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IN THE NAME OF ALLAH, THE MOST
BENEFICENT, THE MOST GRACIOUS
AND THE MOST MERCIFUL!
1
ARTHROGRYPOSIS
MULTIPLEX
CONGENITA
PREPARED BY: -
M.SHAYAN
shayanadnan00@yahoo.com
2
WHAT IS ARTHROGRYPOSIS?
• Arthrogryposis means a child is born with joint
contractures. this means some of their joints don’t
move much and may even be stuck in 1 position.
• often the muscles around these joints are thin,
weak, stiff or missing.
• extra tissue may have formed around the joints,
holding them in place. about 1 baby in 3,000 is
born with arthrogryposis.
• Most contractures happen in the arms and the
legs. They can also happen in the jaw and the
spine. 3
• Arthrogryposis does not occur on its own. It is a feature of many other conditions, most
often amyoplasia (lack in the newborn of muscular development and growth).
• Children with arthrogryposis may have other health problems, such as problems with
their nervous system, muscles, heart, kidneys or other organs, or differences in how
their limbs, skull or face formed.
• This condition is also called arthrogryposis multiplex congenita. “Arthrogryposis” means
the joints are curved or crooked. “Multiplex” means it affects more than 1 joint.
“Congenita” means the condition is present at birth.
4
ARTHROGRYPOSIS IN CHILDREN
• Each child with arthrogryposis is different. In some children, the condition is mild. It
affects only a few joints, and these joints have almost as much movement as normal. In
other children, the condition is more serious. It affects more joints and restricts their
movement more. In extreme cases, arthrogryposis affects nearly every joint.
5
TYPES OF ARTHROGRYPOSIS
• Distal arthrogryposis affects only a few joints, usually in the hands and feet. At least 10
different forms of distal arthrogryposis have been identified including
 Freeman-Sheldon syndrome.
 Gordon syndrome.
 Trismus-pseudocamptodactyly syndrome.
 Multiple pterygium syndrome
 Sheldon-hall syndrome.
6
WHAT ARE THE CAUSE OF ARTHROGRYPOSIS
MULTIPLEX CONGENITA?
• AMC is thought to be related to decreased movement in utero, which can have multiple
causes.
• Neurologic and muscle problems may well be the most common causes of decreased
fetal movement, but connective tissue disorders, maternal illness, and limited space are
also common causes.
7
ETIOLOGY
• It usually occurs due to absence of active fetal movements (akinesia), normally
appearing in the eighth week of fetal life.
• Fetal akinesia lasting over 3 weeks may be sufficient to result in absence of normal
stretching of muscles and tendons acting on the affected joints.
• Consequently fetal akinesia leads to fibrosis and contractures of the affected joints
determined by the passive position of the limb.
• The direct etiological factor causing akinesia in humans remains unknown, but a
number of abnormalities can be found.
8
SYMPTOMS OF ARTHROGRYPOSIS
• Thin, weak (atrophied), stiff or
missing muscles.
• Stiff joints due to extra tissue
(fibrosis or fibrous ankylosis)
• Differences in the skin around their
joints, such as webbing.
9
ARTHROGRYPOSIS DIAGNOSED
• During a routine ultrasound, abnormal limbs may be noted, but no definitive cause can
be ascertained until further tests can be run.
• Clinical and physical exam by orthopedic provider, including imaging studies such as an
x-ray, CT scan or MRI
• Blood work is typical, especially genetic testing which would be completed by a genetics
specialist.
• An electromyography will be performed to make a distinction between myopathic and
neurogenic arthrogryposis.
10
TREATMENT FOR ARTHROGRYPOSIS
• NON-SURGICAL TREATMENT : -
 Physical and occupational therapy
• SURGICAL TREATMENT : -
 Typically, surgical correction is necessary for the correction of clubfeet, knee contractures, hip
dislocations or in upper limbs to correct deformities that limits or interferes with function.
11
COMPLICATIONS ASSOCIATED WITH
ARTHROGRYPOSIS:
• Clubfeet
• Dislocated hips
• Facial asymmetry
• Facial birthmarks
• Hand contractures
• Internally rotated shoulders and limbs
• Intestine, kidney, bladder and genital malformation
• Muscle absence or atrophy
• Respiratory problems and aspiration of fluids
• Scoliosis 12
13

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Arthrogryposis multiplex congenita

  • 1. IN THE NAME OF ALLAH, THE MOST BENEFICENT, THE MOST GRACIOUS AND THE MOST MERCIFUL! 1
  • 3. WHAT IS ARTHROGRYPOSIS? • Arthrogryposis means a child is born with joint contractures. this means some of their joints don’t move much and may even be stuck in 1 position. • often the muscles around these joints are thin, weak, stiff or missing. • extra tissue may have formed around the joints, holding them in place. about 1 baby in 3,000 is born with arthrogryposis. • Most contractures happen in the arms and the legs. They can also happen in the jaw and the spine. 3
  • 4. • Arthrogryposis does not occur on its own. It is a feature of many other conditions, most often amyoplasia (lack in the newborn of muscular development and growth). • Children with arthrogryposis may have other health problems, such as problems with their nervous system, muscles, heart, kidneys or other organs, or differences in how their limbs, skull or face formed. • This condition is also called arthrogryposis multiplex congenita. “Arthrogryposis” means the joints are curved or crooked. “Multiplex” means it affects more than 1 joint. “Congenita” means the condition is present at birth. 4
  • 5. ARTHROGRYPOSIS IN CHILDREN • Each child with arthrogryposis is different. In some children, the condition is mild. It affects only a few joints, and these joints have almost as much movement as normal. In other children, the condition is more serious. It affects more joints and restricts their movement more. In extreme cases, arthrogryposis affects nearly every joint. 5
  • 6. TYPES OF ARTHROGRYPOSIS • Distal arthrogryposis affects only a few joints, usually in the hands and feet. At least 10 different forms of distal arthrogryposis have been identified including  Freeman-Sheldon syndrome.  Gordon syndrome.  Trismus-pseudocamptodactyly syndrome.  Multiple pterygium syndrome  Sheldon-hall syndrome. 6
  • 7. WHAT ARE THE CAUSE OF ARTHROGRYPOSIS MULTIPLEX CONGENITA? • AMC is thought to be related to decreased movement in utero, which can have multiple causes. • Neurologic and muscle problems may well be the most common causes of decreased fetal movement, but connective tissue disorders, maternal illness, and limited space are also common causes. 7
  • 8. ETIOLOGY • It usually occurs due to absence of active fetal movements (akinesia), normally appearing in the eighth week of fetal life. • Fetal akinesia lasting over 3 weeks may be sufficient to result in absence of normal stretching of muscles and tendons acting on the affected joints. • Consequently fetal akinesia leads to fibrosis and contractures of the affected joints determined by the passive position of the limb. • The direct etiological factor causing akinesia in humans remains unknown, but a number of abnormalities can be found. 8
  • 9. SYMPTOMS OF ARTHROGRYPOSIS • Thin, weak (atrophied), stiff or missing muscles. • Stiff joints due to extra tissue (fibrosis or fibrous ankylosis) • Differences in the skin around their joints, such as webbing. 9
  • 10. ARTHROGRYPOSIS DIAGNOSED • During a routine ultrasound, abnormal limbs may be noted, but no definitive cause can be ascertained until further tests can be run. • Clinical and physical exam by orthopedic provider, including imaging studies such as an x-ray, CT scan or MRI • Blood work is typical, especially genetic testing which would be completed by a genetics specialist. • An electromyography will be performed to make a distinction between myopathic and neurogenic arthrogryposis. 10
  • 11. TREATMENT FOR ARTHROGRYPOSIS • NON-SURGICAL TREATMENT : -  Physical and occupational therapy • SURGICAL TREATMENT : -  Typically, surgical correction is necessary for the correction of clubfeet, knee contractures, hip dislocations or in upper limbs to correct deformities that limits or interferes with function. 11
  • 12. COMPLICATIONS ASSOCIATED WITH ARTHROGRYPOSIS: • Clubfeet • Dislocated hips • Facial asymmetry • Facial birthmarks • Hand contractures • Internally rotated shoulders and limbs • Intestine, kidney, bladder and genital malformation • Muscle absence or atrophy • Respiratory problems and aspiration of fluids • Scoliosis 12
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