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CONGENITAL
PSEUDARTHROSIS OF TIBIA
Dr. Sidharth Yadav
Orthopaedic Dept.
N.K.P.SIMS
DEFINITION
 Pseudarthrosis is a
false joint associated
with abnormal
movements at the site
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.
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.
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.
SIGNS OF
NEUROFIBROMAT
OSIS
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.
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.
Congenital pseudarthrosis of tibia
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.
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.
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.
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.
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.
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.
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.
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.
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.
ANDERSEN CLASSIFICATION
 Also divided into 6 types :-
 Club foot
 Cystic
 Late
 Fibular
 Dysplastic
 Angulated
CLASSIFICATION BY PALEY
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.
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 .
TREATMENT
 Is divided into 2 types :-
 Prophylactic :-
 Decreased activity.
 Orthotics or cast.
 Curettage with bone grafting.
 Active :-
 Surgical treatment
TREATMENT
 Bone grafting
 IM fixation
 Ilizarov fixation
 Free vascularized fibular grafting
 Amputation
 Bmp(bone morphogenic proteins).
 Electric stimulation.
Congenital pseudarthrosis of tibia
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.
ILIZAROV FIXATION
 Advantages :-
 Provides stability.
 Enables full wt. bearing.
 Allows limb lengthning &
segmental transport.
 Disadvantages :-
 Pin tract infection
 Ankle stiffness.
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.
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.
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.
Congenital pseudarthrosis of tibia
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.
THANK YOU…

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Congenital pseudarthrosis of tibia

  • 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.
  • 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
  • 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.

Notes de l'éditeur

  1. 5.7% of pt. with NF-1 develops CPT whereas 40% pt’s of CPT found to have NF-1.
  2. Macroscopy :-  Aspect of the pseudarthrosis of the tibia. At the site of insufficient bone healing, white fibrous tissue substitutes the bone. A thickened cuff of periosteum and interruption of the bone compacta are noted. Microscopy :- Histologic section of the periosteum of the control case with some vessels and small nerves, H&E, 40×. (B) By contrast, the periosteum of CPT patients shows a lot of small blood vessels embedded in proliferating fibrous tissue, H&E, 40×. (C) In the area of pseudarthrosis, cellular proliferating fibrous tissue with immature bone is observed, H&E, 40×. (D) Proliferating fibrous tissue of the periosteum.
  3. The anterolateral bowing is due to the dominant muscular forces on the leg from the posterolateral compartment.
  4. Various classification system based on radiographic & morphology exist.
  5. Patient with type 1 CPT usually presents early in life , before 2 years of age & have a good prognosis with treatment. Type 2 presents usually in older childern after experiencing the failed surgical t/t, refracture or an osteotomy to correct a bowed tibia. Type 2 have a relative poor prognosis. Type 3 cases are usually of late onset type which develops as a stress fracture of congenital dysplastic tibia or in a healed tibia after previous bone grafting & has the best prognosis. The siginificant feature of type 2 is there is presem=nce of dead bone coz of the previous sx.
  6. Prophylactic :- pt. with anterolateral bowing of the tibia at the junction of middle & distal third of diaphysis or beyond ,particularly when accompanied by narrowing ,sclerosis & loss of defination of the medullary canal are considered high risk for developing pathological fractures & nonunion. The extremity is protracted by an orthosis & the child is guarded against excess activity. as the longitudinal growth takes places , there is a tendency towards the correction of the deformity though it can take a long time. The threat of the fractures is ever present untill skeletal maturity. Ostetomy are prohibited. If fracture & pseudarthrosis apperas imminent eg. In presence of cyst then curettage & filling with bone grafts.
  7. Sometimes it develop bet. The time of sucessful bone grafting of tibia & skeletal maturity. Lateral malleolus is shifted proximally so valgus deformity develops.