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.

1. CONGENITAL
PSEUDOARTHROSIS TIBIA
Dr. J.FAISAL
Moderator :DR.SIDDHARTH
SHETTY
Dept of Orthopaedics
KSHEMA

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

5. Epidemiology
1 : 250,000 live births
50 % - 90 % associated with
neurofibromatosis ( cutaneous
and osseous lesion)
Usually – left
Bilateral - rare.

6. Aetiology
Aplasia/ dysplasia portion tibial
shaft – nutritional disturbances
Increased Intrauterine pressure
Amniotic bands
Part of neurofibromatosis

7. Pathology
Middle and
lower 3rd
junction.
Ends of bone
sclerosed with
gap

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

10. Boyds Classification

11. Type -I
Anterior bowing and defect in the
tibia at birth.

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

13. Type III
Congenital Cyst
middle & distal
third of tibia
Anterior bowing
may precede or
follow fracture
Excellent results.

14. Type IV
Sclerotic segment
middle/ distal 3rd
No narrowing
Medullary canal
partially/ completely
obliterated
Insufficiency/ stress
Fracture do not heal

15. Type V
Pseudo arthrosis tibia with
dysplastic fibula
Pseudo arthrosis tibia/ fibula/
both
Prgnosis good if only fibula

16. Type VI
Intra osseous neurofibroma /
schwannoma
Rare
Aggressive treatment

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

25. Bone grafting
Prophylactic
Deformed tibia
Before pathological fracture
To strengthen the deformed area
Decrease the risk of pathological
fractures

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

29. Stabilization
Compression plates (difficult in
achieving fixation)
Intramedullary rods

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.

34. PRE-OP
POST OP

36. Fractured dysplastic tibia
IM rod fixation and grafting
Tenuous union achieved
Fibula unhealed - ankle valgus
Distal tibial–fibular fusion -prevents
valgus

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

41. Microvascular Bone Graft
1.
2.
3.
Vascularized rib
Vascularized Iliac crest
Vascularized Fibula grafts – best

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

45. Two months
Extensive
subperiosteal
new bone
formation and
hypertrophy
of the graft

46. Twenty-two months later
The tibia is
healed
Leg is protected
in a knee ankle
foot orthosis

47. Thirty-three months
Tibia has healed
well
Medullary canal
is reforming in
the area of the
vascularized
fibula.

48. Ilizarov-Advantages
• Provides excellent stability
• Complete resection of the
pseudarthrotic area

49. Advantages
• Enables weight bearing ,which
stimulates healing of bone and
soft tissues
• Can transport fibula distally
• Donot interfere other treatment if
it fails

50. Disadvantages
Time-consuming
Not easy to perform
Pin track infections, fracture, ankle
valgus, and ankle stiffness

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.

52. Amputation
Amputation with appropriate
prosthetic fitting allows rapid
rehabilitation and return to normal
function

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

56. Amputation above the
pseudarthrosis site provides
better skin coverage
Problems - Bony overgrowth

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

65. Pseudarthrosis cannot be
satisfactorily healed
Symes amputation and
prosthetic replacement permit
restoration of relatively normal
function

66. Thank you