Craniosynostosis occurs when one or more of the skull's sutures fuse early, before the brain is fully grown. It can be caused by a single suture fusing (simple) or multiple sutures (compound). The suture affected determines the skull deformity produced - sagittal synostosis causes an elongated, narrow skull called scaphocephaly; coronal synostosis causes a wide, short skull called brachycephaly; lambdoid synostosis causes flattening at the back of the skull. Craniosynostosis is diagnosed using 3D imaging or ultrasound and features sutural narrowing and ridging.
Craniosynostosis occurs when one or more of the skull's sutures fuse early, before the brain is fully grown. It can be caused by a single suture fusing (simple) or multiple sutures (compound). The suture affected determines the skull deformity produced - sagittal synostosis causes an elongated, narrow skull called scaphocephaly; coronal synostosis causes a wide, short skull called brachycephaly; lambdoid synostosis causes flattening at the back of the skull. Craniosynostosis is diagnosed using 3D imaging or ultrasound and features sutural narrowing and ridging.
Craniosynostosis occurs when one or more of the skull's sutures fuse early, before the brain is fully grown. It can be caused by a single suture fusing (simple) or multiple sutures (compound). The suture affected determines the skull deformity produced - sagittal synostosis causes an elongated, narrow skull called scaphocephaly; coronal synostosis causes a wide, short skull called brachycephaly; lambdoid synostosis causes flattening at the back of the skull. Craniosynostosis is diagnosed using 3D imaging or ultrasound and features sutural narrowing and ridging.
Craniosynostosis occurs when one or more of the skull's sutures fuse early, before the brain is fully grown. It can be caused by a single suture fusing (simple) or multiple sutures (compound). The suture affected determines the skull deformity produced - sagittal synostosis causes an elongated, narrow skull called scaphocephaly; coronal synostosis causes a wide, short skull called brachycephaly; lambdoid synostosis causes flattening at the back of the skull. Craniosynostosis is diagnosed using 3D imaging or ultrasound and features sutural narrowing and ridging.
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Anatomy
The bones of the skull are the frontal, parietal, temporal,
occipital, and sphenoid bones The place where two bones come together is called a suture At birth, the adjacent bones override each other to allow the infant to pass through the birth canal. As the child grows the sutures allow for skull expansion to accommodate the growing brain. The brain doubles in size by age 6 months and again at age 2 years. These sutures normally begin to fuse around 2 years of age along with the closure of the fontanel (soft spot) Anatomy The seam where two skull bones fuse together is called a suture. The major sutures are called sagittal, coronal, metopic and lambdoid Pathology Craniosynostosis occurs when one or more of the major sutures close early The cause is unknown It is called 'primary' when not associated with any other problem. When there is an underlying disorder it is considered 'secondary heali It may be "simple" with only one suture closed or "compound" when multiple sutures are involved Pathology Craniosynostosis occurs when one or more of the major sutures close early The cause is unknown It is called 'primary' when not associated with any other problem. When there is an underlying disorder it is considered 'secondary Healing rickets and hypophosphatasia Idiopathic hyperclcemia Anemias with diploe involvement Hyperthyroidism Brain atrophy After successful shunting for hydrocephalus Pathology Features Sutural narrowing Sharpening of the edges Sclerosis and ridging along the margins of the suture Bony bridging across the suture line
Three-Dimensional Study: Gold standard
Ultrasound: Preliminary study Sagittal Craniosynostosis Most common Creates an elongated narrow skull called 'scaphocephaly' or 'dolicocephaly The compensatory change causes the front (frontal) and back (occipital) areas of the skull to be pushed outward causing bossing (bulging of the skull) Coronal Craniosynostosis Second most common Caused by early closure of the coronal suture Produces a calvarium that is short from front to back and wide from side to side, called bradycephaly or brachycephaly Bregma is somewhat high in position, and calvarium is wide in frontal projection Marked uptilting of the sphenoid wings Harlequin appearance of the orbits Coronal Craniosynostosis Frontospenoid synchondrosis is also probably involved Often the primary deformity in Crouzons disease and Aperts syndrome Craniofacial dysostosis Unilateral Coronal Synostosis Features Flattening of the ipsilateral frontoparietal region Elevation of the ipsilateral sphenoid wing Unilateral harlequin appearance of the involved orbit Coronal suture on the involved side shows the usual shape of premature closure Narrowness of the suture line Sharpening of the sutural edges Sclerosis along the bony margins Skewed Nasal Septal Sign nasal septum is obliquely tilted Lambdoid Craniosynostosis Isolated synostosis of the lambdoid sutures is less common When present, Marked flattening and underdevelopment of the lower portion of the posterior fossa Severe overgrowth of the bregma Oxycpehalic or turricephalic skull Metopic Craniosynostosis Forehead becomes pointed Trigonocephaly Mixed Craniosynostosis Cloverleaf Skull most grotesque deformity Prominent bulging of the temporal region is see Universal Craniosynostosis All sutures can close prematurely Severe microcephaly is seen Markedly increased inner table convolutions and other signs of chronically increase intracranial pressure (vs. brain atrophy) Summary Name of Skull Deformity from Craniosynostosis Suture(s) Fused Early Type of Skull Deformity Sagittal suture Scaphocephaly (most common) One coronal suture Anterior plagiocephaly Both coronal sutures Brachycephaly One lambdoid suture Posterior plagiocephaly Metopic suture Trigonocephaly All skull sutures and basilar Oxycephaly skull sutures Fusion of all sutures except Kleeblattschadel (Cloverleaf metopic and squamosal skull)