OVERVIEW OF CONGENITAL

MUSCULOSKELETAL DISORDER

Dicky Mulyadi, dr.SpOT(K)

Introduction
There are many kinds of congenital
musculoskeletal disorders
Need thorough examination and therapy
HISTORY TAKING
Prenatal History
Bleeding
Infection
Metabolic disease
Sexual Transmitted Disease
Medicine
Trauma
Fetal Movement
Labor History
Age of the pregnancy, labor duration, birth
weight and height. Baby presentation ( in the
breech position congenital dislocation of the
hips and congenital musculus torticalis is
frequently founded), spontaneous or induction
labor, APGAR score.
Development History
The steps of the development, included
physically and mentally.

Family History
It is needed to find a hereditery factor.
SALTER : insidency of congenital deformity
can be detected in the newborn is 3% and 6%
1 year old.
Physical examination
examination is done in undressed
and systematically

look-feel-move
HEAD

Shape and size of the head

Defect of the face

Open Fontanel

Baby is stimulated with sound and light

NECK

Swelling
(inspection and palpation)
Flexion-extension-deflection lateral- rotation

Congenital Deformity :
Torticolis Muscles
Klippel Feil Syndrome
Torticalis muscles
Klippel Feil Syndrome
UPPER LIMB

check the active movement of the upper limb

Shoulders
injury of the brachial plexus during labor
often happen, sometime can be related with
clavicle fracture that can cause pseudo
paralysis of the upper limb.
Craniocleidodisostosis
FOREARM
Normal flection
Extension - arthrogryposis multiplex congenital
Full supinasi dan pronasi radio-ulnar
synostosis

Dysplasia or no radial bone radial club

hand
Hand
normal fist position.

Thumb is flexion and the others extension:

Clasp Thumb and Trigger Thumb.

Another congenital deformity: Congenital

amputation, brachidactily, syndactily or
polydactily.
Syndactily
Polydactily
Spine
Check for birth mark, lump or hairy patch.

1.Brevi collis shape and position of

scapulae
2.Sprengels deformity
3.Spina bifida
4.Meningocele
5.Meningomyelocele.
Sprengels deformity
Lower Limb and Body Posture
First step look
Standing position and shape of the body from
forward, backward and sideward
See if there any :
Defect of the spine or deformities of the limb
Diminution of the normal curve spine
Pelvic inclination
Position of the head, scapulae,shoulders, gluteal and popliteal
Imbalanced shoulders
Deviation of the trunk
Scoliosis
Hip more prominent than the other
Spasm of para vertebrae muscles
Limitation of motion of the vertebral column
DDH
CTEV
Trendelenburg Test positive
weakness of hip abductor muscle
GAIT
The Gait cycle consist of two phases : Stance
and Swing
Muscles weakness of :
1. Gluteus Medius Trendelenberg Test (+)
2. Quadriceps Femoris difficult in climbing
stairs
3. Gluteus Maximus + Quadriceps Femoris
Patient can walk is by supporting the front of his
thigh with his hand
Quadriceps Paralysis
Deformity
Deformity is determined based on :
Type and actual site
Soft tissues, bones, joints
Severity
Fixed, passively or actively corrected
Muscle spasm
Local tenderness or pain on motion
 Test of deformity
a. Obers test
b. Thomas test
c. Galleazi test
d. Ellis test
Thomas Test
Obers Test
 Motor Power Muscle Testing
Muscle strength and power may be
classified as :
a. kinetic
b. static
Paresis or weakness impairment of
strength
Paralysis total loss of strength
DDH
(Developmental Dysplasia of Hip)
Epidemiology
0.7 to 35 per 1000 live births
Depends on geography
Depends on type of study
Retrospective studies tend to report
lower incidence
Screening programmes tend to report
higher incidence
Predisposing Factors

Female
Breech delivery
First born
Family history
Oligohydramnions
Other congenital abnormalities
Clinical Screening
Baby must be relaxed
Examiner must not use excessive force
1. Asymmetric Abduction
2. Barlows Sign: 3. Ortolani Sign: Clunk
Clunk of Dislocation of Entry
Provocative test Reduction maneuver
4. Galeazzi Sign
 Hip Ultrasonography
Graf (1980)
Harcke and Grissom (1990)
dynamic US
 Imaging
AP Pelvis
Useful after 6-8 months
Monitoring hip/acetabular development
Detection of complications
 Management Protocol

1-6 months
Pavlik harness for 3-4 weeks.
Closed surgical reduction if harness fails.
Management Protocol
Infancy
Pavlik Harnes
Problem:
Extreme position
Avascular necrosis
Tight shoulder straps
Nerve palsies
Femoral nerve palsy
extreme flexion
Uncomfortable
Inferior dislocation
2. Abduction Splint
Proper size
Full time for first 3
months
Confirm resolution with
USG/ X-ray
Closed reduction
Traction
Adductor tenotomy
Closed reduction and spica
Arthrogram is helpful in visualizing reduction

3 months casting, change at 6 weeks

Night hip abduction brace for 6 months
Treatment
6-18 months.
Closed or open surgical reduction
Hip spica cast

Some time we combined with

pinning for maintain reduction +
hip spica cast
Open Reduction

Indications
Failed closed after trial of casting
Interposition of soft tissue on arthrogram
Limbus shape on arthrogram
Age over 1 year without concentric reduction
Treatment
18 months - 8 years
Open reduction with femoral osteotomy or
/and pelvic osteotomy
Femoral osteotomy (Shortening)
Pelvic osteotomies
Primary acetabular dysplasia
Residual subluxation of the hip
Failure of gradual improvement of
radiographic dysplasia following reduction
of a dislocated hip
When necessary to stabilize reduction
during or after open reduction of the hip.
Salter Innominate Osteotomy
Complications
The most common complication of
treatment of DDH is osteonecrosis of the
femoral head
Growth disturbance of proximal femoral
physis
Gait abnormality
Club Foot/ CTEV
 Term describe a complex congenital contractural
malalignment bones & joints of the foot & ankle.

Deformities :
Hind foot : equinus
Subtalar joint : varus
Fore foot on hind foot : cavus
Fore foot on mid foot : adductus

Congenital talipes equinocavovarus

 FOREWORDS

CLUB FOOT : Aetiology

Controversies Pathology
Classification
Still Not well understood Treatment rationale
Assesment of results
Forewords
100.000 babies w/ CTEV each years
80 % developing countries
Mostly untreated or poorly treated
Cause of physical, social, psychological &
financial burden
Most often : otherwise a healthy child
Operative & Conservative Treatment
current trend : toward more conservative
Incidence
Varies widely (race & gender)
0.39 per 1000 (Chinese)
1.2 per 1000 (Caucasians)
6.8 per 1000 (Polynesians)
Bilateral : 50%
Unilateral : right > left
Male-to-female ratio of 2.5:14 (Lochmiller
et al)
etiology : still uncertain
Genetic
- 24.4% (+) family history, race variance
- Siblings: 30x risk
Intra Uterine factors
Uterine compression & oligohydramnios ( Hippocrates )
Asymmetrical positioning of the fetus in the womb (
Turco)
Interruption in the development of the normal foot
Histologic anomalies
Retraction fibrosis, primary germ defect, collagen
synthesis
Vascular anomalies
Absence of anterior tibial artery
Muscle anomalies
Clinical features
Clubfoot
Equinus Cannot corrected passively
Varus Severity varies

Cavus
Adductus

Posterior creases, empty heel, medial creases,

smaller calf, shorter leg (18%)1
Physical Examination
Examine the entire body !!!
Associated anomalies:
upper extremities, back, legs & abnormal reflexes
Information about the etiology
A standardized examination: performed initially
& after each interval of treatment
Clinical Features
 Pirani Severity Score
Lateral curve
Medial crease
Talar head coverage
Posterior crease
Rigid equinus
Empty heel

6 elements score : 0 0,5 18

Midfoot & Hindfoot score
Tenotomy if MS <1, HS > 1

Pirani S et al
 Radiographic Examination
Have limited role for initial diagnosis
Follow up after surgical management
Talocalcaneal angle
Anteroposterior view: 30 to 55 degrees
Dorsiflexion lateral view : 25 to 50 degrees
Tibiocalcaneal angle
Stress lateral view : 10 to 40 degrees
Talo-first metatarsal angle
Anteroposterior view : 5 to 15 degrees
Clubfoot management
Main purpose to gain :
- plantigrade
- supple Functional & cosmesis
- pain free

Management toward conservative

Ponseti technique : gold standard
Ponseti : 2 4 months ,70 % need Achilles tenotomy
Kite & Lovell : 22 24 months, 95 % without surgery
 Management
- Started as soon as possible, immediately
following birth
- First three weeks of life are the golden
period, because the ligamentous tissues are
still lacks under the influences of maternal
sex hormones
- Managements extends until adolescent
- Exam every 6 months until skeletal
maturity
Management
Conservative
Kite & Lovell technique
Ponseti technique(current gold standard)
Operative
Carrol
Cincinnati
Turco , modified Turco
Ala carte approach
Details of the Ponseti Technique
Details of the Ponseti Technique
Serial casting ( 4 5 or more )
Reduce the cavus
Manipulation
Locate head of talus
Stabilize the talus
Manipulate the foot
Reduce cavus
Positioning
forefoot
hindfoot
alignment
supination
until arch norm
Locate head of talus
Stabilize the talus, manipulate the foot
2nd, 3rd , 4th cast
Adductus & varus
corrected
Equinus gradually
improves w correction
of adductus & varus
Casting, Molding
Removal
Equinus correction & 5th cast
Bracing

Immediately after last cast removed

Unilateral : 75 degree affected side- 45 degree normal side
Bilateral : 70 degree
Worn : 24 hours 3 months after cast
12 hours at night + 4 hours at days until 3 4 years
Operative Management
Resistant clubfoot
Late presenting cases
Soft tissue and hard tissue procedure
a la carte approach
Various technique
Followed by casting for 3 6 weeks
Often produce stiff scarred ankle & foot
 Operative Treatment

Indication
Do not respond to conservative treatment
Significant rigid clubfoot deformity
Objectives
1. To achieve reduction of the dislocation or
subluxation of the talocalcaneonavicular joint
2. To maintain the reduction
3. To restore normal articular alignment of the
tarsus & the ankle
4. To establish muscle balance between the evertors
& invertors, and the dorsiflexors & plantarflexors
5. To provide a mobile foot with normal function &
weight bearing
Postero-medial release
Relaps Management
Relaps : 6 % for compliance pts, 80 % for
non compliance pts
Etiology : non-compliance for bracing
program
Management :
Re- casting for 3 4 times
Repeat bracing protocol
If necessary consider surgery
Thank you