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Our Guide to Pediatric Chiari Malformations-Long Island Brain and Spine

November 22, 2023

Chiari malformations are structural abnormalities involving the base of the skull and cerebellum, the part of the brain that controls balance. While this condition can occur in individuals of all ages, its manifestation in children can lead to significant harm. These malformations can lead to various symptoms and associated conditions, some of which might persist into adulthood.

Fortunately, many treatment options are available at Long Island Brain and Spine. Our experienced adult and pediatric neurosurgeon, Dr. David Phillips, specializes in Chiari malformation surgery in children and adults and has cared for many children and families in times of great need. In this article, we'll delve into the types of Chiari malformations and discuss their characteristics, conditions, and crucial signs to watch for so you can ensure that you or your family member gets the best possible care.

Types of Chiari Malformations

These are the different types of Chiari malformations:

- Chiari 1 Malformation: The most common type in children and adults, Chiari 1 involves an extension of the cerebellar tonsils into the spinal canal. Although it can be present at birth, symptoms often don't manifest until late childhood or adulthood. Common Chiari 1 malformation symptoms include headaches, neck pain, and sometimes problems with coordination.

- Chiari Type 2 Malformation: More severe than its Type 1 counterpart, Chiari Type 2 is typically associated with myelomeningocele – a form of spina bifida where the spinal canal and backbone don't close properly. Children with Chiari Type 2 malformation often have symptoms from birth or early infancy, including breathing and swallowing difficulties. Some children develop complications of Chiari Type 2 later in life or need long-term follow-up.

- Other Chiari Malformations: While Chiari 1 and Type 2 are the most commonly diagnosed, other types, though rare, do exist. Their manifestation and severity can differ, but their core characteristic remains a structural defect at the base of the skull and cerebellum.

Identifying the specific type of Chiari malformation is crucial, as it influences both the symptomatology and the approach to treatment for your child.

Associated Conditions and Symptoms

Chiari malformations are often intertwined with other conditions, emphasizing the complexity of diagnosis and treatment:

- Hydrocephalus in Adults: Hydrocephalus is a buildup of cerebrospinal fluid in the brain and may be linked to Chiari malformations. In children, it's essential to address hydrocephalus promptly to avoid any lasting complications like cognitive and motor function impairments.

- Myelomeningocele: A severe form of spina bifida, myelomeningocele entails the spinal cord and its lining protruding from an opening in the spine. Its association with Chiari Type 2 malformation amplifies the need for a comprehensive medical approach, addressing both conditions simultaneously.

- Syrinx of the Spinal Cord: A syrinx is a cyst filled with fluid that forms within the spinal cord. Chiari malformations can cause cerebrospinal fluid to flow irregularly, leading to syrinx formation. Symptoms might include stiffness, weakness, numbness or pain in the back, shoulders, arms, or legs.

Understanding these associated conditions paints a holistic picture of Chiari malformations, underlining the intricate nature of pediatric neurological health.

Signs and Symptoms

Recognizing the indications of Chiari malformations is vital for timely intervention. The symptoms can vary based on the type of malformation and any associated conditions. Here's a comprehensive list to help parents and caregivers discern potential signs:

- Headaches: Headaches are one of the most common symptoms, especially with Chiari 1 malformation. They often begin at the back of the head and neck and may intensify with coughing or straining.

- Neck Pain: Often correlated with headaches, neck pain can be persistent and might exacerbate upon exertion.

- Swallowing Difficulties: Especially prevalent in Chiari Type 2 malformation, difficulty swallowing can sometimes lead to gagging or choking.

- Breathing Problems: In severe cases, especially in infants with Chiari Type 2, there can be episodes of apnea, where the child momentarily stops breathing.

- Coordination Issues: Balance and hand-eye coordination might be affected, leading to unsteady walking or difficulty performing tasks requiring fine motor skills.

- Numbness or Weakness: Some children might feel tingling, numbness, or even weakness in their arms or legs.

- Voice Changes: Hoarseness or other voice changes can sometimes be observed.

- Scoliosis: An abnormal sideways curvature of the spine might develop in some children with Chiari malformation.

Promptly noting and addressing these symptoms can lead to early diagnosis, enhancing the chances of effective treatment and improved quality of life for the child. If your child is experiencing any of these symptoms, promptly bringing them in for an assessment is of utmost importance. Our experienced practitioners are trained to work with these specific and pressing symptoms. We prioritize providing the best care for your child at Long Island Brain and Spine.

Treatment and Surgical Options

For many affected children, proactive intervention becomes crucial. While some may remain asymptomatic or show minimal signs, others may experience significant symptoms warranting medical attention:

- Observation: Not every child with a Chiari malformation requires immediate treatment. Regular monitoring through neurological exams and imaging studies like MRIs can be sufficient in cases with mild or no symptoms at all.

- Medication: Some symptoms, especially pain, can be managed with medications. These may include pain relievers for headaches or medications to reduce cerebrospinal fluid production.

- Chiari malformation surgery: Surgery becomes viable when symptoms are severe or when cerebrospinal fluid flow is obstructed. The most common procedure is posterior fossa decompression, which creates more space for the cerebellum and relieves pressure on the spinal cord. This surgery can improve or halt the progression of symptoms in many patients.

- Choosing the Right Surgeon: The intricacies involved in Chiari malformation surgery emphasize the importance of selecting an experienced neurosurgeon. Our specialized Chiari malformation neurosurgeon, Dr. Phillips, is equipped with the expertise to navigate the challenges of this condition. Discussing potential risks, benefits, and post-operative care with a medical professional is crucial when considering surgery.

Dr. David Phillips is an Adult and Pediatric Neurosurgeon specializing in caring for children and adults with various intracranial and spinal problems. Some of his specialties include hydrocephalus, spina bifida, epilepsy, and Chiari malformations. You can be sure your child is in excellent hands with Dr. Phillips. His approach contains upfront communication, regular lifelong follow-up, and discussing all appropriate surgical options. He is skilled in minimally invasive and endoscopic surgery and works with a great team of surgeons, including fellowship-trained scoliosis experts.

Treatment options are tailored to the child's needs. This approach will ensure the best outcomes for the patient. While the prospect of surgery might be daunting, it's worth noting that many children experience substantial relief and improved quality of life post-operation.

Treatment at Long Island Brain and Spine

Chiari malformations, although complex, are increasingly well-understood thanks to medical advancements and dedicated research. Early diagnosis, spurred by awareness of symptoms and associated conditions, can significantly shape a child's developmental and health trajectory. As with many medical conditions, knowledge and proactive intervention are critical. At Long Island Brain and Spine, we leverage the latest research, treatments, and surgical innovations to ensure that the best options are always available to your child.

Remember, while Chiari malformations present challenges, they are surmountable. With proper care and attention, many children with Chiari malformations can lead fulfilling, active lives. Our leading experts at Long Island Brain and Spine are with you every step of the way.

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