Pseudo arthrosis of tibia

1. PSEUDOARTHROSIS OF
TIBIA
Dr Sanjay K
Junior resident
Orthopaedics
KMCT Medical College

2.  ETIOLOGY
 PATHOPHYSIOLOGY
 CLINICAL FEATURES
 CLASSIFICATION
 INVESTIGATIONS
 DIFFERENTIAL DIAGNOSIS
 TREATMENT
 COMPLICATIONS

3.  Congenital pseudoarthrosis
of tibia (CPT) first described
by paget in 1891
 CPT refers to nonunion of a
tibial fracture that develops
spontaneously or after trivial
trauma in a dysplastic bone
segment of the tibial
diaphysis
 unilateral, located at junction
of middle and distal thirds of
the tibial segment
 Fibula affected in more than
half the cases

4.  CPT is rare, with an incidence of
approximately one in 250,000 live
births.
 50% to 90% association of this
disorder with the stigmata of
neurofibromatosis, including skin and
osseous lesions.
 Usually develops during first 2 years
of life

5. Etiology
 The exact cause of CPT is not known .
 Inheritant inability of the bone to form
callus at the site of fracture.
 Intrauterine trauma
 Birth fractures
 Metabolic disease
 Vascular malformation : NF 1

6. Physiopathology
 Fibrous hamartoma replaces the healthy
periosteum.
 Fibrous hamartoma leads to osteolysis and
vascular constriction of the bone.
 The bone is viable despite the osteolysis,
atrophy and hamartomatous constriction.
 Medullary canal obliterated due to sclerosis.
 Osteocytes produce lower levels than normal of
BMP.
 Increased osteoclasts and osteoclasis

7. CLINICAL FEATURES
 Anterolateral or anterior angulation /
bowing of leg at birth
 foot deformity
 Ankle valgus
 Skin dimple
 Signs of neurofibromatosis :
cafe-au-lait spots

8. Associated problems
 Leg length discrepancy,
 multilevel multidirectional tibial deformity
 proximal migration of the fibula,
 fibular nonunion,
 ankle mortise valgus
 ankle-joint dorsiflexion,
 valgus contracture,
 cavovalgus foot deformity, and
 persistent dorsiflexion contracture
before surgery

9. CLASSIFICATION
 Multiple classification systems
 more descriptive of the radiographic
appearance of the lesion at a
particular course in the disease
 provide little insight into the correct
type of treatment or prognosis

10.  Boyd
 Crawford
 Anderson

11. Boyd classification
Type I pseudarthrosis
 occurs with anterior bowing and a
defect in the tibia present at birth.

12. Type II pseudarthrosis
 occurs with anterior bowing
and an hourglass
constriction of the tibia
present at birth
 Spontaneous fracture, or
fracture after minor trauma,
commonly occurs before 2
years of age.
 The tibia is tapered,
rounded, and sclerotic, and
the medullary canal is
obliterated.
 This type is the most
common, is often associated
with neurofibromatosis, and

13. Type III pseudarthrosis
 develops in a
congenital cyst, usually
near the junction of the
middle and distal thirds
of the tibia.
 Anterior bowing may
precede or follow the
development of a
fracture.

14. Type IV pseudarthrosis
 originates in a sclerotic
segment of bone in the
classic location without
narrowing of the tibia.
 The medullary canal is
partially or completely
obliterated.
 An “insufficiency” or
“stress” fracture develops
in the cortex of the tibia
and gradually extends
through the sclerotic bone

15. Type V pseudarthrosis
 occurs with a dysplastic fibula.
 A pseudarthrosis of the fibula or tibia or
both may develop.
 The prognosis is good if the lesion is
confined to the fibula.
 If the lesion progresses to a tibial
pseudarthrosis, the natural history
usually resembles that of type II
pseudarthrosis.

16. Type VI pseudarthrosis
 occurs as an intraosseous
neurofibroma or schwannoma that
results in a pseudarthrosis.
 This is extremely rare.
 The prognosis depends on the
aggressiveness and treatment of the
intraosseous lesion.

17. Crawford classification
Non-dysplastic type
 I. Anterolateral bowing with increased
density and sclerosis of medullary
canal
Dysplastic type
 II. Anterolateral bowing with failure of
tubularization
 III. Cystic changes
 IV. Frank pseudarthrosis

19. Anderson classification
 Clubfoot
 Cystic
 Late
 Fibular
 dysplastic
 Angulated

20. Investigations
 Although diagnosis can be
made from plain radiographs,
MRI provides excellent details
of the extent of the
pathological process
 CT confirms radiographic
findings
 Total bone Scintigraphy : level
of pseudoarthrosis

21. Differential Diagnosis
 Congenital postero-medial bowing of the
tibia and antero-medial bowing
associated with fibular hemimelia.
 congenital longitudinal deficiency of tibia
(paraxial tibial hemimelia)
 Fracture nonunion
 Osteogenesis imperfecta
 Ehlers Danlos syndrome
 Fibrous dysplasia
 Rickets
 Post osteomyelitic pathological fracture

22. TREATMENT
 Treatment depends on the age of the patient
and the presence or absence of a fracture.
 Before walking age, little treatment is
required for a pseudarthrosis
 once the child begins to ambulate, the leg
should be immobilized in a clamshell orthosis
or PTB orthosis and protected.
 If no fracture is present, the child can be
treated in a brace until skeletal maturity with
close follow-up.
 Once a true pseudarthrosis of the tibia
develops, it cannot be expected to heal when
treated by casting or bracing alone.

23. Initial surgical management of tibial
pseudarthrosis involves 3 principles
 resection of the entire pseudarthrosis
and surrounding hamartomatous
tissue,
 restoration of mechanical alignment
and
 intramedullary fixation.

24. These three basic principles often are
augmented by a combination of
 primary shortening,
 bone transport,
 supplemental bone grafting, and
 bone morphogenetic protein

26. INTRAMEDULLARY FIXATION
 Most commonly used ,described by
Anderson
 The pseudarthrosis is often quite distal
in the tibia, making intramedullary
fixation alone inadequate and
unstable.
 Therefore, the ankle joint often must
be crossed by the rod to provide
additional stability in these very distal
pseudarthroses

27.  The rod can migrate with growth,
resulting in restoration of some ankle
motion over time or
 can be surgically advanced to a position
above the ankle once solid union has
been achieved.
 For those lesions that appear more
proximal in the tibia, it might be possible
to avoid crossing the ankle joint.
 In these cases, larger rod diameter or
an interlocking option could aid in
stability.

28. Insertion of Peter Williams rod for congenital pseudarthrosis of tibia

29. VASCULARIZED GRAFT
 Resection of the pseudarthrosis
with reconstruction using a free
vascularized bone graft with either
fibular or iliac crest grafts .
 The procedure requires experience
with microvascular techniques,
 two surgical teams are
advantageous, one to harvest the
graft while the second prepares
the pseudarthrosis site to receive
the graft.
 Vascularized fibular grafts may be
indicated for pseudarthroses with
gaps of more than 3 cm and for
pseudarthroses in which multiple
surgical procedures have failed.

31. ILIZAROV
 good preliminary results
were reported with the
Ilizarov technique,
 problems have included
difficulty transporting the
proximal tibia, “docking”
malalignment, and poor
quality of regenerated
bone, leading to
refracture.
 The Ilizarov approach
with bone transport does
offer the advantage of
maintaining or gaining
tibial length.

33. BONE MORPHOGENETIC
PROTEIN
 Multiple reports have documented the
successful use of recombinant human bone
morphogenetic protein (rhBMP)
 Both currently available forms (rhBMP-2 and
rhBMP-7) of this protein have been used.
 used in conjunction with other accepted
forms of bony stabilization such as
intramedullary fixation.
 Early union rates have been favorable, but
long-term follow-up and prospective
comparative studies are needed to better
understand the long-term efficacy and safety
of these treatments.

34. Prognostic Factors
 Factors reported to negatively affect
union :
1) neurofibromatosis;
2) age at treatment less than three
years;
3) previous failed surgery; and
4) years of follow- up after treatment.

35. COMPLICATIONS
 STIFFNESS OF THE ANKLE AND
HINDFOOT
 REFRACTURE
 VALGUS ANKLE DEFORMITY
 TIBIAL SHORTENING

36. STIFFNESS OF THE
ANKLE AND HINDFOOT
 A stiff ankle should be
expected until the distal
tip of the rod is proximal
to the ankle joint after
longitudinal growth of the
distal end of the tibia
 Even if stiffness persists,
it rarely hampers
functional results.

37. REFRACTURE
 common in patients with pseudarthroses,
despite apparently solid clinical and
radiographic union.
 Refracture can be managed with casting
or removal and replacement of the
intramedullary rod with additional bone
grafting.
 Because of the likelihood of refracture,
removal of the rod after union is not
recommended until skeletal maturity has
been reached

38. 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.
 Intraoperative fluoroscopy is
useful for monitoring this
procedure.
 Long-term bracing is
mandatory during the growth
years to minimize progressive
valgus ankle deformity, or
 surgical treatment with the
Langenskiöld procedure may
be indicated.

39. TIBIAL SHORTENING
 Tibial shortening should be
anticipated in almost all
children.
 can be treated by a well-
timed contralateral
epiphysiodesis or limb
lengthening of the proximal
tibia.
 The ilizarov technique may
be useful initially in severe
cases with significant
shortening and a wide
nonunion or in patients in
whom medullary nailing and
standard bone grafting
procedures fail

40. REFERENCES
 Campbell’s operative orthopaedics 13th edition
 Apley and Solomon’s system of orthopaedics and
trauma 10th edition
 National library of medicine - Pubmed central
(Congenital pseudarthrosis of the tibia:
Management and complications)
 https://www.orthobullets.com/pediatrics/4056/anter
olateral-bowing-and-congenital-pseudoarthrosis-of-
tibia

41. THANK YOU