The concurrence of hydrocephalus and craniosynostosis

Abstract

✓ The clinical course and pneumoencephalographic findings of 14 patients with the associated anomalies of craniosynostosis and hydrocephalus are reviewed. Postmortem examination in two cases, one of which had aqueductal stenosis, confirmed the presence of hydrocephalus. Eight of the 14 had associated anomalies of the extremities consistent with the diagnosis of Apert's syndrome and six patients had dull to normal intelligence. Various types of sutural involvement were present including isolated closure of the sagittal suture. The hydrocephalus, which appears to be an associated anomaly rather than a direct consequence of the craniosynostosis, is most often communicating, but stenosis and atresia of the aqueduct may be present. No single pathogenetic mechanism seems responsible for the abnormal ventricular enlargement, but when the dilation becomes progressive, surgical intervention may be indicated.