Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome.

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.