Craniosynostosis

Craniosynostosis is a congenital condition characterized by the premature fusion (ossification) of one or more cranial sutures — the fibrous joints between the bones of the skull. Normally, these sutures remain open during infancy and early childhood to allow the brain and skull to grow properly. When one or more sutures close too early, the skull cannot expand normally in the direction perpendicular to the fused suture, leading to abnormal head shape, and in some cases, increased intracranial pressure. Craniosynostosis may occur as an isolated (nonsyndromic) finding or as part of a recognized genetic syndrome such as Crouzon syndrome, Apert syndrome, Pfeiffer syndrome, Muenke syndrome, or Saethre-Chotzen syndrome. The condition primarily affects the skeletal system (skull and craniofacial bones) and can secondarily impact the central nervous system. Key clinical features depend on which suture is involved: sagittal synostosis (the most common form) produces a long, narrow skull (scaphocephaly); coronal synostosis causes a flattened forehead on the affected side (anterior plagiocephaly) or a short, wide skull if bilateral (brachycephaly); metopic synostosis results in a triangular forehead (trigonocephaly); and lambdoid synostosis leads to posterior flattening. Symptoms may include an abnormal head shape apparent at birth, a palpable bony ridge along the affected suture, asymmetric facial features, and in more severe or multi-suture cases, signs of elevated intracranial pressure such as headaches, visual disturbances, developmental delay, and papilledema. Treatment is primarily surgical and aims to release the fused suture, allow normal brain growth, reduce intracranial pressure, and improve cranial and facial appearance. Surgical options include open cranial vault remodeling, strip craniectomy, endoscopic suture release (often combined with postoperative helmet therapy), and spring-assisted cranioplasty. The timing and type of surgery depend on the patient's age, the number and location of fused sutures, and whether the condition is syndromic. Multidisciplinary care involving neurosurgery, craniofacial surgery, ophthalmology, genetics, and developmental pediatrics is standard. Long-term outcomes are generally favorable for isolated single-suture craniosynostosis when treated early, though syndromic forms may require multiple surgeries and ongoing monitoring.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Craniosynostosis