The Chiari II Malformation
Definition
Definition: Herniation of the vermis, medulla, and fourth ventricle into the spinal canal. Occurs in >90% of patients with myelomeningocele (spina bifida) and is exclusive to this population.
Signs and Symptoms
Symptoms of Chiari II include dysfunction of CN 9 and 10 leading to dysphagia and repiratory distress. May present as a neurosurgical emergency in up to 20%. A child with Chiari II and any respiratory abnormality requires close observation. These symptoms appear from age 2 or 3 months up to 2 years).6 It is unusual for newborns to be symptomatic. Older children usually present with a spinal cord syndrome and/or syringomyelia, similar to Chiari I patients.3,4,7
Diagnostic Studies
MRI shows descent into the upper cervical spine of the vermis, medulla and fourth ventricle, along with multiple other associated anomalies.2,5
Treatment
The complex anatomy and significant risk of neurological injury and deterioration necessitate a referral to a pediatric neurosurgeon. Most of these patients have hydrocephalus and are usually shunted shortly after birth. Shunt malfunction can mimic Chiari II symptoms!!! Prior to decompression, the shunt must be shown to be functional. Shunt taps and normal or small ventricular appearance on radiographs should not be used as proof of shunt function in these patients. Instead, surgical shunt exploration is preferred.1“It is the shunt until proven otherwise.” The bony decompression focuses on the hindbrain herniation and generally need not involve the posterior fossa or foramen magnum. Cervical laminectomy to the level of the lowest hindbrain tissue is sufficient. The dura is opened and dense arachnoid adhesions are dissected using the operative microscope. The procedure is generally considered over when the avascular floor of the fourth ventricle can be easily visualized. This often requires microsurgical dissection of the vermis off the fourth ventricular floor, and possibly placing a stent between the 4th ventricle and cervical subarachnoid space.
Prognosis and Outcomes
The key to good prognosis is early recognition of symptoms and early treatment. Patients with less severe symptoms at diagnosis are much more likely to improve with surgical intervention. Previously, results of surgery for Chiari II were less than satisfactory. However, these results were reported prior to the recognition of shunt malfunction without ventricular dilatation. Most recent series report better outcomes.
References
1. Iskandar B, Oakes W. The Chiari Malformations, in Albright A, Pollack I, Adelson P (eds): Principles and Practice of Pediatric Neurosurgery, ed 1st. New York, NY: Thieme Medical Publishers, 1999.
2. Naidich TP, Pudlowski RM, Naidich JB, Gornish M, Rodriguez FJ. Computed tomographic signs of the Chiari II malformation. Part I: Skull and dural partitions. Radiology 1980;134:65-71.
3. Oakes WJ. Chiari Malformations, Hydromyelia, and Syringomyelia, in Wilkins R, Rengachary S (eds): Neurosurgery, ed 2nd. New York: McGraw-Hill, 1996, pp 3593-3616.
4. Oakes WJ. The Chiari Malformations of the Child, in Menezes AH, Sonntag V (eds): Principles of Spinal Surgery, ed 1st. New York, NY: McGraw-Hill, 1996, pp 379-394.
5. Schmitt HP. "Inverse Chiari type II syndrome" in untreated hydrocephalus and its relationship to typical Arnold-Chiari syndrome. Brain Dev 1981;3:271-275.
6. Wealthall SR, Whittaker GE, Greenwood N. The relationship of apnoea and stridor in spina bifida to other unexplained infant deaths. Dev Med Child Neurol 1974;16:107-116.
7. Worley G, Erwin CW, Schuster JM, Park Y, Boyko OB, Griebel ML, et al. BAEPs in infants with myelomeningocele and later development of Chiari II malformation-related brainstem dysfunction. Dev Med Child Neurol 1994;36:707-715.
