This document provides information on congenital pseudarthrosis of the tibia (CPT), including: - CPT is a nonunion of the tibia that develops spontaneously in early life and is associated with bowing of the tibia. It occurs more commonly in patients with neurofibromatosis type 1. - Surgical treatment aims to completely excise fibrous tissue, correct deformity, stimulate bone healing, and properly fix bone fragments. Options include bone grafting, internal fixation, Ilizarov fixation, vascularized fibular grafting, and amputation in severe cases. - Classification systems divide CPT into types based on clinical features and radiographic findings to help determine prognosis and guide treatment

1. CONGENITAL
PSEUDARTHROSIS OF TIBIA
Dr. Sidharth Yadav
Orthopaedic Dept.
N.K.P.SIMS

2. DEFINITION
 Pseudarthrosis is a
false joint associated
with abnormal
movements at the site

3. INTRODUCTION
 Congenital pseudarthrosis of tibia refers to nonunion of
tibial fracture that develops spontaneously or after trival
trauma in a dysplastic bone segment of tibia diaphysis.
 CPT is rare & Usually develops in first 2 yrs of life.
 Etiology is unclear.
 Incidence is 1: 250,000
 There is a strong association of CPT with
neurofibromatosis type 1.

4. CLINICAL FEATURES
 Associated with anterolateral
bowing of tibia.
 Bowing usually occurs at the
junction of middle & distal third.
 Deformity may be associated with
skin dimple, limb shortening,
dysplasia of fibula & ankle valgus.
 Usually unilateral.

5. NEUROFIBROMATOSIS
 NF-1 occurs due to mutation on the gene coding for
NEUROFIBROMIN on chromosome 17.
 Neurofibromin is expressed in a broad range of cells & tissue
type.
 It negatively regulates Ras activity ( cell proliferation &
function)
 It’s deficiency leads to increased Ras activity.
 Affects Ras-dependent MAPK( mitogen activated protein
kinase) activity which is essential for osteoclast function &
survival.

6. SIGNS OF
NEUROFIBROMAT
OSIS

7. DIAGNOSTIC CRITERIA OF
NEUROFIBROMATOSIS
 6 or more café-au-lait macules (>5mm before puberty &
>15mm after puberty).
 Axillary or inguinal freckling.
 2 or more neurofibromas or 1 plexiform neurofibroma.
 2 or more Lisch nodules.
 Optic glioma.
 A distinctive osseous lesion such as sphenoid dysplasia or
thinning of long bone cortex with or without pseudarthrosis.
 A first degree relative with NF-1.

8. PATHOLOGY
 Unclear
 Recent studies have shown that there is hyperplasia of
fibroblast with the formation of dense fibrous tissue.
 This invasive fibromatosis is located in the periosteum &
between broken bones ends causing compression,
osteolysis & persistance of pseudarthrosis.

10. PATHOLOGY
 Paley et al theorized that pathology of pseudarthrosis is not
bony but rather its periosteal in origin.
 This theory was also considered by CODAVILLA a century
ago.
 This theory is supported by following observation :-
 Thickening with hamartomatous transformation of periosteum.
 Appearance of strangulation of bone with atrophic changes
followed by avascular changes.
 Failure of remodelling of pin tracts leading to stress fractures.

11. PATHOLOGY
 Pathologic analysis of HERMANNS-SACHWEB et al
confirmed that pathologic periosteum is the cause of
CPT.
 There finding was :-
 Neural cells form a tight sheath around the periosteal
vessels.
 Peiosteum undergoes hypoxemic changes resulting in the
formation of a thick fibrous cuff.
 Leads to impaired oxygen & nutrient supply to the
subperiosteal bone & atrophic changes are observed.

12. CLASSIFICATION
 There is no universally agreed system based on both
clinical features & radiographic findings.
 CAMURATI - 1930
 ADGLEY - 1952
 BOYD - 1958
 APOIL - 1970
 ANDERSEN - 1973
 CRAWFORD - 1986
 CRAWFORD - 1999
 BOYD & ANDERSEN are commonly used.

13. BOYD CLASSIFICATION
 Boyd divided CPT into 6 types :-
 Type 1 :-
 Pseudarthrosis occurs with anterior
bowing.
 A defect in tibia present at birth.
 Other congenital deformities may be
present which may affect the
management of pseudarthrosis.

14. BOYD CLASSIFICATION
 Type 2 :-
 Pseudarthrosis occur with anterior bowing & a hourglass constriction of
the tibia is present at birth.
 Spontaneous fractures or after minor trauma.
 Commonly occur before 2 yrs of age.
 Also known as HIGH RISK TIBIA.
 Tibia is tapered, rounded, sclerotic & obliteration of medullary canal.
 Most common type.
 Associated with NF-1
 Poorest prognosis.

15. BOYD CLASSIFICATION
 Type 3 :-
 Pseudarthrosis develops in a congenital cyst
usually near the junction of middle & distal
third of tibia.
 Anterior bowing may precede or follow the
development of fracture.
 Recurrance of fracture is less common after
treatment.

16. BOYD CLASSIFICATION
 Type 4 :-
 Originates in a sclerotic segment of
bone.
 Without narrowing of tibia.
 Medullary canal is partially or
completely obliterated.
 An insufficiency or stress fracture
develops in the cortex of tibia &
gradually extends through the sclerotic
bone.
 Prognosis is good.

17. BOYD CLASSIFICATION
 Type 5 :-
 Pseudarthrosis of tibia occurs with a dysplastic fibula.
 Pseudarthrosis of both bone may develop.
 Prognosis is good if the lesion is confined to fibula.
 If the lesion progress to tibia then the natural h/o usually
resembles type 2.
 Type 6 :-
 Occurs as an intraosseous neurofibroma or schwannoma
 Extremely rare.

18. CRAWFORD CLASSIFICATION
 Divided broadly divided into 2 types:-
 Non-Dysplastic
 Anterolateral bowing with increased density & sclerosis of
medullary canal.
 Dysplastic
 Anterolateral bowing with failure of tubularization.
 Cystic changes.
 Frank pseudarthrosis.

19. ANDERSEN CLASSIFICATION
 Also divided into 6 types :-
 Club foot
 Cystic
 Late
 Fibular
 Dysplastic
 Angulated

20. CLASSIFICATION BY PALEY

21. TREATMENT
 Treatment of CPT depends upon age of the patient & type of
pseudarthrosis.
 Decision has to be taken whether to attempt to secure union
or amputation is the treatment of choice.
 No single treatment approach has proven ideal .
 True pseudarthrosis does not heal when treated with casting
alone.

22. TREATMENT
 Goals of treatment :-
 Complete excision of the soft tissue fibromatosis at the
site of pseudarthrosis.
 Correction of angular deformity.
 Stimulation of bone healing.
 Proper fixation of bone fragments.
 Postoperative protection .

23. TREATMENT
 Is divided into 2 types :-
 Prophylactic :-
 Decreased activity.
 Orthotics or cast.
 Curettage with bone grafting.
 Active :-
 Surgical treatment

24. TREATMENT
 Bone grafting
 IM fixation
 Ilizarov fixation
 Free vascularized fibular grafting
 Amputation
 Bmp(bone morphogenic proteins).
 Electric stimulation.

26. VASCULARISED FIBULA GRAFTING
 Advantages :-
 Primary bone lengthening
 Correction of deformity.
 Union occur in a relative short
period.
 Disadvantages :-
 Development of valgus deformity of
normal ankle.

27. ILIZAROV FIXATION
 Advantages :-
 Provides stability.
 Enables full wt. bearing.
 Allows limb lengthning &
segmental transport.
 Disadvantages :-
 Pin tract infection
 Ankle stiffness.

28. AMPUTATION
 Anticipated shortening of more then 2 or 3 inches.
 Multiple failed surgical procedure.
 Stiffness & decreased function of the limb that will be
more useful after amputation & fitting with prosthesis.

29. BONE MORPHOGENIC PROTEIN
 16 different BMP have been identified.
 BMP-2 & BMP -7 are the only current available for the
clinical use in non-union & paeudarthrosis.
 Clinical studies have shown that BMP-2,6,9 plays an
important role in early differentiation of mesenchymal
progenitor cells to preosteblasts.
 BMP-7 promotes early differentiaiton of preosteoblast to
osteoblast.

30. PSEUDARTHROSIS OF FIBULA
 Pseudarthrosis of fibula often precedes or
accompanies the same condition in ipsilateral tibia.
 Several grades are seen :-
 Bowing of fibula without pseudarthrosis.
 Pseudarthrosis without ankle deformity.
 With ankle deformity.
 Fibular pseudarthrosis with latent tibia pseudarthrosis.
 Progressive valgus deformity is developed.

32. TREATMENT
 Until skeletal maturity –ankle foot orthosis.
 At maturity :- supramalleolar osteotomy
 Langenskiöld has devised an operation for children to
prevent valgus deformity & halt its progression—
SYNOSTOSIS.

33. THANK YOU…