Arthrogryposis

Joint contractures that develop before birth (prenatally) and are evident at birth (congenitally). A newborn with arthrogryposis lacks the normal range of motion in one or more joints. In normal embryonic development, the joints begin to develop by about 5 to 6 weeks of gestation. There are joint spaces by 7 weeks, and the limbs can be seen moving by 8 weeks. This motion of joints is clearly essential to the proper development of the joints and structures around the joints. Limitation of joint motion before birth leads to joint contractures. Prenatal limitation of joint mobility can result from: {{}}Neurologic deficits: Including anencephaly, defects of the spine such as spina bifida (meningomyelocele), and nerve deficiencies. Muscle deficits: Including failure of muscle development (agenesis of muscle), fetal diseases of muscle (fetal myopathies), myotonic dystrophy, and myasthenia gravis. Connective tissue and skeletal defects: Including fusion of bones (synostosis), failure of a joint to develop, prenatal fixation of a joint, excess laxity and of dislocation of joints, and fixation of soft tissue around the joint. Fetal crowding or constraint: Not enough room for the fetus to move around freely in multiple births. Fetal constraint occurs from lack of amniotic fluid (oligohydramnios) due to underproduction of fluid by the fetal kidneys, failure to swallow and recirculate the fluid, or chronic leakage of fluid. Arthrogryposis means crooking of the joint. It comes from the Greek "arthro-" (joint) + "gryposis " (crooking).

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Congenital defect of the limbs characterized by severe contractures of multiple joints. [arthro- + G. gryphosis, a crooking]

- a. multiplex congenita limitation of range of joint motion and contractures present at birth, usually involving multiple joints; a syndrome probably of diverse etiology that may result from changes in spinal cord, muscle, or connective tissue. Several forms exist, autosomal dominant [MIM*108110, 108120, 108130, 108140, 108145, 108200], recessive [MIM*208080, 208081, 208085, 208100, 208150, 208155, 208200], and X-linked [MIM*301830] SYN: amyoplasia congenita.

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ar·thro·gry·po·sis .är-(.)thrō-gri-'pō-səs n

1) congenital fixation of a joint in an extended or flexed position

2) any of a group of congenital conditions characterized by reduced mobility of multiple joints due to contractures causing fixation of the joints in extension or flexion

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n.

congenital defects of limbs characterized by contractures, both flexion and extension, causing fixed deformities of the joints if untreated. Arthrogryposis multiplex congenita is a congenital disease with multiple causes in which there are contractures, stiff joints, and absent skin creases, as a result of the muscles being replaced by fibrous tissue. Treatment is by manipulation and splintage of deformed joints; surgical correction is sometimes required.

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ar·thro·gry·po·sis (ahr″thro-grə-poґsis) [arthro- + gryposis] persistent flexure or contracture of a joint.