in the survivors. The preferred treatment is early operative closure and CSF shunting. We report a case of a newborn infant with a Chiari III malfor mation with displacement of the brainstem and cerebellum into the cervical encephalocele which precluded immediate operative closure of the defect. In stead, a ventriculoperitoneal shunt was placed and the patient was followed with serial imaging studies. The child survived. The shunt allowed the bra instem and cerebellum to regress into the cervical spinal canal as the dila ted cerebral aqueduct and fourth ventricle decompressed. A delayed closure of the cervical encephalocele was performed at 30 months of age. Cerebrospi nal fluid diversion with delayed closure may be an option for large lesions .