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What Is a Chiari Malformation?
Chiari malformations are abnormal formations of the brain, specifically affecting the cerebellum and brainstem. The cerebellum, located in the base of the skull above the brainstem, controls the motor functions, balance, coordination and muscle tone.
Chiari malformation neck pain may be associated with other conditions. Syringomyelia is a degenerative disorder when a syrinx, a fluid-filled cyst on the spinal cord, puts pressure on the nerve bundles. Such compression may cause neuralgia, pain along the nerve’s pathway, in the head or neck.
Conditions We Treat
Chiari Simple: tonsillar herniation with a small posterior fossa
Chiari Plus: Chiari malformation, Type I with comorbidity (another chronic condition or disease) such as
- Connective tissue disorders
- Craniocervical instability
- Pseudotumor cerebri
- Tethered cord
Chiari Redux: failed CMI surgery
Causes of Chiari Malformations
The most common Chiari malformations, Type I, may not have any symptoms or they may occur in late childhood or adulthood. Sometimes this congenital defect is not detected until an associated condition is diagnosed. Other suspected causes include exposure to harmful substances, infections or head traumas.
Type II and Type III Chiari malformations are generally diagnosed before birth or shortly thereafter. Causes of structural defects during fetal development are not completely understood, but theories suggest that drug use (prescribed or illegal), alcohol use, lack of proper nutrition or exposure to harmful substances may contribute to the condition along with a genetic predisposition.
When there isn’t enough bony space for the brain tissue of the cerebellum and part of the brain stem to sit above the foramen magnum (opening to the spinal canal), the brain tissue may get pushed into the spinal canal opening. This can block the circulation of the cerebrospinal fluid (CSF) and may lead to hydrocephalus.
Symptoms of Chiari Malformations
Since the cerebellum controls the motor functions and balance, many of the symptoms relate to coordination:
- Balance issues
- Stiffness in the neck
- Neck pain or pain in the back of the head
- Poor hand coordination
- Swallowing difficulty, sometimes accompanied by gagging or choking
- Clearing throat an unusual amount
- Vision or speech problems
- Muscle weakness
- Tingling sensation
- Scoliosis (spinal curvature)
Although some symptoms overlap, some tend to be more common depending on the type of Chiari malformation.
Less common symptoms that may relate to Type I Chiari Malformations:
- Tinnitus (ringing or buzzing in the ear)
- Slowed heart rate
- Symptoms that tend to relate to Type II or Type III Chiari Malformations:
- Rapid eye movements
- Developmental delays in holding the head up or sitting up
- A weak cry
- Breathing problems
Advanced Chiari Malformation Treatments in the Long Island, New York Region
Recent advancements and continued research have led to a range of options when treating Chiari malformations. No one single technique works for everyone. The surgeon and patient need to work together to determine the best approach. Dr. Bolognese performs 200 CMI (Chiari Malformation, Type I) or CMI-related pathologies each year, including Posterior Fossa Decompressions and Posterior Fossa Revisions.
Simple Chiari Malformation Treatment
- Extradural techniques: cutting the bone alone
- Minimally invasive approaches: small incisions or the use of small ports to access and surgically treat the malformation
- MIST (minimally invasive subpial tonsillectomy)
Surgical Management of Complex Chiari Malformations
The surgical approaches for treating Chiari that also involve cranial cervical instability or abnormal anatomy of the craniocervical junction are more complex: Posterior Fossa Decompression (PFD) and a Craniocervical Fusion (CCF) with Transoral Odontoidectomy, if necessary. The use of heftier screws in the occipital condyles reduces the need for 4-5 inches of clunky hardware attached to thin bone and instead is a 1.25 inch of stronger hardware attached to a much thicker bone.
Convenient Long Island, New York Locations
World-class Chiari treatment, close by. Staffed by physicians of multiple specialties, NSPC has developed to meet the needs of a wider patient population. Thanks to our locations throughout the greater tri-state area, more patients have access to comprehensive, collaborative quality care.
Chiari Malformations Educational Videos by Dr Paolo Bolognese
PART I: Discovery, Definition and Classification of Chiari Malformations
PART II: Modern Classification and Subclassification of Chiari Malformations
PART III: Trends & History of Surgery for Chiari Malformations
PART IV: Surgical Techniques Chiari Malformations
Our Chiari Malformation Specialist