Kraniosynostose

CT-scan of a
skull of a patient with coronal synostosis as part of craniofrontonasal dysplasia.
Wikipedia • CC BY-SA 3.0
Infant with
flattening of the left posterior head. 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.The diagnosis was left lambdoid suture craniosynostosis.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with a
pointy head. 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.The diagnosis was metopic suture craniosynostosis.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with a
bump on the forehead. 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.The diagnosis was metopic suture craniosynostosis.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with a
palpable ridge on the top of the head. 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.The diagnosis was sagittal craniosynostosis.
pediatricimaging.org • CC-by-nc-sa 4.0
Frank-ter
Haar syndrome associated with sagittal craniosynostosis and raised intracranial pressure. A-D: Patient 1. A: Antero-posterior (AP) view showing facial features including hypertelorism. B: Lateral view showing brachycephaly and micrognathia. C: X-ray (XR) right radius/ulna showing broad appearance of the radius at the junction between the proximal and middle thirds. D: XR left hand showing crowding of the carpal bones, broad metacarpals, proximal and middle phalanges, and flexion at the MCP and PIP joints. E-G: Patient 2. E: AP view showing facial features including hypertelorism. F: Lateral view showing brachycephaly. G: 3D CT scan showing absence of the sagittal suture. H-J: Patient 3. H: AP view showing facial features including hypertelorism. I: Lateral view showing class III malocclusion and brachycephaly. J: 3D CT scan showing absence of the sagittal suture.
bmcmedgenet.biomedcentral.com • CC-by-2.0
Acquired
craniomeningocele in an infant with craniosynostosis: a case report. Tri-dimensional computed tomography scan confirmed the fusion in the coronal and lambdoid sutures and diffuse skull changes with honeycomb configuration and beaten copper appearance.
jmedicalcasereports.biomedcentral.com • CC-by-2.0
Acquired
craniomeningocele in an infant with craniosynostosis: a case report. Skull X-ray shows fusion of both coronal and lambdoid sutures with diffuse beaten copper appearance.
jmedicalcasereports.biomedcentral.com • CC-by-2.0
Newborn with
a shortened appearance of the head. 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.The diagnosis was bilateral coronal craniosynostosis.
pediatricimaging.org • CC-by-nc-sa 4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Craniosynostosis refers to the premature closure of the cranial sutures. The skull shape then undergoes characteristic changes depending on which suture(s) close early.

Epidemiology

There is a 3:1 male predominance with an overall incidence of 1 in 2000-2500. 8% of cases are syndromic or familial.

Pathology

Primary forms are either sporadic or familial. Secondary craniosynostosis occurs in relation to a variety of causes:

  • endocrine disorders
    • hyperthyroidism
    • hypophosphatemia 
    • vitamin D deficiency 
    • hypercalcemia
  • hematologic disorders causing bone marrow hyperplasia
    • sickle cell
    • thalassemia
  • inadequate brain growth
    • microcephaly
    • shunted hydrocephalus 
Ages of normal sutural/fontanelle closure
Types

The sagittal suture is most commonly involved (≈50%), where the lateral growth of the skull is arrested while anteroposterior growth continues, producing a narrowly elongated skull known as scaphocephaly (meaning boat-shaped) or dolichocephaly (from the ancient Greek for long, δολιχός: dolichos).

The next most common sutures in terms of involvement are:

  • coronal (~20%)
  • lambdoid (~5%)
  • metopic (~5%)
Associations

Most occur as isolated anomalies but syndromic associations can be seen in a small proportion of cases (~10%):

Radiographic features

Restriction of skull growth is perpendicular to the affected suture line. Characteristic dysmorphic head shapes are associated with each type of craniosynostosis.

Ultrasound

Ultrasound may be useful in subtle cases. General features include:

  • sutures are normally hypoechoic
    • there may be a loss of normal decreased echogenicity in the region of the fusion
  • lack of suture patency
  • ridging of the sutures
CT

CT with 3D image reformations is the best modality used for evaluation of sutures .

Treatment and prognosis

Treatment is often with a cranioplasty. Abnormal intracranial pressure may affect neurocognition.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Kraniosynostose:
Choanalatresie
 
Wikipedia • CC BY-SA 3.0
Pfeiffer-Syndrom
 
www.eurorad.org • CC by-nc-sa-4.0
Apert-Syndrom
 
jkamprs.springeropen.com • CC-by-4.0
Kleeblattschädel
 
pediatricimaging.org • CC-by-nc-sa 4.0
Crouzon-Syndrom
 
ijpeonline.biomedcentral.com • CC-by-2.0
Dolichocephalie
 
bmcmedgenet.biomedcentral.com • CC-by-2.0
trigonocephaly
 
pediatricimaging.org • CC-by-nc-sa 4.0
kranio-fronto-nasale
Dysplasie
Wikipedia • CC BY-SA 3.0
persistierende
Sutura frontalis mediana
Wikipedia • CC BY-SA 4.0