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