Treatment Of Congenital Pseudarthrosis Of The Tibia Research Articles

Comparative study of different extendable intramedullary rods combined with surgery in the treatment of congenital pseudarthrosis of the tibia

BackgroundWhen using traditional extensible intramedullary rods to treat congenital pseudarthrosis of the tibia (CPT), there were cases of re-fracture and internal fixation fracture. Therefore, the authors propose a research hypothesis that a thicker distal extensible intramedullary rod can better protect the tibia and reduce the incidence of refracturePurposeTo investigate the clinical efficacy of new and traditional extensible intramedullary rods in the treatment of CPT in childrenMethodsFrom January 2017 to December 2021, the clinical data of 49 children with CPT who were treated with traditional extensible intramedullary rod combined surgery (group A) and new extensible intramedullary rod combined surgery (group B) in our hospital were collected. Inclusive criteria: ① Crawford type IV CPT children; ② The operation was performed by the same team. Exclusion criteria: patients with multiple tibial angulation. During follow-up, the initial healing, proximal tibial valgus, tibial length, ankle valgus, refracture and intramedullary rod displacement of CPT children in the two groups were evaluatedResultsIt was a retrospective investigation. In group A, 26 cases met the inclusion criteria, 24 cases achieved primary healing, with an primary healing rate of 92%, including 1 case of nonunion due to osteomyelitis complications after surgery, and 1 case of delayed healing, with an average healing time of 4.7 ± 0.8 months. 17 cases (68%) had unequal tibia length, with an average difference of 1.6 ± 0.8 cm. Ankle valgus occurred in 10 cases (40%) with an average of 14.4°±4.8°; Proximal tibial valgus occurred in 6 cases (24%) with an average of 7 °± 1.8 °. 20 cases (80%) had tip of the rod migration.10 cases (40%) had re-fracture; The average follow-up time was 2.4 ± 0.4 years. In group B, 22 patients achieved primary healing, and the primary healing rate was 95%, including 1 case with delayed healing. The average healing time was 4.7 ± 1.7months. 14 cases (61%) had unequal tibia length, with an average difference of 1 ± 0.5 cm. Ankle valgus occurred in 4 cases (17%) with an average of 12.3 °±4.9°; The proximal tibia valgus occurred in 9 cases (39%), with an average of 7.7 °±2.5 °. 14 cases (61%) had new type of intramedullary rod displacement. 3 cases (13%) had re-fracture; The average follow-up time was 2.3 ± 0.6yearsConclusionCompared with the traditional extended intramedullary rod combined operation, the new type of extended intramedullary rod combined operation has a lower incidence of re-fracture after CPT, but it still needs to be verified by large sample and multi-center research

THE CURRENT STATE OF DIAGNOSIS AND TREATMENT OF THE CONGENITAL TIBIA PSEUDOARTHROSIS

Congenital pseudarthrosis of the tibia (CPT) is a rare disease that is detected with a frequency of 1 in 140–250,000 newborns. The disease is characterized by a wide range of clinical and radiological signs from progressive antecurvature deformation of the tibia to nonunion with a significant bone defect. Changes in the CPT area are caused by the influence of pathologically altered periosteum, which forms a fibrous hamartoma and is responsible for the deformityof the biomechanical properties of bone tissue. CPT can be formed at the moment of birth or developed spontaneously or as a result of minimal trauma in the early years. The main method of treatment of CPT is a surgery. Nowadays a number of surgical techniques, which are actively used and improved by specialists in the world, has been developed, The most used methods are the Ilizarovʼs method, application of intramedullary fixators, techniques with the use of vascularized tibial autograft, «induced membrane» technique. However, there are a few studies on comparing the effectiveness of different techniques or metal fixatives, most of them are presented in the format of a retrospective analysis of clinical cases series. This is due to the rarity of the disease and the lack of unified approaches on the choice of surgical treatment techniques. The main aim of surgical treatment of CPT is to achieveconsolidation in the area of pseudoarthrosis, which may restore the limb resistance. The part of primary consolidation of CPT after using the surgical treatment various techniques varies very much, range from 60 to 100 %. The percentage of children with CPT tibial amputations has decreased significantly over the past 30 years, which generally indicates an improvement of the results of surgical treatment of the mentioned pathology. However, CPT still remains one of the most difficult diseases of pediatric orthopedics due to the large number of unsatisfactory results and complications after surgery.

Efficacy of the \u201cEiffel tower\u201d double titanium elastic nailing in combined management of congenital pseudarthrosis of the tibia: preliminary outcomes of 17 cases with review of literature

BackgroundDifficulty in obtaining union, recurrent fractures, and residual deformities remain the problems challenging the management of congenital pseudarthrosis of the tibia (CPT). We applied the “Eiffel Tower” double titanium elastic nails (TENs) in the existing combined approach, which takes advantages of TEN’s mechanical stability with the protection against refracture, Ilizarov’s high fusion rate with alignment control and the biologic environment provided by bone grafting for bony union. The results of this procedure are presented and discussed.MethodsSeventeen patients with CPT treated by combined surgery including pseudarthrosis resection, the “Eiffel Tower” double TENs technique, autogenous iliac bone grafting, and Ilizarov fixation between 2013 and 2019 were retrospectively investigated. Signs of bone union, limb length discrepancy (LLD), rate of refracture, and degree of residual deformities were reviewed. The AOFAS Ankle Hindfoot scale and measurement of ankle motion were used to evaluate ankle function. The mean follow-up time was 40.5 (11 to 91) months.ResultsThe mean age at index surgery was 6.2 (2.5 to 15) years. Union of the pseudarthrosis was achieved in 100% of cases. Among them, 15 (88.2%) patients obtained union of the pseudarthrosis on the first attempt (primary union). The average time to primary union was 3.8 (2 to 6) months. The rest 2 cases achieved union after additional surgeries (secondary union). In terms of complications, refracture occurred in 2 patients (11.8%) and 4 patients (23.5%) developed pin infection. The mean limb length discrepancy at the final follow up was 33.4 (6–141) mm. The average AOFAS score improved from 38.2 (27 to 51) pre-operatively to 77 (63 to 87) post-operatively (p < 0.01).ConclusionsThe “Eiffel Tower” double TENs technique is an ideal intramedullary fixation method in the surgical treatment of CPT. The combination of TENs technique with bone grafting and Ilizarov fixation has the advantages of early bone union, less injury on metaphysis, and early functional recovery.Level of evidenceLevel IV.

Combined massive allograft and intramedullary vascularized fibula transfer: the Capanna technique for treatment of congenital pseudarthrosis of the tibia

Background and purpose — Congenital pseudarthrosis of the tibia (CPT) is caused by local periosteal disease that can lead to bowing, fracturing, and pseudarthrosis. Current most successful treatment methods are segmental bone transport and vascularized and non-vascularized bone grafting. These methods are commonly hampered by discomfort, reoperations, and long-term complications. We report a combination of a vascularized fibula graft and large bone segment allograft, to improve patient comfort with similar outcomes.Patients and methods — 7 limbs that were operated on in 6 patients between November 2007 and July 2018 with resection of the CPT and reconstruction with a vascularized fibula graft in combination with a bone allograft were retrospectively studied. The mean follow-up time was 5.4 years (0.9–9.6). Postoperative endpoints: time to discharge, time to unrestricted weight bearing, complications within 30 days, consolidation, number of fractures, and secondary deformities.Results — The average time to unrestricted weight bearing with removable orthosis was 3.5 months (1.2–7.8). All proximal anastomoses consolidated within 10 months (2–10). 4 of the 7 grafts fractured at the distal anastomosis between 6 and 14 months postoperatively. After reoperation, consolidation of the distal anastomosis was seen after 2.8 months (2–4). 1 patient required a below-knee amputation.Interpretation — This case series showed favorable results of the treatment of CPT through a combination of a vascularized fibula graft and large bone segment allograft, avoiding the higher reintervention rate and discomfort with ring frame bone transport, and the prolonged non-weight bearing with vascularized fibula transfer without reinforcement with a massive large bone segment allograft.

Amputation Outcomes in Congenital Pseudarthrosis of the Tibia.

Successful radiographic union in the treatment of congenital pseudarthrosis of the tibia (CPT) may be complicated by persistent pain, recurrent fracture, and poor function necessitating further intervention, including amputation. The long-term functional, radiographic, and clinical outcomes of patients who have undergone amputation as treatment for CPT are unknown. A retrospective study of patients with a diagnosis of CPT secondary to neurofibromatosis and eventual treatment with amputation were included. Clinical and radiographic data, including initial Crawford classification, type of amputation, subsequent operative procedures, and evidence of radiographic healing were collected. A total of 17 patients with a mean age of 4.5 years (range, 0.7 to 9.2 y) at the time of amputation met inclusion criteria. Clinical follow-up averaged 11.1 years (range, 2.1 to 18.4 y), with radiographic follow-up averaging 9.1 years (range, 2.1 to 16.4 y). The mean number of surgeries before amputation was 2.2 procedures. Four patients underwent amputation as the primary procedure (3 Boyd, 1 below knee amputation (BKA)). At the time of amputation, a Boyd amputation was performed in 13 patients with stabilization of the pseudoarthrosis achieved with retrograde Rush rodding of the tibia and local autograft. A transtibial amputation (BKA) was performed in 4.After the Boyd procedure, 4 of the 13 patients (31%) demonstrated persistent nonunion of the pseudoarthrosis and required secondary procedures to gain union. At the most recent follow-up, 12 of 13 patients demonstrated successful radiographic healing of the pseudoarthrosis. Two patients, one for persistent pain and the other for refracture, were later converted to a BKA during the late teen years. All patients functioned well with the use of prosthetic devices. Union of the pseudoarthrosis occurred in >90% of cases following amputation. However, secondary procedures were required in 13 of the 17 patients (76%). Early amputation in the treatment of CPT provides a stable extremity and potential for a high level of function with the use of an adequate prosthesis. Level IV-case series.

Successful treatment of congenital pseudarthrosis of the tibia: still a challenge

The congenital pseudarthrosis of the tibia (CPT) is one of the most challenging problems in pediatric orthopedics. The primary treatment goals are outlined as osteosynthesis, stabilization of the ankle mortise by fibular stabilization and lower-limb-length equalization. Despite the fact that each of the aforementioned goals is difficult to be achieved regardless the surgical option, the main biological consideration is the same: pseudarthrosis resection, biological bone bridging of the defect by stable fixation and the correction of any angular deformity. The external fixation method is suggested as valuable treatment of CPT because it can address not only pseudarthrosis but also all complex deformities associated with this condition. However, treatment of CPT is impaired with complications due to the complex nature of the disease thus failure is common. The most common of these are refracture, growth disturbance, poor foot and ankle function with stiffness. Of these, refracture is the most common and serious complication after primary healing and might result in the reestablishment of pseudarthrosis. Therefore, an effective, safe and practical treatment method that minimizes the residual challenges after healing and accomplishes the multiple goals of treatment is needed. In this article, we report a patient with CPT treated successfully with external fixation. Level of evidence IV retrospective.

Masquelet's procedure and bone morphogenetic protein in congenital pseudarthrosis of the tibia in children: A case series and meta-analysis

A type 2 recombinant human bone morphogenetic protein (rhBMP2) and Masquelet's procedure were used in three children presenting with congenital pseudarthrosis of the tibia (CPT). Recent studies on CPT suggested the presence in situ of pathologic tissues promoting pseudarthrosis. The authors hypothesized that large segmental resection of pseudarthrosis could improve prognosis of the CPT. Masquelet's procedure and rhBMP2 have been advocated for the treatment of long bone defect. The authors report three cases of CPT in children treated with Masquelet's procedure and application of rhBMP2. They analyzed all published cases of CPT similarly treated. In the present study, Masquelet's procedure did not improve the results in the treatment of CPT, but segmental bone reconstruction was possible. Bone healing was obtained in three out of the five applications of rhBMP2. In one case, the patient's parents asked for leg amputation. Analysis of the 33 published cases with the application of BMP in CPT points to a 62% healing rate in this pathology. The authors confirmed that segmental bone reconstruction is possible in CPT using Masquelet's procedure. In the literature, the success rate of the application of rhBMP in CPT appears to be lower than the healing rate usually reported without BMP. Nevertheless, the strict selection of patients, limited number of cases, and their heterogeneity make interpreting the results difficult. However, the theoretical risk which the children are exposed to during the use of BMP makes rigorous selection of the indications necessary. Finally, the interest of rhBMP2 application in Masquelet's procedure remained to be proven.

A regenerative approach for bone repair in congenital pseudarthrosis of the tibia associated or not associated with type 1 neurofibromatosis: correlation between laboratory findings and clinical outcome

Congenital pseudarthrosis of the tibia (CPT) is a rare orthopedic disease presenting spontaneous fractures that do not heal. The treatment of CPT is characterized by repeated surgical procedures that often fail, with the inevitable outcome of severe disability and amputation. We tested the hypothesis that CPT may benefit from regenerative strategies based on mesenchymal stromal cells (MSC) combined with platelet-rich fibrin (PRF) as a source of growth factors. The aim of the study was to verify whether laboratory testing to assess the osteogenic properties of MSC and the osteo-inductive activity of PRF correlated with the clinical outcome. Ten patients affected by refractory CPT were treated by using MSC derived from the iliac crest (IC-MSC), PRF and lyophilized bone. In six patients, CPT was associated with type 1 neurofibromatosis (NF1). Biochemical, functional and molecular assays were performed to assess the intrinsic osteogenic potential of IC-MSC (cells cultured with fetal calf serum) and the osteo-inductive properties of PRF (cells cultured with autologous serum). Bone consolidation was obtained in three patients who had CPT and NF1. In these patients, the IC-MSC exposed to autologous serum were able to form mineral nodules in vitro, while the mineralizing ability was totally abrogated in patients with a poor clinical outcome. Cell therapy may be a useful tool for the treatment of refractory CPT because it increases the opportunity to achieve effective bone tissue regeneration. Our data suggest that the presence of pro-osteogenic growth factors is an essential requirement for bone healing.

Ilizarov Treatment of Congenital Pseudarthrosis of the Tibia: A Multi-Targeted Approach Using the Ilizarov Technique

Congenital pseudarthrosis of the tibia (CPT) is one of the most challenging problems in pediatric orthopaedics. The treatment goals are osteosynthesis, stabilization of the ankle mortise by fibular stabilization, and lower limb-length equalization. Each of these goals is difficult to accomplish but regardless of the surgical options, the basic biological considerations are the same: pseudarthrosis resection, biological bone bridging of the defect by stable fixation, and the correction of any angular deformity. The Ilizarov method is certainly valuable for the treatment of CPT because it can address not only pseudarthrosis but also all complex deformities associated with this condition. Leg-length discrepancy can be managed by proximal tibial lengthening using distraction osteogenesis combined with or without contralateral epiphysiodesis. However, treatment of CPT is fraught with complications due to the complex nature of the disease, and failure is common. Residual challenges, such as refracture, growth disturbance, and poor foot and ankle function with stiffness, are frequent and perplexing. Refracture is the most common and serious complication after primary healing and might result in the re-establishment of pseudarthrosis. Therefore, an effective, safe and practical treatment method that minimizes the residual challenges after healing and accomplishes the multiple goals of treatment is needed. This review describes a multi-targeted approach for tackling these challenges, which utilizes the Ilizarov technique in atrophic-type CPT.

Preliminary experience with the combined use of recombinant bone morphogenetic protein and bisphosphonates in the treatment of congenital pseudarthrosis of the tibia

Congenital pseudarthrosis of the tibia (CPT) is a rare but serious disorder in children. No single approach has clearly emerged as superior in terms of operative procedure, fixation, optimal time for surgery or adjunctive pharmaceutical intervention. CPT is frequently associated with neurofibromatosis type 1 (NF1), a condition featuring deficient bone anabolism and excessive catabolism. We have therefore combined the use of bone morphogenetic proteins (BMP) with bisphosphonates (BP) as an adjunct to surgical intervention. Between 2002 and 2008 we administered BMP-7 (OP-1) at the time of surgery followed by BP (pamidronate or zoledronic acid) in eight Crawford type IV CPT cases in seven patients (six with a confirmed diagnosis of NF1) with a median age of 7years (range 2years 11months to 12years) at surgery. In six of eight cases, this approach led to primary healing after a mean of 5.5months (range 4-7months). One of these cases represented 17months after primary healing of a proximal CPT with a new further distal fracture that required multiple operations to finally unite at 19months. The two remaining cases ultimately reached union after multiple operations at 14 and 30months, respectively, but required recent treatment for refractures. Based on these clinical data (primary healing in 6/8 cases) and prior pre-clinical findings, we propose that BP therapy may be helpful in preserving the BMP-induced bone formation by inhibiting the osteoclastic bone loss. Key factors to achieve union in CPT include sufficient fixation, meticulous resection of the dysplastic tissue and the establishment of a net anabolic environment for bone healing. Whether our biological concept of balancing the anabolic and catabolic responses with BMP and BP improves healing rates in the complex treatment of NF1 CPT remains uncertain and warrants larger prospective multicentre trials.

Attempt to treat congenital pseudarthrosis of the tibia with mesenchymal stromal cell transplantation

Congenital pseudarthrosis of the tibia (CPT) caused by neurofibromatosis type 1 (NF1) is a refractory disease occurring in childhood. We present two cases that had failed all earlier treatment attempts and, as a last treatment attempt, the patients were chosen to receive mesenchymal stromal cell (MSC) transplantation prior to amputation. The MSC from bone marrow (BM) were harvested from the iliac crest and cultured in osteoinductive medium for 3 weeks. The cultured MSC were injected in solution into BM canals of the tibia and around the resection line or bone defect in a 3-dimensional collagen sponge scaffold. After the MSC transplantation, the patients were monitored during a 10-month follow-up period. In both cases, bone formation at the pseudarthrosis site was observed and two of three treated bone defects healed. For clinical reasons not related to cell transplantation, such as new infection and pseudarthrosis and severe shortening of the leg, both extremities were finally amputated and bone samples were analyzed to evaluate MSC therapy effect and safety. MSC transplantation normalized bone remodeling, promoted bone resorption and improved the overall structure of bone. The number of osteoclasts in the cortical bone was 2-fold higher compared with the monitored situation before MSC transfer. In addition, the mineral content of the bone improved after transplantation. We could see no sign of aberrant bone formation or malignant transformation. Our data suggest that MSC transplantation is a possibility for treatment of CPT caused by NF1 in less severe cases without adjunct defects.

Vascularized free fibular bone graft in the management of congenital tibial pseudarthrosis

Congenital pseudarthrosis of the tibia (CPT) remains one of the most challenging problems confronting the orthopaedic surgeon. The operative results are frequently less than successful; many cases require several surgical procedures, and a significant number of them ending in amputation. The purpose of this study was to access the surgical results, complications, secondary procedures, and long-term results of free vascularized fibular graft (FVFG) in the treatment of congenital pseudarthrosis of the tibia. Between 1992 and 2007, nine patients with CPT were treated consecutively at our clinic with free fibula transfer. There were six females and three males. The mean age at the time of operation was 6.5 years (range, 1-12 years). Stability, after reconstruction with FVFG, was maintained with internal fixation in five patients, unilateral frame external fixation in three patients, and intramedullary pin in one patient. Average postoperative follow-up time was 9 years (range, 2-15 years). In seven patients, both ends of the graft healed primarily within 3.7 months (range, 1.5-6 months). In one patient, the distal end of the graft did not unit. This patient required three subsequent operations to achieve union. Stress fracture occurred in the middle of the grafted fibula in one patient, who underwent four additional operations before union, was achieved. Despite the relatively high-complication rate, FVFG remains a valid method for the treatment of CPT. However, even achieving union of pseudarthrosis is not enough for the resolution of the disease. This is only half of the problem; the other half is to maintain union. Long-term follow-up beyond skeletal maturity, if possible, is necessary to evaluate surgical results.

Treatment of Congenital Pseudarthrosis of the Tibia

Treatment of congenital pseudarthrosis of the tibia (CPT) remains a challenge. To clarify the current situation in treatment, a multicenter study was carried out to obtain information on the results of CPT treatment. The objective of this study was to propose appropriate treatment guidelines for CPT. Records of 73 patients with CPT who underwent surgical treatment were collected from 32 hospitals. The modality of the treatment was 26 with Ilizarov technique, 25 with free vascularized fibular graft, 7 with a combination of the two techniques, 6 with intramedullary nailing with free bone grafting, 5 plating with free bone grafting, and 4 with other treatments. Fifty-four procedures resulted in union, 7 resulted in delayed union, 7 were left un-united, 1 underwent amputation, and the results were unknown in 4. According to the results of this study, the most acceptable methods of treatment of CPT are the Ilizarov method and the vascularized fibular graft.

Treatment of congenital pseudarthrosis of the tibia by microsurgical fibula transfer

Conventional methods for the treatment of congenital pseudarthrosis of the tibia (CPT) often lead to unsatisfactory results and so microsurgical fibula transfer (MFT) has appeared as an option to treat this challenging problem. In this series of 27 patients with CPT, we obtained satisfactory results in 23 as assessed by the timing of bone union and bone hypertrophy of the fibular transplant. The overall functional results were also good; in 18 patients, the residual shortening was less than 2 cm (with no limb shortening in 4) and in 7, shortening ranged between 2 and 4 cm. All 25 patients resumed normal walking without the help of braces after an average of 12 months. This report suggests that microvascular fibula transfers provide superior results in the treatment of CPT, especially when considering that many of such cases represented previous surgical failures.