Download to read offline
More Related Content
Similar to Congenital pseudarthrosis of tibia - Dr. Sachin M
Congenital pseudarthrosis of tibia - Dr. Sachin M
- 1. CONGENITAL PSEUDOARTHROSIS OF TIBIA PRESENTED BY:DR. SACHIN. M. 2ND YEAR PGT, DEPT OF ORTHOPAEDICS, SMCH MODERATOR: DR. BIPUL BORTHAKUR PROFESSOR, DEPT OF ORTHOPAEDICS, SMCH
- 2. LEARNING OBJECTIVES INTRODUCTION ETIOPATHOGENESIS CLASSIFICATION INVESTIGATIONS TREATMENT COMPLICATIONS
- 3. INTRODUCTION Congenital pseudarthrosisis a specific type of a non-union Pseudarthrosis – “false joint” Either present or incipient at birth Most commonly involves distal half of the tibia and ipsilateral fibula
- 4. CONGENITAL PSEUDARTHROSIS OF TIBIA Rare disorder with an incidence of 1 in 2,50,000 live births Non-union of a tibial fracture that occurs spontaneously or after a minor trauma Usually develops within first 2 years of life; can be seen before birth also
- 5. ETIOPATHOGENESIS Exact causeis not known, but closely related to patients with Neurofibromatosis or related stigmata in 50% cases (NF1 gene, 17q11) Fibrous dysplasia in 10% of cases Others are – constriction band syndromes and Osteofibrous dysplasia Non-union occurs due to poor healing following fracture of the bone
- 6. ETIOPATHOGENESIS Most acceptedtheory is linked to the periosteum An accumulation of nerve cells (NF1 haplopotent MSCs) around the periosteal vasculature, that destroy its blood supply. Hamartomatous thickened fibrous tissue with limited vascular ingrowth is seen at the site of pseudarthrosis
- 7. CLASSIFICATION ANDERSEN’S CLASSIFICATIONSYSTEM - Based on morphology Dysplastic Cystic (no association with NF) Sclerotic Clubfoot type that arises due to associated abnormalities
- 8. CLASSIFICATION CRAWFORD’S CLASSIFICATIONSYSTEM –Radiological description of lesions NON-DYSPLASTIC DYSPLASTIC DYSPLASTIC DYSPLASTIC Hourglass constriction Cysts Frank pseudarthrosis
- 9. CLASSIFICATION – BOYD’S BOYD’S CLASSIFICATION OF CPT TYPE I: Anterior bowing and a defect in the tibia present at birth TYPE II: Anterior bowing + hourglass constriction of the tibia present at birth (Crawford’s type IIA) Most common type High risk tibia with poorest prognosis Tibia is tapered, rounded and sclerotic with obliterated medullary canal Re-fracture is common during the growth period, ceases after skeletal maturity
- 10. CLASSIFICATION TYPE III:Anterior bowing + congenital cysts in the bone Near the junction of middle and distal thirds of the tibia Less chances of re-fracture TYPE IV: occurs in a sclerotic segment of the bone without narrowing of the tibia Partial or complete obliteration of the medullary canal Starts as stress or insufficiency fracture – complete fracture – non-union Good prognosis if treatment is started before completion of the insufficiency fracture
- 11. CLASSIFICATION TYPE V:pseudarthrosis of the tibia occurs with a dysplastic fibula Dysplastic fibula may end up in pseudarthrosis – poor prognosis TYPE VI: Intra-osseous neurofibroma or schwannoma that results in the pseudarthrosis Extremely rare Prognosis – depends on the aggressiveness of intra-osseous lesions and its treatment
- 12. CLASSIFICATION – PALEY’S EL ROSASSY – PALEY – HERZENBERG’S CLASSIFICATION: based on Geometry and mobility of the bone ends: thick & stiff OR thin & mobile Previous, unsuccessful surgery TYPE I: Atrophic (narrow) bone ends Mobile pseudarthrosis No previous surgery
- 13. CLASSIFICATION – PALEY’S TYPE II: Atrophic (narrow) bone ends Mobile pseudarthrosis Previous unsuccessful surgery TYPE III: Hypertrophic (wide) bone ends Stiff pseudarthrosis With or without previous surgery
- 14. CLASSIFICATION – JOHNSONET AL Can be used to guide the treatment and prognosis Based on Presence or absence of fracture Age of first fracture Fractured tibia – require surgical treatment Intact tibia – Observation and splinting
- 15.
- 16. INVESTIGATIONS MRI -provides valuable information on the extent of the disease and is helpful for the preoperative planning in that the borders for resection can be defined precisely CT scan - confirm radiographic findings, showing osteolytic lesions containing solid tissue Total bone scintigraphy - a slight uptake at the beginning of the dynamic venous phase and a high uptake during terminal phase
- 17. TREATMENT Primary goalsare: Union No re-fractures Correction of diaphyseal deformity Secondary goals are: Correction of ankle-knee valgus Treatment of limb length discrepancy Correction of foot deformity
- 18. TREATMENT Depends onthe age of the patient and presence or absence of the fracture Before walking age – little treatment (casting) Once ambulation begins – casting or bracing (Clamshell orthosis or PTB orthosis), if there is no fracture; can be continued till skeletal maturity Once fracture occurs – surgical management
- 19. TREATMENT SURGICAL MANAGEMENT Basic principles include: Resection of the entire pseudarthrosis and surrounding hamartomatous tissue Restoration of mechanical alignment Intramedullary fixation Augmentation techniques: Primary shortening, bone transport, supplemental bone grafting and BMPs
- 20. TREATMENT SURGICAL MANAGEMENT Amputation – rarely required in initial stages Anticipated shortening of > 2-3 inches History of multiple failed surgical procedures Stiffness and decreased function of a limb that would be more useful after an amputation and prosthetic fitting
- 21. TREATMENT INTRAMEDULLARY FIXATION– William’s IM rod (Anderson et al) Most commonly used technique In very distal tibia CPT – may require to cross the ankle joint to provide additional stability Rod can migrate with growth or can be surgically reposition the rod above the ankle to restore the ankle motion In proximal tibia CPT – possible to avoid crossing the ankle joint Larger diameter rod or the one with interlocking option aid in stability
- 22. TREATMENT INTRAMEDULLARY FIXATION FassierDuval telescopic nailWilliam’s IM rod
- 23. TREATMENT VASCULARIZED BONEGRAFTS Reconstruction of the resected pseudarthrosis with vascularized bone graft – can be harvested by fibula or iliac crest Requires experience in microvascular techniques Two surgical teams – one for harvesting the graft and the other for preparing the pseudarthrosis site, would be helpful Vascularized fibular grafts – CPT with gap > 3cm and CPT with multiple failed surgeries
- 24. TREATMENT BONE MORPHOGENICPROTEINS Can be used as an adjunctive to the surgical stabilization of the pseudarthrosis Both rhBMP-2 and rhBMP-7 have been used Early union rates have been reported Long term follow-up is needed to understand the long term efficacy and safety of these treatments
- 25. TREATMENT ILIZAROV FIXATION Offer the advantage of maintaining or gaining tibial length Disadvantages include – Difficulty transporting the proximal tibia Docking malalignment Poor quality of regenerated bone
- 26. TREATMENT PALEY’S INSTITUTETREATMENT STRATEGY FOR CPT It’s a culmination of 28 years of Dr. Paley’s treatment technique Comprises of 4 papers, recent one being published in 2012 Ilizarov fixator + bone graft + IM nailing (FD telescopic nail) + periosteal graft + BMP insertion + Zolidronic acid infusion + tibio-fibular cross union Locking plates can also be used instead of Ilizarov fixator 100% union rate with 0% re-fracture rate
- 27.
- 29. PROGNOSIS Factors leadingto poor prognosis are: Lower age at treatment Previous failed treatment Neurofibromatosis Increased follow up
- 30. COMPLICATIONS Stiffness ofthe ankle and hindfoot: usually disappears once the IM nail is proximal to the ankle joint If persists, rarely hampers the functional results Re-fracture: most common complication after surgical treatment of CPT (50% to 75%) Can be managed with casting or exchange nailing IM nail should be left in place till skeletal maturity
- 31. COMPLICATIONS Valgus ankledeformity: occurs when the distal tibia fragment is not fixed during insertion of IM nail More frequently seen when the fibula is left intact than when fibular osteotomy is done Long term bracing during growth years to prevent progressive deformity Langenskiold procedure may be indicated
- 32. COMPLICATIONS Tibial shortening: Can be treated by a well-timed contralateral epiphysiodesis or limb lengthening of the proximal tibia Ilizarov technique in patients with significant shortening and a wide non-union; in patients with failed IM nail and bone grafting procedures