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Tuesday, July 19, 2011

Amyoplasia Congenita Syndrome

Name: 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

References:

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