Dolichocephalie

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
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
Radiopaedia • CC-by-nc-sa 3.0 • de

Scaphocephaly (also known as dolichocephaly) is the most common form of craniosynostosis, where premature closure of the sagittal suture results in an impediment to the lateral growth of the skull while anteroposterior growth continues, producing a narrow and elongated skull. Causes are primary, or secondary to certain hematologic disorders, metabolic disorders, bone dysplasias, and syndromes.

Epidemiology

Scaphocephaly accounts for approximately 50% of all cases of craniosynostosis and has a male predilection with an M:F ratio of 3:1.

Pathology

Associations

Treatment and prognosis

Treatment is often with a cranioplasty. In contrast to other craniosynostoses, there is near normal IQ and no hydrocephalus.

History and etymology

The term is derived from the Ancient Greek words "skaphe" meaning a light boat and "kephale" meaning head and describes an elongated head resembling an inverted boat.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Dolichocephalie:
Kraniosynostose
 
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