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Our approach

Many children with Chiari malformations do not have any symptoms and do not need treatment. However, when treatment is needed, Cohen Children’s pediatric neurosurgeons are experts, handling the largest volume of these cases in the region. We offer a variety of Chiari malformation treatment options, all of which are highly successful.

Overview

A Chiari I malformation (or hindbrain herniation) is when the back of the brain (cerebellum) pushes down through the bottom opening of the skull (the foramen magnum), which is only meant for the spinal cord to pass through. This can sometimes obstruct the flow of cerebrospinal fluid through that opening, which leads to headaches and, sometimes, spinal cord injury. The pain can radiate into the neck, shoulders and hands and can be extremely debilitating. Sometimes symptoms can include pain upon exertion, sneezing, coughing or even laughing.

At 15, tennis champ Maddie Germano lost her mom to cancer. Then she found out she had a rare brain condition called Chiari malformation. She underwent neurosurgery at Cohen Children’s, and now she’s back on her feet—and the tennis court.

Causes & risk factors 

Chiari malformation can occur when the section of the skull containing the cerebellum is too small for that portion of the brain, thus putting pressure on and crowding the brain. "Chiari malformation" as a set of diagnoses can also be associated with a form of spina bifida called myelomeningocele.  This is called Chiari II malformation and the symptoms and signs may be quite different from Chiari I malformations.

Symptoms

Depending on the type and severity, Chiari malformations can cause a number of symptoms. Some of the most common symptoms are:

  • Severe headaches, usually after sudden coughing, sneezing or straining
  • Neck pain
  • Lack of balance
  • Poor hand coordination and fine motor skills
  • Numbness and tingling of the hands and feet
  • Dizziness
  • Difficulty swallowing
  • Blurred or double vision
  • Hoarseness or other speech problems

Many symptoms of Chiari malformation can also be associated with other disorders, so if you experience any of them, it’s important to see your doctor for a thorough medical evaluation.

Diagnosis & testing

A detailed diagnostic workup may include an MRI, Cine flow MRI, dynamic X-rays, 3D CTs, high-definition imaging and morphometrics. Once the patient is diagnosed, a determination will be made whether or not surgery is needed, and which type of surgery is best, depending on each specific case.

Treatment options

We offer a variety of Chiari malformation treatment options, all of which are highly successful. Some are minimally invasive, where a neurosurgeon can simply make a small incision and open up the blocked passage in the skull opening to be a little bigger. Other treatments are more invasive, where a surgeon opens up the lining of the brain and expands that area with a patch. 

Since these surgeries can cause discomfort, we have an ongoing study of using a long acting local analgesic to dramatically help with postoperative pain control.

It’s important to note that some Chiari malformations do not need treatment, especially if they are not causing significant symptoms.  A conservative, nonsurgical approach is always primarily considered when our surgeons provide a recommendation.

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