Fact-sheet: Craniosynostosis
Updated on 09/17/2021 at 12:16 PM
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Definition
Craniosynostosis = premature fusion of one or more cranial sutures. It may be isolated or associated with a polymalformative syndrome (syndromic craniosynostosis). Craniosynostosis may be: - Simple: involvement of a single suture. - Complex: involvement of multiple sutures.
Clinical features
In response to premature closure of a suture, compensatory growth occurs through the remaining patent sutures, resulting in a skull deformity characteristic of each type of craniosynostosis.
The main risk is the development of intracranial hypertension, which may potentially lead to mental retardation and visual disturbances.
Different forms:
Scaphocephaly = sagittal synostosis: premature closure of the sagittal suture with anteroposterior elongation of the skull.
Trigonocephaly = metopic synostosis: sclerosis of the metopic suture with a triangular deformity of the forehead.
Plagiocephaly = unilateral coronal synostosis: premature closure of the coronal suture on one side, leading to elevation and deviation of the orbit on the affected side.
Brachycephaly = bilateral coronal synostosis: premature closure of the entire coronal suture, with recession of the lower forehead and a wide, prominent upper forehead.
Cloverleaf skull: results from synostosis of both parieto-squamosal sutures, with or without involvement of the coronal and lambdoid sutures.
Radiography
Frontal and lateral radiographs:
the affected suture shows a bridging appearance with a bony ridge, hyperdense sclerosis, or loss of the serrated pattern.
CT
Bridging appearance with a bony ridge at the affected suture, hyperdense sclerosis, or loss of the serrated pattern.
Preoperative workup ++
Value of 3D reconstructions.
MRI
Performed as part of a syndromic workup: search for intracranial abnormalities.
Search for intracranial hypertension.
Management
Surgical management as early as possible, to prevent the onset of intracranial hypertension.
Surgery consists of a craniectomy, more or less extensive depending on the involvement.
Classification
Different forms: Scaphocephaly = sagittal synostosis: premature closure of the sagittal suture with anteroposterior elongation of the skull. Trigonocephaly = metopic synostosis: sclerosis of the metopic suture with a triangular deformity of the forehead. Plagiocephaly = unilateral coronal synostosis: premature closure of the coronal suture on one side, leading to elevation and deviation of the orbit on the affected side. Brachycephaly = bilateral coronal synostosis: premature closure of the entire coronal suture, with recession of the lower forehead and a wide, prominent upper forehead. Cloverleaf skull: results from synostosis of both parieto-squamosal sutures, with or without involvement of the coronal and lambdoid sutures.