AIMS: The authors analyse a series of @entity1 with @entity91 in order to detail the definition of this @entity91 and its functional prognosis. @entity1 AND METHODS: The medical records of 129 oxycephalic @entity1 were reviewed. Skull X-rays, ophthalmologic examination, mental level assessment, intracranial pressure monitoring and CT scan were analysed. The more recent @entity1 were also analysed by MRI. Operated on or not, the @entity1 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 @entity1 came from North Africa, where @entity91 seems predominant. Mean age at diagnosis was 6 years. Past history of rickets was found in 15% of the @entity1 . On X-rays, the vast majority of the @entity1 presented with multisutural @entity8213 involving both coronal and sagittal sutures, and diffuse digital prints. At the first mental assessment, one third of the @entity1 had an IQ below 80. @entity1829 was found in 17%. The monitoring of intracranial pressure showed an increased pressure in almost two thirds of the @entity1 . Sixty-four percent of the @entity1 with @entity428 had a normal fundoscopy. Out of 16 @entity1 explored by MRI, 12 had a @entity1645 . Postoperatively, all @entity1829 disappeared, and the intracranial pressure returned to normal in all cases with preoperative @entity428 . The mental level seemed to stabilize, the mean postoperative IQ being strongly correlated with the preoperative level. In non-operated @entity1 , the mental level worsened significantly. CONCLUSIONS: @entity91 is a late-appearing @entity91 , with a high risk of @entity532 . Based on the present series, the operation seemed effective in preventing these complications.

[ XXXX , a severe @entity91 . Apropos of a series of 129 cases].

@entity1 :: ('9606', 'Species') :: ['patients', 'PATIENTS'] @entity428 :: ('MESH:D019586', 'Disease') :: ['increased intracranial pressure'] @entity8213 :: ('MESH:D013580', 'Disease') :: ['synostosis'] @entity1645 :: ('MESH:D001139', 'Disease') :: ['Chiari I malformation'] @entity532 :: ('MESH:D003925', 'Disease') :: ['ophthalmologic and mental complications'] @entity1829 :: ('MESH:D010211', 'Disease') :: ['papilledemas', 'Papilledema'] @entity91 :: ('MESH:D003398', 'Disease') :: ['Oxycephaly', 'oxycephaly', 'craniosynostosis']
@entity91 :: (MESH:D003398,Disease) :: ['Oxycephaly']
