Amyoplasia Congenita Disruptive Syndrome

General

• Part of Arthrogryposis Multiplex Congenita
• Multiple joint contractures
• Two forms:
  • Neuropathic: reduced number of anterior horn cells; demyelinated motor roots; fewer axons in peripheral nerve roots.
  • Myopathic: contractors due to lack of intrauterine movement.

Airway

• Micrognathia
• Cleft palate

Respiratory

• Hypoplastic lungs
• Diaphragmatic hernia
• Hydrothorax

Gastrointestinal

• Gastroschisis
• Pyloric stenosis

Musculoskeletal

• Scoliosis
• Contractures
• Joint deformities

Considerations

• Difficult airway
• Positioning nightmare
• Difficult intravenous access possible
• No relationship between arthrogryposis syndromes and MH

Links

• Arthrogryposis Multiplex Congenita
• Guerin-Stern Syndrome
• Otto Syndrome
• Rocher-Sheldon Syndrome
• Rossi Syndrome
• Congenital Arthromyodysplastic Syndrome
• Myodysplasia Foetalis Deformans
• Myodystrophia Foetalis Deformans