Syringomyelia in Childhood

Childhood syringomyelia is rare and its clinical symptoms differ from those in adults. From 1982 to 1985, the authors studied 19 children with syringomyelia with high-resolution computed tomography (Siemens Somatom II) and with magnetic resonance imaging (MRI)(0.15 tesla imager, Toshiba MRT 15A). There were 14 patients with spina bifida (eight with meningomyeloceles associated with Chiari malformation, and six with lipomas), four with Chiari malformation (type undetermined) and one with idiopathic syringomyelia. Scoliosis and pes cavus were predominant symptoms. Scoliosis was demonstrable in 80% and pes cavus in 40% of the patients with Chiari malformation and idiopathic syringomyelia. Surgery was performed in four patients with Chiari malformation and in two with lipoma. Five patients were treated with a syringo-subarachnoid shunt and one patient with a syringostomy. Five patients improved neurologically and one remained unchanged. The results indicate that syringomyelia in children is not as rare as previous reports suggest, and should be diagnosed and treated early. MRI appears to be very useful in the diagnosis of this disorder, which, in children, is frequently expressed as scoliosis and pes cavus. Subarachnoid shunting is effective for the patient with Chiari malformation, provided the syrinx is large and associated with swelling of the spinal cord. (Shinkeigeka 27: 973–978, 1987)