Arthrogryposis- Information, Treatment & Prevention

by : Juliet Cohen



Arthrogryposis, too known as Arthrogryposis multiplex congenita, is an uncommon inborn disorder that causes dual multilateral contractures and is characterized by muscle failing and fibrosis. It is a non-progressive disease. The leading reason of arthrogryposis is fetal akinesia payable to fetal abnormalities or paternal disorders (eg, transmission, drugs, trauma, new paternal illnesses. The condition is presently used in link with a really heterogeneous group of disorders that all include the popular characteristic of dual inborn multilateral contractures. The main reason is persistently decreased fetal movements payable to either fetal or paternal abnormalities. Consanguinity increases the opportunity that both parents transport the same disease gene. Consanguinity is more popular in families with uncommon recessive diseases than in those with popular recessive diseases.

Arthrogryposis is more popular in isolated populations such as finland and the bedouin community in israel. During earlier embryogenesis, multilateral growth is nearly ever natural. Motion is vital for the natural growth of joints and their adjacent structures, lack of fetal campaign causes additional connective tissue to produce around the joint. Arthrogryposis is perceptible at birth or in utero using ultrasonography. Some chromosomal abnormalities dramatically increase with paternal age, and single-gene predominant mutations can increase with maternal age. Muscle abnormalities are comparatively uncommon causes of arthrogryposis. Some related diseases include inborn sinewy dystrophies, inborn myopathies, intrauterine myositis, and mitochondrial disorders. Amyoplasia is an intermittent circumstance and has not been observed in siblings or progeny.

In some cases, few joints may be affected and the scope of movement may be almost natural. In the almost popular character of arthrogryposis, hands, wrists, elbows, shoulders, hips, feet and knees are affected. There are numerous symptoms for this group of diseases. Some of the much popular signs and symptoms are associated with the shoulder, elbow (extension and pronation malformation), wrist, hand, pelvis, knee (flexion malformation) and foot. Other associated syndromes and conditions include focal femoral dysplasia, hand-muscle atrophy and sensorineural deafness, Kuskokwim syndrome, Larsen dysplasia, leprechaunism, nemaline myopathy, oculodentodigital syndrome, ophthalmomandibulomelic dysplasia, otopalatodigital syndrome, Pfeiffer syndrome, pseudothalidomide syndrome, sacral agenesis, tracheoesophageal.

While there is no cure, symptoms and deformities may still be alleviated with various methods due to multiple contractures and weakness. Early vigorous physical therapy to stretch contractures is very important in improving joint motion and avoiding muscle atrophy. Patients with amyoplasia or distal arthrogryposis respond well to physical therapy with excellent functional outcome. Splints can also help stretch joints, especially at night. Orthopedic surgery may also be able to relieve or correct joint problems. Joint manipulation during the first few months of life may produce considerable improvement. Orthotics may help. Wrist flexion deformities may be treated with tendon transfers and bony procedures to change the alignment of the wrist. Surgery may be needed later to align the angle of ankylosis, but mobility is rarely enhanced.