Chiari Malformation Surgery

What is Chiari malformation surgery?

Chiari decompression surgery is a surgical procedure that is administered to remove the back of the skull and spine.

The floor of your skull has a cavity known as the cranial fossa. This cavity encompasses the cerebellum and brainstem.

The cavity may condense because of particular situations, including a brain tumor, or a traumatic injury of Chiari malformation.

Chiari malformation is a hereditary condition whereby a section of your cerebellum moves downwards onto the upper section of the spinal column, putting pressure on the spinal cord and brain.

Chiari malformation surgery seeks to relieve compression on your spinal cord and brain.

It also promotes the normal flow of the cerebrospinal fluid.

Why do you need posterior fossa decompression?

If you have the following symptoms, you may be a candidate for Chiari malformation surgery:

• In case the cerebrospinal fluid collects unusually in the spinal cord

• If a cine MRI shows an obstructed flow of the cerebrospinal fluid and this leads to worsening or severe symptoms

What are the symptoms of Chiari malformation?

Not all people with Chiari malformation manifest in any symptoms. Your doctor will order an MRI scan of your brain to confirm the diagnosis.

When a Chiari malformation presents with symptoms, they include:

• Neck pain

• Muscle weakness

• Double or blurry vision, light sensitivity, etc.

• Hearing loss

• Headaches exacerbated by sneezing, bending over, or coughing

• Problems with swallowing

• Insomnia

• Clinical depression

Some people with Chiari malformation also encounter problems while walking or using their hands. Others find it hard to control the bowel or bladder.

After a Chiari malformation diagnosis make sure to consult your healthcare provider.

You will be advised on the right course of action to take in seeking treatment for your condition.

In case the symptoms exacerbate, or you develop symptoms.

What are the types of Chiari malformation?

Chiari malformation falls under four categories:

Type 1

This type of Chiari malformation occurs in infancy. It happens when the cerebellar tonsils of the brain are displaced downward into the higher section of the spinal canal.

When this happens, there is an interruption in the flow of cerebrospinal fluid.

Symptoms manifest in late adolescence or adulthood.

Chiari 1 malformation may present with the following symptoms:

• Weakness in the arms and hands

• Symptoms of severe pain in the head and neck

• Dizziness

• Sleeping problems

• Choking/swallowing difficulties

• Balance problems

Type II Chiari malformation

This type of Chiari malformation happens when the brainstem and cerebellar tonsils

exert pressure at the base of your skull.

The pressure extends into the upper spinal canal.

Like Type, I Chiari, type II is also prevalent at birth.

The symptoms of Type II Chiari include:

• Variation in breathing pattern

• Rapid, downward, and involuntary movement of the eyes

• Weakness in the arm

Chiari II and IV malformations

This is the rarest form of Chiari malformation. In this case, the cerebellum is unable to develop normally and can be life-threatening.

Chiari III and Chiari IV are characterized by the following symptoms:

• Bladder and bowel dysfunction

• Chronic pain

• Spasticity and muscle weakness

• Headaches

• Motor impairment

How is Chiari’s malformation diagnosed?

It is very hard to diagnose Chiari malformation because the symptoms are not always evident.

Doctors rely on a magnetic resonance imaging (MRI) test to make an accurate diagnosis.

Physicians can also use MRI images to check if a child has a tethered spinal cord or a syrinx. A syrinx refers to a  cavity that develops in the spinal cord and is usually full of fluid.

The medical team will have to conduct follow-up MRIs to establish if the Chiarim malformation exacerbates or changes over time.

After the medical team makes a diagnosis of Chiari malformation, they still need to conduct additional tests. These are useful in assessing the patient’s condition. Some of the additional tests that your medical team might order include:

• Ultrasound scan – The aim of an ultrasound scan is to assess the movement of the cerebellar tonsils as the medical procedure is being administered
• Computed tomography (CT) scan – A CT scan reveals checks for blockage in your spinal canal
• Sleep study – A sleep study is helpful because it reveals the medical team can check the patient’s breathing patterns. It is also possible to establish they are getting sufficient oxygen during sleep. A sleep study is beneficial to children who have a snoring problem or noisy sleeping.
• Evoked potentials – These are a set of electrical tests that give information on the functionality of the brainstem. The tests also further determine the functionality of the spinal cord, peripheral nerves, and brain function.

Treating Chiari malformation

Treatment for Chiari malformation differs from one person to another. It is determined by the severity and symptoms being manifested.

Your primary healthcare provider will talk to you about the severity of symptoms and the treatment options available to you.

Chiari malformation does not affect your day-to-day activities. Some people will only require periodic monitoring by doctors after diagnostic imaging. For other patients, a physician will prescribe medication to relieve pain and headache.

Preparing for treatment

After the MRI scan results, your general physician will refer you to a neurologist who specializes in conditions of the brain and the nervous systems.

An appointment with a neurologist can be very brief. Make sure you are prepared to gather as much information as possible.

Preparation for your initial appointment with your neurologist:

• Note down all the symptoms you have presented with
• Your key personal information such as recent changes in life and major stress should also, be noted
• If you are being treated for other medical conditions, make sure your neurologist knows of this
• You should tag along a family member or friend for the appointment. They may recall certain information that you cannot remember
• If you have questions for your neurologist, make sure they are in writing

Surgery

In case medication cannot relieve the symptoms or stop the damage caused by Chiari malformation from advancing to the central nervous system, surgery is the only option available to you.

Chiari malformation surgery helps to stabilize and improve symptoms. You may require several surgical procedures to treat Chiari malformation.

Posterior fossa decompression is the most common surgical procedure to treat Chiari malformation.

The surgery is conducted under general anesthesia.

The surgical team will shave a section of your scalp in readiness for the procedure.

This is the portion near the base of your skull.

The team will also secure your head to avoid movement.

The neurosurgeon will make an incision along the back of the head to reach the upper neck.

The surgeon will gently peel back the neck muscles lying near the first vertebrae and skull.

The surgeon removes a small part of the bone at the base of your skull. This is done at a high level of precision with the help of a high-speed cutting burr.

The ensuing procedure is called a suboccipital craniotomy, and it aims to create additional space for your spinal cord and brain.

If the surgeon determines there is a need to offer extra pressure relief to the spinal canal and brain, he or she will administer a cervical laminectomy. This is a procedure that removes the vertebrae’s bony roof.

The cerebellar tonsils may also be removed in a procedure called electrocautery.

This frees up additional space for the brain and spinal canal.

In case the child or infant has a defect of the spinal cord or backbone, a condition referred to as a myelomeningocele, they might need a surgical procedure to help reposition the spinal cord. The surgery also helps to close the opening at the back.

This surgical procedure is more effective if it is conducted prenatally as opposed to post-natal. It restores the brain stem and cerebellum to their normal positions.

In case of abnormal accumulation of cerebrospinal fluids in the brain cavities, a condition also known as hydrocephalus, your surgeon can treat this by inserting a shunt system to aid in the drainage of such extra fluid. The shunt system eases pressure inside your skull.

The shunt is inserted surgically inside the head and the extra fluid gets drained into the abdomen or chest cavity. This allows for ease of absorption by your body.

The neurosurgeon will suture together the skin and strong neck muscles. Thereafter, he will place a dressing over the incision.

After surgery

After Chiari decompression surgery, you will be wheeled by the surgical nurse to the recovery area. When you wake up, your throat will feel sore. This is because of the tube inserted by the medical team to assist with breathing during the procedure.

The surgical team will constantly monitor your heart rate, blood pressure, and breathing.

In case you have a headache or feel nausea after Chair malformation surgery, you will receive medication for that.

Once the neurosurgeon has ascertained that your condition is stable, you will be allowed to go home. This often takes about 2 days.

Make sure there is someone at home who can monitor your progress and assist with your daily chores for at least the next 1-2 days.

Your surgeon will give you instructions that you need to follow closely while you recover at home. These instructions relate to:

Incision Care

When you shower, use water and soap only on the site of the incision. Avoid scrubbing the area.

Avoid soaking the incision in a bathtub or bath.

Avoid applying any ointment or lotion on the site of incision.

In case of fluid accumulation around the incision, notify your surgeon immediately. It could be a leakage of cerebrospinal fluid.

Medication

You are likely to experience headaches after Chiari malformation surgery.

Acetaminophen can help to resolve the headaches.

Follow your surgeon’s guidelines on pain medication. Reduce the frequency and amount as the pain reduces.

It is recommended that you eat food rich in fiber and drink plenty of water to avoid narcotics-induced constipation.

Some people manifest balance problems and drowsiness as side effects of Chiari malformation surgery. Your doctor will prescribe anti-seizure medication.

Activities

Avoid engaging in activities that are likely to exert pressure on your head. These include straining, bending over, and prolonged coughing.

Avoid lifting heavy objects.

Do not engage in strenuous housework.

Applying ice packs around your shoulder and neck for 20 minutes helps to relieve muscle spasms and pain.

Call your doctor immediately, if you notice the following symptoms:

• In case the incision shows signs of infection, such as colored drainage or spreading

• Redness

• Worsening speech and vision

• Fever above 101.5º even after taking Tylenol

• Seizure

• Accumulation of fluid beneath the skin around the site of incision

Recovery

Recovery after Chiari malformation surgery takes between 4 and 6 weeks. This is primarily determined by your overall health.

Your neurosurgeon will schedule follow-up appointments about 2 weeks after Chiari malformation surgery. The purpose of such an appointment is to monitor your recovery and to remove the sutures.

Most people resume work after 4 to 6 weeks following surgery.

After 6 months to 1 year, your surgeon will schedule a follow-up cine MRI to ascertain surgery was successful and that the Chiari malformation has been resolved.

Benefits of Chiari malformation surgery

Chiari malformation surgery relieves symptoms brought about by the condition, including neck pain, headaches, memory issues, dizziness, and problems with vision.

Chiari malformation surgery avoids further damage by interfering with the disease progression.

Risks/complications

Like all other surgeries, Chiari malformation surgery comes with certain risks. These include:

• Infection

• Bleeding

• Temporary or permanent nerve damage

• Stroke

• Paralysis

• Cerebrospinal fluid leakage

• Blood clots

Cost of Chiari malformation surgery in India

Chiari malformation treatment in India starts from Rs. 293040 to Rs. 390720. There are highly qualified neurosurgeons in India who specialize in Chiari malformation treatment.

The cost payable for this treatment will be dictated by the following:

• Duration of stay in hospital

• Level of expertise of your neurosurgeon

• Cost of diagnostic tests

• Follow-up appointments

For international patients, it is important to liaise with your healthcare provider in India to find out if they offer visa processing support and transport and accommodation.

References

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