Oxycephaly
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Turricephaly is a type of
cephalic disorder Cephalic disorders () are congenital conditions that stem from damage to, or abnormal development of, the budding nervous system. Cephalic disorders are not necessarily caused by a single factor, but may be influenced by hereditary or genetic c ...
where the head appears tall with a small length and width. It is due to premature closure of the
coronal suture The coronal suture is a dense, fibrous connective tissue joint that separates the two parietal bones from the frontal bone of the skull. Structure The coronal suture lies between the paired parietal bones and the frontal bone of the skull ...
plus any other
suture Suture, literally meaning "seam", may refer to: Arts, entertainment, and media * ''Suture'' (album), a 2000 album by American Industrial rock band Chemlab * ''Suture'' (film), a 1993 film directed by Scott McGehee and David Siegel * Suture (ban ...
, like the
lambdoid The lambdoid suture (or lambdoidal suture) is a dense, fibrous connective tissue joint on the posterior aspect of the skull that connects the parietal bones with the occipital bone. It is continuous with the occipitomastoid suture. Structure Th ...
, or it may be used to describe the premature fusion of all sutures. It should be differentiated from
Crouzon syndrome Crouzon syndrome is an autosomal dominant genetic disorder known as a branchial arch syndrome. Specifically, this syndrome affects the first branchial (or pharyngeal) arch, which is the precursor of the maxilla and mandible. Since the branchial ...
. Oxycephaly (or acrocephaly) is a form of turricephaly where the head is cone-shaped, and is the most severe of the craniosynostoses.


Presentation


Common associations

It may be associated with: * 8th cranial nerve lesion *
Optic nerve In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual information from the retina to the brain. In humans, the optic nerve is derived fro ...
compression *
Intellectual disability Intellectual disability (ID), also known as general learning disability in the United Kingdom and formerly mental retardation, Rosa's Law, Pub. L. 111-256124 Stat. 2643(2010). is a generalized neurodevelopmental disorder characterized by signif ...
*
Syndactyly Syndactyly is a condition wherein two or more digits are fused together. It occurs normally in some mammals, such as the siamang and diprotodontia, but is an unusual condition in humans. The term is from Greek σύν, ''syn'' 'together' and δά ...


Diagnosis


Treatment


See also

*
Acrocephalosyndactylia Acrocephalosyndactyly is a group of autosomal dominant congenital disorders characterized by craniofacial (craniosynostosis) and hand and foot (syndactyly) abnormalities. When polydactyly is present, the classification is acrocephalopolysyndactyl ...


References


Further reading


NINDS Overview
*


External links

Congenital disorders of musculoskeletal system Rare diseases {{congenital-malformation-stub