Chiari Malformation Type 2: Causes, Symptoms, and Treatment
Medically reviewed by Sumiran Racherla, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Chiari Malformation Type 2?
A pediatric neurologist (a doctor who treats brain and nerve problems in children) usually diagnoses Chiari malformation type 2, and a pediatric neurosurgeon (a doctor who does brain and spine surgery on children) treats it. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is Chiari malformation type 2?
Chiari malformation type 2 happens when parts of the brain push down through the foramen magnum (the opening at the base of the skull) into the spinal canal (the space inside the spine). The parts that move include the cerebellum (the part of the brain that controls balance) and the lower brain stem. Doctors also call it Arnold-Chiari malformation. It is present at birth.
Type 2 happens almost only in babies who have a myelomeningocele (a form of spina bifida, or an open spine defect). The spine does not close properly around the spinal cord before birth. This can cause swelling. It can also cause partial or complete paralysis (loss of movement) below the point where the spine did not close.
Symptoms
Symptoms can include:
- Breathing pauses (apnea), sometimes with a bluish skin color.
- Trouble swallowing, and a weak gag reflex (the reflex that helps prevent choking).
- Uncontrolled eye movements.
- Low muscle tone (hypotonia) or muscle stiffness (spasticity).
- Poor coordination (ataxia).
- Weakness in the arms.
- Headaches near the base of the skull.
Fluid can also build up in the brain. Doctors call this hydrocephalus.
Causes and who gets it
Chiari malformation type 2 forms before birth, while the brain and skull are growing. It often happens because the brain and spinal cord do not develop properly before birth. Type 2 is present at birth. It is a rare condition.
How doctors diagnose it
Doctors can see Chiari malformation type 2 before birth with an ultrasound (a scan that uses sound waves). After birth, the main test is an MRI (magnetic resonance imaging), which uses magnets and radio waves to make pictures of the brain and spinal cord. Doctors also do a physical exam and check reflexes and movement.
Treatment
The myelomeningocele is repaired with surgery, usually within the first few days after birth. A surgeon moves the nerves back into the spine, repairs the layers over them, and closes the muscles and skin on the baby's back. If fluid builds up in the brain, a shunt (a tube that drains extra fluid) may be placed.
Doctors treat the Chiari malformation if the baby has symptoms and no complications from hydrocephalus. For type 2, this surgery is usually limited to the tissues in the spinal canal. Surgery can help keep symptoms from getting worse. However, nerve damage that has already happened usually cannot be reversed.
When to see a doctor
In many babies who develop symptoms from Chiari malformation type 2, the symptoms start and get worse fast, and this needs urgent or emergency care. Call 911 if a baby has breathing pauses, trouble breathing, or bluish or grey skin. Call 911 for a seizure. Call 911 if a baby cannot feed or swallow well, or is very sleepy.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. For Chiari malformation type 2, this doctor can follow your child's growth and development over time and refer you to a pediatric neurologist or pediatric neurosurgeon when needed.
Common questions
Is Chiari malformation type 2 the same as Arnold-Chiari malformation?
Yes. Doctors also call Chiari malformation type 2 Arnold-Chiari malformation.
Can Chiari malformation type 2 be found before birth?
Yes. Doctors can see it on an ultrasound during pregnancy. It is present at birth.
Do babies with type 2 always have spina bifida?
Almost always. Type 2 happens almost only in babies who have a myelomeningocele, a form of spina bifida. The spine does not close properly around the spinal cord before birth.
What surgery do babies with type 2 need?
The myelomeningocele is repaired with surgery, usually within the first few days after birth. Doctors treat the Chiari malformation if the baby has symptoms and no complications from hydrocephalus. If fluid builds up in the brain, a shunt may be placed.
Sources
- National Organization for Rare Disorders (NORD) - Chiari malformation type II
- National Institute of Neurological Disorders and Stroke - Chiari Malformation
- American Association of Neurological Surgeons - Chiari Malformation
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
