Medical Conditions

Signs of Chiari Malformation in Babies

Medically reviewed by Dr. Michael Okonkwo, MD, FAAP · Board-Certified Neonatologist

Content reviewed against published NIH, AAP, Mayo Clinic guidelines

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If your baby has been diagnosed with or you suspect signs of chiari malformation in babies, here is what the evidence says.

The short answer

Chiari malformation occurs when part of the brain (cerebellar tonsils) extends below the skull base into the spinal canal. In babies, symptoms may include difficulty swallowing, stridor (noisy breathing), weak cry, apnea episodes, arm weakness, and head tilt. Chiari I may be asymptomatic and found incidentally, while Chiari II is associated with myelomeningocele (spina bifida). Symptomatic cases may require surgical decompression. Many children with Chiari I are monitored without surgery if symptoms are mild or absent.

Key takeaways

  • Chiari malformation occurs when part of the brain (cerebellar tonsils) extends below the skull base into the spinal canal. In babies, symptoms may include difficulty swallowing, stridor (noisy breathing), weak cry, apnea episodes, arm weakness, and head tilt. Chiari I may be asymptomatic and found incidentally, while Chiari II is associated with myelomeningocele (spina bifida). Symptomatic cases may require surgical decompression. Many children with Chiari I are monitored without surgery if symptoms are mild or absent.
  • Usually normal when: Incidentally found mild Chiari I that is asymptomatic and stable on imaging
  • Call your doctor if: Your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari
  • Varies by age — see the age-by-age breakdown below
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What Parents Should Know

According to NIH, AAP, Mayo Clinic guidelines, chiari malformation occurs when part of the brain (cerebellar tonsils) extends below the skull base into the spinal canal. In babies, symptoms may include difficulty swallowing, stridor (noisy breathing), weak cry, apnea episodes, arm weakness, and head tilt. Chiari I may be asymptomatic and found incidentally, while Chiari II is associated with myelomeningocele (spina bifida). Symptomatic cases may require surgical decompression. Many children with Chiari I are monitored without surgery if symptoms are mild or absent. At 0-3 months, chiari II malformation associated with myelomeningocele is typically diagnosed prenatally or at birth. Symptomatic Chiari in young babies may present with feeding difficulties, stridor, apnea, and weak cry. These symptoms need urgent neurosurgical evaluation as brainstem compression can be life-threatening. It is generally considered normal when incidentally found mild Chiari I that is asymptomatic and stable on imaging. However, you should contact your pediatrician promptly if your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari.

Sources: [1], [2], [3]

Normal vs. Concerning

Usually Normal
Worth Discussing
Incidentally found mild Chiari I that is asymptomatic and stable on imaging
Your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari
Your child with Chiari is monitored regularly and has no new symptoms
Sudden worsening of neurological symptoms in a child with known Chiari malformation
Your child underwent successful surgical decompression and is recovering well
Your child with known Chiari develops new headaches, neck pain, or swallowing difficulty

By Age

What to expect by age

0-3 months

Chiari II malformation associated with myelomeningocele is typically diagnosed prenatally or at birth. Symptomatic Chiari in young babies may present with feeding difficulties, stridor, apnea, and weak cry. These symptoms need urgent neurosurgical evaluation as brainstem compression can be life-threatening.

3-12 months

Feeding and swallowing difficulties, recurrent aspiration, and breathing abnormalities may indicate symptomatic Chiari. MRI is the definitive diagnostic study. Some babies with incidentally found Chiari I are monitored with serial MRIs without intervention if asymptomatic.

12-24 months

Symptoms may develop as the child grows. Watch for progressive difficulty with swallowing, changes in voice, neck pain (shown as irritability), balance problems, or new weakness. If surgery is recommended, posterior fossa decompression is the standard procedure.

2-3 years

Children with known Chiari malformation need regular monitoring with neurology. Headaches (especially with coughing, straining, or Valsalva) and neck pain may develop. Many children with Chiari I remain asymptomatic and do not require surgery.

3-5 years

Continued monitoring for changes. Older children may be able to describe headaches or neck pain. Participation in contact sports may need to be discussed with your neurosurgeon. Most children with properly managed Chiari malformation do well long-term.

What to Tell Your Pediatrician

  • Describe when you first noticed signs of chiari malformation in babies and how it has changed over time.
  • Note your baby's current age and which age-specific patterns you are seeing.
  • Mention if your child with known Chiari develops new headaches, neck pain, or swallowing difficulty.
  • Mention if you notice changes in your child's balance, coordination, or hand function.
  • Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
  • Bring a list of any questions or observations you want to discuss at the appointment.

What Should You Do?

When to take action

Probably normal when...
  • Incidentally found mild Chiari I that is asymptomatic and stable on imaging
  • Your child with Chiari is monitored regularly and has no new symptoms
  • Your child underwent successful surgical decompression and is recovering well
Mention at your next visit when...
  • Your child with known Chiari develops new headaches, neck pain, or swallowing difficulty
  • You notice changes in your child's balance, coordination, or hand function
  • Your child was found to have a Chiari malformation and you have questions about monitoring
Act now when...
  • Your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari
  • Sudden worsening of neurological symptoms in a child with known Chiari malformation

What You Can Do at Home

  • Keep track of when you notice signs of chiari malformation in babies — noting the time of day, duration, and any triggers can help your pediatrician.
  • Remember that incidentally found mild Chiari I that is asymptomatic and stable on imaging — this is generally within the range of normal.
  • At 0-3 months, focus on observation rather than intervention unless your pediatrician advises otherwise.
  • Follow any care instructions from your pediatrician. Keep a written log of symptoms to bring to appointments.
  • While monitoring at home, seek immediate care if your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari.

Signs of Hydrocephalus in Babies

Hydrocephalus occurs when cerebrospinal fluid accumulates in the brain ventricles, causing increased pressure. In babies, the most noticeable sign is rapidly increasing head circumference (head growing faster than expected). Other signs include a bulging or tense fontanelle, prominent scalp veins, downward gaze of the eyes ("sunsetting"), irritability, vomiting, and developmental delays. Treatment typically involves surgical placement of a shunt or endoscopic third ventriculostomy to drain excess fluid.

Signs of a Tethered Spinal Cord in Babies

A tethered spinal cord occurs when the spinal cord is abnormally attached to the surrounding tissue, restricting its movement as the child grows. Signs include skin markers on the lower back (deep dimple, tuft of hair, skin tag, or hemangioma above the buttock crease), changes in bladder or bowel function, lower extremity weakness or asymmetry, and foot deformities. Diagnosis is made by MRI. Surgical untethering prevents progressive neurological damage.

When Should My Baby See a Pediatric Neurologist?

A pediatric neurologist specializes in disorders of the brain, spinal cord, nerves, and muscles in children. Referral is appropriate for seizures, abnormal head size or growth, significant hypotonia or hypertonia, developmental regression, movement disorders, headaches, suspected neuromuscular conditions, and abnormal neurological examination findings. These specialists perform detailed neurological evaluations and may order EEGs, MRIs, and other specialized testing.

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Month-by-Month Development

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Guide

When to Call the Doctor

General guide on when to call the pediatrician, visit urgent care, or go to the ER.

Frequently asked questions

Is signs of chiari malformation in babies normal?
Chiari malformation occurs when part of the brain (cerebellar tonsils) extends below the skull base into the spinal canal. In babies, symptoms may include difficulty swallowing, stridor (noisy breathing), weak cry, apnea episodes, arm weakness, and head tilt. Chiari I may be asymptomatic and found incidentally, while Chiari II is associated with myelomeningocele (spina bifida). Symptomatic cases may require surgical decompression. Many children with Chiari I are monitored without surgery if symptoms are mild or absent.
When should I call the doctor about signs of chiari malformation in babies?
Your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari Sudden worsening of neurological symptoms in a child with known Chiari malformation
When is signs of chiari malformation in babies normal?
Incidentally found mild Chiari I that is asymptomatic and stable on imaging Your child with Chiari is monitored regularly and has no new symptoms Your child underwent successful surgical decompression and is recovering well
What causes signs of chiari malformation in babies?
Chiari malformation occurs when part of the brain (cerebellar tonsils) extends below the skull base into the spinal canal. In babies, symptoms may include difficulty swallowing, stridor (noisy breathing), weak cry, apnea episodes, arm weakness, and head tilt. Chiari I may be asymptomatic and found incidentally, while Chiari II is associated with myelomeningocele (spina bifida). Symptomatic cases may require surgical decompression. Many children with Chiari I are monitored without surgery if symptoms are mild or absent. Common explanations include: Incidentally found mild Chiari I that is asymptomatic and stable on imaging. Your child with Chiari is monitored regularly and has no new symptoms.
What should I mention to my pediatrician about signs of chiari malformation in babies?
You should mention signs of chiari malformation in babies at your next visit if: Your child with known Chiari develops new headaches, neck pain, or swallowing difficulty. You notice changes in your child's balance, coordination, or hand function. Your child was found to have a Chiari malformation and you have questions about monitoring.
Is signs of chiari malformation in babies normal at 0-3 months?
Chiari II malformation associated with myelomeningocele is typically diagnosed prenatally or at birth. Symptomatic Chiari in young babies may present with feeding difficulties, stridor, apnea, and weak cry. These symptoms need urgent neurosurgical evaluation as brainstem compression can be life-threatening.
Is signs of chiari malformation in babies normal at 3-12 months?
Feeding and swallowing difficulties, recurrent aspiration, and breathing abnormalities may indicate symptomatic Chiari. MRI is the definitive diagnostic study. Some babies with incidentally found Chiari I are monitored with serial MRIs without intervention if asymptomatic.
Should I go to the ER for signs of chiari malformation in babies?
Seek emergency care if your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari, or if sudden worsening of neurological symptoms in a child with known Chiari malformation. When in doubt, call your pediatrician's after-hours line for guidance.
Does signs of chiari malformation in babies go away on its own?
In many cases, signs of chiari malformation in babies resolves on its own, especially when incidentally found mild Chiari I that is asymptomatic and stable on imaging. By 3-5 years, continued monitoring for changes. Older children may be able to describe headaches or neck pain. Participation in contact sports may need to be discussed with your neurosurgeon. Most children with properly managed Chiari malformation do well long-term.
NIHAAPMAYO CLINIC

References

  1. [1]National Institute of Neurological Disorders and Stroke. Chiari Malformations. NIH
  2. [2]American Academy of Pediatrics. Chiari Malformation. HealthyChildren.org. AAP
  3. [3]Mayo Clinic. Chiari malformation. Mayo Clinic

Doctor Visit Checklist

Bring this checklist to your next pediatrician visit to discuss Signs of Chiari Malformation in Babies.

Things to mention

  • Describe when you first noticed signs of chiari malformation in babies and how it has changed over time.
  • Note your baby's current age and which age-specific patterns you are seeing.
  • Mention if your child with known Chiari develops new headaches, neck pain, or swallowing difficulty.
  • Mention if you notice changes in your child's balance, coordination, or hand function.
  • Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
  • Bring a list of any questions or observations you want to discuss at the appointment.

Observations to share

  • Your child with known Chiari develops new headaches, neck pain, or swallowing difficulty
  • You notice changes in your child's balance, coordination, or hand function
  • Your child was found to have a Chiari malformation and you have questions about monitoring

Urgent signs to report immediately

  • Your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to Chiari
  • Sudden worsening of neurological symptoms in a child with known Chiari malformation

My notes

From ismybabyalright.com — free, evidence-based baby health guides

All content follows our editorial policy and is reviewed against published clinical guidelines.

2,705 evidence-based guides6 authoritative medical sources5 medical advisory board members

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Bottom line

Most cases of signs of chiari malformation in babies are normal. Talk to your pediatrician if your baby has breathing difficulty, apnea episodes, or severe feeding problems that may be related to chiari.

Trust your instincts. If something feels wrong, reach out to your pediatrician. Worrying about your baby means you care — that is a good thing.

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Signs of Hydrocephalus in Babies

Hydrocephalus occurs when cerebrospinal fluid accumulates in the brain ventricles, causing increased pressure. In babies, the most noticeable sign is rapidly increasing head circumference (head growing faster than expected). Other signs include a bulging or tense fontanelle, prominent scalp veins, downward gaze of the eyes ("sunsetting"), irritability, vomiting, and developmental delays. Treatment typically involves surgical placement of a shunt or endoscopic third ventriculostomy to drain excess fluid.

Signs of a Tethered Spinal Cord in Babies

A tethered spinal cord occurs when the spinal cord is abnormally attached to the surrounding tissue, restricting its movement as the child grows. Signs include skin markers on the lower back (deep dimple, tuft of hair, skin tag, or hemangioma above the buttock crease), changes in bladder or bowel function, lower extremity weakness or asymmetry, and foot deformities. Diagnosis is made by MRI. Surgical untethering prevents progressive neurological damage.

When Should My Baby See a Pediatric Neurologist?

A pediatric neurologist specializes in disorders of the brain, spinal cord, nerves, and muscles in children. Referral is appropriate for seizures, abnormal head size or growth, significant hypotonia or hypertonia, developmental regression, movement disorders, headaches, suspected neuromuscular conditions, and abnormal neurological examination findings. These specialists perform detailed neurological evaluations and may order EEGs, MRIs, and other specialized testing.

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