Arthrogryposis is defined as a group of neurologic, muscular, and

connective tissue disorders that are characterized by multiple severe

bony deformities and joint contractures in newborns, which can

cause muscle weakness and fibrosis. This disease affects 1 in 3000

newborn children. (Dimitraki et al., 2011; Seattle Children’s Hospital, 2016)

Diagnosis

X-ray• Always include x-ray of spine &

pelvis

• Bony Abnormalities

• Missing bones

• Skeletal dysplasias

• Scoliosis

• Fractures from difficult delivery

Ultrasound• Fixed contractures & lack of mobility

• Abnormal limb positioning

• Club feet

• Scoliosis

CT & MRI• Assess muscle mass

• Abnormal Tissues in CNS

Electromyogram• Measures activity of muscles at rest

and during contraction

Nerve Conduction Studies• Measures how efficiently nerves

send signals to muscles in the body(Seattle Children’s Hospital, 2016)

Causes

•Neurologic: CNS, Spinal cord

•Muscular: Tendons, Bones, Joints

•Connective Tissue: Fibrosis

Intrinsic Factors

•Family History

•Maternal Illness: Rubella, Diabetes Mellitis

•Trauma

•Drug Abuse: Alcohol, Smoking, Drugs

Extrinsic Factors

• The actual cause of

AMC is unknown

• The main contributing

factor is fetal akinesia

• Fetal akinesia results

from both intrinsic and

extrinsic factors(Kalampokas et al., 2012; Seattle Children’s Hospital, 2016)

Types

• Most common type

• 25-30% of affected individuals

• Affects both arms & legs

• Lack muscular development &

range of motion in joints

• Deformation of joints

• Affects internal organs as well as

muscles & joints

• Breathing and feeding

complications

• Speech disorders

• Developmental delays

• Generally affects only a few

joints, usually in the hands & feet

• Limited range of motion

Amyoplasia

Syndromic

Distal

Symptoms

• Thin, weak, stiff, or missing muscles

• 2 or more joint malformations

• Curving or bowing of the bones above or below affected

joints

• Stiff joints due to fibrosis

• Webbing of the skin around the joints(Hall, 2013; Kalampokas et al., 2012; Seattle Children’s Hospital, 2016)

Treatments

Surgical

Open Reduction• Return dislocated joint to

proper place

• Performed in older children or

children whose joints do not

move back with closed

reduction

Tenotomy• Surgical cutting of tendon

• Releases tight tendon

• Most often used for clubfoot

Osteotomy• Surgical cutting of bone to

shorten or lengthen

• Realigns bone above and

below affected tendon

Capsulotomy• Surgical cutting through joint

capsule if it restricts any

movement

Non-Surgical

Closed Reduction• Non-surgical procedure to set a

bone into proper position

• Allows bone to grow normally

Physical/Occupation al Therapy• Begin at birth and continue

throughout the lifetime

• Improves strength and flexibility

Casts & Braces• Used after reduction procedure

or osteotomy

• Further corrects joint damage

Genetic Counseling• Aids in support for affected

individuals and their families(Hall, 2013; Seattle Children’s Hospital, 2016)

Prognosis

• Not a progressive disorder

• Typically have normal speech and learning capabilities

• Ability to live a successful and rewarding life