Congenital deformities

Lecture
Congenital
Muscular
Torticollis
 Congenital muscular torticollis (CMT)
is caused by fibromatosis within the
sternocleidomastoid muscle. A mass
either is palpable at birth or becomes
so, usually during the first 2 weeks. It is
mo-re common on the right side than
on the left side. It may involve the
muscle diffusely, but more often it is
localized near the cla-vicular
attachment of the muscle
 Various hypotheses of the cause of
CMT include malposition of the fetus
in utero, birth trauma, infection, and
vascular injury
 CMT holds second place in congenital
deformities in children, average of 5-
12%.
 Any permanent torticollis slowly
becomes worse during growth.
 The head becomes inclined toward the
affected side and the face toward the
opposite side.
 If the deformity is severe, the
ipsilateral shoulder becomes elevated,
 conservative treatment
 conservative treatment is indicated
during infancy. The parents should be
instructed to stretch the sternocleido-
mastoid muscle by manipulating the
child's head manually.
conservative treatment
 conservative treatment
Congenital Muscular Torticollis
 did not resolve spontaneously if it
persisted beyond the age of 1 year
 exercise program is more likely to be
successful if the restriction of motion is
less than 30 degrees and there is no
facial asymmetry
 Nonoperative therapy after the age of 1
year is rarely successful.
 severe deformity could and should
be prevented by surgery during
early childhood.
 Surgery performed before the age
of 6 to 8 years may allow
remodeling of any facial asymmetry
and plagiocephaly.
 Usual Surgery performed in age
after 2 years
 Оpen tenotomy of the
sternocleidomastoid
muscle could be followed
by tethering of the scar to
the deep structures, re-
attachment of the clavi-
cular head or the sternal
head of the sternocleido-
mastoid muscle,
 clamp SCM muscle
and resect 2.5 cm of
their inferior ends.
S urgical correction in children with severe
deformity operation usually requires a bipo-
lar release of the sternocleidomastoid muscle.
 A, Skin incisions. B,
mastoid attach-ment
of SCM mus-cle is
cut, and Z-plasty is
performed on
sternal origin.
 C - Completed
operation;
 Postoperative period

 Physical therapy, consisting of stretching,

muscle strengthening, and active range-of-
motion exercises, is instituted in the early
postoperative period. Head-halter traction
or a cervical collar also can be used during
the first 6 to 12 weeks after surgery
 Postoperative period
 The use of plaster casts or braces usually is
unnecessary
 At 1 week physical therapy, including
manual stretching of the neck to maintain
the overcorrected position, is begun.
Manual stretching should be continued
three times daily for 3 to 6 months;
Congenital Elevation of
Scapula
(Sprengel Deformity)
 Clinical manifestation

• The scapula lies more

superiorly than it should
in relation to the thoracic
cage and usually is hypo-
plastic and misshapen.
 Clinical manifestation
• Other congenital anomalies may be
present such as cervical ribs,
malformations of ribs, and
anomalies of the cervical vertebrae
(Klippel-Feil syndrome);
 Clinical manifestation
• if the deformity is
severe, the scapula is
very small and can be
so elevated that it
almost touches the
occiput.
• The patient's head often is deviated
toward the affected side. Sometimes an
extra ossicle is present;
this is a rhomboidal pla-
que of cartilage and
bone that extends from
the superior angle of
the scapula to the spi-
nous process.
 Operative treatment
• If deformity and impairment are mild,
no treatment is indicated;
• if they are more severe, surgery may be
indicated
• An operation to bring the scapula
inferiorly to near its normal position
can be attempted after the child is
about 3 years old.
 Operative treatment
• The earlier surgery is performed
after 3 years of age, the better the
results, because the operation
becomes more difficult as the child
grows.
• In older children an attempt to
bring the scapula inferiorly to its
normal level can injure the brachial
plexus.
 Operative treatment
• Some surgical techniques are commonly
used
• Surgical release of muscles from the scapula
along with excision of the supraspinatus
portion of the scapula and any omoverteb-
ral bone.
• The scapula is then moved inferiorly to a
more normal position and the muscles are
reattached
Skin has been incised in midline. Origins of
trapezius and of rhomboideus major and
minor have been freed from spinous proces-
ses, and have been retracted laterally. Any
omovertebral bone, is to be excised.
 Scapula and attached sheet of muscles have
been displaced inferiorly, and aponeuroses of
trapezius and rhomboids have been
reattached to spinous processes at more
inferior level. D - free upper and below edges
have been overlapped and sutured in place.
• State and function
after operative
treatment
Congenital Anomalies of
Lower Extremities
 Congenital Clubfoot
(Talipes Equinovarus)
Congenital Clubfoot (Talipes
Equinovarus) The incidence of con-
genital clubfoot is approximately 1 in
every 1000 live births.
Bilateral deformities occur in 50%
of patients.
 Etiology
 Several theories have been proposed
regarding the cause of clubfoot. One is
that a primary germ plasm defect in
the talus causes continued plantar
flexion and inversion of this bone, with
subsequent soft tissue changes in the
joints and musculotendinous
complexes.
 Etiology
 Another theory is that primary soft
tissue abnormalities within the
neuromuscular units cause secondary
bony changes. Clinically, children with
clubfoot have a hypotrophic anterior
tibial artery in addition to the obvious
atrophy of the musculature about the
calf
 Clinical manifestation
 The three basic components of club-

foot are equinus, varus, and adduction

deformities. However, the deformity
varies in severity; and the condition
may be much less severe, with the foot
being in only a mild equinus and varus
position
 Late clinical manifestation
 Clubfoot is accompanied by internal
tibial torsion. The ankle, midtarsal, and
subtalar joints all are involved in the
pathological process.
 If the clubfoot is allowed to remain
deformed, many other late adaptive
changes occur in the bones.
Врожденная косолапость
 Late clinical manifestation

 These changes depend on the severity

of the soft tissue contractures and the
effects of walking. In untreated adults,
some of the joints may spontaneously
fuse, or they may develop degenerative
changes secondary to the contractures.
 NONOPERATIVE
TREATMENT

 The initial treatment of clubfoot is nonope-

rative. Various treatment regimens have
been proposed, including the use of correc-
tive splinting, taping, and casting.
Treatment consists of weekly serial
manipulation and casting during the first 6
weeks of life, followed by manipulation and
casting every other week until the foot is
clinically and roentgenographically
corrected
Congenital club foot
- Treatment of club foot by staging elastic
dressing.
- by staging plaster casts
- Orthopaedic shoes
 NONOPERATIVE
TREATMENT
 The order of correction by serial
manipulation and casting should be as
follows: first, correction of forefoot
adduction; next, correction of heel varus;
and finally, correction of hindfoot equinus.
Correction should be pursued in this order
so that a rocker-bottom deformity will be
prevented by dorsiflexing the foot through
the hindfoot rather than the midfoot.
 NONOPERATIVE TREATMENT
 If the clubfoot is corrected by the time the
child is 6 months of age, this should be
documented by both the clinical
appearance and repeated anteroposterior
and dorsiflexion lateral stress
roentgenograms. The foot can be placed in
an ankle-foot orthosis that can be used
part-time on children with compliant
families.
 OPERATIVE TREATMENT
 The more rigid the initial deformity,
noncorrectedinitial deformity the
more likely that surgical treatment
will be required
 Operative treatment
 Surgery in clubfoot is indicated for

deformities that do not respond to con-

servative treatment by serial manipu-
lation and casting.
Surgery in the treatment of clubfoot
must be tailored to the age of the child
and to the deformity to be corrected.
 club foot - Operative treatment
elongation of elongation
- incisions tendones of heel tendon,
capsulotomy
 Triple arthrodesis and
talectomy generally are salvage
operations for uncorrected
clubfoot in older children and
adolescents
Triple arthrodesis and talectomy generally
are salvage operations for uncorrected
clubfoot in older children and adolescents
- Area between
broken lines repre-
sents amount of
bone
removed from mid-
tarsal region and
subtalar joint in mo-
derate fixed defor-
Congenital and Developmental
Dysplasia of Hip (DDH)
or
Congenital dislocation of Hip
(DDH)
 Congenital dysplasia of the hip generally
includes subluxation (partial dislocation)
of the Congenital Anomalies femoral
head, acetabular dysplasia, and complete
dislocation of the femoral head from the
true acetabulum.
 The incidence of congenital dysplasia of
the hip has been estimated to be
approximately 1 in 1000 live births
 Etiology
 Several theories regarding the cause of
congenital dysplasia of the hip have
been proposed, including mechanical
factors, hormone-induced joint laxity,
primary acetabular dysplasia, and
genetic inheritance. Breech delivery,
with the mechanical forces of abnormal
flexion of the hips, can easily be seen as
a cause of dislocation of the femoral
head.
 An increased incidence of congenital
dysplasia of the hip has been reported
in cultures that place infants in
swaddling clothes with the hip in
constant extension.
 And decreased incidence of congenital
dysplasia has been reported in
cultures that place infants with the hip
in constant abduction (African people)
 In a newborn with true congenital
dislocation of the hip the femoral head
can be dislocated and reduced into and
out of the true acetabulum.
 In an older child the femoral head
remains dislocated and secondary
changes develop in the femoral head
and acetabulum
 clinical presentation
 The clinical presentation of congenital
dysplasia of the hip varies according to the
age of the child.
 In newborns (up to 6 months of age) it is
especially important to perform a careful
clinical examination because
roentgenograms are not absolutely reliable
in making the diagnosis of congenital
dysplasia of the hip in this age group.
 clinical presentation
Routine clinical screening should include
both the Ortolani test and the provocative
maneuver of Barlow.
The provocative maneuver of Barlow detects
any potential subluxation or posterior
dislocation of the femoral head by direct
pressure on the longitudinal axis of the
femur while the hip is in adduction. Both
these tests require a relaxed and pacified
child
 clinical presentation
 The Ortolani test is performed by gently
abducting and adducting the flexed hip to
detect any reduction into or dislocation of
the femoral head from the true acetabulum.
 clinical presentation
 As the child reaches the age of 6 to 18
months, several factors in the clinical
presentation change. Once the femoral head
is dislocated and the ability to reduce it by
abduction has disappeared, several other
clinical signs become obvious. The first and
most reliable is a decrease in the ability to
abduct the dislocated hip because of a
contracture of the adductor musculature
newborn 7 months
 Clinical signs of congenital dislocation
of hip in 13-month-old girl
 Decrease in abduction of right hip with
adduction contracture.
 Asymmetrical skin folds are
commonly mentioned as a sign to look
for, but unfortunately this sign is not
always reliable because normal children
may have asymmet-
rical skin folds, and
children with disloca-
ted hips may have
symmetrical folds.
Positive Galeazzi
sign with apparent
shortening of right
lower extremity.
 The Galeazzi sign is noted when the
femoral head becomes displaced not
only laterally but also proximally,
causing an apparent shortening of the
femur on the side of the dislocated hip
Bilateral dislocations may appear
symmetrically abnormal
In a child of walking age
with an undetected
dislocated hip, families
describe a "waddling"
type of gait, indicating
dislocation of the
femoral head and a
Trendelenburg gait
pattern.
 Although roentgenograms are not
always reliable in making the diagnosis
of congenital dysplasia of the hip in
newborns, screening roentgenograms
may reveal any acetabular dysplasia or
a teratological dislocation.
 As a child with a dislocated hip ages
and the soft tissues become contracted,
the roentgenograms become more
reliable and helpful in the diagnosis
and treatment
X-ray of the child 3 months old with hip
dysplasia
X-ray of the normal hip joints of the
child 3 months old
X-ray of hip joints of helthy child in
1 year old
Schemes for X-ray interpretation
The most commonly used lines of reference
are the vertical line of Perkins (b) and the
horizontal line of Hilgenreiner (A). Both used
to assess the position of the femoral head.
Reference lines for the evaluation of the
acetabulum include the acetabular index
and the center edge (CE) angle of
Wiberg.
 The acetabular index (ά) in a newborn
generally is 30 degrees or less. Any
significant increase in this measurement
may be a sign of acetabular dysplasia.
In addition, the Shenton line will be
disrupted in an older child with a dislo-
cated hip.
 the superior gap (distance between the
proximal metaphysis and Hilgenreiner's
line) and the medial gap (distance between
the femoral calcar and the lateral pelvic
wall at that level)
Normally the metaphyseal beak of the
proximal femur lies within the inner
lower quadrant of the reference lines
noted by Perkins and Hilgenreiner.
Treatment
 The treatment of
congenital or
developmental
dysplasia of the hip is
age related and
tailored to the specific
pathological condition
 Treatment
Five treatment groups related to age
have been designated:
 (1) newborn, birth to 6 months of age,
 (2) infant, 6 to 18 months of age,

 (3) toddler, 18 to 36 months of age,

 (4) child and juvenile, 3 to 8 years of age,

 (5) adolescent and young adult, beyond 8

years of age.
Newborn (Birth to 6 Months of Age)

 From birth to approximately 2 - 2.7

months of age, treatment is directed at
stabilizing the hip that has a positive
Ortolani or Barlow test or reducing the
dislocated hip with abducted sweddling
or abducted pamperses
Newborn (Birth to 6 Months of Age)

During the first few

months of life
Children have been
treated in the Pavlik
harness.
 When properly applied and
maintained, the Pavlik harness is a
dynamic flexion abduction orthosis
that can produce
excellent results in the
treatment of dysplastic
and dislocated hips in
infants approximately 4
to 6 months of age.
A roentgenogram of the patient in the
harness can help to confirm that the
femoral neck is directed toward the
triradiate cartilage.
After several weeks
of harness wear,
when the hip feels
stable clinically,
ultrasound is helpful
to confirm reduction
of the hip.
The Pavlik harness should be worn full-time
until stability is attained, as determined by
negative Barlow and Ortolani tests.
 If any of patterns of dislocation or
subluxation persist for more than 3 to 6
weeks, treatment in the Pavlik harness
should be discontinued and a new
program initiated; in most patients,
this consists of optional traction, closed
or open reduction, and casting.
Infant (6 to 18 Months of Age)
 Once a child reaches crawling age
(approximately 4 to 6 months), success
with the Pavlik harness decreases
significantly. A child aged 6 to 18
months with a dislocated hip probably
will require either closed manipulation
or open reduction.
 Infant (6 to 18 Months of Age)

 Children in this age group often are

seen initially with a shortened
extremity, limited passive abduction,
and a positive Galeazzi sign. If the child
is walking, a Trendelenburg gait may
be present.
Roentgenographic changes include
delayed ossification of the femoral
head, lateral and proximal
displacement of the femoral head, and
a shallow, dysplastic acetabulum.
 Infant (6 to 18 Months of Age)
 Treatment in this age group should
follow a standard regimen, which
includes adequate preoperative
traction, adductor tenotomy, and
closed reduction and arthrogram or
open reduction in children with a failed
closed reduction.
 Preoperative traction, adductor
tenotomy, and gentle reduction with an
acceptable "safe zone" are especially
helpful in the prevention of avascular
necrosis of the femoral head.
Preoperative traction
 Lower limb trac-
tion in upright
direction. I position

 Lower limb
traction in level
direction.
II position
Gentle closed reduction is accomplished
with the child under general anesthesia.

 Adductor Tenotomy. A percutaneous or

open adductor tenotomy under sterile
conditions can be performed for a mild
adduction contracture.
 The findings of the clinical examination
and of arthrography at the time of
attempted closed reduction determine
if the hip will be stable or may require
open reduction.
A, Developmental dislocation of left hip in 5-
month-old girl.

B - Intraoperative
arthrogram of
irreducible left hip
showing excessive
pooling of dye.
 Application of Hip Spica


After confirmation of a stable reduction, a

hip spica cast is applied with the hip joint in
95 degrees of flexion and 40 to 45 degrees of
abduction. This "human position" as best
for maintaining hip stability and
minimizing the risk of avascular necrosis.
 The final view of the spica with about 40 to
45 degrees of abduction and 95 degrees of
flection. Excessive abduction should be
avoided.
 Spica cast immobilization is
continued for 4 months.
- The cast can be changed at 2 months
with the patient under general anesthesia.
Roentgenograms can be obtained to be sure
that the femoral head is reduced anatomi-
cally into the acetabulum. Clinical and
roentgenographic follow-up is essential
until the hip is considered normal. CT
scanning is useful in the postoperative
assessment of reduction.
A - Anteroposterior roentgenogram of pelvis
obtained with patient in spica cast after
closed reduction. Note difficulty in assessing
position of femoral head.

B - CT scan of pelvis to
confirm bilateral
reduction of femoral
head into true
acetabulum.
 Open Reduction
 In children in whom efforts to reduce a
dislocation without force have failed,
open reduction is indicated to correct
the offending soft tissue structures and
to reduce the femoral head
concentrically in the acetabulum.
Anterior open reduction in congenital
dislocation of hip. A, incision. B, Division of
sartorius and rectus femoris tendons. C-D, T-
shaped incision of capsule, capsulotomy of
hip. E-F, Reduction and capsulorrhaphy
Roentgenograms or CT scans can be used
to confirm reduction of the femoral head
into the acetabulum.
 The cast is worn for 10 to 12 weeks.
 If roentgenograms show satisfactory
position of the hip 4 to 6 weeks after
surgery, the portion of the cast below the
knee is removed to allow knee motion and
some hip rotation.
After removal of the
total cast, an abduction
brace is worn full-time
for 4 to 8 weeks;

Then it is worn only at

night and during naps
for 1 to 2 years, until
normal acetabular
development is evident
 Toddler (18 to 36 Months of Age)
 Because of widespread screening of new-
borns, it is becoming less common for con-
genital dysplasia of the hip to go undetected
beyond the age of 1 year. The older child
with this condition has a wide perineum,
shortened lower extremity, and hyperlor-
dosis of the lower spine as a result of femo-
ropelvic instability.
 For these children with well-established
hip dysplasia, open reduction with
femoral or pelvic osteotomy, or both,
often is required.
These techniques are described on Infant
(6 to 18 months of age), and Toddler (18 to 36
months of age) respectively,
Scheme of technique for open reduction,
primary femoral shortening, and Salter
osteotomy. A, Segment of femur is resected.
B, Proximal femur is abducted, capsule is
incised, head of femur is reducted. C, Salter
osteotomy is com-
pleted with graft
in place.
Femoral frag-
ments are fixed
with pediatric hip
screws
 Solter′s
osteotomy
 Juvenile or Child (3 to 8 Years
of Age)
 The management of untreated congenital
dislocation of the hip in a child over 3 years
of age is difficult. By this age adaptive
shortening of the periarticular structures
and structural alterations in both the
femoral head and the acetabulum have
occurred. Dislocated hips in this age group
require open reduction. Preoperative
skeletal traction should not be used.
 Adolescent and Young Adult (Older
Than 8 to 10 Years of Age)
 In children older than 8 to 10 years of
age or in young adults in whom the
femoral head cannot be repositioned
distally to the level of the acetabulum,
only palliative salvaging operations are
possible. A reconstructive operation such
as a total hip arthroplasty may be indicated
at the appropriate age.
In bilateral dislocations in this age group
the hips should be left unreduced and
total hip arthroplasties may be carried
out during adulthood