OXYCEPHALY, A SEVERE CRANIOSYNOSTOSIS - A NALYSIS OF 129 CASES

Aims. - The authors analyse a series of patients with oxycephaly in or der to detail the definition of this craniosynostosis and its function al prognosis. Patients and methods, - The medical records of 129 oxyce phalic patients were reviewed. Skull X-rays, ophthalmologic examinatio n menial level assessment, intracranial pressure monitoring and CT sca n were analysed. The more recent patients were also analysed by MRI. O perated on or not, the patients were followed-rep, particularly as Jar as the mental evolution is concerned. Mean follow-up was 3 years 7 mo nths. Results, - One third of the patients came from North Africa, whe re oxycephaly seems predominant. Mean age at diagnosis was 6 years. Pa st 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 print s. Al the first mental assessment, one third of the patients had an IQ below 80. Papilledema was Sound in 17%. The monitoring of intracrania l pressure showed an increased pressure in almost two thirds of the pa tients. Sixty-Sour 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 dis appeared and the intracranial pressure returned to normal in all cases with preoperative increased intracranial pressure. The mental level s eemed to stabilize, the mean postoperative IQ being strongly correlate d with the preoperative level. In non-operated patients, the mental le vel worsened significantly. Conclusions. - Oxycephaly is a late-appear ing craniosynostosis, with a high risk of ophthalmologic and mental co mplications. Based on the present series, the operation seemed effecti ve in preventing these complications.