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Symptomatic Chiari I malformation in childhood: a report of 7 cases
R C Dauser1, M A DiPietro, J L Venes
1Division of Neurosurgery, University of Michigan, Ann Arbor.
Insights
Chiari I malformation is significant in children, often presenting with hydrosyringomyelia, scoliosis, and weakness. Surgical intervention improved or stabilized symptoms in all pediatric patients reviewed.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Medical Imaging
Background:
- Chiari I malformation was historically considered significant only in adolescents and adults.
- Recent recognition highlights its importance in the pediatric population.
Observation:
- A review of 7 pediatric cases (under 12 years) with Chiari I malformation was conducted.
- Associated conditions included hydrosyringomyelia, scoliosis, and motor weakness.
- Intraoperative ultrasound proved a valuable surgical aid.
Findings:
- All pediatric patients underwent posterior fossa and cervical decompression with dural grafting and syringosubarachnoid shunts.
- Symptoms and signs improved or stabilized in all cases post-surgery.
- Increased use of noninvasive neuroimaging, particularly MRI, likely contributes to more frequent reporting in young children.
Implications:
- The study suggests a potential underdiagnosis of Chiari I malformation in children with conditions like scoliosis.
- Early recognition and surgical management can lead to positive outcomes in pediatric patients.
- Further research into the incidence of undiagnosed Chiari I malformation in pediatric populations is warranted.
Abstract:
The Chiari I malformation, once thought to be a disorder of clinical importance only in the teen years and beyond, is now recognized as being significant in the pediatric population as well. We have reviewed 7 cases of Chiari I malformation in children less than 12 years of age. Hydrosyringomyelia was often, but not invariably, an accompanying feature, and this in turn was associated with scoliosis and motor weakness. All patients had posterior fossa and cervical decompression with dural grafting procedures, as well as various types of syringosubarachnoid shunts placed. Intraoperative ultrasound was found to be a valuable surgical adjunct. Symptoms and signs were improved or stabilized in all patients. We feel that this disorder is now more commonly reported in young children because of the increased availability of noninvasive neuroimaging techniques, especially magnetic resonance imaging. Questions are raised as to the actual incidence of undiscovered cases of this disorder in children with commonly seen conditions such as scoliosis.