Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome
Gregory W Albert1, Arnold H Menezes, Daniel R Hansen
1Department of Neurosurgery, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, Iowa 52242, USA.
Insights
Early surgical treatment for Chiari malformation Type I (CM-I) in young children leads to positive outcomes. Prompt diagnosis and intervention can prevent the need for additional therapies for related conditions.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Developmental Pediatrics
Background:
- Chiari malformation Type I (CM-I) can present with varied symptoms in young children.
- Understanding age-specific presentations is crucial for timely diagnosis.
Purpose of the Study:
- To evaluate unique signs and symptoms of CM-I in children under 6.
- To highlight the benefits of early surgical intervention in this pediatric population.
Main Methods:
- Retrospective review of medical records for 39 patients under 6 years old.
- Analysis of patient presentation, surgical procedures, and outcomes between 1984 and 2007.
Main Results:
- Infants (0-2 years) often showed oropharyngeal dysfunction (77.8%).
- Older children (3-5 years) more commonly presented with syringomyelia (85.7%), scoliosis (38.1%), and headache (57.1%).
- Surgical interventions included posterior fossa craniectomy, with some requiring cervical laminectomy, duraplasty, transoral decompression, or occipitocervical fusion, leading to symptom resolution or improvement.
Conclusions:
- Early recognition and surgical treatment of CM-I in young children yield favorable outcomes.
- Timely surgery can often prevent the need for further therapies for oropharyngeal dysfunction, syringomyelia, and scoliosis.
Object:
The authors conducted a study to evaluate the unique presenting signs and symptoms of Chiari malformation Type I (CM-I) in children younger than 6 years of age and highlight the benefits of early surgical treatment in this patient population.
Methods:
The authors reviewed the medical records of patients who presented to the neurosurgery department before their 6th birthday and subsequently underwent surgery for CM-I. They identified 39 patients who had been evaluated between 1984 and 2007 and examined the medical records for presentation, surgical intervention, and outcome.
Results:
Children aged 0-2 years commonly presented with oropharyngeal dysfunction (77.8%). Children aged 3-5 years more frequently presented with syringomyelia (85.7%), scoliosis (38.1%), and/or headache (57.1%). All patients underwent posterior fossa craniectomy. Additionally, in many patients cervical laminectomy and/or duraplasty was performed. A few patients required transoral decompression and occipitocervical fusion. In most cases, surgery led to resolution or dramatic improvement of initial symptoms.
Conclusions:
Early recognition and surgical treatment of CM-I in young children leads to good outcomes in the majority of patients. Additional therapies for oropharyngeal dysfunction, syringomyelia, and scoliosis can frequently be avoided.
