This document discusses congenital pseudarthrosis tibia, a rare birth defect where the tibia fails to properly heal. It has a prevalence of 1 in 250,000 live births. The document covers the definition, causes, presentations, classifications, surgical treatment options including bone grafting, plating, rodding, electrical stimulation, and amputation. The goal of treatment is to achieve bony union while maintaining limb length and function. Early surgical intervention and long-term bracing are recommended to prevent fractures and deformities as the child grows. The best surgical options are vascularized fibula grafting or intramedullary rodding with bone grafting.
2. 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
3.
4.
5. Epidemiology
1 : 250,000 live births
50 % - 90 % associated with
neurofibromatosis ( cutaneous
and osseous lesion)
Usually – left
Bilateral - rare.
8. Biopsy
Dense, cellular, fibrous connective
tissue with areas of cartilage formation
Fibroblasts rather than Schwann cells or
perineural cells
Rarely – neurofibromatosis tissue
Hamatomatous tissue.
9. 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
12. Type-II
Anterior bowing and
hour glass
constriction at birth
Fracture < 2 years
of age
Tibia tapered,
sclerotic , medullary
canal obliterated
Associated with
neurofibromatosis
17. Prognosis
Simple – best
Cystic
Scerlotic
Sclerotic type with
pseudarthrosis of
the fibula worst
18. 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
19. When to discontinue orthosis
Tibia has straightened sufficiently
Medullary canal has reconstituted
Adequate cortical thickness
Skeletal maturity is approached
20. Long-term reports of successful
orthotic management in adolescents
or adults not available
21. Goals of surgery
1.
2.
3.
Obtaining union at the
pseudarthrosis site
Maintaining union throughout
growth and development
Obtaining an acceptable limb
length at maturity
22. 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
23. Surgical options
Bone grafting alone
Bone grafting and internal fixation
Electrical stimulation
Microvascular bone grafting
Ilizarov external fixation
Amputation
26. 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
27. Paterson - Indicated primarily for
cystic prepseudoarthrosis
Tachdjian - Suggested concomitant
curettage and bone grafting of any
cystic lesions
28. Bone Grafting & Internal Fixation
Excision of pseudarthrosis
Correction of angular deformity
Rigid internal fixation
Bone graft – good outcome. Better
primary union
30. 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
31. Postoperatively
Unilateral hip spica cast - long-leg
cast - knee ankle foot orthosis
Anderson et al. - 10 of 13
pseudarthroses healed by
intramedullary rod technique
32. 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
33. Fern et al
Outer sleeve across the
pseudarthrosis site
Provide more strength and
reduce refracture.
Expand up to a maximum of 6.4
cm.
36. Fractured dysplastic tibia
IM rod fixation and grafting
Tenuous union achieved
Fibula unhealed - ankle valgus
Distal tibial–fibular fusion -prevents
valgus
37.
38. 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
39. 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
40. Mode Of Action
Induce bone formation
Alone
effective in 50%
Remainder, additional procedures
are necessary before primary
union can be achieved
42. 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
43. •
•
•
Fixation of the vascularized bone
in situ
Microvascular anastomosis
Skin closure
44. 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
49. Advantages
• Enables weight bearing ,which
stimulates healing of bone and
soft tissues
• Can transport fibula distally
• Donot interfere other treatment if
it fails
51. Used in four ways
Compression of the pseudarthrosis
2. Compression with metaphyseal
tibial lengthening
3. Compression followed by distraction
for hypertrophic nonunion
4. Distraction alone for hypertrophic
nonunion
1.
53. 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
54. 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
55. B/K amputation at pseudarthrosis
poor end-bearing stump
Abnormal tissue and previous
surgical scar -poor skin coverage
increased breakdown
Overgrowth and frequent revision
57. 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
58. 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
59. 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
60. Complications
Stiffness of the Ankle and Hindfoot
Refracture - casting or removal and
replacement of the intramedullary
rod with additional bone grafting
61. 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)
62. 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.
63. Conclusion
Minimize the number of
operative procedures
Maintain as normal function as
possible
Prevention of fractures - critically
important
64. Best results with respect to
union are achieved with a
vascularized fibula graft or
intramedullary rod
Initial surgical procedure should
be the latter