Congenital multiple arthrogryposis

Congenital multiple arthrogryposis is characterized by multiple contractures of the joints (especially the upper limbs and neck) and amyoplasia, usually without other serious congenital anomalies. Intelligence is relatively normal.

[1], [2], [3], [4], [5], [6]

What causes congenital multiple arthrogryposis?

Any condition that leads to impaired fetal movements in the prenatal period (for example, malformations of the uterus, multiple pregnancies, oligohydramnios), can lead to congenital multiple arthrogryposis (BMA). Congenital multiple arthrogryposis is not a genetic disease, although in some genetic diseases (for example, type I spinal muscular atrophy, trisomy on the 18th chromosome), the probability of arthrogryposis is increased. Congenital multiple arthrogryposis can be a consequence of neurogenic diseases, myopathies or connective tissue diseases. It is suggested that the causes of concomitant amyplasia are congenital myopathies, diseases of the neurons of the anterior horn of the spinal cord and myasthenia gravis in the mother.

Symptoms of congenital multiple arthrogryposis

Deformations are evident even at birth. Congenital multiple arthrogryposis does not progress; however, the cause that caused the development of the WMA (for example, muscular dystrophy) may progress. In the affected joints, a flexural or extensor contracture is formed. The shoulders are usually brought and are in a state of internal rotation, the elbows are unbent, and the wrists and fingers are in a state of flexion. There may be a dislocation of the hip joints, as a rule, the legs in the hip joints are slightly bent. Knees are unbent; Equinovarus stops are often noted. The muscles of the legs are usually hypoplastic, the limbs tend to be cylindrical and completely even. Sometimes there is thinning, membranous soft tissue from the ventral side of the joints with flexion contractures. The child may have scoliosis. With the exception of the thinning of the long bones, the skeleton is radiographically normal. Physical disorders can be severe, disabling. Intellect is usually not broken or slightly reduced.

Other anomalies that rarely accompany arthrogryposis include microcephaly, cleft palate, cryptorchidism, heart and urinary tract defects.

Diagnosis of congenital multiple arthrogryposis

The examination should include a careful search for a combination of anomalies. For the diagnosis of neuropathy and myopathies, you can use electromyography and muscle biopsy. With muscle biopsies, amioplasia is usually found with fatty and fibrous tissue replacement.

[7], [8], [9], [10]

Treatment of congenital multiple arthrogryposis

Congenital multiple arthrogryposis requires an early referral to the orthopedist and physiotherapist. Exercises and physiotherapy with joints during the first months of life can lead to a significant improvement. Can be effective use of orthopedic devices. The operation may be required later to reduce ankylosis, but an increase in the volume of movements is rarely observed. Moving muscles (for example, surgical movement of the triceps so that it can bend the arm at the elbow) can improve the function. Many children feel good; 2/3 are outpatient patients after treatment.