2. INTRODUCTION
Nonunion of tibia fracture that develops
spontaneously or after trivial trauma in a dysplastic
bone segment of diaphysis.
usually develops in first 2 years
misnomer
Etiology is not clear.
Incidence is 1: 250,000
3. Strong association with NF type 1.
55% of cases associated with NF.
Some authors: association in nearly every instance.
Fibrous dysplasia: 15% of patients with anterolateral
bowing.
4. NEUROFIBROMATOSIS
NF-1: due to mutation on the gene coding for
NEUROFIBROMIN on chromosome 17.
It negatively regulates Ras activity.
Affects Ras-dependent MAPK( mitogen activated protein
kinase) activity-essential for osteoclast function & survival.
5. •
•6 or more café-au-lait macules
• Axillary or inguinal freckling.
• 2 or more neurofibromas or 1 plexiform neurofibroma.
• 2 or more Lisch nodules.
•Optic glioma.
•Distinctive osseous lesions.
•Family history
6. •Recent studies :Hyperplasia of fibroblasts
•Osteolytic fibromatosis
•Paley et al: it is not bony but periosteal
POINTS FAVOURING:
•Thick & harmartomatous periosteum
•Avascular & atrophic changes in bone
•Failure of remodelling
8. BOYD CLASSIFICATION
TYPE 1: Anterior bowing, defect at birth,
other congenital abnormalities
TYPE2: Mc type
Anterior bowing & a hourglass constriction.
Spontaneous #s or after minor trauma.
Commonly occurs before 2 years
HIGH RISK TIBIA.
Associated with NF-1
Poorest prognosis
9. TYPE 3 :
Congenital cyst.
• Anterior bowing may precede or follow the
development of #.
TYPE 4 :
• Originates in a sclerotic segment.
• Medullary canal obliterated.
• Insufficiency or stress # develops
and gradually extends.
• Prognosis is good.
10. TYPE 5 :
• Pseudarthrosis of tibia with dysplastic fibula.
• Prognosis good if confined to fibula only.
• Lesion resembles type 2
TYPE 6 :
• Occurs as an intraosseous neurofibroma or
schwannoma .
• Extremely rare.
11. CRAWFORD CLASSIFICATION
1-Non-Dysplastic
Anterolateral bowing with increased density &
sclerosis of medullary canal.
2-Dysplastic
2a Anterolateral bowing
with failure of tabularization.
2b Cystic changes.
2c Frank pseudarthrosis.
12. DIAGNOSIS
CLINICAL FEATURES:
cutaneous signs of NF
anterolateral bowing of tibia.
bowing usually at junction of middle & distal third.
Associated with skin dimple, limb shortening, dysplasia
of fibula & ankle valgus.
Usually unilateral.
13. IMAGING
MRI:
Extent of disease
Preoperative planning.
Hyper intense on T2-weighted images and hypo intense on
T1-weighted images.
CT SCAN
Confirm radiographic findings.
TOTAL BONE SCINTIGRAPHY
Level of the pseudarthrosis .
16. STRATEGIES TO ACHIEVE UNION
Microvascular graft transfer-vascularized fibula
The Ilizarov technique
Bone grafting with internal fixation-plating or IM devices.
Excision of the pseudarthrosis should be an integral part of
the procedure.
STRATEGIES TO PREVENT REFRACTURE
Splint the limb in an orthosis until skeletal maturity.
Retain an intramedullary nail until skeletal maturity.
17. STRATEGIES FOR DEALING WITH SHORTENING OF
THE LIMB
Minimize the extent of shortening.
Limb equalization procedures
STRATEGIES FOR MINIMIZING VALGUS DEFORMITY
OF THE ANKLE
Ensure union of fibular pseudarthrosis.
Retaining an IM rod that crosses ankle.
18. BONE GRAFTING
MCFARLAND:
Corticocancellous graft from opposite tibia
Placed posteriorly
VASCULARISED FIBULAR GRAFT
fibula along with vascular pedicle.
Transferred into the gap created.
Vessels anastomosed to local vessels.
19. Procedure of choice for gaps > 3cm.
92%- 95% union rate
Refracture.
Langenskiold procedure to prevent ankle valgus.
In addition weakness may ensue in the donor leg due to
resection of origins of flexor muscles.
PERIOSTEAL GRAFTING: has been tried
20.
21. INTRA MEDULLARY FIXATION
Resection, shortening and fixation with an IM rod &
autogenous bone grafting.
Union 85%.
WILLIAMS TECHNIQUE
Threaded male and female components of the rod
Can be placed antegrade & brought out the bottom of
the foot.
After retrograde insertion back in to the proximal tibia -
male end is unscrewed and removed from bottom -
female threaded rod left intraosseously in tibia or across
the ankle in talus/calcaneus.
22. ILIZAROV TECHNIQUE
Provides excellent stability
Complete resection of pseudo-arthrotic part.
Enables weight bearing which aids healing
Compression of pseudo-arthrosis
Limb lengthening procedures can be done
23. BONE MORPHOGENIC PROTEIN
BMP2
BMP7
ELECTRICAL STIMULATION
Limited to the earlier phases when union is the primary
goal.
24. AMPUTATION – Maybe the better option in some
situations
McCARTHY CRITERIA
Failure after 3 surgeries
LLD 5cm or more
Deformed foot
Prolonged hospitalization
High medical costs
25. COMPLICATIONS
1. REFRACTURE
14% to 60%.
Anatomic alignment minimizes the risk.
IM rod and external bracing -protection against re-
fractures.
26. 2. MALALIGNMENT
Procurvatum
valgus deformity
3. LIMB LENGTH DISCREPANCY
Residual limb length discrepancy common
Growth abnormalities noted with CPT.
27. 4. ANKLE VALGUS
Progressive ankle valgus is a problematic
postoperative donor-site morbidity of a vascularized
fibular graft in children.
Tibiofibular metaphyseal synostosis (Langenskold
procedure) useful.
28.
29. 5. ANKLE STIFFNESS
Progressively regresses after IM removed from
ankle.
Pain secondary to degenerative changes of ankle-
limitation of activity and shoe modification.
Severe pain – ankle arthrodesis
30. CONCLUSION
Challenging for the surgeon
Poor tendency to heal
Excision of the hamartomatous tissue and pathological
periosteum is the KEY.
31. “yogasthaḥ kuru karmāṇi saṅgaṃ tyaktvā dhanañjaya
siddhyasiddhyoḥ samo bhūtvā samatvaṃ yoga ucyate”
By being established in Yoga, O Dhananjaya, undertake actions,
casting off attachment
and remaining equipoised in success and failure. Equanimity is
called Yoga.
THANK YOU.