HYDROCEPHALUS AND CRANIOSYNOSTOSIS

Object. A retrospective study of 1727 cases of craniosynostosis was un dertaken to determine the interrelationship between abnormal cerebrosp inal fluid (CSF) hydrodynamics and craniosynostosis. Methods. The pati ents were divided into two groups: nonsyndromic craniosynostosis and s yndromic craniosynostosis. Cases of occipital plagiocephaly without su ture synostosis and cases of shunt-induced craniosynostosis were exclu ded from the study. The majority of patients (1297) were treated surgi cally for their cranial deformity; 95% of these patients had a postope rative follow-up review period lasting 5 years. Clinical and radiograp hic charts covering the time from presentation through the follow-up p eriod were reviewed. Conclusions. Abnormal intracranial CSF hydrodynam ics was found in 8.1% of the patients (3.4% of whom had received shunt s and 4.5% of whom had not). Three types of CSF hydrodynamic disturban ce were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spa ces. Hydrocephalus occurred much more frequently in patients with synd romic craniosynostosis (12.1%) than in those with isolated craniosynos tosis (0.3%). In fact, patients with kleeblattschadel exhibited hydroc ephalus as a constant feature and patients with Crouzon's syndrome wer e far more likely to have hydrocephalus than those with other syndrome s. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of s yndromic craniosynostosis, venous sinus obstruction and/or chronic ton sillar herniation were found. Their role in the pathophysiology of hyd rocephalus in craniosynostosis is discussed.