Chiari malformation and syriingomyelia is often present from birth (congenital), but symptoms may not appear until later in life. Some people with Chiari and syringomyelia may not experience noticeable symptoms until adolescence or adulthood, while others might have symptoms from a young age. This delayed onset can make diagnosis challenging.

Children are not small adults! Research suggests that early intervention in pediatric cases can prevent long-term complications.

• Children may not be able to communicate symptoms and are therefore more likely to present with objective symptoms, such as scoliosis or neural tube defects. Trouble swallowing is a common symptom with pediatric CM I.
• Pediatric patients typically present 8-9 years old while adults typically present symptoms in their 40s.
• Syringomyelia is often more common in children and is a motivating symptom for decompression surgery.
• The occurrence of pediatric CM I is about the same in boys and girls; however, CM I occurs in about 3x more adult women than men.
• CM II patients are diagnosed at birth and require shunting to restore the CSF flow. Shunting is roughly two times more common in children than adults due to the spina bifida population.
• Decompression surgery is very successful at reducing headache symptoms, especially for children, and roughly 80-90% successful at reducing syrinx size.
• Pediatric Chiari patients require multidisciplinary care, including neurosurgery, orthopedic surgery, primary care, neurology and pain specialty.

Chiari I Abnormality in Childhood by Arnold H. Menezes, MD

The clinically symptomatic child with the hindbrain herniation syndrome (Chiari I) can differ significantly from the adult patient. A prospective analysis of these children has brought an understanding of the various presentations.

The primary symptoms below age 5 have been failure to thrive secondary to repeated aspirations, gastroesophageal reflux and headaches. Between ages 3-10 years, headaches and scoliosis were the main symptoms together with neurological abnormalities secondary to the cerebellar tonsillar impaction and syringohydromyelia.

The factors affecting treatment have been clinical symptoms/signs, posterior fossa volume, craniovertebral junction abnormalities and instability. Taken together, these substrates provide for successful outcome.

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Watch this video relating to Children with Chiari and Syringomyelia

Histological Investigation of the Posterior Atlanto-Occipital Membrane in Pediatric Patients with Chiari I Malformation – Vijay M Ravindra, MD and Douglas Brockmeyer, MD

Quality Indicators in Pediatric Chiari I – Gerald Grant, MD

Amplified Imaging of the Pediatric Brain – Michael Moseley, MD

Long-term outcomes for children with incidentally discovered Chiari malformation type 1 – Robert F Keating, MD

Disease Progression of Pediatric Patients …CMI – Kerri Thorn, MD

Comparing Children with Chiari malformation Type I and Controls

Long-term Outcomes for Children Chiari Malformation

Additional Pediatric Research

FDA News Release: Treatment of Scoliosis in Children

The Chiari I Abnormality in Childhood