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Scaphocephaly or sagittal craniosynostosis is a type of cephalic disorder which occurs when there is a premature fusion of the sagittal suture. Premature closure results in limited lateral expansion of the skull , resulting in a characteristic long, narrow head. [ 1 ]
It is estimated that craniosynostosis affects 1 in 1,800 to 3,000 live births worldwide. [3] three out of every four cases affect males. Sagittal synostosis is the most common phenotype, representing 40% to 55% of nonsyndromic cases, [3] whilst coronal synostosis represents between 20% and 25% of cases. [3]
Craniosynostosis-Dandy-Walker malformation-hydrocephalus syndrome (HCDPH1, also known as Sagittal craniosynostosis, Dandy-Walker malformation and hydrocephalus, Dandy-Walker malformation with sagittal craniosynostosis and hydrocephalus, Braddock-Jones-Superneau syndrome, or simply Hydrocephalus, autosomal dominant) is an autosomal dominant syndrome characterized by sagittal craniosynostosis ...
Craniosynostosis, a condition in which the sutures of the head (joints between the bones of the skull) prematurely fuse and subsequently alter the shape of the head, is seen in multiple conditions, as listed below. The level of involvement varies by condition and can range from minor, single-suture craniosynostosis to major, multisutural ...
Saethre–Chotzen syndrome (SCS), also known as acrocephalosyndactyly type III, is a rare congenital disorder associated with craniosynostosis (premature closure of one or more of the sutures between the bones of the skull). This affects the shape of the head and face, resulting in a cone-shaped head and an asymmetrical face.
Craniosynostosis, Philadelphia type is a rare autosomal dominant syndrome characterized by sagittal craniosynostosis (scaphocephaly) and soft tissue syndactyly of the hands and feet. This condition is considered a form of acrocephalosyndactyly. [1] [2] [3]
The primary diagnostic factor is a malformation of the skull. The two most common types of craniosynostosis are sagittal and bicoronal. Sagittal craniosynostosis manifests itself by causing a long narrow skull, resembling a football. It is quantitatively determined by measuring the anterior to posterior (front to back) diameter of the skull.
Leptocephaly is a rare form of complex craniosynostosis (usually considered a form of scaphocephaly) in which the sagittal and metopic suture simultaneously close. [1] [2] [3] Leptocephaly is characterized by equal narrowing of the head (from full sagittal fusion) and a tall and narrow (rather than long) head shape.