Non-syndromic bicoronal and sagittal craniosynostosis

Non-syndromic bicoronal and sagittal craniosynostosis (Orphanet code 620205) is a rare congenital craniofacial condition characterized by the premature fusion of both coronal sutures and the sagittal suture of the skull. Unlike syndromic forms of craniosynostosis, this condition occurs in isolation without additional systemic anomalies or an identifiable genetic syndrome. The premature closure of these three sutures significantly restricts normal skull growth, leading to an abnormal head shape. Because both the coronal and sagittal sutures are involved, the resulting cranial deformity can be complex, often presenting with a shortened and widened or turricephalic (tower-shaped) skull, depending on the compensatory growth patterns of the remaining open sutures. The condition primarily affects the skeletal system of the skull and can secondarily impact the brain and eyes. Premature fusion of multiple sutures raises the risk of elevated intracranial pressure, which if left untreated may lead to headaches, visual impairment (including papilledema), developmental delays, and in severe cases, cognitive impairment. Affected infants typically present at birth or in early infancy with a visibly abnormal head shape, and the fused sutures can be detected on physical examination and confirmed with imaging studies such as CT scan. Treatment is primarily surgical and aims to release the fused sutures, reshape the skull to allow normal brain growth, and reduce or prevent elevated intracranial pressure. Surgical approaches may include open cranial vault remodeling or, in select cases, endoscopic strip craniectomy followed by postoperative helmet therapy. The timing of surgery is typically within the first year of life. Long-term follow-up with a multidisciplinary craniofacial team—including neurosurgeons, plastic surgeons, ophthalmologists, and developmental specialists—is essential to monitor for recurrence of synostosis, intracranial pressure changes, and neurodevelopmental outcomes.

Common questions about Non-syndromic bicoronal and sagittal craniosynostosis