Arthrogryposis and Amyoplasia - Alpha Hand Surgery...
Transcript of Arthrogryposis and Amyoplasia - Alpha Hand Surgery...
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Arthrogryposis and AmyoplasiaTerence Tay
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Terminology
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Arthrogryposis� Syndrome of non progressive joint
contractures present at birth � multiple congenital joint contractures !
� arthrogryposis multiplex congenita (AMC) Stern WG: Arthrogryposis multiplex congenita. JAMA
1923;81:1507-1510.
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Arthrogryposis� Encompasses a broad spectrum of
diseases, common phenotype of multiple congenital contractures
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Amyoplasia (A= no; myo=muscle; plasia=growth)
� Distinct form of arthrogryposis !
� Described by Hall et al !� characterized by quadrimelic involvement
and replacement of skeletal muscle by dense fibrous tissue and fat
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Amyoplasia� may not specifically describe one single group
of patients -sacral agenesis :-form of primary segmental amyoplasia in which there is an absence or greatly reduced number of muscle fibers in an otherwise normal extremity -a decrease in anterior horn cells and white matter, indicating a primary neurogenic cause, has been documented in the autopsy results of a patient with amyoplasia
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Epidemiology� Multiple congenital pathologic
contractures (arthrogryposis),occur in about 1 of every 3,000 live births
� amyoplasia occurs in 1 of every 10,000 live births
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InheritanceMay be inherited in several patterns � Autosomal dominant � Autosomal recessive � X-linked recessive � Sporadic � Mitochondrial � Can be caused by single gene defects
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Etiology� Fetal akinesia (limited fetal movement)
seems to be a common element in the development of most types of arthrogryposis
-oligohydramnios, ‘tight package’
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� Freeman-Sheldon syndrome � Has characteristic facial
features and condition affecting hands and feet !!
� Beals’ syndrome � Contractural
arachnodactyly with PIPJ flexion contractures
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Upper limb� Shoulder- adducted, internally rotated � Elbow – extended � Wrist – flex and ulnar deviated � Fingers- flex and thumb adducted
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Lower limb� Hip- adducted and flexed (30%- dislocated) Knee- flexed or extended/ dislocated Foot- equinovarus, congenital vertical talus
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General appearance� Scoliosis- 30% � Muscle mass reduced � Fusiform limb with no skin crease over joint � Webbing across joint � Loss of deep tendon reflexes
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Other things need to look for..� Hypoplasia of labial fold � Inguinal hernia � Crytorchism � Abdominal wall defect � Bowel atresia � gastroschisis
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Distal arthrogryposis� group of inherited diseases that primarily
involve the hands, feet, or both
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Distal Arthrogryposes� group of autosomal dominant disorders
that mainly involve the distal parts of the limbs !
� Categorized into 9 different groups � Classified by Hall et al and Goldberg and
later Bamshad et al
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Distal Arthrogryposis Type 1 (DA1)
� characterized largely by camptodactyly and clubfoot
� The shoulders and hips are less frequently affected.
� Isolated hypoplasia of the distal interphalangeal crease of the fifth digit to severely clenched fists and ulnar deviation of the wrist..
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Distal Arthrogryposis Type 2 (DA2)
� phenotypically similar to a condition called Freeman-Sheldon syndrome
� characterized by oropharyngeal abnormalities, scoliosis, and a distinctive face
� “whistling-face syndrome.”
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Distal Arthrogryposis Type 5 (DA5)
� individuals have ocular abnormalities � ptosis, restricted movement of the
extraocular muscles, and/or strabismus !
� pulmonary hypertension as a result of restrictive lung disease *recent findings
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Distal Arthrogryposis Type 7 (DA7)
� trismus-pseudocamptodactyly syndrome, TPS
� shortened hamstring muscles and short stature
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Distal Arthrogryposis Types 3, 4, and 6 (DA3, DA4, and DA6)
� Very rare � DA3, or Gordon syndrome , is
distinguished from other distal arthrogryposes by short stature and cleft palate. Hearing impairment
� DA4 has scoliosis, torticollis and cervical vertebrae fusion
� DA6 has sensorineural hearing loss
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Distal Arthrogryposis Type 8� Also called autosomal dominant pterigium
syndeome � Pterigium in the neck, axilla, elbow and
knee
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Distal Arthrogryposis Type 9� Contractural aracnodactyly or Beal
syndrome
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Central Nervous System Causes of Arthrogryposis� Developmental abnormalities affecting
the forebrain (e.g., hydranencephaly, microcephaly, or forebrain neuronal migration disorders), !
� joint contractures are probably due to diminished corticospinal tract activation of spinal cord motor neurons
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Example of neurological causes� X-linked spinal muscular atrophy, a
progressive motor neuron disease � infantile spinal muscular atrophy (Werdnig-
Hoffmann disease)
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Neuromuscular Causes of Arthrogryposis� Neuromuscular junction blockade in
fetuses carried by mothers with myasthenia gravis
� Congenital myopathies- mutations of genes that encode fetal skeletal-muscle myosin heavy chains !
� Electromyography is useful
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Treatment
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? Fixed Joint Contracture
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? Distal Arthrogryposis
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General Management� Individualized to each child’s needs Goals � Independent function i.e. for feeding � Increase ROM !Team approach between physicians and therapists
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Non operative treatment� Frequent passive movement of
all involved joints for increased mobilization
� Use of static progressive splints � Serial casting � Orthotics � These are most effective for
distal contractures, and not usually effective for contractures in amyoplasia
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Surgical Management� Recommended for fixed joint contractures
that preclude or interfere with upper-limb function
� Timing of surgery is controversial � Usually recommended before 4-5 years of
age to minimize compensatory movements and maximize school function
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Surgical correction of elbow contracture
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!!!
J Hand Surg 2012;37A:1078–1082. Copyright © 2012 by the American Society for Surgery of the Hand. All rights reserved
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Introduction� Loss of elbow flexion limits in function like
feeding and self care !
� Any surgery is dependent on preoperative level of contracture and limb function
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Selection of patient� Young patient, good triceps function � Pre op elbow flexion beyond 45 degree !
� When conservative management fails…..
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Surgical technique for posterior elbow release and humeral osteotomy
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First part of surgery 1. Release of posterior elbow � Posterior skin incision � Locate the ulnar nerve and preserve
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� Incise the tendon of the triceps in a distally based, V-shaped incision just distal to the musculotendinous junction.
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� Release posterior capsule � Take care not to release the main bands
of the medial or lateral collateral ligaments
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2nd part of surgery2. Humeral external rotational osteotomy- only when there is internal rotation of shoulder � elevate the triceps extraperiosteally off the
humerus in a medial to lateral direction, exposing the distal half of the medial diaphysis and the posterior and medial metaphysis
� Perform a transverse osteotomy
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3rd part of surgery3. Closure � Repair the triceps in a lengthened position
in a V-to-Y fashion with non absorbable braided suture
� Cast for 3-4 weeks and start active ROM
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� Able to achieve passive elbow flexion � Shoulder in good position to optimize hand
to mouth function
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Steindler Flexorplasty
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The Steindler Flexorplasty for theArthrogrypotic Elbow Charles A. Goldfarb, MD, Michelle S. Burke, BS, William B. Strecker, MD, Paul R. Manske, MD, St Louis, MO
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Steindler flexorplasty� Initially decribed to treat paralytic
condition !
� Transfer the proximal osseous origin of the wrist and finger flexor from medial epicondyle to more proximal and lateral to the humerus !
� Mayer and Green
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Surgical technique
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Steindler flexorplasty� Flexor pronator muscle identified !
� small wafer of the bony/cartilaginous medial epicondyle is separated in continuity with the muscle mass
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Eight elbows were treated with screw fixation; 4 with heavy, nonabsorbable sutures; 3 with metal sutures; and 2 with K-wire fixation
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Post op� Place in posterior splint that block
extension but allow passive flexion exercise for 4 weeks
� Start active ROM at 6weeks and strengthening at 3 months
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Criticism about this technique� Insufficient strength � Limit elbow extension and supination � Increased in wrist and finger flexion
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Other technique
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Tendon transfer� Triceps to Biceps Transfer � Pectoralis to Biceps Transfer � Latissimus Dorsi to Biceps Transfer
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Triceps to biceps transfer� Triceps can be transferred with minimal
morbidity because gravity can assists elbow extension
� Muscle strength of grade 4 Van Heest et al, Williams
� Lack of power in extension can prohibits crutch walking
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Pectoralis to Biceps Transfer
� Unipolar vs bipolar transfer � not always aesthetically pleasing !- unipolar transfer adducts the arm
substantially and creates an abnormally large anterior axillary fold
!- bipolar transfer is a more complex procedure that deprives the arm of an adductor and can lead to significant scarring
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Latissimus Dorsi to Biceps Transfer
� Similar fashion with bipolar pectoralis transfer
� Good ROM arc and no flexion contracture -reported by Van Heest el al � hypoplastic in some cases of
arthrogryposis
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Literature review of all technique
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Capsular release and triceps lengthening
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Triceps to biceps transfer
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Pectoralis transfer
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Steindler flexorplasty
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Arthrogryposis Wrist and hand
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Classical features � symmetric positioning of the limbs � wrist flexion, and hand ulnar
deviation � digits are postured in flexion and
are stiff � contracted clasped thumb � devoid of skin creases, muscle
wasting, paucity of skin crease
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Treatment� Non surgical management !
� passive movement of all involved joints !
� Static progressive splinting and prolonged stretch
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Surgical treatment� timing of surgery � proximal row carpectomy � dorsal wedge osteotomy of the distal
radius or mid-carpus, � soft tissue distraction � arthrodesis
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Dorsal Carpal Wedge Osteotomy in theArthrogrypotic Wrist
Ann E. Van Heest, MD, Rudy Rodriguez, MDJ Hand Surg 2013;38A:265–270. Copyright © 2013 by the American Society for Surgery of the Hand
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Surgical technique� Ezaki and Carter
� A dorsal wedge osteotomy at the level of the midcarpus
!� place with 2 cross K-wires !
� May be coupled with tendon transfer, ECU, ECRB
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After surgery� The limb is immobilized in a long arm splint
for 6 weeks !
� The osteotomy site is protected with intermittent wearing of a splint for a minimum of 3 months
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Arthrogryposis� describe position of limb � single or multiple joints � distal or proximal involvement or both � presence of webbing, joint crease, muscle
bulk � look for associated syndrome eg beals,
freeman- sheldon, etc by checking for archnodactyly, ocular involvement, whistling facies
95
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Conclusion� Identify the problem � Think of non surgical management first !
� Surgical treatment come last
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Thank you