Arthrogryposis

Mentor : Dr. dr. Adrian Khu, Sp. OT, FICS

Presentator : Irmayanti Ginting (133307010072)
Definition

Arthrogryposion comes from

Greek, namely "arthro,
namely joint and" Gryp "which
means curved. Can be
concluded Arthrogryposis is a
curved joint in many (multiple)
areas in the body that arise in
infancy (congenital)
 Comprises nonprogressive conditions charaterized by
multiple joint contractures found at birth
 1 in 3000 live births
 Multiple congenital contractures
Etiology
 Neuropathic abnormality
 Abnormality of muscle structure or function
 Abnormality of connective tissue
 Space limitation
 Maternal disease
 Impaired intrauterine or fetal vascularit
Classification

 Amyoplasia
This is primarily a muscle disorder that affects all four
extremities, usually symmetrically
 Distal Arthrogryposis
This is a group of genetic disorders, they differ from the
sporadic classic arthrogryposis in that their inheritance is
autosomal dominant.
Diagnosis
1. Laboratory studies:
a. General laboratory tests are seldom useful
b. Markers of the infectious process like cytomegalovirus,
coxsackievirus, enterovirus when AMC accompanies
intrauterine growth retardation, eye involvement, and
hepatosplenomegaly
c. Maternal antibodies of neurotransmitters when the
newborn presentation suggests myasthenia gravis.
2. Imaging studies
2. Imaging studies
Treatment
 Most children have good prognosis
 May be progressive but no new joints are involved
 Passive stretching exercise followed by serial splinting with
custom made thermoplastic splints
 Plantigrade standing and walking
Non-Surgical Treatment
 Occupational/ Physical Therapy
In some cases it may be possible to correct arthrogryposis to
some degree with therapy alone. Therapy may include
mobilization, casting, and splinting that would be ongoing in
addition to any other treatments
 Casting/splinting
The primary treatment for wrists, hands and clubfeet and often
the only treatment needed. It can be perfomed by me and/ or
therapists and may be utilized at all ages.
 Examples of orthotic management for upper and lower extremities’
deformations in children with AMC:
 A – wrist-hand orthosis (WHO) correcting palmar flexion contracture
 B – elbow and wrist orthosis increasing elbow flexion
 C – knee-ankle-foot orthosis (KAFO);
 D – KAFO used for walking improving knee active extension
Surgical management of the
lower limb

 The hip
- Contractures of the hip are present in nearly 90% of AMC
children
- Surgical management involves releases (transection) of
contracted soft tissues (including the rectus femoris and
sartorius muscles, the iliopsoas muscle, and the hip joint
capsule), or, in the older child, proximal femoral extension
osteotomy
 Knee
- Knee contractures are observed in up to 85% of AMC
patients and include flexion and extension contractures
Soft Tissue Release

In soft tissue releases, contractures are

relaxed by cutting the tight muscle, tendon, or
ligament which allows the limb or extremity to
move more freely
 Tendon transfer
• Tendons may be
Tendon moved from one part of
the body to another to
transfer correct the positioning
and enable movement
of a limb
Osteotomy
An osteotomy is a surgical procedure where a curved or
deformed bone is broken and reset. Internal pins or plates and
screws are utilized and at times external frame called “fixators”
are used to hold/guide the bone into the correct position and
proper alignment