Chiari I Malformation with Scoliosis and Syringomyelia

Abstract

We reviewed retrospectively the cases of 8 children who were surgically treated for Chiari I malformation with scoliosis and syringomyelia. The patients were 5 males and 3 females, ranging in age from 7 to 16 years (mean, 10.8 years). The follow-up periods ranged from 12 to 51 months. Clinical symptoms found in all patients included scoliosis (100%), dysbasia (38%), headache (25%), nocturnal enuresis (25%). The duration of symptoms ranged from 12 to 60 months. All patients underwent foramen magnum decompression (FMD) and duraplasty (DP) with preservation of the arachnoid membrane. Simultaneously, four patients underwent C1 laminectomy, 3 patients underwent C1 laminoplasty, and 1 was unspecified. Postoperatively, scoliosis improved in 6 cases, remained unchanged in one case, and progressed in one case. Mean Cobb’s angle decreased from 54.0°(range, 31-78°) to 44.4°(range, 16-76°). Cobb’s angles less than 25°were achieved in 2 patients (25%) with scoliosis less than 40°and they were able to discontinue use of the brace. Three patients (37.5%) with scoliosis that remained sever (>50°) required an additional corrective operation. Three other patients (range, 25-50°) continue to use the brace. Clinical symptoms improved in 4 cases, but nocturnal enuresis continued in 1 case. Magnetic resonance images showed a decrease in size of the fistula in 4 patients, and an increase in one patient. It was thought that the operation when Chiari I malformation was less than 40°Cabb’s angle resulted in improvement of the spinal deformity.