Spine Surgeons treating Chiari Malformation

CHIARI MALFORMATION

Chiari malformation is a structural abnormality of the brain.  In this case, part of the lower back of the brain protrudes through an opening at the base of the skull. Clearly, this condition puts pressure on the cerebellum, brainstem, and spinal cord, potentially affecting their function. While the exact cause is unknown, it’s often congenital but may go undiagnosed until adulthood.

Scoliosis Quiz on the Herniated Disc page
Pain Quiz on the herniated disc page

3 percent of individuals with progressive curvature may eventually experience severe problems that can include scoliosis and back pain, spinal problems, and nerve compression causing numbness, weakness, and leg pain.

Understanding Chiari Malformation

Chiari MalformationChiari malformation is a structural abnormality in the base of the skull where the brain and spinal cord meet. Basically, this condition occurs when the lower part of the cerebellum extends through the opening at the base of the skull. This displacement of brain tissue can obstruct the flow of cerebrospinal fluid (CSF) and disrupt normal neurological function. Chiari malformation can have a significant impact on a person’s quality of life,  As an example, it can lead to symptoms ranging from headaches and neck pain to more severe neurological deficits if left untreated.

Causes of Chiari Malformation

The cause of Chiari malformation is not fully understood, but it is believed to occur from genetic and environmental factors. Some cases may be congenital, meaning they occur during fetal development.  While others cases may develop later in life due to factors such as trauma, infection, or abnormal skull growth. Certain genetic mutations and conditions, such as spinal cord tethering or hydrocephalus, may also increase the risk of Chiari malformation.

Symptoms of Chiari Malformation

Symptoms of Chiari malformation can vary widely depending on the severity of the condition and individual factors. Common symptoms may include:

  • Headaches, often worsened by coughing, sneezing, or straining
  • Neck pain or stiffness
  • Dizziness or vertigo
  • Difficulty swallowing
  • Nausea and vomiting
  • Sensory disturbances, such as tingling or numbness in the hands or feet
  • Muscle weakness or coordination problems
  • Ringing in the ears (tinnitus)
  • Breathing difficulties, particularly during sleep
  • Vision problems, such as double vision or blurred vision

Questions and Answers

How is Chiari malformation diagnosed, and what tests are involved?

Diagnosis of Chiari malformation typically involves a combination of a detailed medical history, thorough physical examination, and imaging studies. Ultimately, the most common diagnostic tool is magnetic resonance imaging (MRI).  With this tool, doctors are provided with detailed images of the brain and spinal cord. Hence, this non-invasive procedure helps identify any herniation of the cerebellar tonsils into the foramen magnum. In some cases, additional tests like computed tomography (CT) scans or electromyography (EMG) may be recommended.  Lastly, this test will rule out other potential causes of symptoms and assess nerve function.

What are the non-surgical treatment options for Chiari malformation, and how effective are they?

Non-surgical treatment options for Chiari malformation aim to manage symptoms and improve the patient’s quality of life. Pain management with over-the-counter or prescription medications can help alleviate headaches and discomfort. Physical therapy plays a crucial role in enhancing muscle strength, flexibility, and posture. Lifestyle modifications, such as avoiding activities that trigger symptoms and maintaining a healthy lifestyle, are also essential. While non-surgical treatments may not cure Chiari malformation, they can be effective in reducing symptoms and improving overall well-being.

When is surgical intervention necessary for Chiari malformation, and what does it involve?

Surgical intervention for Chiari malformation is considered when symptoms are severe, progressive, or associated with neurological deficits. Also, the most common surgical procedure is posterior fossa decompression.  Basically, this is where a small portion of the skull or the back of the first cervical vertebra (C1) is removed.  This will create more space within the skull.  As such, it will relieve the pressure on the brainstem and cerebellum. Duraplasty may be performed concurrently to expand the protective membrane surrounding the brain and spinal cord. In some cases, a syringe-subarachnoid shunt placement is necessary to drain fluid from cysts within the spinal cord. Therefore, the decision for surgery is based on individual symptoms, neurological findings, and the degree of herniation observed in diagnostic imaging.

2 / 2

 

 
 
 

 

 

 

 
 
 
 

 

 
 
 
 

Diagnosis of Chiari Malformation

Diagnosing Chiari malformation typically involves a combination of medical history review, physical examination, and diagnostic imaging studies. Magnetic resonance imaging (MRI) is commonly used to visualize the brain and spinal cord and identify any abnormalities.  The MRI can show a herniation of the cerebellar tonsils into the foramen magnum. Additional tests, such as computed tomography (CT) scans or electromyography (EMG), may be ordered to assess nerve function.

Risk Factors for Chiari Malformation

While the exact risk factors for Chiari malformation are not fully understood, certain factors may increase the likelihood of developing the condition. These may include:

  • Genetic predisposition or family history of Chiari malformation
  • Abnormalities in skull or spinal cord development
  • Traumatic injury to the head or spine
  • Certain medical conditions, such as hydrocephalus or syringomyelia

Non-Surgical and Surgical Treatment Options

Treatment for Chiari malformation depends on the severity of symptoms and the extent of neurological involvement. In mild cases or asymptomatic individuals, observation and symptom management may be sufficient. Non-surgical treatment options may include:

  • Pain management with over-the-counter or prescription medications
  • Physical therapy to improve muscle strength, flexibility, and posture
  • Lifestyle modifications, such as avoiding activities that exacerbate symptoms or trigger headaches
  • Monitoring and regular follow-up appointments to track any changes in symptoms or neurological function

In cases where symptoms are severe or progressive, or if there is evidence of neurological deficits or CSF obstruction, surgical intervention may be necessary. The goal of surgery is to relieve pressure on the brain and spinal cord by creating more space within the skull. Surgical procedures for Chiari malformation may include:

  • Posterior fossa decompression surgery removes a small portion of the skull or the back of the first cervical vertebra (C1).  This will alleviate pressure on the brainstem and cerebellum
  • Duraplasty is a procedure to expand the dura mater (the protective membrane surrounding the brain and spinal cord).  This procedure will increase CSF flow and prevent further herniation
  • The Syringo-subarachnoid shunt placement is a procedure to drain excess fluid from syrinx (cyst) within the spinal cord

Complications and Long-term Benefits of Treatment

Complications of Chiari malformation may include persistent symptoms, recurrent headaches, neurological deficits, and the development of syringomyelia (fluid-filled cysts) within the spinal cord. However, with appropriate treatment and management, many individuals with Chiari malformation can experience significant improvement in their symptoms and quality of life. Surgical intervention can provide long-term relief of symptoms and prevent further neurological deterioration, allowing patients to resume normal activities and enjoy a better quality of life.

Physical Therapy Recommendations and Living with Chiari Malformation

Physical therapy plays a crucial role in the management of Chiari malformation, helping to improve muscle strength, flexibility, balance, and posture. A qualified physical therapist can design a personalized exercise program to address specific symptoms and functional limitations, incorporating techniques such as stretching, strengthening exercises, manual therapy, and postural retraining. Additionally, lifestyle modifications, such as maintaining a healthy weight, practicing good posture, avoiding activities that exacerbate symptoms, and managing stress, can also help individuals with Chiari malformation manage their condition more effectively and improve their overall well-being.

Prevention and Related Conditions

While it may not be possible to prevent Chiari malformation entirely, certain preventive measures may help reduce the risk of complications and improve outcomes. These may include early detection and intervention, regular monitoring and follow-up appointments with a healthcare provider, adherence to treatment recommendations, and lifestyle modifications to minimize symptom triggers and maintain overall health and wellness. It’s also essential for individuals with Chiari malformation to be aware of related conditions, such as syringomyelia, spinal cord tethering, and hydrocephalus, and to seek appropriate medical care if new symptoms or complications arise.

Why Choose the Southwest Scoliosis and Spine Institute

The Southwest Scoliosis and Spine Institute, led by renowned spine surgeons Dr. Richard A. Hostin, Dr. Devish Ramnath, and Dr. Ishaq Syed, offers comprehensive and compassionate care for patients with complex spine conditions, including Chiari malformation. With offices in Dallas, Plano, and Frisco, Texas, the institute provides state-of-the-art diagnostic and treatment options tailored to each patient’s unique needs and preferences. Finally, the team of experienced spine specialists utilizes the latest advancements in surgical techniques, including minimally invasive spine surgery approaches, to achieve optimal outcomes and improve the quality of life for individuals living with Chiari malformation. From diagnosis to treatment and beyond, patients can trust the Southwest Scoliosis and Spine Institute to provide expert care and support every step of the way.

____________________

American Association of Neurological Surgeons:  Chiari Malformation

 

 

We’re here to help STOP THE PAIN
If you are an adult living with scoliosis or have a child with this condition and need a doctor who specializes in orthopedic surgery,
call the Southwest Scoliosis and Spine Institute at 214-556-0555 to make an appointment today.