Craniosynostosis Health Dictionary

Craniosynostosis: From 1 Different Sources


n. premature fusion of some of the cranial bones, usually before birth, so that the skull is unable to expand in certain directions to assume its normal shape under the influence of the growing brain. Depending on which cranial *sutures fuse early, the skull may become elongated from front to back, broad and short, peaked (oxycephaly or turricephaly), or asymmetrical. Craniosynostosis is a feature of several related inherited disorders (see acrocephalosyndactyly). Compare craniostenosis.
Health Source: Oxford | Concise Colour Medical Dictionary
Author: Jonathan Law, Elizabeth Martin

Craniopharyngioma

A rare, non-hormone-secreting tumour of the pituitary gland. Symptoms of a craniopharyngioma may include headaches, vomiting, and defective vision. If a craniopharyngioma develops in childhood, growth may become stunted and sexual development may not occur. Craniopharyngiomas are usually removed surgically. Untreated, they may cause permanent brain damage. craniosynostosis The premature closure of one or more of the joints (sutures) between the curved, flattened bones of the skull in infants. If all the joints are involved, the growing infant’s brain may be compressed and there is a risk of brain damage from pressure inside the skull. If the abnormality is localized, the head may be deformed. Craniosynostosis may occur before birth and, in some cases, is associated with other birth defects. It may also occur in an otherwise healthy baby, or in a baby affected by a disorder such as rickets. If the brain is compressed, an operation may be performed to separate the fused skull bones.... craniopharyngioma

Fontanelle

One of the 2 membranecovered spaces between the bones of a baby’s skull. At birth, the skull bones are not yet fully fused, and 2 soft areas can be felt through the scalp. These are the anterior fontanelle, which is diamond-shaped and usually closes up by

age 18 months, and the posterior fontanelle, which is triangular and closes up within the first 2 months. It is normal for the fontanelles to become tense and bulge out when a baby cries. Persistent tension at other times may indicate an abnormality, particularly hydrocephalus (the accumulation of fluid in the skull). A sunken fontanelle may be a sign of dehydration. If a fontanelle is abnormally large, or takes a long time to close, the cause may be a brain abnormality or a disorder, such as rickets, affecting the skull bones. Early closure of the fontanelles results in a deformity called craniosynostosis.

Occasionally, a third fontanelle is present between the other 2; this occurs in Down’s syndrome.... fontanelle

Acrocephalosyndactyly

n. any one of a group of related inherited disorders, including *Apert syndrome, resulting in abnormalities of the skull (*craniosynostosis), face, and hands and feet (*syndactyly).... acrocephalosyndactyly

Apert Syndrome

a hereditary disorder characterized by *craniosynostosis, underdevelopment of the midfacial tissues resulting in a sunken facial appearance, and *syndactyly (fusion) of 2–5 digits (‘mitten glove’). Variable mental deficits and cleft palate may result. The condition may be associated with *Crouzon syndrome, in which case the fusion of the digits is less marked. See also acrocephalosyndactyly. [E. Apert (1868–1940), French physician]... apert syndrome

Craniostenosis

n. premature closing of the *sutures and fontanelles between the cranial bones during development, resulting in the skull remaining abnormally small. Compare craniosynostosis.... craniostenosis

Crouzon Syndrome

(craniofacial dysostosis) a genetic disorder characterized by premature fusion of the skull sutures, leading to distortion in the shape of the head. It is a generalized form of *craniosynostosis, with a wide skull, high forehead, widely spaced eyes (ocular *hypertelorism), and *exophthalmos.See also Apert syndrome. [O. Crouzon (1874–1938), French neurologist]... crouzon syndrome

Oxycephaly

(turricephaly) n. a deformity of the bones of the skull giving the head a pointed appearance. See craniosynostosis. —oxycephalic adj.... oxycephaly



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