Chiari II Malformation

Chiari II malformation is present in the majority of children with meningomyelocele. Chiari II malformation consists of malformation of the cerebellum, hindbrain, and brainstem. Aqueductal stenosis is commonly associated. Symptomatic Chiari malformation is characterized by apnea, vocal cord paralysis, stridor, oral motor dysfunction, visual dysfunction, and upper limb weakness and incoordination in the infant. In older children it presents with visual dysfunction, motor incoordination, headache, and hand weakness.11 Even mild cervical hyperflexion-extension injuries may result in symptoms. Of greatest concern is the young child or infant who presents to the ED with stridor and meningomyelocele. Suspicion of a Chiari malformation should be present, and evaluation should be ordered on an expedited basis. Stridor may be associated with vocal cord paralysis and may proceed to complete airway obstruction in a small number of patients. Evaluation is by MRI of the craniocervical junction. For children with severe respiratory compromise, immediate hospitalization is required. If respiratory tract function is not severely impaired and the process is static by history, a case-by-case decision should be made regarding hospitalization based on availability of prompt outpatient services from neurosurgery, otorhinolaryngology, and radiology for MRI. When significant respiratory compromise is associated with Chiari II malformation, tracheostomy may be required. Tracheostomy should be reserved for patients in whom intubation is not possible.12

Individuals with myelomeningocele and Chiari II malformation may have instability of the craniocervical junction. If the Chiari malformation has been symptomatic, it may have been treated with decompression surgery. The surgery may involve removal of a posterior segment of the cranium at the foramen magnun and/or laminectomy of the cervical vertebrae. Fusion of the cervical vertebrae may be done to improve cervical stability. If the child is in a motor vehicle accident, there should be a high index of suspicion for upper cervical spine injury. Routine precautions should be instituted, and complete evaluation, including flexion and extension films of the neck, should be done if indicated.

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