Transcript Congenital deformations. Developmental dysplasia of the hip

Congenital deformations.
Developmental dysplasia of the hip.
Congenital muscular torticolis. Clubfoot.
Congenital muscular torticolis.
Congenital torticollis is a deformity of the
neck characterised by tilting of the head to
one side. There is a rotation of the occiput
to one side and the chin to the opposite
side.
The basic feature of this condition is a
contracture of the sternomastoid muscle
on one side. The exact pathology leading
to the contracture is not known. It may be
a myodysplasia of genetic origin.
Clinical features
• The child may be brought soon after birth
when the mother notices the tilt in the
head to one side and often a swelling in
the neck. There may be a firm swelling in
the junction of the middle and distal third
of the stemo-mastoid muscle. This is
called the sternomastoid tumour. In cases
due to birth trauma, it may be associated
with fractures of the clavicle or Erb's palsy.
In a left sternomastoid torticollis, the chin
is tilted upwards to the right side and the
occiput is tilted down on the left side.
Radiography
• Radiography may show congenital
anomalies in the cervical spine like
hemivertebrae or partial fusion of the
cervical vertebrae in cases where the
stemomastoid is normal.
In neglected cases, the contracture is
severe and cannot be stretched passively.
There is facial asymmetry, as the face in
the affected side is not well developed. A
new macula develops in the fundus to
make the vision level in the tilted position
of the head.
Radiography
• In older children
torticolis is due to
the rotatory
subluxation which
may be due to
infection, trauma
Treatment
• A position in bed.
• In the newborn stage, the stemomastoid is
stretched passively by the physiotherapist.
• After 4-6 weeks the child is taught neck
exercises.
• The treatment in later cases is mainly
surgical tenotomy of the lower end of the
stemomastoid.
Developmental dysplasia of the hip.
General definitions
• There are 2 types of congenital dislocation
of the hip. In the more common type,
excessive laxity of the hip joint capsule
and ligaments result in subluxation and
dislocation.
• In the other type, dysplasia or poor
development of the acetabulum is the
cause for the dislocation.
Clinical Features
In a new born child suspected to have
congenital dislocation of the hip, look for any
obvious shortening of the leg or any additional
crease in the posterior dislocation. Two tests are
performed.
With the baby on its back, the hip and knee are
held flexed and the limb is slowly abducted at
the hip. If the hip is dislocated, the head of the
femur is felt to slip in to the acetabulum with a
click. This is the Ortolani's test. Barlow's test is a
slightly modified one in which the head could be
pushed in and out of the acetabulum with a click.
Clinical Features
With the child lying on its back, the hip and
knee are held flexed to 90 degrees, the
pelvis is steadied with the left hand and
the knee is held in the right hand. On
pushing down and pulling up at the knee
vertically, the head could be felt by the left
hand to move down and up in the gluteal
region. This is the telescopy test.
Clinical Features
The condition often escapes notice till the
child begins to walk when a limp is obvious.
On examination, there is limitation of
abduction of the flexed hip. The dislocated
head of the femur can be palpated in the
gluteal region. When the hips are flexed to 90
degrees and the feet rested on the table, the
level of the knee of the affected leg is lower.
This is called the Galeazzi's sign. The gait will
be unstable. This is called the Trendelenberg
gait due to the dislocated hip telescoping
upward on putting weight on the leg.
Bilateral dislocation
• There will be no difference between the
two legs in length. The perineum will be
wider than normal. Telescoping will be
present in both hips. The older child walks
with a gait swaying from side to side with a
marked increase in the lordosis of the
lumbar spine (Sailor's gait).
Radiological features
• In the newborn baby, before the femoral
epiphysis has appeared, the condition is to be
suspected if the joint space is excessively broad
and the neck of the femur lies far away from the
acetabulum. In A.P. view of pelvis with both hips
in abduction, normally a line drawn along the
shaft of the femur prolonged proximally will pass
through the centre of the acetabulum. When the
hip is dislocated, this line will pass above the
acetabulum. More recently ultra sound scanning
is used to detect the position of the unossified
cartilaginous head. In a child aged one year and
above, the dislocated side shows the epiphysis of
the head lying outside the acetabulum and at a
higher level. The roof of the acetabulum slopes
upwards when compared to the normal side.
Treatment
• The treatment should be started as soon as
possible after birth. The aim of the treatment is
to reduce the hip and maintain it in the reduced
position till it remains stable. The treatment is
age related and depends on the age at which
the child is brought.
• In Infants below 3 months
Splints like Von Rosen's splints are applied to
retain the position of the head.
Treatment
Children from 3 to 6 months
• The dislocation is reduced by gentle manipulation under general anaesthesia and
immobilised in plaster cast, with the hip at 90
degree flexion and 45 degree abduction.
This is the "human position" as described by
Kumar. The hips is left in an older should
not be immobilised in the extreme position of
the so called Frog position of 90 degrees
• flexion and 90 degrees abduction. The
excessive stretching of the capsule in this
position interferes with the blood supply to
the capital epiphysis.
Treatment
•
•
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Children from 6 to 12 months
A preliminary traction and gradual abduction is done
for 2-3 weeks before reduction under general
anaesthesia. Some of these children may need
adductor tenotomy if there is resistance to reduction
by its tightness. The plaster cast is then applied.
Children 1 to 3 years
In this age group, after preliminary traction. surgery
will be required to reduce the head of femur into the
acetabulum. In cases with excessive valgus and
anteversion of the femoral neck, a femoral varus
derotation osteotomy is done as a second stage.
Children 3 to 6 years
In this age group, after open reduction of the hip and
femoral osteotomy, an osteotomy of the acetabulum is
made to retain the head in stable position.
• Neglected congenital dislocation of the hip
• Neglected congenital dislocation of the hip may
present after the age of 10 years with pain in the
hip and unstable gait. These will require pelvic
stabilising operations like Schantz osteotomy.
• Complications
• Avascular necrosis of the head of the femur is a
complication of forceful reduction of the hip in
childhood. Neglected cases develop painful
instability of the hip and in later years
osteoarthrosis of the hip.
Club Foot
(CONGENITAL TALIPES EQUINO VARUS)
Congenital talipes equino varus is one of the
most common congenital deformities in
India when compared to the Western
countries. The word Talipes is derived from
'Talus' and 'Pes' and was applied to those
walking on their neglected deformities
wherein the talus rested on the ground as
the foot (Pes). It is characterised by a foot
plantar flexed at the ankle, inverted at the
subtalar joint and adducted at the forefoot.
The deformity is bilateral in approximately
50% of cases and is more common in
males.
Etiology
• The exact cause of this deformity is not known.
According to Hippocrates, the talipes deformity
is of mechanical origin and is caused by an
abnormal intra uterine position of the fetus.
Another theory 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
musculo tendinous corn-plexus. The third
hypothesis is that primary soft tissue
abnormalities within the neuromuscular units
cause secondary bony changes.
Types of CTEV
• One type is a less severe deformity with
an elevated heel and is mobile
and is comparatively easy to correct by
manipulation. This is called the extrinsic
type.
• The other type is a severe deformity with a
small heel and extremely resistant to
treatment and tends to relapse. This is the
intrinsic type (so called vicious feet).
Pathological Anatomy
• The structural changes are most evident in the talus and
the calcaneum. The talus is plantar flexed and the
superior articular surface is exposed. The neck becomes
elongated and deflected medially and downwards. The
calcaneum is inverted so that its medial tuberosity
approaches the medial malleolus and its posterior aspect
is elevated. The navicular is rotated on its axis and its
tubercle may almost come into contact with the medial
malleolus.
• The soft tissues on the posterior aspect of the ankle and
medial aspect of the foot are contracted. The tendo
achilles, the tibialis posterior, the plantar fascia, the
spring ligament, the deep plantar ligaments, flexor
hallucis longus, flexor digitorum longus and abductor
hallucis are contracted. The capsules of the subtaloid,
talo navicular, naviculo cunieform joints are also
contracted.
Clinical features
The shape and contour of the foot is
characteristic. There is equinus deformity
at the ankle, varus deformity at the
subtaloid joint and adduction deformity at
the tarsometatarsal joints. The deformity is
graded as mild, moderate or severe. The
foot is on the whole smaller than normal
and the heel is poorly developed. There is
a crease across the medial border of the
foot. The lateral malleolus is very
prominent while the medial malleolus is
buried in a depression.
Radiological features
• Radiograph of the feet AP and lateral views are
taken to assess the severity of the deformity.
The alignment of the talonavicular, calcaneum
and the first metatarsal bone is studied in AP
view of foot.
• Normally, the line along the axis of the talus
roughly passes anteriorly through the navicular
and the shaft of the 1-st metatarsal bone. In the
club foot, this line is deviated lateralward and
passes along the 3rd or even the 4th metatarsal
bone, due to the inward displacement of the
navicular and metatarsalbones.
Methods of Treatment
The methods used in the treatment of club
feet are as follows.
• Gentle stretching and strapping.
• Manipulative correction and plaster
casting.
• Surgery.
Conservative treatment In the first three weeks
• The mother (usually the grand mother) is taught
to mould the infant's foot twice a day. As the skin
is very soft, strapping is not done.
Three weeks to three months
• The surgeon manipulates the foot once in two
weeks and the position is maintained by
strapping with adhesive plaster.
Three months to one year
• Manipulation is done under general anaesthesia
and the corrected position maintained in a
plaster of paris cast this extends from mid thigh
to the toes, with the knee in flexion. This is
repeated once in three or four weeks. The
plaster cast is protected by a water proof plastic
bag.
Club foot Boots
After 4 or 5 manipulations, the foot is usually in
normal shape but it needs a retentive shoe. The
special I club foot boots maintain the correction
and prevent lapse. The feet need careful
watching till the child walks independently.
• The forefoot part is turned out, outer side of heel
and sole are raised and there is a strap across
the front of the ankle to keep the heel down.
• Denis Browne special night splints are worn after
the foot gets corrected.
Surgical Treatment
• Surgery should be resorted to only in cases where
conservative treatment has failed. The following are the
surgical procedures used in the treatment of club feet.
• 1. Soft tissue operations
• a. Tendo Achilles lengthening and posterior capsulotomy.
• b. Postero Medial soft tissue release, c. Complete
Subtaloid release.
• 2. Bone operations
• Lateral wedge resection and calcaneo cuboid fusion.
• Soft Tissue operations
• a) Tendo Achilles lengthening and posterior capsulotomy.
• This is done in cases where manipulations have corrected
the inversion and adduction deformities, but the equinus
persists. The contracted tendo achilles tendon is
lengthened. The contracted posterior capsule of the ankle
and talo calcaneal joints are also divided.
• b) Postero Medial soft tissue release.
• In this operation in addition to the lengthening of the
tendo achilles, the tibialis posterior, flexor hallucis longus
and flexor digitorum longus tendons are also lengthened.
All the ligaments and soft tissues on the plantar and medial surfaces of the calcaneum, talus and navicular
bones are divided.
• Recently, it is being felt that better results can be
obtained by adopting surgical correction much earlier
even at the age of 4-8 months.
• c) Complete subtaloid release
• Currently the surgical treatment followed is a complete
release of all tight structures in the posterior, medial and
lateral aspects of the subtaloid and ankle joint.