Congenital pseudoarthrosis of tibia is a failure of normal bone formation in the distal half of the tibia, often leading to a segmental defect and bowing of the bone. It is most commonly caused by neurofibromatosis, which is present in 40-80% of cases. Pathology shows thickened periosteum and fibrous tissue surrounding the tibia, preventing callus formation and bone healing. Treatment aims to fully excise the soft tissue, correct angular deformity, stimulate bone healing, and properly fixate bone fragments, often through the Ilizarov technique or intramedullary nailing with bone grafting. Complications can include non-union, refracture, malalignment and

1. Congenital pseudoarthrosis of tibia

2. INTRODUCTION
Failure of normal bone formation in the distal half of the tibia
 segmental defect of bone
Anterolateral angulation
Pathological fracture

3. Etiology
 NEUROFIBROMATOSIS:
most common cause in about 40-80% cases
hallmarks like café-au-lait spots and skin nodules
Absent at birth and appears later
 Fibrous dysplasia
 Idiopathic

4. PATHOLOGY
 Site is usually surrounded by thickened periosteum and fibrous tissue
 Thickened periosteum results in hamartomatous tissue
 Failure of callus formation resulting in pseudoarthrosis
 Appearance of strangulation of bone with atrophic changes followed by
avascular changes
 Failure of remodeling of bone results in stress fracture

5. Histopathology
 Fibrous hamartoma is the key pathology
 Low osteogenicity and high osteoclastogenicity
 Soft tissue at the pseudoarthrotic site is composed of variable of fibrous
tissue, fibrocartilage and hyaline cartilage with evidence of endochondral
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 pseudoarthrosis

7. ANDERSON CLASSIFICATION
 Dysplatic type
 Cystic type
 Late type
 Club foot type
 Angulated pseudoarthrosis

8. Dysplastic
 Diameter of tibia is narrowed
 Hourglass constriction is characteristic
 Tibia is bowed anteriorly or anterolaterally
 Prone to non- union and refracture
 Neurofibromatosis is always present in the dysplastic type
 It may be present at birth and develop at an average of 12 months

9. Cystic type
 Cyst like lesion in the affected segment
 No significant narrowing of diameter of tibia
 It may be present at about 8 months
 Neurofibromatosis not associated

10. Late type
 Affected leg shows length discrepancy
 After minimum trauma a stress fracture like break occurs with consequent
development of pseudoarthrosis.
 Not associated with neurofibromatosis
 Anterior bowing develops between age of 4 and 12 years

11. Club foot type
 Fracture present at birth in a leg with marked anterior angulation
 The involved or contralateral lower limb has other associated congenital
abnormalities such
constriction band
club foot

12. Angulated pseudoarthrosis
 Due to corrective osteotomy of anterior bowing of tibia
 Osteotomy results in pseudoarthrosis
 Therefore,simple osteotomy of anterior angulation is contraindicated

13. BOYDS CLASSIFICATION
 Type I
 Type II
 TypeIII
 TypeIV
 Type V
 Type VI
 Anterior bowing with tibia defect
 Pseudoarthrosis with hour glass constriction
 Pseudoarthrosis with bone cyst
 Sclerotic segments,March fracture
 Dysplastc fibula
 Interosseous neurofibroma

21. CLINICAL FEATURES
 Anterolateral,anterior rarely anteromedial bowing of the dysplastic tibia and
fibula
 Tibia and fibula affected at the same level
 Bowing increases during the first two years of life and eventually develops
pseudoarthrosis
 Patient walks with a limp or unable to walk

23. Criteria for diagnosis
 Mutiple café-au-lait spots(smooth edged,>0.5cm)
 Positive family history of neurofibromatosis
 Definite biopsy
 Bony lesions like pseudoarthrosis of the tibia

24. Radiological features
 Segment of tibia or tibia and fibula show hourglass thinning, sclerosis and
loss of medullary cavity
 Angulation at two levels proximally and distally with dysplastic changes

27. Treatment
 Complete excision of the soft tissue fibromatosis at the site of
pseudoarthrosis
 Correction of angular deformity
 Stimulation of bone healing
 Proper fixation of bone fragments

28. Treatment methods
 Ilizarov method
 Vascularized fibular graft
 Extending intramedullary nailing and bone grafting
 Electrical stimulation

29. Ilizarov technique

31. Intramedullary rod fixation
 The procedure of choice for the first attempt to gain union entails resection
of pseudoarthrosis, shortening and fixation with an intramedullary rod and
autogenous bone grafting
 The procedure can be performed can be performed at an y age and rates
of union of around 85% .

33. Complications
 Refracture
 Malalignment of tibia
 Limb length discrepancy
 Ankle valgus
 Ankle stiffness

34. Refracture
Shortening
Valgus deformity
 Splint the limb until skeletal
maturity,retain intramedullary nail
 Union of pseudo arthrosis as early as
possible, limb equalization procedure
 Ensure union of fibular pseudoarthrosis
 Retain intramedullary rod that crosses
the ankle joint

35. Thank you