[Oxycephaly, a severe craniosynostosis. Apropos of a series of 129 cases].

AIMS: The authors analyse a series of patients with oxycephaly in order to detail the definition of this craniosynostosis and its functional prognosis. PATIENTS AND METHODS: The medical records of 129 oxycephalic patients were reviewed. Skull X-rays, ophthalmologic examination, mental level assessment, intracranial pressure monitoring and CT scan were analysed. The more recent patients were also analysed by MRI. Operated on or not, the patients were followed-up, particularly as far as the mental evolution is concerned. Mean follow-up was 3 years 7 months.
RESULTS: One third of the patients came from North Africa, where oxycephaly seems predominant. Mean age at diagnosis was 6 years. Past history of rickets was found in 15% of the patients. On X-rays, the vast majority of the patients presented with multisutural synostosis involving both coronal and sagittal sutures, and diffuse digital prints. At the first mental assessment, one third of the patients had an IQ below 80. Papilledema was found in 17%. The monitoring of intracranial pressure showed an increased pressure in almost two thirds of the patients. Sixty-four percent of the patients with increased intracranial pressure had a normal fundoscopy. Out of 16 patients explored by MRI, 12 had a Chiari I malformation. Postoperatively, all papilledemas disappeared, and the intracranial pressure returned to normal in all cases with preoperative increased intracranial pressure. The mental level seemed to stabilize, the mean postoperative IQ being strongly correlated with the preoperative level. In non-operated patients, the mental level worsened significantly.
CONCLUSIONS: Oxycephaly is a late-appearing craniosynostosis, with a high risk of ophthalmologic and mental complications. Based on the present series, the operation seemed effective in preventing these complications.