Congenital pseudo arthrosis of tibia
- 1. CONGENITAL PSEUDO ARTHROSIS OF TIBIA- CPT Dr. Rejul K Raj, CMC Ludhiana
- 3. INTRODUCTION. • Type of non union. • Present at birth or incipient. • rare pathology. 1 in 2,50,000 live births. • neurofibromatosis. • Cause- unknown. • Most commonly in disal tibia.
- 4. Introduction – contd. • extremely unfavorable. • Poor healing potential at the pseudoarthrosis. • there is a little or no tendency for the lesion to heal spontaneously. • It is challenging to treat effectively • Hamartomatous thickened fibrous tissue with limited vascular ingrowth.
- 5. • thin and atrophic tibial bone • pointed distal fragment. • The false joint is in the distal third of the shaft. • The fibula is affected
- 7. Problems- Primary 1. Obtaining union 2. Shortening of the limb – 1. At birth itself. 2. Pseudarthrosis remains ununited. 3. Repeated unsuccessful operations 3. Tendency for refracture- diminishes with skeletal maturity.
- 8. Problems- secondary deformities a) abnormal inclination of the proximal tibial physis b) posterior bowing of the proximal third of the tibial diaphysis c) proximal migration of the lateral malleolus d) fibular hypoplasia e) fibular pseudarthrosis f) ankle valgus and calcaneus deformity
- 10. CLASSIFICATIONS • Andersen's classification – 1973 • Boyd's classification- 1982 • Crawford's classification- 1986 • EI-Rosasy-Paley-Herzenberg's classification- 2007
- 11. BOYD’S- 1982, 6 types • Type I - anterior bowing with tibial defect. • Type II – Most common – with hour glass constriction – spontaneous fracture – HIGH RISK TIBIA ( tapered ,rounded, sclerotic , obliterated ) – Poorest prognosis. • Type III - with congeital cyst • Type IV- with sclerotic segments, sterss fractures. • Type V -with dysplastic fibula. • Type VI- an intraosseous neurofibroma or schwannoma is evident
- 13. Crawford's classification- 1986 • Type I- – the medullary canal is preserved and cortical thickening at the apex of the deformity might be observed – good prognosis; • Type II – – presence of thinned medullary canal, cortical thickening, and trabeculation defect. • type III - – cystic lesion, which may be fractured; patients with this type of pseudarthrosis tend to experience early fracture and, therefore, require early treatment. • Type IV- – pseudarthrosis is present with tibial and possibly fibular nonunion.
- 14. EI-Rosasy-Paley-Herzenberg's classification- 2007 1. patient's condition at time of presentation (relation to previous treatment), 2. radiological (atrophic or hypertrophic), 3. mobility of pseudarthrosis site (stiff or mobile). • Type 1 – Atrophic (narrow) bone ends – Mobile pseudarthrosis – No previous surgery • Type 2 – Atrophic (narrow) bone ends – Mobile pseudarthrosis – Previous unsuccessful surgery • Type 3 – Hypertrophic (wide) bone ends – Stiff pseudarthrosis
- 16. AIMS 1. Achieve union 2. Prevent refracture 3. Correct limb length inequality 4. Correct associated growth abnormalities 5. Prevent ankle deformity and arthritis.
- 17. 1. Strategies to achieve union 1. Microvascular free fibular transfer. 2. the Ilizarov technique 3. Bone grafting with intramedullary nailing. • Excision of the pseudarthrosis should be an integral part of the procedure.
- 18. 2. Strategies for minimizing the risk of refracture • Splint the limb in an orthosis until skeletal maturity. • Retain an intramedullary nail until skeletal maturity.
- 19. 3. Strategies for dealing with shortening of the limb ● Minimize the extent of shortening by obtaining union of the pseudarthrosis as early as possible. ● Established shortening can be addressed by limb equalization procedures
- 20. 4. Strategies for minimizing valgus deformity of the ankle ● Ensure union of the fibular pseudarthrosis. ● Retaining an intramedullary rod that crosses the ankle joint can also prevent ankle deformity although the motion is lost.
- 21. IMAGING Magnetic resonance imaging • extent of the disease • preoperative planning in that the borders for resection can be defined precisely. • The area of the pseudarthrosis is hyper intense on fat-suppressed and T2-weighted images and slightly hypo intense on T1-weighted images with contrast enhancement after administration of gadolinium. Computed tomography scan Confirm radiographic findings. Total bone scintigraphy Level of the pseudarthrosis .
- 22. PATHOGENESIS • exhibits insufficient mechanical strength and osteogenetic capability. • An abnormal highly cellular fibrovascular tissue grows at expense of the bony cortex. • incoordinate osteoclastic bone resorption that does not have a normal bone modeling purpose. • Reactive changes leads to excess trabecular bone and hence medullary sclerosis. • Excessive bone resorption accounts for intracortical cysts. • Impaired vascularization
- 23. • L shaped excision specimen pseudoart hrosis, left tibia Pujani M, Madan NK, Shukla S. Congenital pseudoarthrosis tibia with fibrous hamartoma in a child with neurofibromatosis. J Lab Physicians 2013;5:68-9
- 24. Histopathology • Fibrous hamartoma is the key pathology = low osteogenicity and high osteoclastogenicity. • The soft tissue at the pseudarthrotic site is composed of variable admixture of fibrous tissue, fibrocartilage, and hyaline cartilage with evidence of enchondral 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 pseudarthrosis
- 25. • Figure 2: Tissue section from site of pseudoart hrosis showing mature lamellar bone along with soft tissue showing spindle cell proliferati on: Fibrous hamartom a (H and E, ×400 Pujani M, Madan NK, Shukla S. Congenital pseudoarthrosis tibia with fibrous hamartoma in a child with neurofibromatosis. J Lab Physicians 2013;5:68-9
- 26. TREATMENT HISTORY Shah H, Rousset M, Canavese F. Congenital pseudarthrosis of the tibia: Management and complications. Indian J Orthop 2012;46:616-26 • Charnley (1956) first treated cases of CPT with intramedullary rod. • Coleman added autogenous iliac bone graft to intramedullary rod for the treatment of CPT. • In 1974, Ostrup et al. demonstrated the presence of active osteocytes in vascularized bone graft and their absence in nonvascularized bone graft. • Taylor et al. (1975) succeeded in grafting the first vascularized fibula and 3 years later, the same technique was applied by Weiland et al. for treating patients with CPT. • Shah et al. used cortical bone grafting with intramedullary nailing for the treatment of the CPT. – Cortical bone grafting from contralateral tibia resist resorption better than cancellous bone hence primary union rate is higher with use of cortical bone graft.
- 27. SURGICAL OPTIONS 1. Vascularized fibular graft 2. External fixation 3. Intramedullary rod 4. Amputation
- 28. 1. Vascularized fibular graft • The procedure entails harvesting a long segment of the opposite fibula along with its vascular pedicle. • This is transferred into the gap created after radical excision of the pseudarthrotic segment. • The vessels of the transferred fibula are anastomosed to the local vessels. The transferred fibula is fixed securely to the tibia.
- 29. 1. Vascularized fibular graft Charnley-Williams technique • the intramedullary rod in tibia was inserted along with vascularized fibular graft according to the technique described by O'Brien. • The goal is to obtain bone union, by mixing propitious biological environment with the vascularized bone graft, and the intramedullary rod is responsible for stability. • The high success rate of primary and secondary union are the distinct advantage of vascularised fibular graft. The limitations of this technique are • cost, technical complexity, poor protection against re-fracture, failure to correct limb length discrepancy, and deformities of leg and ankle simultaneously at time of primary surgery.
- 30. 2. External fixation • Circular external fixation as the treatment of choice of CPT • allows total resection of pathological tissue • ensures stability regardless of the amount of resected tissue • allows extension of the member, correction of axial deformities • full support immediately after the intervention
- 31. • The Ilizarov frame is applied, • the pseudarthrosis is excised. • the fragments are compressed.
- 32. 3. Intramedullary rod • provides stability and does not disturb distal tibial epiphysis. • Intramedullary rod prevents refracture; therefore, it is inadvisable to remove the rod after union
- 33. 3. Intramedullary rod • 1. Excision of the pseudarthrosis – until fresh bleeding. – the thick periosteum is excised. • 2. Bone grafting - autogenous bone grafting facilitates union • 3. Intramedullary rodding Three struts of cortical bone are placed around the site of the excised pseudarthrosis after ensuring that the fragments are well apposed.
- 38. 4. Amputation • For resistant pseudarthrosis when other extensive surgical procedures have not achieved a functional extremity • either due to persistent nonunion or due to dysfunctional angular deformity, shortening, atrophy, and stiffness, the amputation is entirely appropriate.
- 39. 5. Ultrasound- Low-Intensity pulsed ultrasound stimulation (LIPUS) • Okada et al. reported a case of CPT of the tibia (Boyd type IV) successfully treated with low-intensity pulsed ultrasound stimulation (LIPUS) administered for 20 min/day. • The treatment was continued for 1 year until solid fusion on radiographs and subsequent full-weight-bearing was achieved. • • The underlying mechanisms of action of LIPUS remain unclear. However, in experimental studies conducted in rats, LIPUS application facilitates union and increase mechanical strength of bone
- 40. complications 1. Refracture 2. Malalignment of the tibia 3. Limb length discrepancy 4. Ankle valgus 5. Ankle stiffness
- 41. 1. Refracture. • 14% to 60%. • Anatomic alignment of the tibia and fibula minimize the risk of re-fracture. • Intramedullary rod and external bracing must be continued as effective protection against re-fractures.
- 42. 2. Malalignment of the tibia • Diaphyseal malalignment of the tibia (procurvatum or valgus deformity) are progressive and do not remodel
- 43. 3. Limb length discrepancy • Residual limb length discrepancy following successful union is a major problem. • Growth abnormalities of the tibia, fibula, and the ipsilateral femur abnormalities are also noted with CPT • which include inclination of the proximal tibial physis, posterior bowing of the proximal third of the tibial diaphysis, proximal migration of the lateral malleolus.
- 44. 4. Ankle valgus
- 45. 4. Ankle valgus • Compromises functional outcome. • Progressive ankle valgus is a problematic postoperative donor-site morbidity of a vascularized fibular graft in children. • • Tibiofibular metaphyseal synostosis (the Langenskiöld procedure)
- 46. 5. Ankle stiffness • progressively regresses once intramedullary rod is removed from ankle. • Pain secondary to degenerative changes of the ankle can be treated with limitation of activity and shoe modification. • Severe pain may require ankle arthrodesis
- 47. Follow up. • till skeletal maturity to identify and rectify residual problems.
- 48. Take Home message. • Rare. • The natural history of the disease is extremely unfavorable and • Little or no tendency for the lesion to heal spontaneously. • challenging to treat effectively • Aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture. • The key to get primary union is to excise hamartomatous tissue and pathological periosteum. • Age at surgery, status of fibula, associated shortening, and deformities of leg and ankle play significant role in primary union and residual challenges after primary healing. • Surgical options such as intramedullary nailing, vascularized fibula graft, and external fixator, have shown equivocal success rate in achieving primary union • Amputation must be reserved for failed reconstruction, severe limb length discrepancy and gross deformities of leg and ankle..
- 49. Refereneces. • Shah H, Rousset M, Canavese F. Congenital pseudarthrosis of the tibia: Management and complications. Indian J Orthop 2012;46:616- 26 • Campbell’s – 12th edition. • Paediatric orthopaedics –A system of decision making, by Benjamin Joseph.
- 50. THANK YOU.
Editor's Notes
- #9: Several of these growth abnormalities develop if the tibial pseudarthrosis remains ununited and they become more severe as the child grows. This implies that the sooner union of the pseudarthrosis is achieved the less pronounced these growth abnormalities will be.5
- #11: ANDERSON’S ( 1973) differentiates the morphology of the pseudarthrosis as dysplastic, cystic, or sclerotic types, in addition to a clubfoot type that arises because of accompanying abnormalities.
- #12: Widely accepted.
- #15: drawbacks of the classification are that it does not predict prognosis neither in ascending or descending order and short-term results. The classification seems promising, but still it has to be validated in long term results and multicenter study.
- #30: However, many potential complications have been reported. Sometimes, secondary bone grafting is necessary to obtain bone union. [23],[30] Simonis et al. recommended the use of this method when limb length discrepancy is more than 5 cm and pathological tissue length is greater than 3 cm. [47] New-onset fractures are not rare, and they may be observed at the graft site extremity, [23],[40] or in the bone graft itself. [48] Other complications include malalignment (anterior bowing and valgus deformity), as no remodeling occurs in these progressive angular deformities. [23],[30],[49],[50] Valgus deformity on the donor site has also been reported. [48],[50],[51] In order to prevent this complication, distal tibiofibular metaphyseal synostosis (Langenskiold procedure) is recommended by some authors. [23],[50]However, this procedure may only delay, but may not prevent, the development of ankle valgus. [50]
- #31: Fabry and Plawecki reported initial bone union in all patients treated with external fixation after one or more surgical procedural failure. [54],[70] Paley et al. (1992) reported initial union in 15 out of 16 children after one treatment, and union after one additional treatment in 1 child.[42] Mean age at the time of treatment was 8 years. Boero et al. (1977) reported less enthusiastic results, as circular external fixation failed in 8 out of 21 patients with CPT in the setting of Type 1 Neurofibromatosis. [27] The age at the time of surgery may have influenced bone union results. Consolidation was obtained in 12 out of 14 patients who were operated when they were 5-years old, and only in 1 out of 7 patients who were treated before 4 years of age. The European Paediatric Orthopaedic Society (EPOS) report found an overall rate of consolidation of 75.5% with mean age of 7.5 years. [9] These data are close to those from Ohnishi et al., as consolidation was obtained in 86% of patients, with a mean age of 4.5 years at the time of surgery. [26] Cho et al. reported re-fracture-free rate of cumulative survival of 47% at 5 years and did not change thereafter. The risk of re-fracture was significantly higher with a younger age at surgery (less than 4 years), low cross-sectional area of the healed segment, recurrence of tumor, when associated with persistent fibular pseudarthrosis, residual ankle valgus, removal of an intramedullary rod, and noncompliance with bracing. [56] Fixation can correct deformities of leg and ankle and limb length discrepancy simultaneously and has a high rate of primary union in children older than age 6 years. However, the external fixator can be cumbersome for small children and it fails to prevent ankle valgus. Moreover, there is high frequency of re-fracture following external fixator and involution of the regenerate bone tissue has been observed following use of circular external fixator. Circular external fixation procedure takes a long time, is complex and associated with a high risk of infection. If the child has been protected with internal splint (intramedullary nailing) and bracing till skeletal maturity, the frequency of the re-fracture can be reduced significantly. [6],[10],[26],[36],[52],[55] Authors preference We use external fixator as primary treatment in children older than 8 years of age, with significant limb length discrepancy and associated deformities of the leg. We observe that significant limb length discrepancy and deformities of the leg are more in children who present after 8 years of age. We prefer to correct limb length discrepancy, deformities of leg with achieving union of the pseudarthrosis site simultaneously. The external fixation provides better axial, angular, and rotational stability of pseudarthrosis site, which is essential for primary union. We prefer to add intramedullary nailing with external fixation primarily, as it helps to realign the tibial segments and minimize the shearing force at the pseudarthrosis site during healing. We also prefer to leave intramedullary nailing after primary healing to protect against re-fracture. We allow weight bearing early following circular external fixation application. Children can resume back to school and social activities soon after the surgery.
- #32: The Ilizarov frame is applied, the pseudarthrosis is excised and the fragments are compressed. The compression can be applied in different ways including closed longitudinal compression, side-toside compression, segmental bone transport and resection with acute compression.Resection and acute compression is recommended as bone grafting can also be combined with the procedure. A metaphyseal osteotomy is also performed and tibial lengthening is begun after seven to ten days The highest union rates have been reported for this approach as opposed to longitudinal compression, side-to-side compression or segmental bone transport.
- #46: To prevent this complication, tibiofibular metaphyseal synostosis (the Langenskiöld procedure) has been recommended.
- #49: Rare. The natural history of the disease is extremely unfavorable and once a fracture occurs, there is a little or no tendency for the lesion to heal spontaneously. challenging to treat effectively aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture. The key to get primary union is to excise hamartomatous tissue and pathological periosteum. Age at surgery, status of fibula, associated shortening, and deformities of leg and ankle play significant role in primary union and residual challenges after primary healing. Surgical options such as intramedullary nailing, vascularized fibula graft, and external fixator, have shown equivocal success rate in achieving primary union although they are often associated with acceptable results. Amputation must be reserved for failed reconstruction, severe limb length discrepancy and gross deformities of leg and ankle..