What is a Chiari malformation?

A Chiari malformation, previously referred to as an Arnold-Chiari malformation, is a condition in which the lower part of the brain extends down into the spinal canal.

It is due to a structural abnormality such as a smaller-than-expected size of the skull. In case your skull is not large enough, part of the brain, particularly your cerebellum, will grow downwards where there is spare space in an opening at the base of your skull known as the foramen magnum.

This part of the brain helps in your muscle movements, posture, balance, speech, and coordination. Its symptoms affect the workings of this part of the brain like loss of balance.

It grows to the extent that your brain starts pressing at the base of your skull. This growth anomaly also blocks cerebrospinal fluid flow. It cushions the spinal cord and the brain and also circulates nutrients and chemicals and removes the waste products.

What are the symptoms of a Chiari malformation?

Some common symptoms of Chiari malformations include:

• Changes in vision, such as double vision, fuzzy vision, irregular eye movements, or light sensitivity, issues with hearing, such as hearing loss or tinnitus, a ringing sound.
• Difficulty in speaking, eating, drinking, or swallowing;
• Drooling, vomiting, or gagging
• Scoliosis, or curvature of the spine
• Loss of bowel or bladder control
• Fainting
• Breathing problems while sleeping (sleep apnoea)

Each individual experiences symptoms variably, mildly to severely or not at all. Some children are born with symptoms. Other children develop symptoms in late childhood or adulthood. Also, the symptoms may change or worsen over time.

What causes a Chiari malformation?

It is unsure what specifically causes Chiari I abnormalities. Though it usually only discovered in adulthood when symptoms appear or an MRI is performed, it tends to be present from birth.

Most cases seem to be the result of part of the skull being less than adequate for the brain.

Other conditions that can cause Chiari I malformations include a tethered spinal cord, a build-up of fluid on the brain called hydrocephalus, and certain types of brain tumour.

Sometimes, Chiari malformations run in families. Some children who were born with it might have had issues with the development of their skulls because they received a defective gene.

But the chances of passing a Chiari malformation on to your children are very low. Additionally, keep in mind that even if your kids inherit it, they may never have symptoms.

What are the risk factors for a Chiari malformation?

Chiari malformation could be affect anybody. You may be more likely to have developed a Chiari malformation if at least one of your relatives in your biological family had the condition

What are the complications associated with Chiari malformation?

Chiari malformation may cause severe health issues and developmental delays. Some complications include:

• Hydrocephalus: This is a life-threatening condition in which your brain has excessive cerebrospinal fluid, or CSF. Hydrocephalus occurs due to the failure of CSF to drain from the brain. As CSF accumulates in the brain, it creates pressure within your skull. This could cause cognitive problems and a deformed head shape. It can be life-threatening if not treated. This condition is most frequently observed in Chiari malformation type II.
• Syringomyelia and hydromyelia: The fluid buildup, if not well circulated between the brain and spinal cord, would cause CSF to accumulate inside your spine, which might harm your spinal cord and present a range of other symptoms such as movement and coordination problems, pains, weakness, spasms of muscles, numbing sensation, decreased sensitivity towards hot and cold, and difficulties in controlling one's bladder or bowel.
• Tethered cord syndrome: Children who are born with myelomeningocele, a severe form of spina bifida, are more likely to grow up to have tethered cord syndrome. This occurs when scarring from closure surgery causes their spinal cord to attach to their spine. Slow and progressive nerve loss that affects the lower body and leg muscles can occur in people with tethered cord syndrome. Their bladder and bowel movements may also be impacted.

Chiari malformation symptoms can also impact your mood, particularly if you suffer from severe headaches or insomnia. Depression strikes some people. Consult a mental health expert or your doctor if this condition has an impact on your mood.

How is a Chiari malformation diagnosed?

Following a thorough physical examination, a medical professional will diagnose a Chiari malformation. Your mobility, balance, and hand and foot sensations will all be tested by your medical specialist. They will also check for developmental delays, learning difficulties, and memory issues in kids.

A doctor may prescribe imaging tests to obtain a thorough picture of your brain and spinal cord in order to confirm a diagnosis. Imaging testing could consist of:

• MRI-Magnetic Resonance Imaging: This imaging test will show the soft tissues and bones. Your doctor will see the brain, the spinal cord and cerebrospinal fluid through this. The degree to which the cerebellum spreads into the spinal canal can be determined using an MRI.
• Cine MRI: A cine MRI is comparable to an MRI. The doctor can see how the cerebrospinal fluid (CSF) flows around the base of your skull using this test.
• Computed tomography (CT): This test can assist in identifying issues with the spinal column and base of the skull bones.
• X-ray: An X-ray will look for any abnormalities in the bones in your neck and skull.

Prenatal ultrasounds can sometimes show Chiari abnormalities before to the baby's birth. An ultrasound is a type of imaging exam that creates images of soft tissues using sound waves.

If you do not have symptoms from a Chiari malformation, you will receive that diagnosis when you have tests to diagnose and rule out another unrelated condition with imaging tests.

How is Chiari malformation treated?

Based on how severe your symptoms are, your doctor will create a treatment plan. It is not necessary to receive treatment if you are symptom-free. Your physician will use routine MRIs to keep an eye on your health.

Treatment for mild symptoms such as a headache or neck pain may include:

• Pain medications or drugs to help you manage your symptoms.
• Massage therapy or physical therapy.
• Limit your physical activities, such as heavy lifting.
• Use of devices to help with vision or hearing problems, such as glasses or hearing aids.

Chiari malformation surgery

Severe Chiari malformation cases may require surgery. Surgical procedures may include:

• Craniectomy: Your surgeon will remove a portion of your skull, relieving pressure on your brain and helping fluid flow throughout your head.
• Posterior fossa decompression: The most popular method for treating a Chiari malformation is this Chiari malformation. Your surgeon removes a portion of the posterior fossa, the back part of the skull, to release pressure on your brain and give it more space.
• Laminectomy: To restore the flow of cerebrospinal fluid and make more space for your spinal cord, your surgeon may remove a small section of the bones covering your spinal column, depending on how severe the Chiari malformation is.
• Duraplasty: The dura, a membrane on the outside of your brain, is opened by the surgeon during this decompression procedure. To give your brain additional space, your surgeon will then sew in a patch to enlarge the membrane. By enlarging the region, this surgery relieves pressure on your brain.
• Electrocautery: Increasing the space for draining cerebrospinal fluid, surgeons can shrink a very small portion of your brain known as the cerebellar tonsils by applying a low amount of electricity. The cerebellar tonsils can be drawn in without any damage to the brain or nerves.
• Placement of a shunt: When diagnosed with hydrocephalus, the surgeon inserts a shunt or tube inside your skull for siphoning away the excessive amount of CSF. Fluid moves out of the skull and your brain through it and settles down in other parts of the body.

Does treatment have some side effects?

Before you start treatment, your physician will go over the side effects so you may make the best decision for your health. There are dangers associated with any procedure. Because the incision and treatment will be close to your brain, surgery around your brain and skull has a high risk. As you become older, you may need to have more procedures.

How soon after treatment will I feel better?

Depending on what treatment you and your healthcare provider choose, you may start feeling relieved of mild symptoms within several days of taking pain medication or attending therapy sessions. It may take weeks to months before you fully recover from the surgery. Your symptoms may disappear or decrease dramatically after undergoing surgery.

Can a Chiari malformation be prevented?

There is no known way to prevent a Chiari malformation.

Talk to your physician about how to keep up healthy behaviours if you intend to get pregnant. To lower the chance of a child being born with spina bifida, your doctor might advise you to take vitamins like folic acid on a regular basis. To determine your chance of having a kid with a genetic condition, your doctor may advise genetic testing.

What is the prognosis for a Chiari malformation?

Your prognosis depends on the severity and type of Chiari malformation. You will need to stay close to your healthcare provider and care team as they monitor your symptoms and devise the right course of treatment for you.

What is the life expectancy of a person with a Chiari malformation?

If you do not have symptoms or you have mild symptoms after being diagnosed with a Chiari malformation, you will most likely have a normal life expectancy. Severe symptoms and some types of Chiari malformation can be fatal. Discuss your expectations with your medical provider.

When should I see a healthcare provider?

A patient should be brought to the doctor if their symptoms worsen or if they have a suspected Chiari malformation. In case the child has this malformation and they are late to developmental stages, seek help from their care provider. Visit your healthcare practitioner throughout the postoperative period if you experience any infection-related symptoms, such as worsening pain, oedema, or an unhealing wound.

What questions should I ask my doctor?

• What kind of Chiari malformation am I suffering from?
• What kind of care would you suggest?
• How frequently should I get imaging tests to keep an eye on my condition?
• Would you advise surgery?
• Does the treatment have any side effects?
• Will this disease affect my future children as well?

Why Tender Palm Super-Speciality Hospital for Chiari malformation?

Tender Palm Hospital, owned by doctors, is renowned for attracting the most experienced professional in the country, with the finest neurologist and neurosurgeons specialized in Chiari malformation. Tender Palm stands out as the premier Neurology hospital in Lucknow, India. Boasting cutting-edge infrastructure and advanced technology, Tender Palm ensures top-notch medical care for its patients.

To seek an expert consultation for any Neurology condition

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Email at care@tenderpalm.com

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