Chiari Malformation is a neurological condition in which the bone at the back of the skull, known as the cerebellar tonsils, extends below the normal position. As a result, there is crowding in the region of the brain called the cerebellum and the brainstem. This crowding can lead to a blockage in the flow of cerebrospinal fluid (CSF), which connects the brain and the spinal cord. Additionally, this crowding can cause symptoms such as swallowing difficulties, instability, and sensory and motor changes in the limbs.
Is it dangerous?
As mentioned, the descent of the cerebellar tonsils into the spinal canal can result in symptoms such as weakness in the hands and legs, weakness, and even imbalance. The accumulation of fluid can lead to hydrocephalus and the enlargement of brain ventricles.
Many patients may not experience any symptoms, and the malformation is detected only during routine examinations. Consequently, in many children, the condition is not diagnosed until symptoms appear, causing disruptions in their quality of life and functional decline.
The impact of the malformation usually becomes more evident during adolescence, and in these cases, it is characterized by headaches, neck pain, tremors, numbness in the hands, and sleep disturbances (breathing pauses during sleep). Often, the symptoms are attributed to psychological disturbances of adolescence, and the underlying cause of the symptoms remains undiagnosed.
The specific symptoms can vary significantly from person to person and depend on the severity of the malformation. In general, patients may complain of neck and head pain aggravated during physical activities and coughing. Other possible symptoms include tremors, unsteadiness while walking, double vision, swallowing difficulties, and breathing pauses during sleep.
It is essential to emphasize that not every patient with a descent of the cerebellar tonsils will require Chiari surgery. Some cases can be monitored, but it is crucial to seek evaluation from a specialized neurosurgeon experienced in such surgeries and with a substantial amount of experience with these cases.
The following symptoms may characterize Chiari Malformation:
1. Headaches radiating to the neck and aggravated by physical activities and coughing.
2. Swallowing difficulties and choking.
3. Breathing pauses during sleep.
4. Weakness in the limbs.
5. Balance disturbances.
Before
After
The goal of the surgery is to relieve the crowding in the area of the hindbrain, create space for the cerebellum and tonsils, release intracranial pressure, and alleviate troublesome symptoms. During the procedure, called posterior fossa decompression, the narrow space between the head and the neck is enlarged, and small bone windows are opened both in the skull and the first cervical vertebra. Additionally, the dura, which encases the cerebellum and the spinal cord, is expanded.
For Chiari Malformation corrective surgery, hospitalization for four to six days may be required, and the recovery and return to regular activities usually take a few weeks. However, during the first two months after the surgery, physical activity is limited to allow the tissues in the surgical area to heal. In most cases, patients can expect a full and normal life after the surgery.