An approach to symptomatic children (ages 4-14 years) with Chiari type I malformation

A review of 16 pediatric patients examined and treated from 1987 to 1991 is presented. The purpose for this review was to examine the different initial clinical presentation in the younger age group versus the older child and to identify the factors impacting on the patients' surgical outcome, including the patients' age, clinical presentation and MRI findings. Patients' ages ranged from 4 to 14 years. Ten were under 6 years of age. Seven males and 9 females were included. The diagnosis was confirmed by MRI in all patients and all were followed by clinical and MRI evaluations for at least 1 year postoperatively. The patients were grouped into three subgroups: (a) group IA patients with a Chiari I malformation, (b) group IB patients with a Chiari I malformation and syrinx, and (c) group IC patients with a Chiari I malformation and hydrocephalus with or without an associated syringomyelic cavity. A uniform surgical approach was used and tailored to the degree of anomalies seen on MRI. Patients with a Chiari IA and IB were treated by a suboccipital craniectomy, upper cervical laminectomy, arachnoidal retraction and duraplasty. Only 1 patient in group B required a syringosubarachnoid shunt initially. Patients with a Chiari IC malformation were treated by a ventriculoperitoneal shunt initially. The clinical presentation of younger children (less than 6 years old) appeared similar to older children (over 6 years of age) except for sleep apnea, which was limited to the younger age group. Patients with pronounced motor and sensory deficits had the worst prognosis for recovery. The patient's age, duration of symptoms, type of Chiari I malformation and degree of downward tonsillar migration on MRI did not appear to have a negative impact on the final outcome.