Hydrocephalus and craniosynostosis

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Object. A retrospective study of 1727 cases of craniosynostosis was undertaken to determine the interrelationship between abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis.

Methods. The patients were divided into two groups: nonsyndromic craniosynostosis and syndromic craniosynostosis. Cases of occipital plagiocephaly without suture synostosis and cases of shunt-induced craniosynostosis were excluded from the study. The majority of patients (1297) were treated surgically for their cranial deformity; 95% of these patients had a postoperative follow-up review period lasting 5 years. Clinical and radiographic charts covering the time from presentation through the follow-up period were reviewed.

Conclusions. Abnormal intracranial CSF hydrodynamics was found in 8.1% of the patients (3.4% of whom had received shunts and 4.5% of whom had not). Three types of CSF hydrodynamic disturbance were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spaces. Hydrocephalus occurred much more frequently in patients with syndromic craniosynostosis (12.1%) than in those with isolated craniosynostosis (0.3%). In fact, patients with kleeblattschädel exhibited hydrocephalus as a constant feature and patients with Crouzon's syndrome were far more likely to have hydrocephalus than those with other syndromes. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of syndromic craniosynostosis, venous sinus obstruction and/or chronic tonsillar herniation were found. Their role in the pathophysiology of hydrocephalus in craniosynostosis is discussed.

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Address reprint requests to: Giuseppe Cinalli, M.D., Service de Neurochirurgie, Hôpital Necker—Enfants Malades, 149 rue de Sevres, 75015 Paris, France.

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    Imaging data obtained in a patient with oxycephaly. A: Preoperative CT scan obtained when the patient was 5 years old. B: Postoperative CT scan obtained 2 weeks after forehead advancement. Note the increased ventricular size and the signs of transependymal resorption. C: A CT scan obtained 4 months after forehead advancement. There is a significant additional increase in ventricular size, which is more marked at the level of the frontal horns, with periventricular lucency. D: Angiogram, lateral view, showing stenosis of both jugular foramina. Note the significant transdiploic collateral circulation at the level of the sagittal sinus in the parietal region and at the level of the posterior fossa. E: Angiogram, posterior view, showing hypoplasia of both lateral sinuses, stenosis of both jugular foramina, and a marked collateral circulation.

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    Imaging data obtained in a patient with Opitz C syndrome. Left: Computerized tomography scan obtained when the patient was 5 days old. Note the mild dilation of the temporal horns and severe cranial deformity. Center: Axial MR image obtained in the child at the age of 2 months, before shunt placement and cranial remodeling. Severe ventricular dilation is apparent. Right: Sagittal MR image obtained at the same time showing good visibility of the sylvian aqueduct and severe crowding of the posterior fossa without tonsillar herniation.

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    Imaging data obtained in a patient with Crouzon's syndrome. A: Computerized tomography scan obtained when the patient was 20 months old, 1 week after forehead advancement and cranal vault remodeling. B: Computerized tomography scan obtained 2 months later. Note the mild increase in vertricular size and the marked dilation of the pericerebral spaces. The craniectomy sites are bulging with severe bone resorption of the partial flaps. C: Sagittal MR image showing chronic tonisillar herniation. D: Magnetic resonance angiography sequence displaying jugular foramen stenosis with hypoplasia of the left lateral sinus and signifigant collateral circulation at the level of the right sigmoid sinus.

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    Imaging data obtained in a patient with Apert's syndrome. Left: Preoperative CT scan obtained when the patient was 4 months old. Center: Computerized tomography scan obtained 2 months after forehead advancement. Note the persistence of a significant pericerebral fluid collection, bulging craniotomy sites with forehead deformity, and bilateral bone resorption. A subduroperitoneal shunt was placed in the patient a few days later. Right: Computerized tomography scan obtained 2 months after placement of the subduroperitoneal shunt. The pericerebral fluid collection has almost completely disappeared, but the craniotomy sites continue to bulge and the ventricular size has increased. A VP shunt was placed in the patient a few days later.

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    Sinusography film obtained in an 8-month-old patient with Crouzon's syndrome showing severe jugular foramen stenosis (arrow), chronic tonsillar herniation, and progressive ventricular dilation. Only the sagittal and lambdoid sutures are involved at this age. The coronal sutures are open and widely enlarged, allowing ventricular dilation to develop.

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