disease | Chiari Malformation |
alias | Arnold-Chiari Malformation |
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bubble_chart Overview Cerebellar tonsillar herniation malformation refers to the herniation of the cerebellar tonsils into the spinal canal, sometimes accompanied by elongation and downward displacement of the medulla oblongata and the fourth ventricle, leading to a series of symptoms; also known as Arnold-Chiari malformation. Clinically, it is further divided into three types:
- mild type, where only the cerebellar tonsils herniate into the spinal canal.
- severe type, where the cerebellar tonsils herniate into the spinal canal, accompanied by elongation and downward displacement of the pons, medulla oblongata, and fourth ventricle.
- most severe type, which, on the basis of the severe type, is accompanied by lumbar spina bifida and meningocele, and results in obstructive hydrocephalus.
The main clinical manifestations include neurological damage symptoms and symptoms of increased intracranial pressure.
The incidence of this disease shows no regional or gender differences, and the condition progresses slowly, with neurological damage symptoms typically appearing only in adolescence. The primary treatment for this condition is surgical decompression, and the prognosis is generally good. However, the earlier the symptoms appear (e.g., during infancy or early childhood), the worse the prognosis.
bubble_chart Clinical Manifestations
- Medullary and upper cervical cord compression manifestations: hemiplegia, quadriplegia, sensory disturbances, sphincter dysfunction, and dyspnea.
- Cranial nerve and cervical nerve involvement symptoms: facial numbness, diplopia, tinnitus, hearing loss, hoarseness, dysphagia, and occipital neck pain.
- Cerebellar symptoms: unsteady gait, ataxia, and ocular tremor.
- Signs of increased intracranial pressure: headache, vomiting, and optic disc edema.
bubble_chart Diagnosis
- Symptoms of posterior cranial nerve, medulla oblongata, or cervical spinal cord involvement: paralysis, sensory disturbances, dysphagia, and occipital pain, among others.
- Signs of intracranial hypertension and cerebellar symptoms: headache, vomiting, optic disc edema, ataxia, and gait instability, among others.
- Cranial and cervical computed tomography and magnetic resonance imaging show cerebellar tonsillar herniation into the spinal canal or accompanied by elongation and downward displacement of the medulla oblongata and fourth ventricle.
bubble_chart Treatment Measures
- Surgical treatment: Surgery is required for patients with obvious neurological damage symptoms. If complicated by hydrocephalus, a cerebrospinal fluid shunt should be performed.
- Prevent infection and provide symptomatic treatment.
bubble_chart Cure Criteria
- Cured: Neurological function is basically restored, able to live independently, and intracranial pressure is normal.
- Improved: Neurological symptoms have improved, able to live independently or still requiring care.
- Not cured: Neurological symptoms show no improvement, unable to live independently.
bubble_chart Prevention
This condition involves the elongation and flattening of the cerebellar tonsils, which herniate through the foramen magnum into the spinal canal, often accompanied by downward displacement of the medulla oblongata and the fourth ventricle. This can lead to symptoms due to compression of the medulla oblongata, cranial nerves, brainstem, and upper cervical spinal cord. It is frequently associated with basilar invagination and spina bifida. Avoid trauma and excessive neck extension to prevent symptom exacerbation or respiratory arrest caused by medullary compression. Patients with significant symptoms should seek consultation from a specialist in neurosurgery at a major hospital for early diagnosis and surgical intervention. Postoperatively, intensive neurofunctional rehabilitation is recommended, including hyperbaric oxygen therapy and acupuncture if available, to promote neurological recovery.