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Arthrogryposis Multiplex Congenita

Codes

IDC-10: Q74.3

ORPHA: 1037

Report from the Questionnaire Report from the Observation Chart

General information

Estimated occurrence
1:4,500 live births.
Cause
There are more than 150 different diseases within this diagnosis and the genetic cause varies. The etiology may be classified as:
  • Muscular causes (various congenital muscular diseases).
  • Neurogenic causes (deformities of the central nervous system, loss of motor neurons in the spinal marrow, loss of peripheral nerves).
  • Connective tissue diseases.
  • Reduced mobility of the fetus owing to insufficient space in the uterus (lack of amniotic fluid, twin pregnancy, heart-shaped uterus).
  • Maternal illness during pregnancy.
General symptoms
Stiffness in several joints is the common denominator. The disease is not progressive; on the contrary treatment may result in improvement. It is common for these children to be born with one dislocated hip and a club foot.

Orofacial/odontological symptoms

The jaw-joints may also be affected, which results in a restricted ability to open the jaws. An overjet and open bite in the region of the front teeth, a narrow, high palate and a small lower jaw all occur. Many individuals have restricted oral motor skills, which affects their ability to suck, chew, swallow, and speak. Drooling and teeth grinding occur frequently.

Advice on follow-up and treatment

  • It is important that individuals with Arthrogryposis Multiplex Congenita receive dental preventive treatment, with extra prophylactic care and information on oral hygiene from an early age.
  • When the jaws do not open properly, the function of the jaw joint should be investigated, and appropriate treatment thereafter prescribed (such as jaw-stretching exercises or surgery).
  • Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
  • Teeth grinding should be followed up, and be managed with a splint when necessary.
  • Training in oral motor skills and extra stimulation in cases of eating disorders, speech difficulties and drooling.

Sources


Updated: 0001-01-01 00:00