Introduction: Spine fractures are common manifestation of osteoporosis. After an acute osteoporotic vertebral compression fracture pain persisting even after 3 months and clinical tenderness should raise the suspicion of pseudarthrosis.
PURPOSE. To report the experience and evolution of treatment of Congenital
CPT is a uniquely difficult condition, often associated with Neurofibromatosis (NF1), where bone healing is compromised. Although rare, the severity of this condition and the multiple procedures often entailed in treating it, warrant research attention. As study material is limited, animal models of the disorder are desirable for testing new treatments. We sought to create a model of CPT where both copies of the NF1 gene were ablated at the fracture site, as has been found in some clinical specimens. NF1 floxed mice had fracture surgery; both closed fracture and open osteotomy were performed. Either a Cre- or control GFP-adenovirus was injected into the fracture site at day zero. Recombination was confirmed in ZAP reporter mice. Additionally, cell culture studies were used to examine the possible responses of NF1+/+ (wild type) NF1+/− or NF1−/− to drugs which may rescue the dysregulated Ras/MAPK pathway in NF1. In closed fractures, radiographic bridging was 100% in NF1+/+ calluses and <40% in NF1−/− calluses (P<0.05). In open fractures, radiographic bridging was 75% in NF1+/+ calluses and <30% in NF1−/− calluses (P<0.05). In both fracture repair models the NF1−/− state was associated with a significant up to 15-fold increase in fibrotic tissue invading the callus by week 3. In NF1−/− fractures, large numbers of TRAP+ cells were observed histologically in the fibrotic tissue. Closed fractures also showed a significant increase in BRDU labelled proliferating cells in the callus. In cell culture models of NF1 deficient osteogenesis, NF1−/− progenitors were found to be significantly impaired in their capacity to form a calcified matrix as measured by Alizarin Red S staining and osteogenic markers (Runx2, Osteocalcin, Alp expression). However, when differentiated calvarial NF1 floxed osteoblasts were treated with Cre adenovirus, mineralization was not affected, suggesting that NF1 impacts on osteogenic differentiation rather than mature cell function. Treatment with MEK inhibitor PD0325901 was found to rescue the NF1−/− progenitor differentiation phenotype and permit robust mineralization. Treatment with the JNK inhibitor SP600125 was also able to improve ALP activity and mineralization in NF1+/− osteoprogenitors compared to control cells. This model of NF1 −/− induction at a fracture or osteotomy site closely replicates the clinical condition of CPT, with lack of bone healing and fibrous tissue invasion. Underlying defects in bone cell differentiation in NF1 deficiencies can be at least partially rescued by JNK and MEK inhibitors.
The problem of retained drain fragments is a well known but under reported complication in the literature. The authors present the case of a 66 years old male, who suffered a right distal humerus fracture luxation six years ago that was treated conservatively. He went to the emergency service with fever and right elbow purulent drainage. Physical examination showed deformity, swelling and fluctuation of the right elbow with purulent drainage through cutaneous fistula. The x-ray showed instable inveterate pseudarthrosis of the distal humerus. Leucocytosis and neutrophylia with increased CRP were presente in the blood tests and the patient started empiric treatment with Ceftiaxone IV. A MRSA was isolated in cultural exam of the exsudate, and a six weeks treatment with Vancomycin IV was iniciated. Exhaustive surgical cleaning was performed and two plastic foreing bodies (fragmented drains) were removed. At the time of discharge the patient was afebrile, with normal analytical parameters and negative culture tests. The orthopaedic surgeon should considerate the presence of a foreign body in patients with infected abcess and traumatic or surgery previous history.
Three or more years after completion of treatment, we re-examined 16 patients with orthopaedic problems associated with neurofibromatosis I (NF-I) who were treated at our institution between 1976 and 1999. Seven boys and five girls between the ages of 5 and 15 years presented with congenital pseudarthrosis of the tibia (CPT). All had undergone previous surgery elsewhere. The patients had typical skin lesions and the associated radiological appearances of pseudarthrosis of the tibia. There were two cystic types of CPT, five hourglass and five normotrophic types, mostly at the level of the distal third of the tibia. Primary consolidation of the CPT was not obtained in any patient. Three patients underwent below-knee amputation after multiple surgical procedures. Eight had consolidations of the pseudarthrosis after multiple operations, but all had residual deformities and/or shortening. One patient remained with a non-consolidation. The surgical procedures included intramedullary rodding with or without bone grafting, fibular bypass grafting, Soffield turn-about rodding, electrical stimulation, and, in patients seen since 1989, various Ilizarov techniques including lengthening and bone transport. Our results suggest that the best treatment of this perplexing paediatric orthopaedic problem remains undetermined. Considerations for the selection of treatment include the pathologic anatomic pattern of NF-I and the patient’s age and expectations. A National Orthopaedic Neurofibromatosis Register will be useful in the decision-making process.
Presenters Position: To perform a retrospective audit of the spectrum of management of tibial pseudarthrosis by a single surgeon over a seven year time period. All discharge summaries and operation logs from 2004 to 2011 were reviewed to identify patients, and their case notes and x-rays were examined. Patients were contacted telephonically for follow-up examination.Purpose of Study:
Description of Methods:
Percutaneous vertebroplasty is an effective procedure for the treatment of osteoporotic vertebral compression fractures, spinal metastasis and other pathologic spinal diseases. However, there has been no mention in the relevant literature of the use of percutaneous vertebroplasty for the treatment of spinal pseudarthrosis in ankylosing sponyloarthritis. A 58-year-old male with a long standing ankylosing spondylitis presented with increasing, intolerable and non-intractable back pain. There was a 16- month-old history of a non-significant minor fall. Various radiological imaging technicques showed spinal pseudarthrosis with extensive discovertebral destruction and fracture of the posterior elements at the level T11–T12. Under local anaesthesia, and through a transpedicular approach with the guidance of CT, the cannula of a large bore needle was introduced into the level of spinal pseudarthrosis. Bone cement was then instilled into the affected spinal level. Results were documented by spiral CT and with sagittal reconstructions. Extraosseous cement leakage was seen at the puncture site of the vertebra and in the epidural veins and the paravertebral vessels. However, the patient did not present any immediate or late neurological and systemic complications. Percutaneous vertebroplasty of spinal pseudarthrosis in patients with ankylosing spondylitis is an effective procedure for stabilization of the affected spine segments and pain management.
Congenital pseudarthrosis of the tibia remains difficult to treat. With variable success, children are subjected to many surgical procedures. We review our experience using titanium elastic nails in this condition. We have treated 10 patients, six boys and four girls. Five had associated neurofibromatosis and pseudarthrosis of the fibula. In seven children, a mean of four previous operations had been done. The mean follow-up time was 4 years. In our patients, union occurred at a mean of 18 weeks, with a mean limb length discrepancy of 3 cm. The range of movement in the knee and ankle was good. Our early results are encouraging.
Seventeen patients underwent surgery, at a mean age of 6 years and 4 months. The procedure always included excision of the pseudarthrosis at both ends and internal fixation with a wire or a plate. In only eight cases a bone graft was used. Healing was achieved in fourteen patients. Three patients needed a second surgical procedure. In these 3 cases we had not used bone grafting. All patients had a normal range of shoulder motion, except a twelve year old girl who complained of discomfort of the right upper limb. The cosmetic result was good in eleven cases, one surgical wound was noted as hypertrophic, and one developed a keloid. An asymmetry of the trunk was still noted in seven cases. The x-rays showed symmetric clavicles in ten cases. Eight patients were treated conservatively. All of them had a normal range of motion of the shoulder, six had a good cosmetic result and two cases a poor one. Discussion According to Alldred, the anomaly results from the failed coalescence of the two primary ossification centers of the clavicle. The overview of the literature and our findings (in one case) confirm that the cartilage which covers both ends of the bone is made of growth cartilage. However, the true mechanism of the nonunion is still unknown. The three familial cases of our work suggest a possible genetic transmission of the disease. The diagnosis is based on the following criteria: right side lesion, found in infancy, without previous fracture, increasing size with growth, without major functional consequences, without neurofibromatosis or cleidocranial dysostosis symptom. X-rays or histologic examination will confirm the diagnosis showing the usual findings described above. Complications of the pseudarthrosis of the clavicle are rare and late. Conservative management appears to give good results as seen with our eight patients. However surgical treatment ensures symmetrical shoulder girdles and good function with few complications. Therefore, we recommend performing an excision of the cartilaginous caps, followed by an iliac bone graft and an internal fixation with wire. Surgical management will be preferred in symptomatic patients, in the case of major or increasing deformity, or on parental request.
The “Zemel technique” for scaphoid pseudarthrosis without major carpal collpse is a modification of the Matti-Russe procedure, using only cancelous bone graft and 2 Kirchner wires for fixation – these are introduced under direct vision after a large curetage of the pseudarthrosis, then partially withdrawn and reintroduced after tight local cancelous bone packing. We used it in 51 cases, obtaining bone union in 48. In 9 patients bone, union was not certain after 12 weeks of plaster and K wires were replaced by a screw with no further immobilization, but we still had 3 nonunions. This procedure, technically easy to perform and using current orthopaedic material, seems a good alternative to non-angulated pseudarthrosis of the scaphoid, except of its proximal pole.
This paper reviews the outcome of 13 children with congenital pseudarthrosis of the tibia after intramedullary rodding and autogenous bone grafting. The oldest patient was aged nine years at the time of surgery. The ages of the others ranged from 12 to 24 months. The oldest patient at follow-up was 18 years. All 13 had bone defect and angulation. Ten children had clinical features of neurofibromatosis. Ten had pseudarthrosis involving the distal third of the tibia, two the middle third and one the proximal third. Autogenous iliac crest chips were used following excision of fibrous tissue and dense and atrophic bone. Rodding was done across the ankle joint in 10 patients. Postoperatively an above-knee cast was applied for 6 to 12 months, after which an above-knee brace was used to protect the rodding. At follow-up, which ranged from 10 months to 16 years after surgery, all patients were fully weight-bearing and ambulant. Three patients were lost to follow-up after 2 to 4 years. Complications included refracture and rod breakage (two), rod migration (three), and growth retardation with shortening of up to 5 cm. Ten patients had ankle and subtalar joint stiffness and two had valgus deformities of the ankle. Three patients underwent repeat rodding and bone grafting. Radiological union was observed to be progressing in all patients. Intramedullary rodding of the tibia for congenital pseudarthrosis of the tibia is a simple procedure and can be repeated. It avoids prolonged hospital stay and permits early weight-bearing. Careful supervision is necessary, and until there are signs of good bony union, external support is mandatory.
To determine the preferable treatment for congenital pseudarthrosis of the tibia, we retrospectively reviewed 19 patients (20 limbs) treated consecutively over a 22 year period (1988–2007). Fifteen were followed up to maturity. The patients were assessed for union, leg length discrepancy (LLD), ankle valgus, range of ankle movement and distal tibial physeal injury. The median age at surgery was 3 years. At surgery nineteen of the tibiae had a dysplastic constriction with a fracture (Crawford II-C or Boyd II) lesion. To obtain union in the 20 tibiae, 29 procedures were done. Nine failed primarily and required a second procedure to obtain union. Older patients (≥ 5 years) had a significantly higher success rate. Excision, intramedullary rodding and bone graft (IMR) was done in 14 tibiae: 10 (71.4%) were successful. Six of 10 primary operations and all 4 secondary operations after a previous failed procedure were successful. Ipsilateral vascularized fibula transfer (IVFT) was successful in 5 tibiae (3 primary and 2 secondary). Ilizarov with bone transport only, failed in two patients. Ilizarov with excision, intramedullary rodding and bone graft with lengthening was successful in 2 of 5 cases (40%); two sustained fractures at the proximal lengthening site. A median leg length discrepancy (LLD) of 3 cms occurred post surgery which was treated with contralateral epiphysiodesis. At maturity 3 patients had a LLD of ≥ 2cms. Six limbs had ankle valgus and were treated with stapling and tibio-fibular syndesmosis. Decreased range of movement of the ankle (< 50%) occurred in 7 patients. Distal tibial physeal injury occurred in 4 patients and was associated with repeated rodding.Aim
Results
Congenital pseudarthrosis of the lower limb is not uncommon, with an incidence of approximately 1 in 150,000. In contrast, isolated congenital pseudarthrosis of the fibula without tibial involvement is a very rare. There were only 11 cases of true isolated congenital pseudarthrosis of the fibula described in the English literature till 1999. We describe 3 patients diagnosed and treated in our institution. The current management is also discussed, highlighting the unique treatment options in this rare condition.
We evaluated the clinical outcome of IM nailing for the treatment of femoral shaft pseudarthrosis in patients who had multiple failed plate osteosyntheses. From January 2000 untill April 2001, 20 (19 male-1 female, mean age 28) patients were treated because of femoral shaft non-union in our institution. All patients had two or more failed plate osteosyntheses. There were no septic non-unions in this group. Eight patients had an established non-union on an average of nine months post-op and the remaining eleven had radiological and clinical evidence of implant failure. There was no segmental bone loss, hi all patients the implants were removed and nailing was performed. Extensive periosteal stripping, bone necrosis and soft-tissue scaring were constant findings in all patients. Twelve patients received interlocking nails. Eight femurs were grafted with iliac crest bone graft. All patients were followed by serial x-rays until union. There were no postoperative complications. All pseudarthroses were healed within an average of 9.7 months (8–12). Non-unions which received bone graft (eight out of twenty) in day one, were healed faster than those which didn’t. There were no re-operations among these patients. Among the remaining ten patients five were grafted five to six months postoperatively and three had had nail dynamization. IM nailing for femoral shaft non-unions after multiple failed plate osteosyntheses is a safe and effective method of treatment. Autologous bone graft reduces healing time and re-operation rate.
We present a case of a 19-year-old white female patient with neurofibromatosis type I who, 10 years ago, underwent free vascularized fibular grafting for isolated congenital pseudarthrosis of her left radius. An external fixator was applied for gradual distraction and correction of the deformity of the pseudarthrosic site for five weeks. Wide resection of pseudarthrosis with surrounding fibrotic and thick scar tissue and bridging of the gap with a free vascularized fibular graft followed. Four months postoperatively, union was established in both graft ends. At the last follow-up, 10 years postoperatively, the patient has excellent function with full wrist flexion-extension and forearm pronation-supination. Free vascularized fibula transfer is considered the treatment of choice for congenital radial pseudarthrosis. It allows complete excision of the pathologic tissue and covering of the gap in one operation. Due to the vascularity of the free vascularized fibular graft both sides of fibula unite easily with no additional intervention.
Our aim is to study the epidemiology of delayed union and pseudarthrosis of femoral fractures which were treated in our clinic with intramedullary nailing technique and simultaneously to speculate for the application of the method and for the reasons which might guide to these complications. During a 36 month-period, from 1999 to 2001, 46 patients suffering femoral shaft fractures were treated in our clinic. They were 43 men and 3 women and their average age was 22.8 years. Twenty-eight fractures were in the right leg and 18 in the left. All patients underwent primary closed intramedullary locked nailing with AO type of nail, except 4 patients who also suffered a unilateral intertrochanteric fracture and were treated with AO-PFN nail. Reaming was done in 33 cases. No condition or disease able to inhibit fracture healing was observed. All patients were operated in the first three days after submission by the same team of surgeons. Thirty patients underwent static intranaedullary locked nailing and the rest dynamic from the beginning. The average time of post-operative follow up, clinical and radiological, was seven months. Thirty-three fractures were healed successfully in six months (mean: 4.2 months). In 10 cases delayed union was observed (mean: 7.2 months) and in the rest three pseudarthrosis. The last group underwent revision of the intramedullary nailing with an AO nail of greater diameter. Reasons for delayed union (21, 74%) and pseudarthrosis (6, 52%) are considered: a)soft tissues enclosed between the bony segments, b)lated nail dynamization, c)factors concerning the technique. Undoubtfully, closed intramedullary locked nailing is the method of choice for the surgical treatment of femoral fractures. However, fracture healing sometimes exceeds the usual period of 4–6 months. Unfortunately, whereas infrequently, the surgeon also faces pseudarthrosis which is a difficult to solve problem.