CONGENITAL

PSEUDOARTHROSIS TIBIA
Dr. J.FAISAL
Moderator :DR.SIDDHARTH
SHETTY
Dept of Orthopaedics
KSHEMA
 Definition
It is a specific type of non union
which is either present or incipient
at birth.
Its misnomer ( infantile
pseudoarthrosis).
Most difficult and challenging
deformities
 Epidemiology

1 : 250,000 live births

50 % - 90 % associated with
neurofibromatosis ( cutaneous
and osseous lesion)
Usually – left
Bilateral - rare.
 Aetiology
Aplasia/ dysplasia portion tibial
shaft – nutritional disturbances
Increased Intrauterine pressure
Amniotic bands
Part of neurofibromatosis
 Pathology
Middle and
lower 3rd
junction.
Ends of bone
sclerosed with
gap
 Biopsy
Dense, cellular, fibrous connective
tissue with areas of cartilage formation
Fibroblasts rather than Schwann cells or
perineural cells
Rarely – neurofibromatosis tissue
Hamatomatous tissue.
 Clinical presentation
 Angular deformity since birth
(anterolateral).
 If acute fracture then painful and unstable
 If not bony prominence with dimple over
skin
 Cafe-au-lait spots
 Positive family history
Boyds Classification
 Type -I
Anterior bowing and defect in the
tibia at birth.
 Type-II
Anterior bowing and
hour glass
constriction at birth
Fracture < 2 years
of age
Tibia tapered,
sclerotic , medullary
canal obliterated
Associated with
neurofibromatosis
 Type III

Congenital Cyst
middle & distal
third of tibia
Anterior bowing
may precede or
follow fracture
Excellent results.
 Type IV
Sclerotic segment
middle/ distal 3rd
No narrowing
Medullary canal
partially/ completely
obliterated
Insufficiency/ stress
Fracture do not heal
 Type V
Pseudo arthrosis tibia with
dysplastic fibula
Pseudo arthrosis tibia/ fibula/
both
Prgnosis good if only fibula
 Type VI
Intra osseous neurofibroma /
schwannoma
Rare
Aggressive treatment
 Prognosis
 Simple – best
 Cystic
 Scerlotic
 Sclerotic type with
pseudarthrosis of
the fibula worst
 Preoperative Management and
Planning
 Prophylactic treatment orthosis - delay or
prevent fracture - subsequent
pseudarthrosis
 Orthosis are worn for years.
 Knee ankle foot orthosis
 With growth and in the absence of a
fracture, the tibial bowing usually improves
 When to discontinue orthosis

 Tibia has straightened sufficiently

 Medullary canal has reconstituted
 Adequate cortical thickness
 Skeletal maturity is approached
Long-term reports of successful
orthotic management in adolescents
or adults not available
 Goals of surgery

1. Obtaining union at the

pseudarthrosis site
2. Maintaining union throughout
growth and development
3. Obtaining an acceptable limb
length at maturity
 Timing of surgery
Previously >4 years
Now recommend early surgical
intervention and revision if require
Masserman et al - union related to
pathologic process than the age at
surgery
Earlier union normal growth of the distal
tibial epiphysis and less limb length
discrepancy
 Surgical options
Bone grafting alone
Bone grafting and internal fixation
Electrical stimulation
Microvascular bone grafting
Ilizarov external fixation
Amputation
 Bone grafting

Prophylactic
Deformed tibia
Before pathological fracture
To strengthen the deformed area
Decrease the risk of pathological
fractures
 Mcfarland procedure

Corticocancellous graft from

opposite tibia
Placed posteriorly
Spanning the deformity
In the normal biomechanical axis
of weight bearing
53% best result out of all other
Paterson - Indicated primarily for
cystic prepseudoarthrosis
Tachdjian - Suggested concomitant
curettage and bone grafting of any
cystic lesions
Bone Grafting & Internal Fixation

Excision of pseudarthrosis
Correction of angular deformity
Rigid internal fixation
Bone graft – good outcome. Better
primary union
 Stabilization
Compression plates (difficult in
achieving fixation)
Intramedullary rods
Tibial or dual tibial and fibular
intramedullary rods
Transfix the ankle and subtalar
joints - stabilize the distal tibial
segment
Joints are progressively freed
growth of the tibia
Proximal migration of the rod
 Postoperatively

Unilateral hip spica cast - long-leg

cast - knee ankle foot orthosis
Anderson et al. - 10 of 13
pseudarthroses healed by
intramedullary rod technique
 Extending IM rods + bone graft

These rods extended with growth

Decreasing the need for revision
surgery
Protecting the union until skeletal
maturity
 Do not the include ankle or
subtalar joint
 Fern et al
Outer sleeve across the
pseudarthrosis site
Provide more strength and
reduce refracture.
Expand up to a maximum of 6.4
cm.
PRE-OP POST OP
Fractured dysplastic tibia
IM rod fixation and grafting
Tenuous union achieved
Fibula unhealed - ankle valgus
Distal tibial–fibular fusion -prevents
valgus
 Electrical Stimulation

Used in conjunction with internal

fixation and bone grafting
1.DC bone growth stimulators
Implanted
2.External stimulation devices with
pulsating electromagnetic fields
 Electrical Stimulation

Spindled bone ends, a large gap,

and gross mobility - poor
prognosis
Cystic or sclerotic transverse
fracture and a gap of less than 5
mm - better responses
 Mode Of Action

Induce bone formation

Alone effective in 50%
Remainder, additional procedures
are necessary before primary
union can be achieved
 Microvascular Bone Graft

1. Vascularized rib
2. Vascularized Iliac crest
3. Vascularized Fibula grafts – best
5 basic steps of free vascularized
bone grafts

• Harvest of the vascularized bone

with an intact vascular pedicle
• Excision of the tibial
pseudarthrosis and abnormal
tissue
• Fixation of the vascularized bone
in situ
• Microvascular anastomosis
• Skin closure
 Vascularized fibula
graft - performed at
17 months of age
 Internal fixation
was not used
 Ends of fibula graft
were inserted into
medullary canal
proximally and into
metaphysis distally
 Two months

Extensive
subperiosteal
new bone
formation and
hypertrophy
of the graft
 Twenty-two months later

The tibia is
healed
Leg is protected
in a knee ankle
foot orthosis
 Thirty-three months

Tibia has healed

well
Medullary canal
is reforming in
the area of the
vascularized
fibula.
 Ilizarov-Advantages

• Provides excellent stability

• Complete resection of the
pseudarthrotic area
 Advantages

• Enables weight bearing ,which

stimulates healing of bone and
soft tissues
• Can transport fibula distally
• Donot interfere other treatment if
it fails
 Disadvantages

Time-consuming
Not easy to perform
Pin track infections, fracture, ankle
valgus, and ankle stiffness
 Used in four ways
1. Compression of the pseudarthrosis
2. Compression with metaphyseal
tibial lengthening
3. Compression followed by distraction
for hypertrophic nonunion
4. Distraction alone for hypertrophic
nonunion
 Amputation

Amputation with appropriate

prosthetic fitting allows rapid
rehabilitation and return to normal
function
 McCarthy-criteria/indication

• Failure to achieve bony union after 3

surgical attempts
• Significant lower-extremity length
inequality (usually 5 cm or greater)
• Development of a deformed foot
• Undue functional loss from prolonged
hospitalizations
• High medical costs
Boyd or Symes amputation -
procedure of choice
Preserves the heel pad and distal
tibial epiphysis, which allows end
bearing on the stump
Bone and skin are lengthened as
a unit to avoid problems with
overgrowth
B/K amputation at pseudarthrosis
poor end-bearing stump
Abnormal tissue and previous
surgical scar -poor skin coverage
increased breakdown
Overgrowth and frequent revision
Amputation above the
pseudarthrosis site provides
better skin coverage
Problems - Bony overgrowth
Rehabilitation and Postoperative
Principles
To restore maximum strength and
function after healing
Each surgical procedure has its
specific postoperative regimen
But all share long-term orthotic
management
Protection is required at least until
skeletal maturity and perhaps even
longer
Decision

1. Radiographic appearance of the tibia

2. The degree of residual deformity
3. Presence or absence of a reconstituted
medullary canal
 Extremity needs to be protected with a
plastic ankle foot orthosis (prevent
recurrent refracture)
 Orthosis are worn for years. With growth
and in the absence of a fracture, the tibial
bowing usually improves
 Complications

Stiffness of the Ankle and Hindfoot

Refracture - casting or removal and
replacement of the intramedullary
rod with additional bone grafting
 Valgus Ankle Deformity
The distal tibial fragment must be
fixed so that valgus deformity of the
ankle is corrected at the time of
placement of the intramedullary rod
Long-term bracing is mandatory
during the growth years
Surgical treatment - Langenskiöld
procedure (tibio fibular synostosis)
 Tibial Shortening
Anticipated in almost all children -
Anderson et al. 4 cm.
Contralateral epiphysiodesis or limb
lengthening of the proximal tibia.
Intramedullary nailing with bone
grafting, with or without electrical
stimulation, is recommended for an
established pseudarthrosis.
 Conclusion
Minimize the number of
operative procedures
Maintain as normal function as
possible
Prevention of fractures - critically
important
Best results with respect to
union are achieved with a
vascularized fibula graft or
intramedullary rod
Initial surgical procedure should
be the latter
Pseudarthrosis cannot be
satisfactorily healed
Symes amputation and
prosthetic replacement permit
restoration of relatively normal
function
Thank you