03‐06‐14
Considerations orthopediques
Dystrophinopathies
Duchenne
 Becker

1
03‐06‐14
DMD
2
03‐06‐14
DMD
3
03‐06‐14
DMD

Peut-on prolonger la
marche par la chx ?
 Arret de la marche
○ Faiblesse musculaire
○ Contractures
articulaires
4
03‐06‐14

Chirugie precoce ( 4-6 ans )
 relachements flech hanches, ischios, TA +
transfert TP
○ Pas effets durable

Chirurgie > 10 ans
 Prolongation marche 1-2 ans
 Variable et imprevisible
 Steroides

Scher & Mubarak ( JPO 2002,22:384 )
 Chirurgies multiples
 Resultats variables
 Benefices < attentes ( familles )
 Chx pieds +
5
03‐06‐14
Chirurgie apres arret de la
marche

Fenetre temporelle etroite ( 4-6 mois )
 Fonte musculaire
 Contractures

Physio post-op +++
 Motivation
 Depression
 Obesite
6
03‐06‐14
Habituellement benefique
Apres arret de la marche
Platres 6 semaines
Ortheses tibiales de nuit
7
03‐06‐14
8
03‐06‐14
Muscular dystrophy
Wheel-chair at age 12
 Scoliosis 90% cases
 Collapsing spine

18
9
03‐06‐14
Muscular dystrophy

Spinal orthosis
poorly tolerated and
ineffective
19
Muscular dystrophy
Seating adjustments
 positioning

20
10
03‐06‐14
Muscular dystrophy
Progressive spinal
collapsus
 Pulmonary
insufficiency
 Inability to sit

21
11
03‐06‐14
Muscular dystrophy
(Pulmonary function)
23
Muscular dystrophy
24
12
03‐06‐14
Muscular dystrophy
 Scoliosis
 Indications for surgery
○ Curve > 30°
○ Wheel-chair
 Vital capacity > 30% predicted
values
25
13
03‐06‐14
Muscular dystrophy

Posterior
instrumentation
 Fusion to L5
 Fusion to pelvis
28
14
03‐06‐14
Posterior instrumentation
29
Luque Rods
30
15
03‐06‐14
Unit rod
31
Unit rod
sub laminar wires
32
16
03‐06‐14
Muscular dystrophy
33
17
03‐06‐14

Contractures
 Coudes
 Equinisme
 Scoliose

Cardiopathie
 Bloc cardiaque
 arythmies
18
03‐06‐14
Fusion scapulo-thoracique
19
03‐06‐14
Type 1 Wernig-Hoffman
Type 2 Infantile
Type 3 Kugelberg-Welander
ASP

Contractures
 Hanches
 Genoux
 Chevilles
Subluxation-luxation hanches
 Scoliose

20
03‐06‐14
ASP

Contractures
 Hanches
 Genoux
 Chevilles


Subluxation-luxation
hanches
Scoliose
ASP

Contractures
 Hanches
 Genoux
 Chevilles


Subluxationluxation hanches
Scoliose
21
03‐06‐14
ASP

Contractures
 Hanches
 Genoux
 Chevilles


Subluxationluxation hanches
Scoliose
22
03‐06‐14
ASP

Contractures
 Hanches
 Genoux
 Chevilles


Subluxation-luxation
hanches
Scoliose
23
03‐06‐14
24
03‐06‐14
25
03‐06‐14
26
03‐06‐14
27
03‐06‐14
28
03‐06‐14
29
03‐06‐14
30
03‐06‐14
CMT

Cavovarus pieds
31
03‐06‐14
Cavovarus

Pathophysiologie
 Intinseques




orteils griffes
Paresie jambier ant
chute 1er meta
Retraction fascia plantaire
Paresie peroniers varus avant-pied
Chute 1er meta
varus arriere-pied
32
03‐06‐14
Test de Coleman
Test Coleman
33
03‐06‐14
Cavovarus CMT

Chirurgie
 Corriger diffomites
○ Fascia plantaire
○ Osteotomie ext 1er meta
○ Osteotomie calcaneum
 Ameliorer imbalance musculaire
○ Transferts
 extenseurs orteils
 Jambier post.
 Long au court peronier
34
03‐06‐14
35
03‐06‐14
36
03‐06‐14
37
03‐06‐14
CMT Dysplasie hanche
38
03‐06‐14
Freidreich’s

Scoliose
 63-100%
 Idiopathic like
 Corset
○ mal tolere
○ Peu efficace
 Chx
Pieds creux
 Equinisme

39

Publikationsliste (17.3.2015) Dr. med. Georg

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16 Feb 2016 ～ 22 Feb 2016