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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 ...
Sagittal Dolichocephaly [66] Craniosynostosis-fibular aplasia syndrome [67] Craniosynostosis-hydrocephalus-Arnold-Chiari malformation type I-radioulnar synostosis syndrome Sagittal Scaphocephaly [68] Craniosynostosis-intellectual disability syndrome of 51N and Gettig [69] Craniosynostosis-intellectual disability-clefting syndrome Microcephaly [70]
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]
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.
The distinctive head shape seen in kleeblattschaedel is caused by the closure of the sagittal, coronal, and lambdoid sutures, with subsequent bulging of the cranial contents leading to a trilobate head shape. [7] The condition is also caused by absence of the coronal and lambdoid sutures. [10] Conditions with kleeblattschaedel include: [11] [12]