Synostosis (; plural: synostoses) is fusion of two or more
bone
A bone is a rigid organ that constitutes part of the skeleton in most vertebrate animals. Bones protect the various other organs of the body, produce red and white blood cells, store minerals, provide structure and support for the body, ...
s. It can be normal in
puberty
Puberty is the process of physical changes through which a child's body matures into an adult body capable of sexual reproduction. It is initiated by hormonal signals from the brain to the gonads: the ovaries in a female, the testicles i ...
(e.g. fusion of the
epiphyseal plate
The epiphyseal plate, epiphysial plate, physis, or growth plate is a hyaline cartilage plate in the metaphysis at each end of a long bone. It is the part of a long bone where new bone growth takes place; that is, the whole bone is alive, with ma ...
to become the
epiphyseal line), or abnormal. When synostosis is abnormal it is a type of
dysostosis. Examples of synostoses include:
*
craniosynostosis
Craniosynostosis is a condition in which one or more of the fibrous sutures in a young infant's skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpe ...
– an abnormal fusion of two or more
cranial bones;
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radioulnar synostosis – the abnormal fusion of the
radius
In classical geometry, a radius (: radii or radiuses) of a circle or sphere is any of the line segments from its Centre (geometry), center to its perimeter, and in more modern usage, it is also their length. The radius of a regular polygon is th ...
and
ulna
The ulna or ulnar bone (: ulnae or ulnas) is a long bone in the forearm stretching from the elbow to the wrist. It is on the same side of the forearm as the little finger, running parallel to the Radius (bone), radius, the forearm's other long ...
bones of the forearm;
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tarsal coalition – a failure to separately form all seven bones of the
tarsus (the hind part of the foot) resulting in an amalgamation of two bones; and
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syndactyly – the abnormal fusion of neighboring digits.
Synostosis within
joint
A joint or articulation (or articular surface) is the connection made between bones, ossicles, or other hard structures in the body which link an animal's skeletal system into a functional whole.Saladin, Ken. Anatomy & Physiology. 7th ed. McGraw- ...
s can cause
ankylosis.
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Clinical significance
Radioulnar synostosis is one of the more common failures of separation of parts of the upper limb. There are two general types: one is characterized by fusion of the radius and ulna at their proximal borders and the other is fused distal to the proximal radial epiphysis. Most cases are sporadic, congenital (due to a defect in longitudinal segmentation at the 7th week of development) and less often post-traumatic, bilateral in 60%, and more common in males. Familial cases in association with autosomal dominant transmission appear to be concentrated in certain geographic regions, such as
Sicily
Sicily (Italian language, Italian and ), officially the Sicilian Region (), is an island in the central Mediterranean Sea, south of the Italian Peninsula in continental Europe and is one of the 20 regions of Italy, regions of Italy. With 4. ...
.
The condition frequently is not noted until late childhood, as function may be normal, especially in unilateral cases. Increased wrist motion may compensate for the absent forearm motion. It has been suggested that individuals whose forearms are fixed in greater amounts of pronation (over 60 degrees) face more problems with function than those with around 20 degrees of fixation. Pain is generally not a problem, unless
radial head dislocation should occur.
Most examples of radioulnar synostosis are isolated (non-syndromic). Syndromes that may be accompanied by radioulnar synostosis include
X chromosome polyploidy (e.g.,
XXXY) and other chromosome disorders (e.g.,
4p- syndrome,
Williams syndrome
Williams syndrome (WS), also Williams–Beuren syndrome (WBS), is a genetic disorder that affects many parts of the body. Facial features frequently include a broad forehead, underdeveloped chin, short nose, and full cheeks. Mild to moderate int ...
),
acrofacial dysostosis,
Antley–Bixler syndrome,
genitopatellar syndrome,
Greig cephalopolysyndactyly syndrome,
hereditary multiple osteochondromas (
hereditary multiple exostoses),
limb-body wall complex, and
Nievergelt syndrome.
Craniosynostosis
Craniosynostosis is a condition in which one or more of the fibrous sutures in a young infant's skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpe ...
(from cranio, cranium; + syn, together; + ostosis relating to bone) is a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by turning into
bone
A bone is a rigid organ that constitutes part of the skeleton in most vertebrate animals. Bones protect the various other organs of the body, produce red and white blood cells, store minerals, provide structure and support for the body, ...
(ossification).
Craniosynostosis
Craniosynostosis is a condition in which one or more of the fibrous sutures in a young infant's skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpe ...
Craniosynostosis has following kinds:
scaphocephaly,
trigonocephaly,
plagiocephaly,
anterior plagiocephaly,
posterior plagiocephaly,
brachycephaly
Brachycephaly (derived from the Ancient Greek '' βραχύς'', 'short' and '' κεφαλή'', 'head') is the shape of a skull shorter than average in its species. It is perceived as a cosmetically desirable trait in some domesticated dog and ...
,
oxycephaly
Turricephaly is a type of cephalic disorder where the head appears tall with a small length and width. It is due to premature closure of the coronal suture plus any other Suture (anatomy), suture, like the Lambdoid suture, lambdoid, or it may be u ...
,
pansynostosis.
References
External links
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{{Authority control
Congenital disorders of musculoskeletal system