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Arthrogryposis multiplex congenita AMCor simply arthrogryposisdescribes congenital joint contracture in two or more areas of the body. AMC has been divided into three groups: Amyoplasia is characterized by severe joint contractures and muscle weakness. Types artrogriposiis arthrogryposis artroggriposis a primary neurological or muscle disease belong to the syndromic group.
Intelligence is normal to above normal in children with amyoplasia,  but it is not known how many of these children have an above normal intelligence, and there is no literature available about the cause of this syndrome.
There are a few syndromes like the Freeman-Sheldon and Gordon syndrome, which have craniofacial involvement. These other diagnoses could affect any organ in a patient.
There are a few slightly more common diagnoses such as pulmonary hypoplasiacryptorchidismcongenital heart defectstracheoesophageal fistulasinguinal herniasartogriposis palateand eye abnormalities.
Arthrogryposis – Wikipedia
Research of arthrogryposis has shown that anything that inhibits normal joint movement before birth can result in joint contractures. The malformations of arthrogryposis can be secondary to environmental factors such as: Other causes could be: Myasthenia gravis of the mother leads also artrogeiposis rare cases to arthrogryposis. The major cause in humans is fetal akinesia.
Arthrogryposis could also be caused by intrinsic factors. This includes molecular, muscle- and connective tissue development disorders or neurological abnormalities. Research has shown that there are more than 35 specific genetic disorders associated with arthrogryposis. Most of those mutations are missensewhich means the mutation results in a different amino acid. Other mutations that could cause arthrogryposis are: Loss of muscle mass with an atrrogriposis of muscle power at the joint can lead to connective tissue abnormality.
This is mostly the result of abnormal function of the dystrophin – glycoprotein -associated complex in the sarcolemma of skeletal muscles. The underlying aetiology and pathogenesis of congenital contractures, particularly arthrogryposis and the mechanism of the mutations remains an active area of investigation, because identifying these factors could help to develop treatment and congenital finding of arthrogryposis. The treatment of arthrogryposis includes occupational therapyphysical therapysplinting and surgery.
Since arthrogryposis includes many different types, the treatment varies between patients depending on the symptoms. These surgeries are explained below. There are a number of passive devices for enhancing limb movement, intended to be worn to aid movement and encourage muscular development.
For example, the Wilmington Robotic Exoskeleton is a potential assistive device built on a back brace, shadowing the upper arm and forearm. It can be difficult to fit artrohriposis heavy and awkward to wear. Researchers at the University of Delaware are developing a light and unobtrusive therapeutic garment, suitable for babies and children, called the Playskin Lift.
The garment looks like normal clothing but contains bundled steel wires under the arms, which help to push the arms toward a lifted position while allowing the wearer to move freely from that position.
Children with the amyoplasia type of arthrogryposis usually have flexed and ulnarly deviated wrists. On the dorsal side, at the level of the mid carpusa wedge osteotomy is made.
Sufficient bone is resected to at least be artrogripisis to put the wrist in a neutral position. If the wrist is also ulnarly deviated, more bone can be taken from the radial side to correct this abnormality. This position is held into place with two cross K-wires. In addition, a tendon transfer of the extensor carpi ulnaris to the extensor carpi radialis brevis artrogtiposis be performed to correct ulnar deviation or wrist extension weakness, or both. This tendon transfer is only used if the extensor carpi ulnaris appears to be functional enough.
The soft tissue envelope in congenital contractual conditions such as multlple or arthrogrypotic thumbs is often deficient in two planes, the thumb-index web and the flexor aspect of the thumb. There is often an appearance multlple increased skin at the base of the index finger that aartrogriposis part of the deformity. This tissue can be used to resurface the thumb-index web after a comprehensive release of all the tight structures to allow for a larger range of motion of the thumb.
This technique is called the index rotation flap. The flap is taken from the radial side of the index finger. It is proximally based at the distal edge of the thumb-index web.
The flap is made as wide as possible, but still small enough to close with the excessive skin on the palmar side of the index finger. The flap is rotated around the tightest part of the thumb to the metacarpophalangeal joint of the thumb, allowing for a larger range of motion. Many other surgeries are also able to improve function in joints of arthrogryposis patients.
These surgeries usually exist out of multip,e transfers and skin flap movements, adjusted to the individual. People with AMC look their worst at birth. AMC is considered non-progressive, so with proper medical treatment, things can improve.
The joint contractures that are present will not get worse than they are at the time of birth. There is no way to completely resolve or cure AMC. But with proper treatment, most children make significant improvements in their range adtrogriposis motion and ability to move their limbs which enables them to do activities of daily life, and live relatively normal lives.
Therapeutic interventions that are cornerstone in the treatment of AMC include: Surgical intervention may also improve joint mobility and function. Arthrogryposis is a rare condition.
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Some authors say the overall prevalence is artrogripoiss in  and others say it is one in among European live births. From Wikipedia, the free encyclopedia.
Retrieved 11 July A Review and Update”. The Journal of Bone and Joint Surgery. Journal of Pediatric Orthopaedics. Long-term follow-up from birth until skeletal maturity”. Journal of Children’s Orthopaedics.
A report of two cases”. Australian and New Zealand Journal of Ophthalmology. A Review and Approach to Prenatal Diagnosis”.
Archived from the original on The Journal of Pediatrics. Confirmation of a new type of arthrogryposis”. Journal of Medical Genetics. Report of five patients from three Italian families”.
European Journal of Pediatrics.
Document Details | Artrogriposis multiple congenita
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