1. By: Dr. Kush vyas
Moderator: Dr. sanjeev Reddy

2.  32 days: first evidence of limb buds
appearance
 3rd wk: limb makes their appearance as a
small elevation as buds at side of the trunk
 4th-5th wk: axial part of the mesoderm of the
limb bud becomes condensed converted into
cartilaginous skeletal and ossification center
of the limb formed

3.  6th wk: upper furrow: arm, forearm hand
:Lower furrow: thigh leg foot

6.  Medial side of the leg
 After femur the longest bone to be formed
into body
 1 body
 2 extremities from where tibia bone going to be
formed
 – upper extremity
 lower extremity

7.  Large and expanded into two eminences,
 medial
 lateral
 Borders
 Medial border: smooth ,rounded above and
below and prominent in centre
 Lateral border: a/k/a interossous crest , thin
and prominent where interossous memb.
attaches

8.  Surfaces:
 Medial surface: smooth concave and broader
 Lateral surface:
 narrower than medial
 upper 2/3rd:shalow grove for origin of the tibialis ant.
 Lower 1/3rd : smooth and concave curves gradually
forward to ant aspect of the bone covered by
tendons of tibialis ant. ,EHL , EDL

9.  Post. surface:
 prominent ridge will be popliteal line
 MIDDLE 1/3rd : divided into two part by vertical ridge
 Medial and broader part: will give origin to FDL
 Lateral and narrow part: tibialis post.
 remaining post. Part is smooth and covered by
tibialis post. ,FDL and FHL

10.  Much smaller than upper
 surfaces:
 Ant surface:
 above- smooth and rounded covered by tendon of
extensors
 Lower margins : rough and transverse depression
where articular capsule of ankle joint attaches
 Post surface:
 transverse shallow grove
 Post surface of talus
 Serving passage for tendon of FHL

11.  Lateral surface: triangular rough depression,
inferior interoosoeus ligament connecting with
fibula
 Medial surface: medial malleolus

13.  Specific type of the non union
 Either well established or incompletely
developed
 characterized by osseous dysplasia and
segmental weakness of bone resulting in
anterolateral angulations of tibia with
pathological fracture with lack of bone union

14.  Define: congenital condition of unknown
origin in which discontinuity of the bone at
the junction of middle and distal 1/3rd or
beyond is present at the birth or develops
during growth period and permitting
persisting abnormal mobility and creating
illusion of false joint
 Incident: 1 in 250,000 live birth

16.  1891:Paget one of the first describe a case
of CPT
 1903: codivilla performed a lengthening
through a femoral osteotomy followed by
gradually calcaneal traction and casting
 1921: Putti lengthened the femur using
distal traction and proximal counter traction
followed by nail inserted in the femur

17.  1937: ducroquet and cottard describe
association of CPT with neurofibromatosis
 1951: Mc farland suggeted bone bypass graft
 1961: G.A. Ilizarow discovered that slow
distraction of a corticotomy through process
through process called distraction
osteogenesis could produce new bone in
widening distraction gap

18.  1956: charney treated CPT with intra
meddulary nail
 1982: boyd classified CPT
 1985: pho et all describes efficiency of
vascularised fibular graft

21.  Unknown
 40 % of patient present with typical of
neurofibromatosis or von Recklinghausen’s
disease which is inherited as an autosomal
dominant trait with variable penetrance and
high rate of mutation

22.  A possible relationship of neurofibromatosis,
fibrous dysplasia and CPT is suggested
 The fact the fibrous constriction lesion is
universally present which suggests primary
pathological lesion is in the periosteal
structures found in tibia
 the defect leads to fracture and non union,
mechanical forces accentuate the problem

23.  Tibia is the most commonly affected site
associated with congenital anterolateral
bowing in neurofibromatosis which
progresses to neurofibromatosis
 CPT can also occur in fibula, radius, ulna,
femur and clavicle

26. 1.Complete defect in bone
2.Congenital bone cyst
3.Congenital bowing of tibia

27. typical pseudoarthosis found at birth.
 The upper and lower segment represent
 variable diaphysis, ranging from proximal
segment representing 2/3rd to 3/4th of the bone
 a shorter distal fragment ranging from 1/3rd to
very small portion of the bone
 Nearing the defect the diaphysial portion
become progressively tapered, their ends
sclerotic and medullary canal obligated

28.  The interval between the bone ends are
occupied by very cellular fibrous tissue which
continue with periosteum
 When the fibers are arranged in whorls and
contain an occasional fine bone trabeculum,
it resembles the histological picture of
fibrous dysplasia

29. occurs in the lower third of tibia microscopic
picture closely resembles that seen in fibrous
dysplasia
 Diameter of tibia neither narrowed nor
appreciably expanded
 fracture invariably takes place through the
weaned shaft

30. Complicated by fracture will results in
pseudoarthosis
 Anterolateral bowing is usually predisposed to
this complication
 Postero medial bowing rarely undergoes a
pathological fracture

31.  Boyd classification:
 Type1:
 Ant. Bowing and defect in tibia present at birth
 Other congenital deformities also present which
affect the ultimate management of the
pseudoarthorosis

32.  Type2:
 Most common
 Often associated with NF
 Worst prognosis
 Ant bowing and hourglass constriction of tibia
present at birth
 Spontaneous fracture or fracture following minor
trauma commonly occurs before 2 yr of age
 ‘’HIGH RISK TIBIA’’
 Tibia is tapered and sclerotic and the medullary
canal is obliterated

33.  Type3:
 Develops in congenital cyst usually near the
junction of the middle and distal third of the
tibia
 Ant bowing may precede or follow the
development of fracture
 Recurrence of the fracture after Rx is less
common than type 2
 excellent results after only one operation noted

34.  Type 4:
 Originates in the sclerotic segments of the bone
in classic location without narrowing of tibia
 Medullary canal is partially or completely
obliterated
 Insufficiency or stress fracture develops in the
cortex of the tibia and gradually extends through
the sclerotic bone
 With completion of the fracture, healing fails to
occur and fracture widens and pseudoarthosis
occur
 Prognosis: good to better

35.  Type5:
 Pseudoarthosis of tibia with dysplastic fibula
 May be both bone involved
 Prognosis: good if lesion confined to fibula
 If progresses to tibia mostly prognosis and progression
resembles type 2

36.  Type6:
 Occurs in interaosseous neaurofibroma or
schwanoma that results in pseudoarthosis
 rarest

37.  Type 1 :
 ANTEROLATERAL BOWING OF TIBIA
 Type 2:
 anterolateral bowing
 increased cortical thickness
 narrow medullary canal
 tubular defect
 Type 3:
 cystic lesion
 Type 4:
 presence of fracture, a cyst or frank
pseudoarthosis

39. —7-month-old boy with neurofibromatosis and congenital pseudoarthrosis of the tibia
(Crawford type I).
7-month-old boy with neurofibromatosis and
congenital pseudoarthorosis of the tibia
(Crawford type I).
shows congenital pseudoarthorosis of the
tibia as hyperintense lesion
Note diffuse edema of soft tissue ventral to
the tibia

40. —7-month-old boy with neurofibromatosis and congenital pseudoarthorosis of the tibia
(Crawford type I).
7-month-old boy with neurofibromatosis and
congenital pseudoarthorosis of the tibia (Crawford
type I)
T1-weighted MR image depicts anterolateral
bowing and circumscribed cortical thickening of
the tibia Medullary canal is preserved
Bone marrow in region of anterolateral bowing of
tibia shows slight hypointensity

41. —1-month-old male neonate with congenital pseudoarthorosis of tibia (Crawford type III)
without neurofibromatosis.
ty
—1-month-old male neonate with congenital
pseudoarthrosis of tibia (Crawford type III)
without neurofibromatosis
Lateral radiograph of calf depicts characteristic
cystic lesion in distal part of tibia

42. —1-month-old male neonate with congenital pseudoarthrosis of tibia (Crawford type
III) without neurofibromatosis.
y
—1-month-old male neonate with congenital
pseudoarthorosis of tibia (Crawford type III)
without neurofibromatosis

43. —1-month-old male neonate with congenital pseudoarthrosis of tibia (Crawford type
III) without neurofibromatosis.

44. —1-month-old male neonate with congenital pseudoarthrosis of tibia
(Crawford type III) without neurofibromatosis.

45. —5-year-old boy with neurofibromatosis and congenital tibial pseudoarthrosis
(Crawford type IV).
5-year-old boy with neurofibromatosis and
congenital tibial pseudoarthorosis (Crawford
type IV)
Lateral radiograph of right leg depicts
congenital tibial pseudoarthorosis type IV
with frank pseudoarthorosis of distal tibia.

46. —5-year-old boy with neurofibromatosis and congenital tibial
pseudoarthrosis (Crawford type IV).
5-year-old boy with
neurofibromatosis and congenital
tibial pseudoarthrosis (Crawford
type IV).
pseudoarthrosis is revealed as tibial
non-union with increased signal
intensity (arrowheads) and
hyperintense tissue ventral to
nonunion (arrows).

47. —5-year-old boy with neurofibromatosis and congenital tibial
pseudarthrosis (Crawford type IV).
y
—5-year-old boy with neurofibromatosis and
congenital tibial pseudoarthorosis (Crawford
type IV)
show a hypo intense pseudoarthorosis area
with hypo intense soft tissue ventral to
pseudoarthorosis

48. —5-year-old boy with neurofibromatosis and congenital tibial pseudoarthorosis
(Crawford type IV).
y
5-year-old boy with neurofibromatosis and
congenital tibial pseudoarthorosis (Crawford
type IV). Pseudoarthorosis and soft tissue
(arrows)
show marked contrast enhancement after
administration of gadolinium

49.  frequently noted at birth
 The deformity is evident from the apical
prominence laterally in the leg with the foot
inverted or at least medially displaced in
relation to the lower leg
 If neonatal fracture has occurred, mobility at
pseuarthrosis sight will be evident
 Vast majority of anterolateral deformity are
unilaterally, the shortening and angulations
is easily appreciated when the affected leg is
compared with the normal leg

50.  If signs f neurofibromatosis are present , diagnosis is
readily apparent
 A milder deformity unaccompanied by signs of
neurofibromatosis may not come to medical attention
until much later when a limp due to deformity or an
impending fracture draws attention to the leg
 the foot and ankle may be normal or slightly normal
or slightly smaller than the contra lateral side
 Presence of cutaneous manifestations of
neurofibromatosis clarifies the diagnosis
 The late form of pseudoarthosis presents as fracture
in an older child will probably be companied by of
this findings
 x ray is the diagnostic tool

51.  A dysplasia of the mesodermal and
neuroactodermal tissue ,neurofibromatosis is a
hereditary dysplasia that may involve almost
every organ system of the body .
 Incidences: 1 in 3000 live births .
 50% are autosomal dominant and
 50% are sporadic from spontaneous
mutatations
 Neurofibromatiosis is divided into two types
1.NF1 or von Recklinghausen disease
2.NF2 or bilateral acoustic neuroma
frequency of 9:1NF1 to NF2

52.  Chromosome 17 is defective in NF1 and skin
manifestation are prominent ,such sessile or
pedunculated skin lesions (mollusca fibrosa)
and café au lait spots
 Haemartomas,gliomas, and alignment nerve
sheath tumor are also seen in NF1along with
spiral neaurofibroma.
 Chromosome 22 is defective in NF2 and
there are minimal skin manifestation .
 Bilateral acoustic neuromas meningiomas ,
schwanoma are seen in NF2 along with spinal
schwanoma

53.  Only NF1 manifests skeletal finding with radiographic
features
 At least 50% of patients demonstrate bony changes
 most commonly pit like cortical erosions resulting from
direct pressure from adjacent nuerofibromas ,
 commonly in long ones and ribs .
 The ribs may also appear twisted or ribbon like
 The long bones may demonstrate overgrowth,
cortical defects or, bowling and sclerosis.
 Pseudoarthosis formation , most commonly within
tibia
 The long bones are the sight of fibrous cortical
defects and nonossifying fibromas associated with
neurofibromatosis

60.  AP AND LATERAL VIEW SHOULD BE TAKEN
 Initially,
 bowing of the tibia convex anteriorely or
anterolaterally with narrowing of the tibial shaft
and sclerosis encroaching on the medullary cavity
at apex
 The apex of the of the curve is typically at
junction of middle and distal third of the
shaft of the tibia

61.  Sometimes, shows pointed distal fragment
and cupped proximal fragment which fit
together to form a false joint
 There is often associated tibial shortening
 50% cases, associated With NF will show lytic
lesion within the tibia

71. 1.Fibrious dysplasia
2.Rickets
3.osseous syphilis
Early congenital syphilis
Late congenital syphilis
4.infantile scurvy
5.Weisman Netter Stuhl syndrome
6.osteogenesis Imperfecta
7.Nonunion of fracture
8.blounts disease(infantile tibia vara)

72.  depends on age and type
 A true congenital pseuarthrosis of the tibia
will not heal when treated by casting alone .
treatment categorized as
1. prophylactic
2. directed towards pre pseudoarthosis lesions
3.active directed towards fully developed
congenital pseudoarthorosis

73.  Patients with
 anterolateral bowing of the tibia at the junction of the middle
and distal 3rd of the diaphysis
 accompanied by narrowing and sclerosis
 lose of definition of medullary canal
 considered at a risk for developing pathological fracture
and non union .
 The extremity must be protected by an orthotic and the
child guarded against excessive activity
 As longitudinal growth and remodeling take place, there is
a tendency towards spontaneous correction of the
deformity and restoration of normal architecture
 The threat of fracture is present until skeletal maturity is
reached

74.  If fracture and pseudoarthosis is eminent
particularly when the bone structure is
inadequate and especially in presence of a cyst
,child’s activity cannot be controlled
 The weakened region of the tibia is by passed by
a cortical or rib transplant
 Since congenital cyst in this region invariably
fractures
This is treated by bypass procedure and later
curette the defect, fill it with bone transplant
and immobilize the leg in a cast till the defect is
obliterated and the tibia of the adequate size is
obtained

75.  for an established pseudoarthorosis ,surgical treatment is
necessary
 older the patient more likely success of union so, age of
puberty would seem to be appropriate but by this time ,
the leg is under developed, deformed and short and
amputation frequency preferred
 aggressive surgical interventions is undertaken early in
childhood, because sufficient time reminds for growth
factors to operate so that when skeletal maturity is
reached , a well-developed even normal extremity results
 if the initial surgery fails , the procedure may be repeated
 surgery can be attempted as a early as four years of age
 If surgery is to be postponed , deformity should be
prevented by an orthotic

76. The principles of treatment are as follows :
1.Stabilization
a.dual on lay bone grafts (Boyd)
provides both stability and induction of
osteogenesis
Tapered slender bones will not permit its use

77.  B . Intramedullary rod (charnley)
 Disadvantages :
 Permits the rotary forces which can be controlled
with a thigh length cast with the knee flexed at
45
 Advantage:
 Permits osteogensis inducing axial compressive
forces; controls a small distal fragment by trans-tarsal
insertion of the rod

78.  C. External skeletal fixation
 Useful for maintaining length ,when the defect is
inadequate and when poor soft tissue of the
pseuarthrosis will not permit local fixation device

79.  2.stimulation of osteogenesis
 A . Bone transplants
 autogeneous cancellous bone is preferred
 B. Electrical stimulation :
 used along or as an adjunctive measure with
stabilization and bone transplant
 this will increase the rate of bony union.

80.  3.maintainace of cast immobilization
 This is accomplished until bony bridging is
adequate and the structure and size of the tibia
are sufficient to withstand the possibility of a
pathlogigal fracture.
 The cast must be applied to the upper thigh,
and the knee must be kept flexed at 45 degree to
prevent gravity induced tensile force

81.  4.compression versus non compression stress
 Proponents of intramaddulary rodding advocate
immediate wait bearing to encourage
compression stress that induce osteogenesis

82.  Two tibial cortical grafts and the supply of
cancellous bone chips are removed from a
donor or obtained from a bone bank
 The pseudoarthorosis is exposed, and the
fibrous tissue , which may surprisingly extend
into the surrounding soft tissue, is
completely excised .
 The eburnated bone is removed from the
bone ends and the medullary canal is opened
up by drilling

83.  To correct bowing and the equines deformity an
Achilles tenotomy and a fibular osteotomy are
required if possible ,the fibula should be preserved to
aid postoperative immobilization
 The lateral surface of the tibia above and below are
shaved down flat , and the tibial cortical transplant
are affixed ,one medullary and other laterally .
 A space is left between the ends of tibial fragments
into which are packed the cancelous bone chips .
 Only skin and subcutaneous tissue are closed .
 A cast is applied and immobilization is continued until
union is apparent .
 Fracture and non union are postoperative
complications ,and proyectio of the leg by a lether
lacer brace or a plastic orthosis and until skeleton
maturity is mandatory

84.  Fixation by an interaamadulary rode eliminate
an angulatory strain while permitting beneficial
axial compressive forces desperate the criticism
that is still allows rotational stresses.
 rodding of the tibia by the transtrasal approach
is the most commonly used method of fixation
 it can be used alone with external plaster
support ,but generally autogeneous cancelous
bone or osteo-perosteal bone transplant are
added fixation tarsus will prevent nail from
cutting of short distal segment of the tibia
before introducing the nail
 Achilles tenotomy will overcome the bowing
action that prevents correction of deformity

87.  mcElvenny first called attention to the heavy
cuff of tissue surrounding the bone at
pseudoarthorosis and he reasoned the
presence of these tissues , whether
congenital or a result of a fracture , may
decrease bone production and consequently
healing .
 any operation for congenital
pseudoarthorosis should include complete
excision of this tissue

88.  the operation for prophylactic treatment of
an imminent fracture and pseudoarthorosis a
congenital angulation of the tibia
 a single graft is placed posteriorly so as to
span the pseudoarthorosis except and the
upper and lower sites of implantation the
transplant is entirely exta-periosteal

89.  The operation is also used preliminary to
curettage and bone grafting of a congenital
cyst pre-pseudoarthrotic lesion

90.  Sofield’s multiple osteotomies with internal
fixation by medullary rods:
 Fragmentation
 Reversal of fragments
 realignment

91.  Micro vascular transfer of the contrlateral or
ipsilateral fibula
 Pre operative arteriography is must to determine
anastomosis sight and rule out anomalies
 Disadvantage:
 Non union at one end of the tibia
 Stress fracture
 Morbidity of donor leg

94.  Principle: correct all angular deformity and
maximizes the cross sectional area of union
of pseudoarthorosis
 Therapeutic consideration includes:
 Type of bone graft
 Type of fixation
 Whether to resects the pseudoarthorosis or not
 Electrical stimulation has been used
primary goal: union of the site

95.  Most imp thing to be kept in mind:
 Correct associated problems
 Leg length discrepancy
 Multilevel multi directional tibia deformity
 Proximal migration of fibula
 Fibular non union
 Ankle mortise valgus
 Ankle joint dorsiflexion
 Cavo valgus deformity
 Valgus contracture

96.  Principle of distraction osteogenesis:
 Same as limb lengthening principle i.e distraction
of a bone segment after low energy periosteal
osteotomy by mechanically applied tension force
is primary method of limb lengthening
 For skeletally immature patient:
 Epiphysiolysis of epiphyseal distraction

97.  The site of bone regeneration constitutes the
centre for stimulating the surrounding
musculo-facial neurovascular and cutaneous
tissue
 The degenerated bone undergoes maturation
or consolidation phase at the end of the
lengthening until the new bone is
morphologically and functionally in
distinguishable form

98.  1 mm/day is the critical rate and critical
rhythm in limb lengthening
 (0.25/6hour is the standard format should be
followed clinically)
 Lengthening should be performed without
interruption function of the limb including partial
wt bearing in the leg is actively encouraged

99.  Indication:
 Limb lengthening
 Angular or multiplanar deformities
 Foot deformities including lengthening of the
metatarsal
 Congenital anomaly of the lower limb( tibial and
fibular hemimelia)
 Joint contracture
 Non union and infected non union
 Acute fracture
 Fusion of the joint
 In case of dwarfism

100.  1.when to lengthen??
 2.how much bone can be lengthened??
 3.which external fixator should be used??
 4.what are the stage of the lengthening
proess??
 5.How long should be external fixator used??

101.  Amount of the discrepancy and age of the
patient
 Discrepancy; >5 cm or
 expected discrepancy at the age of skeletal
maturity i.e boys 16 yrs and girls 14yrs
 No strict age demarcation is there but the
idea behind eligibility is child should bear
weight and co operate with the excersise
regimen

102.  If angular deformity is present and
discrepancy is even less than 5 cm:
 Should be corrected surgically and using external
fixator

103.  Yet to be determined
 20 to 25% of original length gived good
results
 More than 25% lengthening lead to higher
incidence of all types of complications

104.  Most of the time is surgeon’s choice
 Ilizarow circular fixator for tibial lengthening
 Uniplanar fixator for femoral lenghtening
 Half pins for uniplanar fixator
 K wire for ilizarow

105.  4 stages
 Distraction
 Consolidation
 Removal of frame
 rehabiliation

106.  Distraction:
 5-7 days post operatively
 Optimum distraction 1mm/day (0.25mm/6 hour)
 Example: 5 cm lengthening will require
approximately 50 days

107.  Consolidation:
 External fixator kept on the patient until the
bone formed consolidates and becomes strong
enough to allow safe removal
 Duration: amount of the lengthening
 Aprox:1 month for 1cm

108.  Removal of the frame:
 Evaluate quality and strength of the new bone
 Plain radiograph should show
 New bone formation
 Should completely bridges and lengthened the site
 New cortex formation
 Followed bt brace or cast support for 6 wks

109.  Rehabilitation:
 After removal of cast and brace
 Physiotherapy
 ROM

110.  As explained, for every 1 cm 1 month to 2
month required
 Depends on discrepancy