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Orthopedic Congenital /

Pediatric Deformities
Class of Medical Program 7th Semester Lecture
Dr. Komang Agung I S, dr.,Sp.OT (K)

Suchong Softworks
Lecture Topics
Congenital torticollis (IIIA)
Congenital Talipes Equino Varus (IIIA)
Developmental Dysplasia of the Hip (IIIA)
Scoliosis (IIIA)
Text Book
Appley System of orthopaedic and fractures
Lovell Pediatric orthopedi
The Art and Practice of Childrens Orthopaedics
Congenital Muscular Torticalis
Congenital torticollis

What is the definition of torticollis?


Torticollis or wry neck is a deformity where the
chin is twisted upwards and towards one side.

Solomon, Louis et al., 2010


Definition Tortous = twist collum = neck

Congenital Muscular
TORTICOLLIS (CMT)
is :
unilateral contracture
of sterno.cleido-
mastoid muscle (s.c.m.)
the head tilted toward
the contracture side
the chin rotated toward
the opposite side
head and face :
asymmetric deformity
Etiology
Fibrosis s.c.m. contracture, shortens
fail to elongate as the child grows
progressive deformity
Cause of fibrosis?
local ischaemia : distorted position in utero
birth history : breech, forcipal delivery
note : sectio caesaria : CMT (+)
compartment syndrome of s.c.m.
75% R-side
CMT with DDH = 1 in 5 CMT (Hummer & Mc Ewen)
20% with lump (tumor)
Congenital torticollis
Pathophysiology
The sternocleidomastoid muscle on one side is
fibrous and fails to elongate as the child grows.
As a result, a progressive deformity develops.

Solomon, Louis et al., 2010


Congenital torticollis
Pathophysiology

Solomon, Louis et al., 2010


Congenital torticollis
Etiology
The cause is unknown but a history of difficult
labor or breech delivery is common.
The muscle may have suffered ischemia from a
distorted position in utero.
Injury during birth is also possible.

Solomon, Louis et al., 2010


Congenital torticollis
Pathogenesis
A lump could be noticed in the first few weeks of
life. The lump is well-defined and involves one
or both heads of the sternomastoid.
At first, there is no deformity or obvious
limitation of movement (within a few months
the lump would disappear)
The deformity would become apparent at the age
of 1-2 years old.

Solomon, Louis et al., 2010


Clinical findings
Deformity :
torticollis may present at birth
or (2-3) weeks age
Head tilted to the contracted muscle
Chin rotated to opposite side
Eyes-shoulder lines : not parallel
Clinical findings
Lump :
Appear at (2-3) weeks
Slowly regress in (6-12) weeks after which the torticollis
become apparent
Face and head
face flattened by external pressure-plagycephally, related to
the sleeping position
prevention : not to sleep in prone position
by growth : face asymmetry increase
Neck (posterior view)
Scoliosis lower C-upper D
Clinical findings
Congenital torticollis
Diagnosis
The head is tilted to one side, so that the ear
approaches the shoulder, the sternomastoid on
that side may feel tight and hard.
Plagiocephaly (asymmetrical development of the
face) may also be apparent as the child grows
History of the patient should be assessed,
radiographs of the patient should also be taken
to exclude bone abnormalities or fracture.

Solomon, Louis et al., 2010


Congenital torticollis
Diagnosis
The cause is unknown but a history of difficult
labor or breech delivery is common.
The muscle may have suffered ischemia from a
distorted position in utero.
Injury during birth is also possible.

Solomon, Louis et al., 2010


Differential Diagnosis of CMT

1. Congenital Anomalies :
postural toricollis
hemivertebrae, cervical-superior dorsal spine
unilateral atlanto-occipital fusion
Klippel-Feil syndrome
unilateral cong.absence of s.c.m.
Pterygium colli
2. Trauma : mainly C1,C2, rotatory subluxation, fracture
3. Inflammatory conditions-unilateral
Cervical lymphadenitis
Cervical spine infection = tb, pyogenic
Rheumatoid arthritis (Juvenile)
Spontaneous hyperaemic subluxation of the atlas
Congenital torticollis
Treatment
Early diagnosis during infancy is proven to be
beneficial as daily stretching by the parents may
prevent incipient deformity.
If the condition persists beyond a year, operative
correction is required.
The contracted muscle is divided and head is
manipulated into a neutral position.
After operation, correction must be maintained
with a temporary rigid orthosis followed by
stretching exercises
Solomon, Louis et al., 2010
Treatment
Conservative :
manipulation
passive stretching
each stretching halted for 10 seconds (15-20) x
a day (4-6) x
by physiotherapist or by parents
result (85-90)% success
Operative
+ 10% fail by stretching
Age > 1 year
recurrent
Technic :
unipolar
bipolar
1874 : William Cheseldon : tenotomy s.c.m.
Treatment
Complications :
lesion of spinal accessory nerve
recurrent :
incomplete release
post op tx : indiscipline
operation at late age
cosmetics :
scar
no s.c.m. (band)
Note : Avoid injury of carotid vessels
n.accessorius
n. phrenicus
n.hypoglossus
Operative technic :
1 case monopolar (Z plasty)
14 cases : bipolar
Postoperative :
Glisson traction
Sand pillows lateral of the head
Diazepam
2 days after surgery : stretching [7 days after
surgery : stitches off]
Collar, 1 cm elevated on the affected side. To be
used for 3 months
Continue stretching programme
Thank you
Early Diagnosis
Early Management
Minimize possibility to do
surgery
Congenital Talipes Equinovarus
What is the definition of Talipes Equinovarus?
Talipes => Talus (Latin = Ankle Bone) and Pes
(Foot)

Talipes Equinovarus: The heel is in equinus, the entire


hindfoot in varus, the midfoot and the forefoot
adducted and supinated.

*equinus: upward bending motion of the ankle joint is


limited
*varus: inward angulation of the distal segment of
bone or joint
Solomon, Louis et al., 2010
34

CTEV
Talipes = talus + pes
ankle + foot
Equinus = horse like
= heel in plantar
flexion
Varus = inverted + adducted
Phatoanatomy
Bone, joint, soft tissue

Talus
Talonaviculare joint
Talocalcaneal
Calcaneocuboid joint
Muscle contracture
36

Dx. at birth = 3 basic deformation :


Forefoot = Adduction
Hindfoot = inversion =varus
Hindfoot = Equinus
Forefoot = Cavus (Ponseti)
Congenital Talipes Equinovarus
Etiology
The cause is unknown, however resemblance to
other disorders suggest several possible
mechanisms:
- Germ Defect
- A form of arrested development
- Neuromuscular disorder
- Associated with arthrogryposis
- Postural deformity caused by a tight packing in an
overcrowded uterus

Solomon, Louis et al., 2010


Congenital Talipes Equinovarus
Pathophysiology
The neck of the tallus points downwards and
deviates medially, whereas the body is rotated
slightly outwards in relation to both the calcaneum
and the ankle mortise.
The posterior part of the calcaneum is held close to
the fibula by a tight calcaneo-fibular ligament, and is
tilted into equinus and varus; it is also rotated
medially beneath the ankle.
The navicular and entire forefoot are shifted
medially and rotated into supination.
Solomon, Louis et al., 2010
Congenital Talipes Equinovarus
Pathophysiology

Solomon, Louis et al., 2010


Congenital Talipes Equinovarus
Pathogenesis
The skin and soft tissues of the calf and the medial
side of the foot are short and underdeveloped, if the
condition is not corrected early, secondary growth
changes occur in the bones, which are permanent.

Even with treatment, at this stage, the foot is liable
to be short and the calf may remain thin.

Solomon, Louis et al., 2010


Congenital Talipes Equinovarus
Diagnosis
At birth: both feet are turned and twisted inward,
so that the sole faces posteromedially.
The heel is usually small and high
Deep creases appear posteriorly and medially, some
the creases are incomplete constriction bands.
There is a limitation in dorsiflexion and eversion, in
severe cases, the deformity is fixed.
Examine other associated disorders (Congenital
Hip Dislocation, spina bifida, etc.)

Solomon, Louis et al., 2010


Differential Diagnosis
AMC
Agenesis tibia
Constriction band
Spina bifida
Sacral agenesis

Pucking syndrome
Pucking Syndrome
44

Dorsofleksion and abduction


Clinical Pictures
Congenital Anomaly
Sreeter disease (constriction band)
Spina bifida
Spina Bifida
Spina Bifida
51

Sacral Agenesis
Agenesis Tibia
Failure of formation
Congenital Talipes Equinovarus
Diagnosis

Clinical Diagnosis
X-Ray AP, Lateral
View After 3 months

Solomon, Louis et al., 2010


Congenital Talipes Equinovarus
Treatment
Conservative
Should begin early
(within a day or two
after birth)
Serial Plastering by
Ponsetti technique
Physiotherapy

Surgical

Solomon, Louis et al., 2010


Treatment
Konservatif mo
Serial Plastering
Gradual stretching
3 mo
Operatif
ATL
PMR/CSTR
Combined soft tissue + Bone/
Triple Arthrodesis
Serial Plastering / Ponsetti technique
Serial Plastering
Serial Plastering
Surgery in neglected case
Neglected case
Pasca Operasi CTEV
Neglected case
Thank you
Developmental Dysplasia of The Hip
What is the definition of
Developmental Dysplasia of
The Hip?
A spectrum of disorders
including acetabular dysplasia
without displacement,
subluxation, and dislocation.

Teratological forms of
malarticulation leading to
dislocation are also included.

Solomon, Louis et al., 2010


Developmental Dysplasia of the HIp
Abnormal development of the hip with
1.) Instability (joint dislocatability) and
2.) Dysplasia or abnormal shape of the acetabulum
Most hip instability resolves shortly after birth
Unresolved instability is often painless and not
obvious since a dislocated hip may function well for
many years
Unresolved, it can lead to pain (often knee pain),
abnormal gait, functional disability and
degenerative hip disease
These problems are preventable if it is caught and
treated early
DDH
Hip laxity and immature acetabula are common in
the first few weeks of life
Approximately 90% of these infants will have
stabilized by 2 months of age after time for further
growth
After school age, the likelihood of spontaneous
resolution is very low
Clinical Picture / Neglected
X Ray DDH
Dislocation R Hip
Normal Left Hip
Risk factors
Breech presentation
Female gender
Family history
Factors related to tight positioning in utero (can see
associated torticollis and metatarsus adductus
Developmental Dysplasia of The Hip

Etiology
Genetic Factors
Hormonal Factors
Intrauterine Malposition
Postnatal Factors

Solomon, Louis et al., 2010


Developmental Dysplasia of The Hip

Pathogenesis and Pathophysiology


Birth
Hip is unstable but normal in
shape, the capsule is stretched
and redundant.
Infancy
The Femoral head dislocates
posteriorly, but with the
extension of hips, it comes to lie
first posterolateral and then
superolateral to the acetabulum.
The cartilaginous femoral head
is normal in size but the bony
nucleus appears late and the
ossification is delayed
throughout infancy.
Solomon, Louis et al., 2010
Developmental Dysplasia of The Hip

Pathogenesis and Pathophysiology


Infancy
The capsule is stretched and the teres ligament
become elongated and hypertrophied.
After weight bearing commences
The changes are intensified
Pressure of the femoral head induces a false
socket to form above the shallow acetabulum
The capsule develops an hourglass appearance
Surrounding muscles become shortened.
Solomon, Louis et al., 2010
Risk factors
Girls with a breech presentation 12 percent
Girls with a positive family history 4.4 percent
Boys with a breech presentation 2.6 percent
Girls 1.9 percent
Boys with a positive family history 0.9 percent
Clinical Features
The earlier DDH is detected, the easier and more
successful the treatment
Presentations vary based on age
Newborn: hip instability
Infant: reduced abduction
Toddler: asymmetric gait
Adolescent: activity related pain
Adult: osteoarthritis
Physical Exam
Hips should be checked at every visit until the
child is walking normally
Exam technique uses adduction and posterior
pressure to feel for dislocation and abduction
and elevation to feel for reduction
Hip Instability: The sensation of dislocation or
reduction is best described as a clunk or a jerk
Clicks and pops not associated with a palpable
clunk are very common and not worrisome
Exam
Examine each hip individually while the
child is calm and not crying
Examine on a stable surface with the
child supine and with hips flexed 90
degrees in neutral rotation
Sign of DDH
DYSPLASIA
Hyperlordotic in Hip problem

PHYSICA
L
EXAM
Exam
Galeazzi test: dislocated hip may
be displaced posteriorly so the
knee appears lower/shorter
Look for asymmetry in skin
folds
Gait asymmetry
Bilateral DDH can be a
challenge- look for widening of
the perineum, symmetric
limited abduction, and short
thigh segments relative to the
child's size. Once the child
begins to walk, hyperlordosis
and a waddling Trendelenburg
gait can be seen
Exam
Bilateral DDH:
widening of the perineum
symmetric limited abduction
short thigh segments relative to the child's size.
Once walking, hyperlordosis and a waddling
Trendelenburg gait
By 3 months: hip often stabilizes and tests for
instability are no longer very helpful
Imaging
Ultrasound
Plain radiographs- limited value early on due to
femoral heads cartilaginous and not ossified
Radiographs should be with hips flexed 20 to 30
degrees, or neutral if the child is older
Management
If abnormal exam:
Referral to orthopedic surgeon
Imaging:
Ultrasound if less than 5 months
Radiograph if >4 months
Breech females: screening is recommended-
ultrasound at 6 weeks or radiograph at 4 months.
This is optional for lower risk groups.
Treatment
Based on age
Abduction splints in younger kids (usually <6
months)
Closed reduction- goal is to reduce the hip, then
keep it stabilized in spica cast
Open reduction
If closed reduction is unsuccessful
Often needed in kids >18 months
Risks vs benefits
Follow up is important; complications, failure
Pavlik Harness
Look
Gallaezis sign
x ray
Developmental Dysplasia of The Hip

Diagnosis
Ideally, best to be diagnosed at birth (ask if
theres a family history of congenital dysplasia)
Ortolanis test
Barlows test
USG
Plain X-Ray (more useful after 6 months)

Solomon, Louis et al., 2010


Ortholani and Barlow test
Adduction/ dislocate
Abduction / reduced
Length of limb
Length of femur
Congenital Torticalis
X Ray DDH
Developmental Dysplasia of The Hip

Treatment/Management
First 3-6 months: Splintage
Persistent 6-18 months: Closed reduction,
splintage, operation
Persistent 18 months 4 years: Traction,
Arthrography, Operation, Splintage

Solomon, Louis et al., 2010


Operative Treatment
Thank you
Recognized earlier
Reduced by closed technique
Retain by Pelvic Harness

Congenital Anomaly : late surgery to improve


function
Solomon, Louis et al. Apley's System Of
Orthopaedics And Fractures. London:
Hodder Arnold, 2010. Print.

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