Syringobulbia in a pediatric population

To better understand the presentation, management, and outcome of syringobulbia in the pediatric age group.

The University of Iowa pediatric neurosurgery database was searched for patients under the age of 18 with a diagnosis of syringobulbia. The patients' records were retrospectively reviewed for demographic data, chief complaint and presenting symptoms, neurological and radiographic findings, treatment, outcome, and complications. Children with open neural tube defects and Chiari II malformations were excluded.

Six pediatric patients were identified as meeting inclusion criteria. The average age at time of surgery was 14.8 years. The chief complaints were vision impairment in three children and numbness, gait instability, and headache worsened with Valsalva in one patient each. Other prominent symptoms included sleep apnea and weakness. All patients showed at least one cranial nerve dysfunction. Radiographs revealed hindbrain herniation and associated syringomyelia in all cases. Two patients had scoliosis. Treatment was posterior fossa decompression with cerebellar tonsillar shrinkage, opening of foramen of Magendie, and duraplasty. Two patients also required concomitant ventral decompression. The cavity of syringobulbia communicated with syringomyelia and the fourth ventricle in most children but was distinct from the fourth ventricle. Two patients received fourth ventricle to subarachnoid shunts. Follow-up averaged 3.2 years, and all patients clinically improved after surgery. Magnetic resonance imaging documented resolution of syringobulbia in all cases, with syringomyelia improving in all cases. There was no permanent morbidity or mortality in the series.

Syringobulbia is strongly associated with Chiari malformation and syringomyelia, and patients often present because of cranial nerve palsies. Posterior fossa decompression is a safe and effective treatment.