short class room presentation for PG student on congenital pseudoarthrosis of Tibia.

1. CONGENITAL PSEUDO ARTHROSIS
OF TIBIA- CPT
Dr. Rejul K Raj, CMC Ludhiana

3. INTRODUCTION.
• Type of non union.
• Present at birth or incipient.
• rare pathology. 1 in 2,50,000 live births.
• neurofibromatosis.
• Cause- unknown.
• Most commonly in disal tibia.

4. Introduction – contd.
• extremely unfavorable.
• Poor healing potential at the pseudoarthrosis.
• there is a little or no tendency for the lesion to heal
spontaneously.
• It is challenging to treat effectively
• Hamartomatous thickened fibrous tissue with
limited vascular ingrowth.

5. • thin and atrophic
tibial bone
• pointed distal
fragment.
• The false joint is in
the distal third of the
shaft.
• The fibula is affected

6. • Untreated CPT

7. Problems- Primary
1. Obtaining union
2. Shortening of the limb –
1. At birth itself.
2. Pseudarthrosis remains ununited.
3. Repeated unsuccessful operations
3. Tendency for refracture- diminishes with
skeletal maturity.

8. Problems- secondary deformities
a) abnormal inclination of the proximal tibial physis
b) posterior bowing of the proximal third of the tibial
diaphysis
c) proximal migration of the lateral malleolus
d) fibular hypoplasia
e) fibular pseudarthrosis
f) ankle valgus and calcaneus deformity

10. CLASSIFICATIONS
• Andersen's classification – 1973
• Boyd's classification- 1982
• Crawford's classification- 1986
• EI-Rosasy-Paley-Herzenberg's classification- 2007

11. BOYD’S- 1982, 6 types
• Type I - anterior bowing with tibial defect.
• Type II – Most common
– with hour glass constriction
– spontaneous fracture
– HIGH RISK TIBIA ( tapered ,rounded, sclerotic ,
obliterated )
– Poorest prognosis.
• Type III - with congeital cyst
• Type IV- with sclerotic segments, sterss fractures.
• Type V -with dysplastic fibula.
• Type VI- an intraosseous neurofibroma or
schwannoma is evident

13. Crawford's classification- 1986
• Type I-
– the medullary canal is preserved and cortical
thickening at the apex of the deformity might be
observed
– good prognosis;
• Type II –
– presence of thinned medullary canal, cortical
thickening, and trabeculation defect.
• type III -
– cystic lesion, which may be fractured; patients
with this type of pseudarthrosis tend to
experience early fracture and, therefore, require
early treatment.
• Type IV-
– pseudarthrosis is present with tibial and possibly
fibular nonunion.

14. EI-Rosasy-Paley-Herzenberg's
classification- 2007
1. patient's condition at time of presentation (relation to previous
treatment),
2. radiological (atrophic or hypertrophic),
3. mobility of pseudarthrosis site (stiff or mobile).
• Type 1
– Atrophic (narrow) bone ends
– Mobile pseudarthrosis
– No previous surgery
• Type 2
– Atrophic (narrow) bone ends
– Mobile pseudarthrosis
– Previous unsuccessful surgery
• Type 3
– Hypertrophic (wide) bone ends
– Stiff pseudarthrosis

16. AIMS
1. Achieve union
2. Prevent refracture
3. Correct limb length inequality
4. Correct associated growth abnormalities
5. Prevent ankle deformity and arthritis.

17. 1. Strategies to achieve union
1. Microvascular free fibular transfer.
2. the Ilizarov technique
3. Bone grafting with intramedullary nailing.
• Excision of the pseudarthrosis should be an
integral part of the procedure.

18. 2. Strategies for minimizing the risk of
refracture
• Splint the limb in an orthosis until skeletal
maturity.
• Retain an intramedullary nail until skeletal
maturity.

19. 3. Strategies for dealing with
shortening of the limb
● Minimize the extent of shortening by
obtaining union of the pseudarthrosis as early
as possible.
● Established shortening can be addressed by
limb equalization procedures

20. 4. Strategies for minimizing valgus
deformity of the ankle
● Ensure union of the fibular pseudarthrosis.
● Retaining an intramedullary rod that crosses
the ankle joint can also prevent ankle
deformity although the motion is lost.

21. IMAGING
Magnetic resonance imaging
• extent of the disease
• preoperative planning in that the borders for resection can be
defined precisely.
• The area of the pseudarthrosis is hyper intense on fat-suppressed
and T2-weighted images and slightly hypo intense on T1-weighted
images with contrast enhancement after administration of
gadolinium.
Computed tomography scan
Confirm radiographic findings.
Total bone scintigraphy
Level of the pseudarthrosis .

22. PATHOGENESIS
• exhibits insufficient mechanical strength and osteogenetic
capability.
• An abnormal highly cellular fibrovascular tissue grows at
expense of the bony cortex.
• incoordinate osteoclastic bone resorption that does not have
a normal bone modeling purpose.
• Reactive changes leads to excess trabecular bone and hence
medullary sclerosis.
• Excessive bone resorption accounts for intracortical cysts.
• Impaired vascularization

23. • L shaped
excision
specimen
pseudoart
hrosis, left
tibia
Pujani M, Madan NK, Shukla S. Congenital pseudoarthrosis tibia with fibrous hamartoma in a
child with neurofibromatosis. J Lab Physicians 2013;5:68-9

24. Histopathology
• Fibrous hamartoma is the key pathology = low
osteogenicity and high osteoclastogenicity.
• The soft tissue at the pseudarthrotic site is composed of
variable admixture of fibrous tissue, fibrocartilage, and
hyaline cartilage with evidence of enchondral ossification.
• marrow spaces are devoid of hematopoiesis.
• This invasive fibromatosis is located in the periosteum and
between broken bone ends and surrounds the tibia causing
compression, osteolysis, and persistence of pseudarthrosis

25. • Figure 2:
Tissue
section
from site
of
pseudoart
hrosis
showing
mature
lamellar
bone
along with
soft tissue
showing
spindle
cell
proliferati
on:
Fibrous
hamartom
a (H and E,
×400
Pujani M, Madan NK, Shukla S. Congenital pseudoarthrosis tibia with fibrous
hamartoma in a child with neurofibromatosis. J Lab Physicians 2013;5:68-9

26. TREATMENT HISTORY
Shah H, Rousset M, Canavese F. Congenital pseudarthrosis of the tibia: Management and complications. Indian
J Orthop 2012;46:616-26
• Charnley (1956) first treated cases of CPT with intramedullary rod.
• Coleman added autogenous iliac bone graft to intramedullary rod for the treatment
of CPT.
• In 1974, Ostrup et al. demonstrated the presence of active osteocytes in
vascularized bone graft and their absence in nonvascularized bone graft.
• Taylor et al. (1975) succeeded in grafting the first vascularized fibula and 3 years
later, the same technique was applied by Weiland et al. for treating patients with
CPT.
• Shah et al. used cortical bone grafting with intramedullary nailing for the
treatment of the CPT.
– Cortical bone grafting from contralateral tibia resist resorption better than cancellous
bone hence primary union rate is higher with use of cortical bone graft.

27. SURGICAL OPTIONS
1. Vascularized fibular graft
2. External fixation
3. Intramedullary rod
4. Amputation

28. 1. Vascularized fibular graft
• The procedure entails harvesting a long segment of the opposite fibula along with its
vascular pedicle.
• This is transferred into the gap created after radical excision of the pseudarthrotic segment.
• The vessels of the transferred fibula are anastomosed to the local vessels. The transferred
fibula is fixed securely to the tibia.

29. 1. Vascularized fibular graft
Charnley-Williams technique
• the intramedullary rod in tibia was inserted along with
vascularized fibular graft according to the technique described
by O'Brien.
• The goal is to obtain bone union, by mixing propitious
biological environment with the vascularized bone graft, and
the intramedullary rod is responsible for stability.
•
The high success rate of primary and secondary union are the
distinct advantage of vascularised fibular graft.
The limitations of this technique are
• cost, technical complexity, poor protection against re-fracture,
failure to correct limb length discrepancy, and deformities of
leg and ankle simultaneously at time of primary surgery.

30. 2. External fixation
• Circular external fixation as the treatment of choice of CPT
• allows total resection of pathological tissue
• ensures stability regardless of the amount of resected tissue
• allows extension of the member, correction of axial
deformities
• full support immediately after the intervention

31. • The Ilizarov frame is applied,
• the pseudarthrosis is excised.
• the fragments are compressed.

32. 3. Intramedullary rod
• provides stability and does not disturb distal tibial epiphysis.
• Intramedullary rod prevents refracture; therefore, it is
inadvisable to remove the rod after union

33. 3. Intramedullary rod
• 1. Excision of the pseudarthrosis
– until fresh bleeding.
– the thick periosteum is excised.
• 2. Bone grafting - autogenous bone grafting facilitates union
• 3. Intramedullary rodding
Three struts of cortical bone are placed around the site of
the excised pseudarthrosis after ensuring that the
fragments are well apposed.

38. 4. Amputation
• For resistant pseudarthrosis when other extensive surgical
procedures have not achieved a functional extremity
• either due to persistent nonunion or due to dysfunctional
angular deformity, shortening, atrophy, and stiffness, the
amputation is entirely appropriate.

39. 5. Ultrasound-
Low-Intensity pulsed ultrasound
stimulation (LIPUS)
• Okada et al. reported a case of CPT of the tibia (Boyd type IV)
successfully treated with low-intensity pulsed ultrasound
stimulation (LIPUS) administered for 20 min/day.
• The treatment was continued for 1 year until solid fusion on
radiographs and subsequent full-weight-bearing was achieved.
•
• The underlying mechanisms of action of LIPUS remain unclear.
However, in experimental studies conducted in rats, LIPUS
application facilitates union and increase mechanical strength
of bone

40. complications
1. Refracture
2. Malalignment of the tibia
3. Limb length discrepancy
4. Ankle valgus
5. Ankle stiffness

41. 1. Refracture.
• 14% to 60%.
• Anatomic alignment of the tibia and fibula minimize the risk
of re-fracture.
• Intramedullary rod and external bracing must be continued as
effective protection against re-fractures.

42. 2. Malalignment of the tibia
• Diaphyseal malalignment of the tibia (procurvatum or valgus
deformity) are progressive and do not remodel

43. 3. Limb length discrepancy
• Residual limb length discrepancy following successful
union is a major problem.
• Growth abnormalities of the tibia, fibula, and the
ipsilateral femur abnormalities are also noted with CPT
• which include inclination of the proximal tibial physis,
posterior bowing of the proximal third of the tibial
diaphysis, proximal migration of the lateral malleolus.

44. 4. Ankle valgus

45. 4. Ankle valgus
• Compromises functional outcome.
• Progressive ankle valgus is a problematic postoperative
donor-site morbidity of a vascularized fibular graft in children.
•
• Tibiofibular metaphyseal synostosis (the Langenskiöld
procedure)

46. 5. Ankle stiffness
• progressively regresses once intramedullary
rod is removed from ankle.
• Pain secondary to degenerative changes of the
ankle can be treated with limitation of activity
and shoe modification.
• Severe pain may require ankle arthrodesis

47. Follow up.
• till skeletal maturity to identify and rectify
residual problems.

48. Take Home message.
• Rare.
• The natural history of the disease is extremely unfavorable and
• Little or no tendency for the lesion to heal spontaneously.
• challenging to treat effectively
• Aims to obtain a long term bone union, to prevent limb length discrepancies,
to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness,
and pathological fracture.
• The key to get primary union is to excise hamartomatous tissue and
pathological periosteum.
• Age at surgery, status of fibula, associated shortening, and deformities of leg
and ankle play significant role in primary union and residual challenges after
primary healing.
• Surgical options such as intramedullary nailing, vascularized fibula graft, and
external fixator, have shown equivocal success rate in achieving primary union
• Amputation must be reserved for failed reconstruction, severe limb length
discrepancy and gross deformities of leg and ankle..

49. Refereneces.
• Shah H, Rousset M, Canavese F. Congenital
pseudarthrosis of the tibia: Management and
complications. Indian J Orthop 2012;46:616-
26
• Campbell’s – 12th edition.
• Paediatric orthopaedics –A system of decision
making, by Benjamin Joseph.

50. THANK YOU.