embryology of skeletal system

2.  The skeletal system is mesoderm in origin with some
neural crest contribution.
 The skeletal system develops from paraxial and
lateral plate (somatic layer) mesoderm and from
neural crest.
 Paraxial mesoderm forms a segmented series of
tissue blocks on each side of the neural tube,
known as somitomeres in the head region and
somites from the occipital region caudally.

5.  Somites differentiate into a ventromedial part, the
sclerotome, and a dorsolateral part, the
dermomyotome.
 At the end of the fourth week sclerotome cells
become polymorphous and form a loosely woven
tissue, the mesenchyme, or embryonic connective
tissue.
 It is characteristic for mesenchymal cells to
migrate and to differentiate in many ways.
 They may become fibroblasts, chondroblasts, or
osteoblasts (bone-forming cells).

7.  The bone-forming capacity of mesenchyme is not
restricted to cells of the sclerotome, but occurs
also in the somatic mesoderm layer of the body
wall, which contributes mesoderm cells for
formation of the pelvic and shoulder girdles
and the long bones of the limbs.
 Neural crest cells in the head region also
differentiate into mesenchyme and participate in
formation of bones of the face and skull.

8.  Occipital somites and somitomeres also contribute
to formation of the cranial vault and base of the
skull.
 In some bones, such as the flat bones of the
skull, mesenchyme in the dermis differentiates
directly into bone, a process known as
intramembranous ossification.
 In most bones, however, mesenchymal cells first
give rise to hyaline cartilage models, which in turn
become ossified by endochondral ossification.

9.  The skeletal system consists of two parts:
 The axial skeleton which includes the vertebral
column, the ribs, the sternum and the skull.
 The appendicular skeleton which consists of the
pectoral and pelvic girdles, and the limb bones.

10. Vertebral Column
 It is during the fourth week of development that cells of
the sclerotomes shift their position to surround both the
spinal cord and the notochord .
 This mesenchymal column retains traces of its
segmental origin, as the sclerotomic blocks are
separated by less dense areas containing intersegmental
arteries.
 During further development the caudal portion of each
sclerotome segment proliferates extensively and
condenses.


11.  This proliferation is so extensive that it proceeds into
the subjacent intersegmental tissue and binds the caudal
half of one sclerotome to the cephalic half of the
subjacent sclerotome.
 Hence, by incorporation of the intersegmental tissue
into the precartilaginous vertebral body, the body of the
vertebra becomes intersegmental.

13. Development of spinal cord in embryonic period

14. Development of Ribs and Sternum
 Ribs form from the costal processes of thoracic
vertebrae and are derived from the sclerotome portion
of paraxial mesoderm.
 The sternum develops separately in somatic mesoderm
in the ventral body wall.
 Two sternal bands are formed on either side of the
midline, and these later fuse to form cartilaginous
models of the manubrium, sternebrae, and xiphoid
process

16. The skull
 The skull can be divided into two parts: the
neurocranium, which forms a protective case
around the brain, and the viscerocranium, which
forms the skeleton of the face.
 The neurocranium is most conveniently
divided into two portions:
 (a) the membranous part, consisting of flat
bones, which surround the brain as a vault;
 (b) the cartilaginous part, or chondrocranium,
which forms bones of the base of the skull.

17.  DURING WEEK 6, the mesenchymal primordia of bones in
the limb buds undergo chondrification to form hyaline
cartilage models of the future bones.
◦ The clavicle initially develops by intramembranous
ossification but does form growth cartilages at both ends.
◦ The pectoral girdle and upper limb cartilages appear before
those of the pelvic girdle and lower limbs. The cartilages
appear in a proximodistal sequence.

18.  OSSIFICATION in long bones begins by the end of the
embryonic period; the primary centers are seen by
week 12 in almost all bones of the extremities; and
secondary ossification centers are seen after birth

19. The limbs
 After four weeks of development, the buds for the limbs
become visible from the ventrolateral body wall.
 It consists of a mesenchymal core that will form the bones
and connective tissues of the limb which is covered by a layer
of cuboidal ectoderm.
 The ectoderm at the distal border of the limb then becomes
thicker which forms after the apical ectodermal ridge (AER).
 As the limbs continue growing, the cells that are farther away
from, the influence of AER begins to form into muscles and
cartilage.

20. THE LIMB BUDS DEVELOP
AS THE EMBRYO GROWS

21.  The limb buds of the six week embryo now become
flattened to form the hand plates and foot plates which
is separated by a circular constriction.
 Fingers and toes are formed.

22.  Development of the upper and lower limb is similar
except that the morphogenesis of the lower limb is
approximately one to two days behind that of the upper
limbs.
 During the seventh week of gestation, the limbs (both
upper and lower) rotate in opposite directions.

23.  The upper limbs rotates ninety degrees laterally so that the
extensor muscles lie on the lateral posterior surface and the
thumbs lie laterally
 the lower limbs rotates ninety degrees medially placing the
extensor muscles on an anterior surface and the big toe
medially.

24.  Bone is actually a very complex and dynamic living
tissue.
 The framework of bones and their cartilages together
constitute the skeletal system.

25.  Diaphysis: long shaft of bone
 Epiphysis: ends of bone
 Metaphysis: b/w epiphysis and diaphysis
 Articular cartilage: covers epiphysis
 Periosteum: bone covering (pain sensitive)
 Medullary cavity: Hollow chamber in bone
- red marrow produces blood cells
- yellow marrow is adipose.
 Endosteum: thin layer lining the
medullary cavity

27.  Histology of bone tissue
Cells are surrounded by matrix.
- 25% water
- 25% protein
- 50% mineral salts
4 cell types make up osseous tissue
Osteoprogenitor cells
Osteoblasts
Osteocytes
Osteoclasts

28.  Osteoprogenitor cells:
- derived from mesenchyme
- all connective tissue is derived
- unspecialized stem cells
- undergo mitosis and develop into
osteoblasts
- found on inner surface of periosteum and
endosteum.

29.  Osteoblasts:
- bone forming cells
- found on surface of bone
- no ability to mitotically divide
- collagen secretors
Osteocytes:
- mature bone cells
- derived form osteoblasts
- do not secrete matrix material
- cellular duties include exchange of
nutrients and waste with blood.

30.  Osteoclasts
- bone resorbing cells
- bone surface
- growth, maintenance and bone repair

32.  Compact bone: forms external layer of all bones
- majority of all long bones
- protection and strength (wt. bearing)
- composed of concentric ring structure called osteons
- blood vessels and nerves from periosteum penetrate
the compact bone through horizontal openings
called perforating (Volkmann’s) canals.

33.  Spongy bone (cancellous bone): internal
layer
- made of irregular lattice work of thin columns of bone
called trabeculae (“little beams”)
- filled with red and yellow bone marrow
- osteocytes in the trabeculae get nutrients directly from
circulating blood.
- short, flat and irregular bone is made up of mostly
spongy bone.

34.  The process by which bone forms is called ossification
 Ossification (osteogenesis) begins around the 6th -7th week of
embryonic life. At this time the embryonic skeleton is made of
fibrous membranes and hyaline cartilage.

35.  Two patterns of bone formation occur.
 Intramembranous ossification: formation of bone
directly on or within fibrous connective tissue
membranes.
 Endochondral ossification: the formation of bone with
in the cartilage.

37.  Intramembranous (within the membrane) ossification: Bone
develops from a fibrous membrane.
- flat bones of skull
- mandible
- clavicles
-mesenchymal cells become vascularized and become
osteoprogenitor cells and then osteoblasts.
- organic matrix of bone is secreted
- osteocytes are formed
- calcium and mineral salts are deposited and bone tissue hardens.
- trabeculae develop and spongy bone is formed
- red marrow fills spaces

38. . Endochondral (intracartilaginous) ossification:
 Replacement of hyaline cartilage with bone
 Most bones are formed this way (i.e. long bones).
 Where bone is going to form:
1- mesenchymal cells differentiate into
chondroblasts (immature cartilage cells) which
produces hyaline cartilage.
2- Chondrocytes (mature) mitotically divide increasing in
length

39.  The key to an accurate diagnosis is a
 A careful history
 Thorough physical examination
 Appropriate radiographic imaging &
 Laboratory testing.

40.  Most important part of the evaluation
 Usually obtained from the parents or guardian ,but the
child if old enough and co-operative can also give
useful information.

41.  The chief complaint is established first.
 This may include
-pain
-deformity
-joint stiffness
-gait disturbances (limp, toe-walking, in-toeing, out
toeing).
-swelling
-generalized muscle weakness.

42. One must ascertain,
 Location & duration of symptoms
 Antescedent factors such as
• Fever
• Trauma
• Radiation of pain
• Neurologic symptoms
 Factors aggravating or alleviating the symptoms
 Previous evaluations or treatments

43.  In children with chronic symptoms the past medical
history is also important.
 the prenatal or pregnancy history should be obtained.
These includes:
• Maternal diseases or illnesses.
• Vaginal bleeding
• Oligohydramnios
• Ingestion of toxic substances or medications
• Trauma.

44.  The birth history should determine
• The length of pregnancy
• Duration of labor
• Type of difficulty, if any with delivery
• Birth presentation
• Birth weight
• APGAR score

45.  The condition of child during the neonatal period is
important
 In older infants & young children evaluation of the
presence and delay of developmental milestones for,
• Posture
• Locomotion
• Dexterity
• Social activities
• Speech etc are important

46.  The family history may give clues to possible genetic
disorders such as
• congenital syndromes
• Muscular dystrophy
• Skeletal dysplasia etc.

47.  Includes four parts.
1. Observation
2. Palpation
3. Assessment of joint range of motion
4. Gait assessment in ambulatory children.

48.  If the child can stand, posture, truncal alignment and
symmetry of the extremities can be evaluated.
 The skin is assessed for cutaneous lesions.
 The presence of café-au-lait spots may indicate
neurofibromatosis
 A maculopapular rash may indicate juvenile
rheumatoid arthritis.

49. café-au-lait spots
maculopapular rash

50. • The involved joint, area of extremity or trunk that is of
concern should be palpated for tenderness,
• massess,
• soft tissue swelling and
• increased warmth.

51.  Palpate the clavicles in the newborn or young infant for
tenderness or a bump that indicates callus formation
with clavicle fracture.

52.  Palpate the affected joint or extremity to detect warmth or
tenderness.
 In the injured child or child with a cast or splint thoroughly
assess the neurovascular status of the affected extremities.
 Palpate the fingers or toes for warmth
 Determine capillary refill time
 Note the presence of sensation or motion
 Palpate pulses distal to injury, noting their strength and
quality.

53.  The range of motion of the involved joints should be
assessed and recorded.
 If the opposite joint is normal , this range should also
be recorded for comparison purposes.
 It must be remembered that the range of motion of
joints changes from infancy through childhood & into
adolescence.

54.  Description of Joint Motions
 Flexion: Act of bending a joint; a motion away from
the zero starting position.
 Extension: Act of straightening a joint; a return
motion to the zero starting position.
 Hyperextension: When the motion opposite to flexion
is an extreme or abnormal extension (as may be seen
with the knee or elbow joint), and the joint extends
beyond the zero starting position.
 Abduction: Lateral movement of the limbs away from
the median plane of the body, or lateral bending of the
head or trunk.

55.  Adduction: Movement of a limb toward the median
plane of the body.
 Supination: Act of turning the forearm or hand so that
the palm of the hand faces upward or toward the
anterior surface of the body.
 Pronation: Turning of the palm of the hand so that it
faces downward or toward the posterior surface of the
body.
 Inversion: An inward turning motion (seen primarily
in the subtalar joint of the foot).

56.  Eversion: An outward turning motion.
 Internal (inward) rotation: Process of turning on an
axis toward the body.
 External (outward) rotation: Process of turning on
an axis away from the body (opposite motion of
internal rotation).

57.  Gait disturbances are one of the most common parental
concerns in children.
 Gait cycle is the time between right heel strike
followed by left toe- off, left heel strike & right toe-off
and ends with right heel strike.

59.  One Gait cycle includes two phases.
• Stance
• Swing
 The stance phase is the period of time during which
one of the two feet is on the ground.
 The swing phase is the portion of the gait cycle during
which a limb is being advanced forward without
ground contact.

60.  The normal 1 yr old child has a wide-based stance &
rapid cadence with short steps.
 A 2 yr old child shows increased velocity & step length
& diminished cadence compared with a 1yr old child.
 Most of the adult gait patterns are present in children
by 3 yr of age.
 The gait characteristics of a 7yr old child are similar to
those of an adult.

61.  Common gait disturbances include
• Limp
• Torsional variations (in-toeing & out toeing)
• Toe-walking.

62.  A careful neurologic evaluation must be performed &
includes,
 Muscle strength testing
 Sensory assessment
 Evaluation of deep tendon & pathologic reflexes such
as the babinski reflex

63.  It is the principal method for the evaluation of the
pediatric musculoskeletal system.
 This can include
 Routine radiographs
 Technetium bone scans
 CT
 MRI
 ultrasonography

64.  They are the first step and consist of anteroposterior
and lateral views of the involved joint, bone or area.

65.  A bone scan is a non-invasive imaging technique that
uses a radioactive substance to visualize the bones,
showing cell activity in the bone.
 A radiopharmaceutical called Technetium-99m MDP is
injected into your child's veins. Technetium-99m MDP
has a tiny amount of radioactive molecules in it.

66.  Once the radioactive substance has travelled through
the bloodstream and into your child's bones, a special
camera, called a gamma camera, is used to take
pictures.
 Common indications include
• Early septic arthritis or osteomyelitis
• Neoplasia such as osteoid osteomas & leukemia.
• Metastatic lesions
• Inflammatory diseases.

67.  Coronal and axial cross section studies with CT can be
beneficial in evaluating complex disorders of the spine,
pelvis & feet.
 It allows the visualization of the bone anatomy and the
relationship of bones to contiguous structures.

68.  This avoids ionizing radiation and is presumed not to
produce biologically harmful effects.
 It produces excellent anatomic images of the
musculoskeletal system.
 MRI distinguishes physiologic changes that occur in
the bone marrow with respect to age & disease such as
avascular necrosis.

69.  It has no ionizing radiation and no biologically harmful
effects.
 The equipment is portable and scans can be obtained in
any planes.
 Major indications are:
• Developmental dysplasia of hip
• Foreign bodies in the soft tissue
• Popliteal cysts of the knee etc.

70.  Hematology
• CBC
• ESR
• C-reactive protein determination
• Blood culture for infectious disorders.
• Rheumatoid factor etc.

71.  Traction
 Application of a pulling force on an extremity or
body part.
 A common method of immobilization
 In running traction the weight pulls directly on the
extremity in only one plane.
 This may be achieved with either skin or skeletal
traction.

72.  Casting
 Application of plaster or fiberglass material to form a
rigid apparatus to immobilize a body part.
 Used to immobilize a bone that has been injured or a
diseased joint.
 When a fracture has occurred, a cast serves to hold the
bone in reduction, thus preventing deformity as the fracture
heels.
 Casts are constructed of a hard material, traditionally
plaster but now more commonly fiber glass.

73.  Splinting
-temporary stiff support of injured area
-indications include temporary fracture reduction,
immobilization and support of sprains.

74.  Fixation
-surgical reduction of a fracture or skeletal deformity
with an internal or external pin or fixation device.

75.  Cold therapy
-application of ice bags, commercial cold packs or
cold compresses.
-Most often used in acute injuries to cause
vasoconstriction , thereby decreasing pain & swelling
 Crutches
-ambulatory devices that transfer body weight from
lower to upper extremities.

76.  Orthotics,braces
 Adaptive positioning devices specially fitted for each child by the physical
or occupational therapist or othotist.
 Used to maintain proper body or extremity alignment, improve mobility
and prevent contractures.
 Physical therapy, occupational therapy
-physical therapy focuses on attainment of gross motor skills
-occupational therapy focuses on refinement of fine motor skills, feeding
& activities of daily living.

79.  Congenital limb deficiencies or reduction
malformations are manifested by a variety of degrees of
loss of functional capacity.
 They are characterized by under development of
skeletal elements of the extremities.

80. i. PHOCOMELIA
It is a reduction deformity (congenital amputation) in
which there is gross reduction in the proximal part of the
extremity so that distal part seems to be approaching the
trunk.
ii. HEMIMELIA
It refers to absence of fore arm and hand or leg and
foot.
iii. AMELIA
It means complete absence of limbs.

81. Causes
 Genetic inheritance
 Prenatal environment insults
* thalidomide intake.
 Deletion or shortening of limbs may be associated with
chronic villus sampling, especially before 10-12 weeks of
gestation.

82.  Pathophysiology
 Limb deficiencies occur as the fetus is developing
 These defects are attributed to an amniotic band
constricting the limb, resulting in either incomplete
development or amputation of the limb.
 Formation of the limbs may be suppressed at the time
of limb bud formation or there may be interference in
later stages of differentiation & growth.

83. Phocomelia

84. Severe congenital fibular hemimelia
Bilateral ulnar hemimelia.
HEMIMELIA

85. AMELIA

86. Treatment
 Prosthetic devices.
 Physical & occupational therapy.

87. Definition
 It is a complex deformity of the ankle and foot that
includes forefoot adduction, midfoot supination,
hindfoot carus and ankle equinus.

88. Variations
 Talipes varus-an inversion or
bending inward
 Talipes valgus- an eversion or
bending outward
 Talipes equinus- plantar
flexion, in which the toes are
lower than the heel
 Talipes calcaneus-
dorsiflexion,in which the toes
are higher than the heel.

89. Incidence
 1/1000 live births
 Boys are affected twice as often as girls
 Bilateral clubfeet occur in 50% cases.

90. Causes
 Precise cause is unknown
 45% cases show a hereditary tendency.
 An abnormal diminution of the amniotic fluid
causes excessive pressure from uterine walls
exerted on the fetus resulting in the fetus becoming
splinted against the fetal body in the equino varus
position.
 Arrested embryonic development.

91. Classification
1. Positional clubfoot
It is believed to occur primarily from intrauterine
crowding and responds to simple stretching & casting.
2. Syndromic (teratologic) clubfoot
It is associated with other congenital anomalies
such as myelomeningocele or arthrogryposis ( congenital joint
contractures in two or more areas of the body).
It is a more severe form of club foot that is often
resistant to treatment.
3. Congenital club foot (idiopathic)
It may occur in an otherwise normal child and has
a wide range of rigidity and prognosis.

92. Diagnostic evaluation
 History collection
 Physical examination
 Ultrasonography
The deformity is readily apparent and early
detected prenatally through Ultrasonography or at birth.
 X-rays.

93. Therapeutic management
 Mild cases are treated by vigorous manipulation of the
feet to the correct position atleast twice daily.
 The Achilles tendon requires stretching to prevent
recurrence.

94.  In more severe forms, a Dennis Brown splint is used
along with adhesive plaster.

95.  Definition
The term flat foot denotes loss of medical
longitudinal arch of the foot.

96.  Clinically flat foot is recognized when the arch touches
the ground on weight bearing or is close to the ground.
 Until age 2-3 yrs foot normally appears flat because of
absence of medial longitudinal arch.

97. Causes
 Congenital
• Calcaneo valgus deformity
A flexible calcaneovalgus foot is a postural deformity in
infants which causes the foot to appear to be pushed up against
the front of the leg.
“Calcaneo” describes an upward-position of the foot, and
“valgus” describes an outward position.
Thus a calcaneovalgus foot is one that is positioned upward
and outward.

98. • Hypermobility (condition that features joints that
easily move beyond the normal range expected for a
particular joint.)
• Rigidity with tarsal anomalies.

99.  Acquired
• Fracture of the talus or calcaneus
• Tear of plantar ligaments
• Muscle imbalance
• Postural
• Bad gait
• Faulty shoes.

100. Treatment
 conservative measures
 Arch support
 Shoe modification
 Exercise
 Orthopedic interventions
 removal of calcaneus
 Arthodesis after 10 yrs.
Is the artificial induction of joint
ossification between two bones via surgery.

101.  The hand is deviated laterally because of partial or total
absence of radius.
 In radial dysplasia:
◦ The affected arm is shorter, with curving of the forearm and stiffness
of the elbow and fingers.
◦ thumb is either very small or missing.
 Absence of thumb, congenital heart defect and a bleeding
diathesis are frequent accompaniments.
 .

103. Treatment
 Orthopedic treatment is centralization of ulna in
relation to hand and reconstruction of thumb by
pollicization ( hand surgery technique in which
a thumb is created from an existing finger) of index
finger.

104.  In this condition the thumb cannot be straightened since
it is locked in flexion because of a nodular swelling of
the long flexor tendon at the base of the thumb.

105.  Treatment is surgical incision of the constricting mouth
of the tendon sheath.
 Injection of the tendon sheath with a corticosteroid is
effective over weeks to months in more than half of
patients.

106.  An extra finger/toe, usually close to the metacarpophalangeal
joint of the little finger or 5th toe or the thumb, may occur as
an isolated trait or as a component of such syndromes as
 Laurence-moon-biedl syndrome
a rare autosomal recessive genetic disorder associated
with retinitis pigmentosa, extra digits, spastic paraplegia,
hypogonadism and mental retardation.

107.  Meckel-gruber syndrome
a rare, lethal, ciliopathic, genetic disorder, characterized
by renal cystic dysplasia, central nervous system malformations (occipital
encephalocele), polydactyly (post axial),hepatic developmental defects,
and pulmonary hypoplasia due to oligohydramnios
 Trisomy 13.
Trisomy 13, also called Patau syndrome, is a chromosomal condition
associated with severe intellectual disability and physical abnormalities in
many parts of the body.

108.  Carpenter syndrome
Is an extremely rare autosomal recessive congenital
disorder characterized by craniofacial malformations, obesity,
syndactyly etc.

109. Incidence
 The condition has an incidence of 1 in every 500 live
births.
 Postaxial hand polydactyly is a common isolated
disorder in African black children, and autosomal
dominant transmission is suspected.
 Postaxial polydactyly is more frequent in blacks than
in whites .
 More frequent in male children.

110. Cause
 Genetic mutation
Treatment
 Orthopedic intervention is in the form of ligation or
excision at birth or amputation at about 1 yr of age.

112.  Fusion of digits/ toes varies from a cutaneous web to a
synostosis and may occur as an isolated trait or as a
component of certain syndromes like
 Apert syndrome
A congenital disorder characterized by
malformations of the skull, face, hands and feet.
 Carpenter syndrome
 Laurence-moon syndrome
 Trisomy 13

113.  De lange syndrome
It is a genetic disorder that can lead to severe
developmental anomalies. It affects the physical and
intellectual development of a child.
 Holt oram syndrome
It is an autosomal dominant disorder that affects
bones in the arms and hands (the upper limbs) and may
also cause heart problems.

115.  Treatment
• No treatment usually required for syndactyly
• Surgical repair is sometimes performed for
cosmetic reasons.

116. Definition
 These are circumferential constrictions in the soft
tissues, more frequently in legs and feet than arms and
hands.
Incidence
 The reported incidence of constriction ring syndrome
varies from 1/1200 and 1/15000 live births.
 The prevalence is equally in male and female.[

117. Causes
 Intrauterine disruption during pregnancy followed by a
cascade of events involving amniotic rupture
 Intrauterine trauma
Clinical features
 Depending on the magnitude of constriction, they
cause obstruction in the circulatory and lymphatic
channels, leading to localized edema.
 Associated foot deformities and super added fractures
of tibia and fibula are common.

119. Treatment
 excision of the constriction band.

120. Definition
 refers to nonunion of a tibial fracture that develops
spontaneously or after trivial trauma in a dysplastic
bone segment of the tibial diaphysis.
 In this condition there is an aplasia of a portion (usually
distal half) of the tibia, giving the impression of a non
bearing fracture in the neonate.

122. Incidence
 1 in 190,000 live births
 Etiology and pathogenesis unclear
Treatment
 Intermedullary mailing with bone grafting, vascularized
fibular graft and electrical stimulation

123. Congenital dislocation of the patella
describes a condition in which the patella
is dislocated laterally at birth permanently
and irreducibly.

124.  It may be congenital
 Ligamentous laxity on medial side of the joint
 Small lateral condyle of femur
 Short quadriceps muscle
 Or may be post traumatic

125.  Two types are known
 Recurrent (occuring at intervals)
 Habitual (occuring whenever knee is flexed)

126. Clinical features
 knee deformity
 flexion contracture
 valgus appearance of the lower leg
 patella possibly palpable at the lateral aspect
of the knee
 empty intercondylar notch
 frequently hypoplastic patellae in syndromes
Treatment
 Orthopedic correction

128.  The term denotes medial angulation of knees
because of outward deviation of the
longitudinal axis of both tibia and femur.
 The concurrent finding is abnormally
divergent ankles (intermalleolar distance >
8cm)
 Physiologic knock knee is common in
toddlers but it always disappears by age 7
yrs.

129.  The commonest variety is idiopathic
 It may also be secondary to bone softening,
post traumatic, paralytic, post infective or
neoplastic
Treatment
 stapling or osteotomy

130.  The term denotes lateral angulation of knee
joints because of inward deviation of
longitudinal axis of tibia & fibula.
 As a result, knees are abnormally divergent
(bow like) where as ankles are abnormally
convergent.
 Physiological bowleg, when the child begins
to walk is quite common. It resolves in due
course.

131. Other causes include,
 Rickets
 Postural
 Traumatic
 Developmental
 Endocrinal
Treatment
Corrective osteotomy

132.  The term refers to a hard , immobile,
fusiform and well circumscribed mass,
around 2cm in diameter, which may be felt in
the middle of the sternomastoid muscle,
usually 10-14 days after birth.

133. Etiology
 The etiology of SMT is unknown,
 but the current theory is that it results from
abnormal intrauterine positioning, leading to
intramuscular compartment syndrome and
ischaemic muscle injury with subsequent
fibrosis and contracture of the sternomastoid
muscle.
 The cause may be a birth trauma, usually
from a difficult breech delivery.

134.  If untreated, the sternomastoid tumour
naturally resolves completely.
 In 50–70% of the cases by 6 months of age,
with muscle shortening.
 Persisting in 5–7% after 1 year.

135. Treatment
 It consists in stretching the affected
muscle to the over corrected position by
gentle manipulation several times daily.
 At 6-12 months of age surgical lengthening
& division of the sternal portion of the muscle
from mastoid process at its origin.

136.  It is a disorder involving the abnormal
development of bones in the skull and collar
(clavicle) area.
Cause
 Autosomal dominant disorder

137. Clinical feature
 It is characterized by absence of the outer
third of each clavicle so that the patient can
• make his shoulders meet in front,
• high- arched palate
• absent Para nasal sinuses
• defective teething
• poorly developed spinal bones

138. Treatment
 There is no specific treatment for the bone
problems.
 An oral surgeon should monitor teeth
regularly.
 An otologist should check for hearing
problems.

139.  In this congenital disease one side of the
body is significantly larger than the other.
 The hypertrophy is usually of the whole one
side , including face, tongue, teeth and
genitalia.

140.  Definition
It refers to abnormalities of the developing
hip that include dislocation, subluxation and
dysplasia of the hip joint.
 Incidence
 The global incidence of this condition varies
from 0.7-15.5/1000 live births.
 It is rather uncommon in India.
 Occurs more frequently in females.

141. Etiology
 In breech presentation and other difficult
deliveries, the head of the infant’s femur may
get dislocated upward and backward.
Its constant pressure over the dorsal aspect
of the ileum may cause development of a false
acetabulum.
 Hereditary
 Presence of oligohydramnios.

142.  While dislocation may occur during a growth
period in utero, the laxity of the newborn’s
hip allows dislocation and relocation of the
hip to occur.
 The hip can develop normally only if the
femoral head is appropriately and deeply
seated within the acetabulum.

143.  The signs & symptoms are variable,
depending on whether the child is brought
prior to or following dislocation.
Before dislocation
The cardinal clinical signs are
 An extra fold in the fat of the thigh and
genital region on he side of dislocation.
 Differences in the length of the limbs.
 Diminuation of spontaneous movements.
 A limitation of abduction.

145. After dislocation
 The asymmetry of the skin folds of the
gluteal, inguinal & knee regions may be
marked.
 In children who have started walking a
distinct limp draws attention to the
unilaterally affected hip.
 External rotation and shortening of the leg on
the affected side are noted on examination.
 A click may be heard on forced abduction
(ortolani sign)

146. Diagnostic evaluation
 History collection
 Physical examination
 Ortolani test
 Barlow test
these tests are most reliable from birth to 2
or 3 months of age.
with Barlow test the thighs are adducted
where as the ortolani test involves abducting
the thighs to test for hip subluxation or
dislocation.

148.  X-rays (before dislocation)
shows hypoplasia of the femoral epiphysis,
increased angulation of the acetabular root and
deformed Shenton’s line ( an imaginary line drawn
along the inferior border of the superior pubic
ramus and along the inferomedial border of the
neck of femur).
 X-rays (after dislocation)
show the upward and outward displacement of
the under developed femoral head, together with a
poorly developed acetabular proof.
 CT scan
 Ultra sonography.

149.  Early diagnosis followed by a period of
traction in abduction which is best achieved
by Gallow’s splints with buttocks raised off
the bed.

150.  New born to age 6 months
 The hip joint is maintained by dynamic
splinting in a safe position with the proximal
femur centered in the acetabulum in an
attitude of flexion.
 Pavlik harness is the most widely used device

151.  When adduction contracture is present other
devices such as skin traction are used to
slowly & gently stretch the hip to full
abduction.
 When there is difficulty in maintaining stable
reduction, a hip spica cast is applied and
changed periodically to accommodate the
child’s growth.
 After 3-6 months sufficient stability is
acquired to allow transfer to a removable
protective abduction brace.

153.  After 6-18 months
 Gradual reduction by traction is used for
approximately 3 weeks.
 After reduction the child is placed in a hip
spica cast for 2-4 months until the hip is
stable, at which time a flexion abduction
brace is applied.

154. Older child
 Operative reduction which may involve
preoperative traction, tenotomy of contracted
muscles and any one of several innominate
osteotomy procedures designed to construct an
acetabular roof is usually required.
 After cast removal and before weight bearing is
permitted, range of motion exercises help
restore movement.
 Successful reduction and reconstruction become
increasingly difficult after the age of 4 yrs.

155. Prognosis
 Without treatment delay in learning to walk
may occur
 If dislocation is bilateral, significant lordosis
and waddling gait result.
Complications
 Avascular necrosis
 Redislocation
 Acetabular dysplasia
 Wound infection.

156.  It is the lateral curvature of the spine that
exceeds 10 degrees.
Incidence
 Idiopathic scoliosis accounts for 65% of all
cases of scoliosis with majority of those
occuring.

157. Etiology
 Unknown etiology
 Genetic factors
 Growth abnormalities
 Bone, disk, or CNS disorders
may contribute to its development.

158.  Idiopathic
o Unknown cause
o Infantile, occurs in the first 3 yrs of life.
o Juvenile, diagnosed between age 4 & 10 yrs or
prior to adolescence.
o Adolescent: age 11 to 17 yrs.
 Neuromuscular
o Associated with neurologic or muscular disease
such as cerebral palsy, myelomeningocele, spinal
cord tumors, spinal muscular atrophy.

159.  Myopathic
o Associated with certain types of muscular
dystrophy.
 Congenital
o Results from anomalous vertebral
development

160.  Uneven musculature on one side of the spine
 A rib prominence or a prominent shoulder blade,
caused by rotation of the ribcage in thoracic scoliosis.
 Uneven hips, arms or leg lengths
 Slow nerve action (in some cases)

162.  History collection
 Physical examination
 X-ray
 CT scan
 MRI
 Myelography
Myelography is a type of radiographic examination
that uses a contrast medium to detect pathology of the
spinal cord, including the location of a spinal cord
injury, cysts, and tumors.

163.  Tomography
Tomography refers to imaging by sections or
sectioning, through the use of any kind of penetrating
wave.

164.  For curves of 20-50 degrees, bracing may be
sufficient to decrease the progression of the
curve.
 Types of braces used to treat scoliosis are
 Under arm: less conspicuous, no visible neck
piece.

165.  Night time bending: creates a curve so severe
that walking is not possible, so can be worn
only at night.

166.  Surgical correction is often required for
curves greater than 45 degrees.
 It is achieved with rod placement & bone
grafting

167.  The term kyphosis refers to an enhanced
angulation in the thoracic or thoracolumbar
spine in the sagital plane or a round back
deformity.
 Clinical and radiologic evaluation is
necessary.
 Treatment is orthotic and/ or operative.

168.  Postural kyphosis the most common type, normally
attributed to slouching (improper posture ), can occur
in both the old and the young.
 Scheuermann's kyphosis is significantly worse
cosmetically and can cause varying degrees of pain,
and can also affect different areas of the spine (the most
common being the midthoracic area).
 Congenital kyphosis can result in infants whose spinal
column has not developed correctly in the womb.

169.  Nutritional kyphosis can result from nutritional
deficiencies, especially during childhood, such as
vitamin D deficiency (producing rickets), which softens
bones and results in curving of the spine and limbs
under the child's body weight.
 Post-traumatic kyphosis can arise from untreated or
ineffectively treated vertebral fractures.

170.  Orthosis (brace)
milwaukee brace
Modern brace for the treatment
of a thoracic kyphosis. The brace
is constructed using a CAD/CAM
device.

171.  Surgery
• Surgical treatment can be used in severe cases.
• In patients with progressive kyphotic deformity due to
vertebral collapse, a procedure called a kyphoplasty
may arrest the deformity and relieve the pain.
• Kyphoplasty is a minimally invasive procedure,
requiring only a small opening in the skin.

172. Definition
 This is a condition where the angle of the
femoral neck is less than normal.

173. Types
 Congenital
 It is seen in a wide range of neck- shaft
deformities, the angle sometimes being < 90
degree.
 It is likely that coxavara is related to the
maldevelopment of the upper part of the
femur.

174.  The acquired variety is more a sign than a
disease.
Causes
 Commonest cause is rickets
 The condition occurs in obese adolescents
with delayed skeletal maturation
 In tall & thin individuals with a recent growth
spurt or in such endocrinal disorders such as
hypopituitarism, hypothyroidism,
pseudohypoparathyroidism.

175.  Painful limp
 Pain in the anterior aspect of the thigh with
radiation to the knee
 External rotation of the hips on flexion is a
pathognomonic sign.

176.  Diagnosis
o Radiographs
 Treatment
o Surgical management
valgus osteotomy to improve hip
biomechanics and length and rotational
osteotomy to correct retroversion and
lengthening.
o Avoiding carrying heavy weights
 Complications
o Osteo necrosis
o chondrolysis

178.  This is a rare condition characterized by
brittle bones, blue sclera, a hereditary
tendency to deafness and lax ligaments.

179. Cause
 This disorder usually occurs as a result of a
defect in the collagen type I gene, usually
through an autosomal recessive or dominant
inheritance pattern.

180. Type I
 It is as an autosomal dominant disorder
characterized by osteoporosis and excessive
bone fragility with fractures, blue sclera and
conductive deafness (in adolescence or adult
hood).

181. Type II (osteogenesis imperfecta congenita)
A lethal syndrome characterized by
 Low birth weight & length
 Hypotelorism with breaking of the nose
 Extremely short, deformed & bent limbs.
 Broad thighs that are flexed at right angles to the trunk
 Crumpled long bones
 Fractured & beaded ribs in x-ray studies.
Whereas 50% are born dead, the remaining 50%
die soon after birth due to respiratory insufficiency as a
result of defective thoracic cage.

182. Type III
 An autosomal recessive disorder
characterized by multiple fractures and blue
sclera which tend to become less blue with
age.

183. Type IV
 An autosomal dominant disorder manifests
anytime from birth to adult life with fractures
and deformities.
 The sclera show a tendency to become less
blue with age.
 Deafness is less frequent.
 In some cases opalescent dentin may be
observed

186.  There is no specific therapy though adequate
nutrition and vitamin supplements, especially
Vit D in optimum amounts are essential.
 Young children suffering from this disease
should have their activities adequately
supervised.
 Magnesium oxide orally 15 mg/kg/day may
help to decrease the fracture rate.

187.  Biophosphonate administration is used for
moderate to severe cases.
 Physical therapy & occupational therapy
prevent contractures & maximize mobility.
 Light weight splints or braces may allow the
child to bear weight earlier.
 Severe cases may require surgical insertion of
rods into the long bones.

188.  It is a disorder of the connective tissue, which
provides the strength, support, and elasticity
to tendons, cartilage, heart valves, blood
vessels, and other vital parts of the body.

189.  It is characterized by
• A gracile habitus(small stature )with long
extremities (span more than height)
• Spider fingers & toes (arachnodactyly)
• Ocular anomalies (cataract, nystagmus,
ectopia lentis)
• High arched palate,
• spine & chest deformities and
• cardiovascular diseases (dissecting
aneurysms of aorta & valvular defects)

190.  Incidence
1 in 5000-10,000 births
 Cause
Autosomal dominant inheritance caused by a
mutation in the gene fibrillin-1, which results in
changes in connective tissue.
Fibrillin-1 protein forms fibers in connective
tissue. In addition to providing structural
support, the normal fibrillin-1 protein also
contributes to cell signaling activity.

192.  Clinical features
• Upper/lower segment ratio after maturity is
remarkably low.
• Length of middle finger is >1.5 times of its
metacarpal.
• 5th finger may show clinodactyly
(Curving of the fifth finger (the little finger)
toward the fourth finger (the ring finger)).
• Great toe is typically long.
• Steinberg sign (wrist sign)
ask patient to close his fist & try to
enclose the thumb within it. Then it would find
that it protrudes beyond the medial edge of the
hand.

193.  Homocystinuria is an important differential
diagnosis and must be ruled out by
demonstrating a negative urinary cyanide
nitroprusside test or specific aminoacid
studies.

194.  Treatment
 Supportive measures
 Control of respiratory infection
 Surgical correction of ocular and spinal
deformities of cardiovascular complications.

195.  It refers to the congenital contractures
(permanent shortening) of multiple joints
 Fibrous tissue replaces the affected muscles,
partially or completely.
 Skin is usually thickened and other
deformities like clubfoot may be there.

196.  Etiology
 Neuropathic origin supported by reduced number
of anterior horn cells
 Myopathic origin supported by diminution of
movements in utero.

197.  Every joint in the body has typical signs and
symptoms like
• the shoulder (internal rotation),
• hand (fingers in fixed flexion and thumb in
palm),
• hip (flexed, abducted and externally rotated,
frequently dislocated),
• elbow (extension and pronation) and
• foot (clubfoot).
• The range of motion capability can be different
between joints because of the different
deviations.

199. Treatment
 Correction of orthopedic deformities by
rehabilitation.