Craniosynostosis

  • Etiology: premature fusion of one or more sutures with skull elongating in direction of fused sutures
  • Imaging:
    — Sagittal – 50% – scaphocephaly / dolicocephaly – normal head volume
    — Bilateral coronal – 20% – brachycephaly – higher incidence of neurological complications including increased ICP / optic atrophy / mental retardation
    — Bilateral lambdoid – 5% – brachycephaly
    — Unilateral coronal – anterior plagiocephaly with deformed skull looking like trapezoid, unilateral harlequin eye deformity due to elevation of orbital roof, ipsilateral frontal bone flattened with contralateral frontal bossing, metopic suture deviated to affected side, trapezoid deformity of calvarium
    — Unilateral lambdoid – posterior plagiocephaly with deformed skull looking like trapezoid
    — Metopic synostosis – occurs in utero, trigonocephaly with ridge along fused metopic suture / pointed head, hypotelorism – quizzical eyes
    — All fused – Kleeblattschadel = cloverleaf skull

Radiology Cases of Craniosynostosis

Radiology Cases of Metopic Craniosynostosis

3D CT of metopic craniosynostosis
Axial CT without contrast of the brain (left) shows fusion of the metopic suture in the midline and a triangular appearance to the anterior aspect of the skull (trigonocephaly). Anterior 3D CT reconstruction (right) shows a prominent midline ridge in the anterior skull and hypotelorism with quizzical eye deformities present bilaterally.
3D CT of metopic craniosynostosis
Axial CT without contrast of the brain (left) shows fusion of the metopic suture in the midline and a triangular appearance to the anterior aspect of the skull (trigonocephaly). Anterior 3D CT reconstruction (right) shows a prominent midline ridge in the anterior skull and hypotelorism with quizzical eye deformities present bilaterally.

Radiology Cases of Unilateral Coronal Craniosynostosis

Radiology Cases of Bilateral Coronal Craniosynostosis

Radiograph of bilateral coronal craniosynostosis
AP and lateral radiographs of the skull show bilateral elevation of orbital roofs resulting in bilateral harlequin eye deformities. The coronal sutures appear sclerotic and fused along their middle and inferior aspects. The sagittal suture and bilateral lambdoid sutures are patent.

Radiology Cases of Sagittal Craniosynostosis

Radiograph and 3D CT of sagittal craniosynostosis
AP (above left) and lateral (above right) radiographs of the skull show the skull to be lengthened (scaphocephaly / dolichocephaly). The sagittal suture appears sclerotic. Superior (below left) and lateral (below right) 3D CT reconstructions of the skull show the skull to be lengthened and complete fusion of the sagittal suture. The remaining sutures are patent.

Radiology Cases of Unilateral Lambdoid Craniosynostosis

3D CT of unilateral lambdoid craniosynostosis
Lateral (above) and superior (below left) and posterior (below right) 3D CT reconstructions of the skull show flattening of the left posterior skull and fusion of the left lamboid suture. The bilateral coronal sutures, sagittal suture, and right lambdoid suture are patent.

Radiology Cases of Bilateral Lambdoid Craniosynostosis

Radiology Cases of Cloverleaf Skull Craniosynostosis

Radiograph and 3D CT of cloverleaf skull
AP (above left) and lateral (above right) radiographs of the skull and anterior (below left) and lateral (below right) 3D CT reconstructions of the skull show fusion of the bilateral coronal sutures, bilateral lambdoid sutures, metopic suture and anterior and posterior aspects of the sagittal suture. There is compensatory widening of the bilateral squamosal sutures. Hypotelorism is present and there are quizzical eye deformities present bilaterally.