Oxycephaly

Isolated oxycephaly is a late-appearing form of nonsyndromic craniosynostosis characterized by premature fusion of both the coronal and sagittal sutures, and, in some cases, of one or both lambdoid sutures. Compensating growth in the anterior fontanel region results in the formation of a pointed or cone-shaped skull and a receding forehead. Only occasionally oxycephaly may be present at birth, usually in the context of a severe genetic abnormality. Some forms evolve from initially unisutural synostoses, and most of the cases are present later in life with harmonious restriction of intracranial volume. Elevated intracranial pressure is a common clinical feature and leads to frequent ophthalmologic complications and moderate to severe intellectual deficit, although individuals may have a completely normal cognitive outcome. When left untreated, it is responsible for severe related complications. Unlike unisutural synostoses, which do not cause intracranial hypertension and for which the aim of surgery is above all the restoration of normal shape, intracranial hypertension is the rule in oxycephaly. For this reason, decompressive surgery is needed even in newborns when signs of increased intracranial pressure are found. During childhood, it should be performed as soon as the clinical diagnosis has been established. The aim of the surgery is mainly to expand intracranial volume. The timing and the surgical technique used for correction will depend primarily on the patient’s age at presentation. Following surgery, the intracranial pressure usually returns to normal, papilledema resolves, and the intellectual deficit seems to stabilize. In infants and older children, fronto-orbital advancement with frontoparietal remodeling is the technique of choice, providing good cranial decompression and satisfactory morphological results.