Chiari I Malformation

When part of the lower brain sits lower than usual

Important. This is general educational information. Your child's situation is unique. Always talk to your child's treating doctor for advice that fits your child specifically.

If an MRI has shown that your child has a Chiari I malformation, or if a doctor has used that term in a clinic, this page explains what it means in plain language. The most important point upfront, many children with this finding have no symptoms at all and need no treatment. This page will help you understand when Chiari I matters, what to look out for, and what the surgery involves in the children who do need it. Your child's neurosurgeon will discuss what applies specifically to your child.

What is a Chiari I malformation?

At the very base of the skull, there is a large opening called the foramen magnum, this is where the brain meets the spinal cord. Just above this opening sits the cerebellum, the part of the brain that handles balance and coordination. The lowest tips of the cerebellum are called the cerebellar tonsils.

In a Chiari I malformation, the cerebellar tonsils sit lower than they should, and they extend a short distance down through the foramen magnum, into the top of the spinal canal. One way to think about it is that the back of the skull is a little smaller than the brain that has to fit inside it.

Chiari I is very different from Chiari II, which is a more complex malformation that occurs with spina bifida (myelomeningocele) and is present from birth. This page covers only the Chiari I form, the more common and milder one, often found in otherwise healthy children.

Many children with Chiari I are diagnosed by chance, when an MRI is performed for something else entirely, for example a headache after a minor head bump, a routine evaluation, or another concern. In these children, the Chiari is often "incidental", meaning that it is real but is not actually causing any problems and may never cause any. Other children do have symptoms that come from the Chiari, and for them surgery can help.

In some children, Chiari I is found alongside a separate finding called syringomyelia, a small fluid-filled cavity inside the spinal cord. When a syrinx is present, it can affect the symptoms and the treatment decisions. Your child's neurosurgeon will look carefully for this on the MRI.

Signs you might notice

Most children with Chiari I have no symptoms at all. When symptoms do occur, they often follow a recognisable pattern, but many of these symptoms can also have completely unrelated causes. The classic Chiari headache is one of the most specific signs to look for.

In babies

  • Feeding difficulty, in particular gagging, choking, or refusal to feed
  • A weak, hoarse, or otherwise unusual cry
  • Episodes of breath holding, noisy breathing, or pauses in breathing
  • Repeated unexplained vomiting
  • Poor head control or an unusual head posture
  • Slow weight gain

In older children

  • A headache at the back of the head that is worse with coughing, sneezing, laughing, straining, or lifting, this pattern is the most typical Chiari headache
  • Neck pain, often localised to the back of the neck
  • Difficulty with balance, walking, or fine hand movements
  • Numbness, tingling, or weakness in the arms or hands
  • Difficulty swallowing, a hoarse voice, or coughing while eating or drinking
  • Snoring, restless sleep, or signs of sleep apnoea
  • A new curvature of the spine (scoliosis), particularly in a younger child, which can sometimes point to a syrinx
  • Episodes of dizziness or brief visual loss when standing up or straining

How is it diagnosed?

Chiari I is diagnosed on an MRI of the brain. The MRI shows the position of the cerebellar tonsils very clearly. There is no single number that decides whether a Chiari is important, your child's neurosurgeon will assess how low the tonsils sit, the shape they take, and most importantly whether they are interfering with the flow of cerebrospinal fluid (CSF) around the brain.

If a Chiari is found, the team will usually also request an MRI of the spine to look for syringomyelia. A specific type of scan called a cine MRI may be performed, this is still an MRI but it produces short "movies" of the CSF as it flows around the foramen magnum. It helps the neurosurgeon to decide whether the Chiari is obstructing flow.

Other tests can be useful depending on the symptoms. A sleep study can check for sleep apnoea. A swallow study or an ENT (ear, nose, throat) examination can evaluate feeding and airway symptoms in babies. A neurological examination is always part of the assessment.

An important part of the assessment is deciding whether the symptoms your child has are genuinely being caused by the Chiari, or whether they could be from a separate problem, many children with Chiari I also have ordinary childhood headaches, for example. This is why the diagnosis is made by combining the MRI with a careful clinical assessment, and not by relying on the MRI alone.

How is it treated?

Treatment depends entirely on whether your child has symptoms from the Chiari, whether there is a syrinx, and how the symptoms are affecting daily life. The two main pathways are careful observation and surgery, and one is not automatically better than the other. The right choice for your child is a decision that you and the neurosurgeon make together.

Watch and follow up (observation)

For most children whose Chiari was found by chance and who have no symptoms, no surgery is needed. The neurosurgeon will recommend follow-up, usually a clinical review and sometimes a repeat MRI after a year, to make sure nothing changes. This is the most common pathway. It is not "doing nothing", it is the right treatment when surgery would carry more risk than benefit.

Posterior fossa decompression surgery

When a child has clear symptoms from the Chiari, or when a syrinx is present and growing, surgery is offered. The operation is termed posterior fossa decompression. The neurosurgeon makes a small opening in the back of the skull and removes a small piece of bone (and sometimes the back arch of the top vertebra) to give the cerebellar tonsils more room. This relieves the crowding at the foramen magnum and allows the CSF to flow normally again.

Bone-only versus dura-opening decompression

There are two versions of the operation. In a "bone-only" decompression, only the bone is removed and the protective covering of the brain (the dura) is left intact. In a more extensive version, the dura is opened and made larger with a patch (a duraplasty). The bone-only version is less invasive, with a quicker recovery and a lower risk of CSF-related complications. The dura-opening version is more thorough and is often preferred when a syrinx is present, but it has a slightly higher rate of CSF leak and meningitis. Your neurosurgeon will discuss which approach is right for your child and why.

Treating syringomyelia

When a syrinx is present, the decompression surgery is usually the first treatment, because relieving the blockage at the foramen magnum often allows the syrinx to shrink over the following months. A separate operation directly on the syrinx is rarely needed in children, and only in particular situations.

The decision to operate is not always straightforward. If your child has mild or vague symptoms and a small Chiari, the neurosurgeon may suggest a period of watching, or a second opinion. If symptoms are clear and life-affecting, surgery has good outcomes, particularly for the classic cough/strain headache. Ask the team to explain their thinking for your child's specific situation.

What can we expect?

If your child is being observed without surgery, the rhythm is usually a clinic appointment once or twice a year, and a repeat MRI at intervals set by the neurosurgeon. Most observed children continue to do well and never need an operation.

If your child does have surgery, the operation itself usually takes two to three hours. Most children spend the first night in the paediatric intensive care unit (PICU) and another two to three nights on a regular ward. The total hospital stay is typically three to five days.

It is normal to have some neck stiffness and discomfort for one to two weeks after the operation. Pain control is straightforward with regular medications. Most children return to school in two to four weeks, and to full activity (including sport) in about six weeks, once the neurosurgeon confirms that healing is complete.

The headache caused by Chiari is the symptom that improves most reliably after a successful decompression, many children notice the difference within the first few weeks. Other symptoms such as numbness, balance problems, or syrinx-related findings tend to improve more slowly, over months. Symptoms that are not actually caused by the Chiari will not, of course, improve with the operation, this is why careful patient selection is so important.

Follow-up after surgery usually includes a clinic visit a few weeks later, and a repeat MRI several months later to confirm that the decompression has done its job, and, if a syrinx was present, that it has begun to shrink. In the long term, most children with Chiari I do very well, whether they have had surgery or not.

Questions you might ask your child's doctor

  • How far down do the cerebellar tonsils sit in our child, and is the CSF flow obstructed?
  • Is there a syrinx? If so, where is it and how big is it?
  • Do you think the symptoms our child is having are actually caused by the Chiari, or could they be from something else?
  • Do you recommend observation or surgery, and why?
  • If surgery is recommended, do you recommend bone-only decompression or opening the dura, and what is the reason for that choice?
  • What are the specific risks of the operation for our child, including CSF leak, infection, and the chance of needing a second operation?
  • What activities should we avoid before and after the surgery?
  • What is the follow-up plan, and how will you decide whether the surgery has worked?
  • What signs at home should make us call you urgently?
  • Is Chiari I something that runs in families, should any other family members have an MRI?

When to call your child's doctor right away

After surgery for Chiari I, most recovery is smooth, but some signs need to be checked right away. Do not wait, contact the neurosurgery team (or go straight to the emergency department) if you notice any of the following:

  • Clear watery fluid leaking from the wound, the nose, or the ear (this can be a CSF leak)
  • A headache that is much worse on sitting up or standing and better on lying flat (also a sign of a CSF leak)
  • Redness, swelling, or discharge from the wound
  • A fever, particularly with neck stiffness or severe headache (this can be a sign of meningitis)
  • A swelling or fluid collection under the skin at the back of the neck that is getting larger
  • New weakness, numbness, or difficulty walking
  • Difficulty swallowing, difficulty breathing, or a new hoarse voice
  • Repeated vomiting, or unusual drowsiness and difficulty rousing
  • A seizure

If your child is difficult to rouse, is having a seizure, or is having difficulty breathing, this is an emergency, go to the nearest emergency department or call the emergency services immediately.

More information from trusted sources

  • Conquer Chiari — A large US-based foundation dedicated to Chiari research and patient education, with comprehensive plain-language information, a patient handbook, and family resources.
    https://www.conquerchiari.org ↗
  • Bobby Jones Chiari & Syringomyelia Foundation — A US-based non-profit supporting children and adults with Chiari malformation and syringomyelia, with information, support groups, and a designated paediatric Chiari centre programme.
    https://www.bobbyjonescsf.org ↗
  • The Ann Conroy Trust (UK) — A UK charity dedicated to people affected by Chiari malformation and syringomyelia, with information leaflets and a family support service.
    https://www.annconroytrust.org ↗
  • American Association of Neurological Surgeons — Patient Information — The patient education pages of the professional society, with an overview of Chiari malformation written in plain language.
    https://www.aans.org/Patients/Neurosurgical-Conditions-and-Treatments ↗
  • HealthyChildren.org (American Academy of Pediatrics) — Trusted patient and family information from the American Academy of Pediatrics, covering many paediatric conditions.
    https://www.healthychildren.org ↗