Arthrogryposis Multiplex Congenita: Difference between revisions
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*Lower Extremity: Hip flexed, abducted, externally rotated, knee flexed, club foot | *Lower Extremity: Hip flexed, abducted, externally rotated, knee flexed, club foot | ||
*2 common variations of AMC (10) | *2 common variations of AMC (10) | ||
*Flexed and dislocated hips, extended knees, clubfeet, internally rotated shoulders, flexed elbows, and flexed and ulnarly deviated wrists | |||
*Abducted and externally rotated hips, flexed knees, clubfeet, internally rotated shoulder, extended elbows, and flexed and ulnarly deviated wrists | |||
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There are four basic groups that babies born with AMC can be divided into: | There are four basic groups that babies born with AMC can be divided into: | ||
#Those with only limbs affected | #Those with only limbs affected | ||
#Those with limbs and trunk involvement | #Those with limbs and trunk involvement | ||
#Those with limb, head/face and organ involvement | #Those with limb, head/face and organ involvement | ||
#Those with limb involvement and severe central nervous dysfunction.(1) | #Those with limb involvement and severe central nervous dysfunction.(1) | ||
<br>Other classification systems divide patients into three main groups: | <br>Other classification systems divide patients into three main groups: | ||
#primarily musculoskeletal involvement; | #primarily musculoskeletal involvement; | ||
#musculoskeletal involvement plus other system anomalies | #musculoskeletal involvement plus other system anomalies | ||
#musculoskeletal involvement plus CNS dysfunction and/or mental retardation (6)<br><br> | #musculoskeletal involvement plus CNS dysfunction and/or mental retardation (6)<br><br> | ||
Revision as of 20:57, 10 April 2016
Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Top Contributors - Casey Sisk, Jess Bell, Kim Jackson, Robin Tacchetti, Uchechukwu Chukwuemeka, Mande Jooste, Evan Thomas, Shelby White, Rucha Gadgil, 127.0.0.1, WikiSysop, Meaghan Rieke, Nupur Smit Shah, Elaine Lonnemann, Naomi O'Reilly, George Prudden and Priyanka Chugh
Definition/Description[edit | edit source]
Arthrogryposis Multiplex Congenita (AMC) is a condition that causes a newborn to have stiff, crooked, and contracted joints at birth that causes a loss of range of motion in more than one joint (hands, feet, hips, knees, elbows, shoulders, wrists, fingers, toes, the jaw and the spine). This lack of joint mobility is often accompanied by fibrous ankylosis, which is an overgrowth or proliferation of tissue in the joint.1 AMC is a non-progressive condition that is diagnosed at birth. The primary impairments of children diagnosed with AMC are decreased joint movement and decreased muscle strength and bulk 10
Prevalence[edit | edit source]
AMC affects roughly 1 in every 3,000 individuals and is a congenital condition present at birth. The condition affects males and females equally throughout its population. The ethnic tendencies are common in the Asian, African and European populations.
Out of those who have the disease, the incidence of specific deformities are as follows:
- Club foot - 1/500
- Congenital dislocated hips - 1/200 - 1/500
- All congenital contractures - 1/100 - 1/250
- Multiple contractures - 1/3000
Characteristics/Clinical Presentation[edit | edit source]
Clinical signs and symptoms of AMC include:
- Decreased or absent movement around small and large joints due to contractures
- Muscles of affected limbs are underdeveloped with decreased strength and bulk
- Long bones of the arms and legs are fragile
- Abnormally slender build
- Cleft palate
- Cognition may or may not be affected
- ⅓ of babies affected have structural or functional abnormalities of the CNS
- Usually painless to the child (4)
Most typical presentations of AMC: (5)
- Upper Extremity: Shoulder internal rotation, elbow extension, pronation, wrist ulnar deviation, hand fisted
- Lower Extremity: Hip flexed, abducted, externally rotated, knee flexed, club foot
- 2 common variations of AMC (10)
- Flexed and dislocated hips, extended knees, clubfeet, internally rotated shoulders, flexed elbows, and flexed and ulnarly deviated wrists
- Abducted and externally rotated hips, flexed knees, clubfeet, internally rotated shoulder, extended elbows, and flexed and ulnarly deviated wrists
There are four basic groups that babies born with AMC can be divided into:
- Those with only limbs affected
- Those with limbs and trunk involvement
- Those with limb, head/face and organ involvement
- Those with limb involvement and severe central nervous dysfunction.(1)
Other classification systems divide patients into three main groups:
- primarily musculoskeletal involvement;
- musculoskeletal involvement plus other system anomalies
- musculoskeletal involvement plus CNS dysfunction and/or mental retardation (6)
Associated Co-morbidities[edit | edit source]
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Medications[edit | edit source]
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Etiology/Causes[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
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