Chiari Malformation Treatment in Delhi, India

Chiari malformation is a structural defect involving the cerebellum and the spinal cord. In this malformation, the brain tissue in the cerebellum grows and extends into the upper part of the spinal canal.

Associated Anatomy of Chiari Malformation

The cerebellum and upper parts of the spinal cord.

Alternate Names of Chiari Malformation

Alternate names include Arnold Chiari malformation, spina bifida, and hindbrain herniation.

Types of Chiari Malformation

Type 1

This is the most common type of Chiari malformation and involves only the lower part of the cerebellum.

Type 2

This type involves the cerebellum and brain stem tissues. It is often associated with a myelomeningocele congenital disability.

Type 3

This involves the extension of brain tissues in the spinal cord. It occurs due to an associated congenital disability in which the fluid accumulation in the brain causes its protuberance.

Type 4

This type is associated with the incomplete development of the cerebellum.

Chiari Malformation Causes

• Developmental defects are the most common cause of Chiari malformation.
• During fetal development, due to a lack of nutrients in the maternal diet, the space at the skull base that encloses the cerebellum becomes small. It creates increased pressure on the cerebellum and blocks the flow of cerebrospinal fluid.
• Some cases of Chiari malformations occur during the later stages of life. In these cases, the cerebrospinal fluid gets drained due to injury, infection, and toxicity.
• Genetic mutations can also cause developmental defects that cause Chiari malformation.

Chiari Malformation Symptoms

Type 1 Chiari malformation is associated with the following symptoms

• Headache
• Neck pain
• Deteriorated coordination of motor skills
• Tingling sensation in the hands and feet
• Dizziness
• Hoarseness of voice
• Ringing sensation in the ear
• Sleep apnea
• Weakness

Type 2 Chiari malformation is associated with

• Enlarged head
• Difficulty swallowing
• Difficulty breathing
• Impaired eye movement
• Weakness in the arms

Chiari Malformation Diagnosis

A physical examination can reveal an abnormality in balance, memory, reflexes, motor skills, and cognitive senses. A few imaging tests used to diagnose this malformation include Magnetic Resonance Imaging (MRI), which helps find the extent of the growth of the cerebellum and spinal cord impairing the brain's normal functioning. Computerized tomography provides a cross-sectional image of the brain tissue and helps identify blood vessels and brain tissue damage.

Chiari Malformation Treatment

Decompression surgery

In the case of symptomatic representation of Chiari malformation, decompression of the nervous tissue and restoration of the flow of cerebrospinal fluid are required.

Resection surgery

Type 2 Chiari malformation associated with symptoms requires surgical treatment. A few cases might require the resection of cerebellar tissue to relieve excessive pressure.

Spinal surgery

In children, spinal surgery helps reposition the spinal cord and relieve excess pressure from cerebral spinal fluid.

Risk Factors of Chiari Malformation

Risk factors associated with Chiari malformation include:

Myelomeningocele

where the opening in the spinal cord persists even at the time of birth and can cause spinal displacement.

Hydrocephalus

In hydrocephalus, excessive accumulation of cerebrospinal fluid in the brain causes undue pressure on posterior parts of the brain.

Syringomyelia

This involves a cyst in the spinal column that causes excessive pressure and leads to Chiari malformation later on.

Possible Complications of Chiari Malformation

Due to the continuous progression of Chiari malformation, some patients may experience complications such as the accumulation of excessive fluid in the brain. Chiari malformation may also hinder the development of the spinal cord covering. The progression of Chiari malformation can possibly cause the formation of cysts in the spinal cord. In these cases, cysts become attached to the spine, causing damage to the nerves and muscles.

Chiari Malformation Prevention

Primary Prevention of Chiari Malformation

• The intake of proper nutrients like folic acid during pregnancy can prevent this congenital malformation.
• Avoiding exposure to hazardous substances like alcohol and drugs also prevents the occurrence of Chiari malformation.
• Maintaining optimal vitamin B12 with the help of supplements before conception also helps prevent congenital disabilities like Chiari malformation.
• Dietary modifications with the introduction of folic acid and vitamin B12-rich sources also help prevent Chiari malformation.

Secondary Prevention of Chiari Malformation

• Most cases of Chiari malformation do not recur after surgery. In a few cases of Chiari malformation that has been treated with surgery, there is a recurrence of symptoms due to the regrowth of the bone.
• Creating CT scan-guided windows in the bone can accumulate brain tissue even after regrowth and prevent a recurrence.
• There is often a recurrence of Chiari Malformation symptoms that is associated with a cyst in the spinal cord, and this requires the repetitive decompression of the posterior fossa.

Epidemiology of Chiari Malformation

• Most cases of Chiari malformation are classified under Type 1 Chiari malformation.
• The occurrence rate of this malformation is 1 in 1,000 births.
• Females have a slightly higher occurrence rate compared to males.
• In most cases, the condition is present at birth.
• In some cases, it becomes predominant in later stages of life.

Expected Prognosis of Chiari Malformation

Surgical treatment causes a reduction in symptoms and provides stability for a longer period of time. In a few cases, more than one surgery may be performed due to the condition's progression.

Natural Progression of Chiari Malformation

Most cases of Chiari malformation might have worsening symptoms due to the blockage of cerebrospinal fluid and increased pressure. It can lead to CSF accumulation in the brain and may be fatal in severe cases.

Pathophysiology of Chiari Malformation

Type 1 Chiari malformation occurs due to the compression of the cerebellum, medulla, or upper spinal cord. Due to disordered anatomy, there may be a disruption in the flow of cerebrospinal fluid through the foramen magnum.

Type 2 Chiari malformation is associated with the compression of the posterior brain, the stretching of the cranial nerves, and disorientation, due to fluid accumulation in brain tissue and its expansion.