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If symptoms are mild, it may be possible to sufficiently treat them with anti-inflammatory and headache medications. In such cases, children are closely tracked so that doctors can quickly intervene should their condition worsen. Even children with mild or asymptomatic cases of CM1 are vulnerable to the compression of brain tissue between the spinal column and the skull, and so they should avoid chiropractic therapy, roller coasters, trampolines and other activities that could result in whiplash. We follow many more children than we treat surgically since the natural history of a child with CM1 is still unknown and CM1 is much more common than previously thought.
Cases in which symptoms are intensifying or are already disabling do require surgery to fix and halt the progress of the structural problems underlying the disorder.
Surgery for CM1 is called posterior fossa decompression surgery, or sometimes just Chiari decompression. It begins with the removal of a portion of the skull bone (craniectomy) on the lower part of the back of the skull to take pressure off the herniated cerebellum and restore the unimpeded flow of CSF. The surgeon then removes a part of the arched, bony roof of the spinal canal, called C1, to make more room and relieve pressure on the spinal cord. Sometimes the surgeon will then round out the cerebellar tonsils to further improve CSF flow. Finally, using a microscope, the surgeon sews a flexible, durable patch over the area to protect the underlying tissue while allowing room for the cerebellar tonsils and aiding the free flow of CSF. The surgery typically takes between two and three hours.
Most children who have Chiari decompression surgery experience improvement in their symptoms within a week or two of the surgery. While most children only need one surgery to correct their CM1, some children require subsequent surgical procedures to treat their CM1. In very young children, for example, removed bone can grow back and compress the cerebellum again, or the lower portions of the cerebellum may again fall below the foramen magnum and impede the flow of CSF. Syrinx may also reform, necessitating surgical treatment with a repeat Chiari decompression or the installation of a shunt into the syrinx. The goal of surgery is to maximize the quality of life for the child so he or she can be a kid again.
In some cases, impeded CSF flow can cause hydrocephalus, which must be treated with the surgical placement of a shunt or, alternatively, an endoscopic third ventriculostomy to divert the CSF underneath the brain.
After Chiari decompression surgery, elevated CSF pressures can worsen and result in benign intracranial hypertension or pseudotumor cerebri, causing symptoms (most notably a frontal headache) that are sometimes mistaken as indication of a tumor. Often, this condition can be simply treated with medication that decreases the production of CSF, but a spinal tap and eye exam may be required for diagnosis. Some more extreme cases may require surgical introduction of a shunt that redirects excess CSF out of the brain.
The time it takes to fully recover from Chiari decompression varies from child to child, but most patients will typically spend three to four days in the hospital and two to three weeks at home before returning to school. Patients may experience some intermittent frontal headaches and post-surgical neck pain during recovery, but these usually pass. Most of the children we treat surgically can expect to return to all normal activities and can enjoy just being kids again.
Lucile Packard Children’s Hospital Stanford in Palo Alto, is a national destination center for Chiari malformations. Patients and their families travel here for the best available diagnosis, treatment and follow-up care. We are constantly working to improve our patients’ experience and quality of life.