Objective: To explore the long-term effect of combined surgery for the treatment of congenital tibial pseudarthrosis in children. Methods: The clinical data of 44 children with congenital tibial pseudarthrosis who underwent combined surgery (tibial pseudarthrosis tissue resection, intramedullary rod fixation, Ilizarov external fixator fixation, wrapped autologous iliac bone graft) from August 2007 to October 2011 at the Department of Pediatric Orthopedics, Hunan Children's Hospital were collected retrospectively. There were 33 males and 11 females. The age at the time of surgery was (3.7±2.2)years (range:0.6 to 12.4 years), including 25 cases under 3 years old and 19 cases above 3 years old.Among them, 37 cases were complicated with neurofibromatosis type 1.The operation status, postoperative complications and follow-up results were recorded. Results: The follow-up time after surgery was (10.9±0.7)years (range:10 to 11 years).Thirty-nine out of 44 patients (88.6%) achieved initial healing of tibial pseudarthrosis, with an average healing time of (4.3±1.1)months (range:3 to 10months).In the last follow-up, 36 cases (81.8%) had unequal tibial length, 20 cases (45.4%) had refractures, 18 cases (40.9%) had ankle valgus, 9 cases (20.4%) had proximal tibial valgus, and 11 cases (25.0%) had high arched feet.Nine cases (20.4%) developed distal tibial epiphyseal plate bridging.17 cases (38.6%) had abnormal tibial mechanical axis.Seven cases (15.9%) developed needle infection, and one case (2.3%) developed tibial osteomyelitis. 21 patients (47.7%) had excessive growth of the affected femur.Five patients (11.3%) had ankle stiffness, and 34 patients (77.2%) had intramedullary rod displacement that was not in the center of the tibial medullary cavity.Among them, 8 cases (18.1%) protruded the tibial bone cortex and underwent intramedullary rod removal.18 children have reached skeletal maturity, while 26 children have not been followed up until skeletal maturity. Conclusion: Combined surgery for the treatment of congenital pseudarthrosis of the tibia in children has a high initial healing rate, but complications such as unequal tibia length, refracture, and ankle valgus occur during long-term follow-up, requiring multiple surgical treatments.
Male ; Female ; Humans ; Child ; Child, Preschool ; Pseudarthrosis/congenital* ; Follow-Up Studies ; Retrospective Studies ; Tibia/surgery* ; Neurofibromatosis 1 ; Tibial Fractures/surgery*

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare disease that affects the sensory and autonomic nervous system. The patients do not have the ability to sense different sensations, such as pain, which tends to lead to different injuries. In addition, the patients suffer from fluctuations in body temperature due to autonomic involvement. The present case was a five-year-old girl with a neglected distal femur fracture. X-rays taken during the follow-up showed marked callus formation and pseudarthrosis of the distal femur. She had biting injuries of the tongue, auto-amputation of the fingers, some developmental delay and a history of recurrent fever with an unknown origin. The electrodiagnostic study was normal. The quantitative sudomotor axon reflex test revealed markedly reduced postganglionic sudomotor axonal responses at all sites recorded on the left. She was diagnosed with CIPA. As the initial presentation of CIPA involves the musculoskeletal system, orthopedic surgeons should have a high index of suspicion.
Autonomic Nervous System ; Axons ; Body Temperature ; Bony Callus ; Female ; Femur ; Fever ; Fingers ; Follow-Up Studies ; Hereditary Sensory and Autonomic Neuropathies ; Humans ; Musculoskeletal System ; Orthopedics ; Pain Insensitivity, Congenital ; Pseudarthrosis ; Rare Diseases ; Reflex ; Sensation ; Surgeons ; Tongue

Nonunion at the lumbosacral junction is a classic complication of long construct and deformity corrections. Iliac fixations have been extensively studied in the literature and have demonstrated superior biomechanical proprieties and lower complication rates. S2 alar iliac screws address the drawbacks of classical iliac screws but demonstrate similar biomechanical advantage. The main aim of this paper was to describe the S1 alar iliac (S1AI) screw fixation technique while evaluating our early results. S1AI screw fixation technique has the advantage of being able to achieve pelvic fixation without dissection to the S2 pedicle entry and is therefore a viable option for salvage of a failed S1 promontory screw.
Congenital Abnormalities ; Lumbosacral Region ; Pseudarthrosis

STUDY DESIGN: Literature review. OBJECTIVES: To review the complications of osteoporotic vertebral compression fractures (OCFs) with regard to risk factors and treatment strategies. SUMMARY OF LITERATURE REVIEW: Understanding the complications and treatment methods of OCFs is important given their increasing prevalence. MATERIALS AND METHODS: Review of the literature. RESULTS: The complications of OCFs include kyphotic deformity due to a delayed collapse of the vertebral body and neurologic deficits. The pathophysiologic mechanism of these complications has not been established; however, the most accepted theory is posttraumatic avascular necrosis of the vertebral body. The risk factors for these complications include fracture at the thoracolumbar junction, systemic usage of steroids, severe osteoporosis, and radiological intravertebral vacuum cleft. Most of these complications require surgical treatment, the indications for which include progressing axial back pain, pseudarthrosis, kyphotic deformity, and neurologic deficits. Surgical approaches for treating the complications of OCFs include anterior, posterior, and combined anterior-posterior approaches. Osteotomies should be done when deformity correction is needed. The decision among these various surgical methods should be made considering patient factors and the surgeon's ability to achieve the best outcomes with solid fusion. CONCLUSIONS: complications of OCFs include kyphotic deformity due to delayed collapse of the vertebral body and neurologic deficits. Most of these complications require surgical treatment, in which achieving solid fusion at the fracture site is the ultimate goal. Patient-related factors and the surgeon's ability should be considered prior to making decisions regarding surgery.
Back Pain ; Congenital Abnormalities ; Fractures, Compression* ; Humans ; Necrosis ; Neurologic Manifestations ; Osteoporosis ; Osteotomy ; Prevalence ; Pseudarthrosis ; Risk Factors* ; Steroids ; Vacuum

Demand for surgical management of adult spine deformity has increased due to a recent increase in the elder population, development of surgical techniques, and increased desire for a better quality of life. More surgeries led to more and various complications. Complications of surgery for adult spine deformity have been reported from 37% to 53% depending on the types of disease, cause, and patient. This rate is higher than complications from general spine surgeries. Complications of surgery for adult spine deformity include; infection, excessive bleeding, nerve injury, pseudarthrosis, adjacent segment disease, and iliac screw complication. Factors influencing these complications include; old age, diabetes, steroid use, and osteoporosis. Proximal junctional kyphosis (PJK) is an abnormal kyphosis at the proximal level of the instrument after spine deformity surgery. Risk factors for PJK include; injury of paravertebral muscles and posterior ligament complex, over- and under-correction of coronal sagittal imbalance, age older than 55 years, and osteoporosis. There is a high risk of unexpected complications during surgery for adult spine deformity. Patients with degenerative spine sagittal imbalance are more prevalent in Korea than in Western countries. They tend to be old, have underlying diseases and osteoporosis, making them susceptible to complications related to instrumentation. Postoperative satisfaction tends to be low thus surgeons need to take care when selecting surgical methods and fusion level.
Adult* ; Congenital Abnormalities* ; Hemorrhage ; Humans ; Korea ; Kyphosis ; Ligaments ; Muscles ; Osteoporosis ; Pseudarthrosis ; Quality of Life ; Risk Factors* ; Spine*

This case report presents two patients who underwent fibular strut grafting for complex revisions of previous lumbar spine arthrodeses. A case review of the Electronic Medical Record at the index institution was performed to evaluate the timeline of events of the two patients who underwent fibular strut grafting for complex revisions of previous lumbar spine arthrodesis, including imaging studies, progress notes, and laboratory results. One patient had developed chronic L3 vertebral body osteomyelitis from a prior fibular allograft and instrumentation placed for a traumatic burst fracture. The second patient had a severe scoliosis recalcitrant to prior arthrodeses in the context of Marfan syndrome and a persistent L4-5 pseudarthrosis. Both patients underwent free vascularized fibular autograft revision arthrodeses. At most recent long-term follow-up, both patients had improved clinically and neither had required further revision. The use of free vascularized fibular grafting is an excellent option for a variety of spinal indications, and these two reports indicate that the technology may have an indication for use after multiple failed surgeries for osteomyelitis or correction of a multi-level large spinal deformity secondary to Marfan syndrome.
Allografts ; Arthrodesis ; Autografts* ; Congenital Abnormalities ; Electronic Health Records ; Fibula ; Follow-Up Studies ; Humans ; Lumbar Vertebrae ; Marfan Syndrome ; Osteomyelitis ; Pseudarthrosis ; Scoliosis ; Spine* ; Transplants

STUDY DESIGN: A case-series study. PURPOSE: To assess the long-term clinical and radiographic outcomes after vertebroplasty using calcium phosphate cement (CPC) for treatment of osteoporotic vertebral fractures (OVF). OVERVIEW OF LITERATURE: Vertebroplasty has become common for the treatment of OVF. However, few studies have reported the clinical application of CPC to vertebroplasty. METHODS: We reviewed 86 consecutive patients undergoing 99 vertebroplasties using CPC. Following repositioning and curettage of the pathological soft tissue of the vertebral body (VB), vertebroplasty using CPC was performed in patients with osteoporotic burst fracture and pseudoarthrosis (procedure A). Vertebroplasty was also performed in patients with osteoporotic compression fractures (procedure B). Back pain and lower back pain were evaluated using the visual analogue scale (VAS). The VB deformity index was measured in a lateral radiograph as the ratio of the VB's height to its longitudinal diameter. RESULTS: The mean age at time of surgery was 77 years old. The mean duration of follow-up was forty-four months. All patients reported decreased pain according to the VAS immediately after vertebroplasty, and pain relief was maintained at the last follow-up in all patients without new OVFs. Complete bone union was observed in all cases by six months after surgery. The mean recovery rate of deformity index was 5.9% in procedure A and 0.02% in procedure B at the final follow-up visit. CONCLUSIONS: Vertebroplasty using CPC gave a satisfactory outcome and no delayed complications in elderly patients with osteoporotic vertebral fractures at follow-up times of at least two years.
Aged ; Back Pain ; Calcium ; Calcium Phosphates ; Congenital Abnormalities ; Curettage ; Dinucleoside Phosphates ; Follow-Up Studies ; Fractures, Compression ; Humans ; Low Back Pain ; Osteoporosis ; Pseudarthrosis ; Vertebroplasty

BACKGROUND: Fibrous hamartoma is the key pathology of congenital pseudarthrosis of the tibia (CPT), which was shown to have low osteogenicity and high osteoclastogenicity. This study further investigated the mechanism of impaired osteoblastic differentiation of fibrous hamartoma cells. METHODS: Fibroblast-like cells were obtained from enzymatically dissociated fibrous hamartomas of 11 patients with CPT associated with neurofibromatosis type I (NF1). Periosteal cells were also obtained from the distal tibial periosteum of 3 patients without CPT or NF1 as control. The mRNA levels of Wnt ligands and their canonical receptors, such as Lrp5 and beta-catenin, were assayed using reverse transcriptase PCR (RT-PCR). Changes in mRNA expression of osteoblast marker genes by rhBMP2 treatment were assayed using quantitative real time RT-PCR. Changes in mRNA expression of transcription factors specifically involved in osteoblastic differentiation by rhBMP2 treatment was also assayed using quantitative real-time RT-PCR. RESULTS: Wnt1 and Wnt3a mRNA expression was lower in fibrous hamartoma than in tibial periosteal cells, but their canonical receptors did not show significant difference. Response of osteoblastic marker gene expression to rhBMP2 treatment showed patient-to-patient variability. Col1a1 mRNA expression was up-regulated in most fibrous hamartoma tissues, osteocalcin was up-regulated in a small number of patients, and ALP expression was down-regulated in most fibrous hamartoma tissues. Changes in mRNA expression of the transcription factors in response to rhBMP2 also showed factor-to-factor and patient-to-patient variability. Dlx5 was consistently up-regulated by rhBMP2 treatment in all fibrous hamartoma tissues tested. Msx2 expression was down-regulated by rhBMP2 in most cases but by lesser extent than control tissue. Runx2 expression was up-regulated in 8 out of 18 fibrous hamartoma tissues tested. Osterix expression was up-regulated in 2 and down-regulated in 3 fibrous hamartoma tissues. CONCLUSIONS: Congenital pseudarthrosis of the tibia appears to be caused by fibrous hamartoma originating from aberrant growth of Nf1 haploinsufficient periosteal cells, which failed in terminal osteoblastic differentiation and arrested at a certain stage of this process. This pathomechanism of CPT should be targeted in the development of novel therapeutic biologic intervention.
Adolescent ; *Cell Differentiation ; Cells, Cultured ; Child ; Child, Preschool ; Female ; Hamartoma/complications/*pathology ; Humans ; Infant ; Low Density Lipoprotein Receptor-Related Protein-5/metabolism ; Male ; Neurofibromatosis 1/complications/*pathology ; Osteoblasts/*pathology ; Periosteum/pathology ; Pseudarthrosis/complications/*congenital/pathology/physiopathology ; Receptors, Wnt/metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Tibia/*pathology ; Transcription Factors/metabolism ; Wnt1 Protein/metabolism ; Wnt3A Protein/metabolism ; beta Catenin/metabolism

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.
Humans ; *Ilizarov Technique/adverse effects ; Pseudarthrosis/*congenital/*surgery ; Tibia/*surgery

OBJECTIVE: Iliac screw fixation has been used to prevent premature loosening of sacral fixation and to provide more rigid fixation of the sacropelvic unit. We describe our technique for iliac screw placement and review our experience with this technique. METHODS: Thirteen consecutive patients who underwent spinopelvic fixation using iliac screws were enrolled. The indications for spinopelvic fixation included long segment fusions for spinal deformity and post-operative flat-back syndrome, symptomatic pseudoarthrosis of previous lumbosacral fusions, high-grade lumbosacral spondylolisthesis, lumbosacral tumors, and sacral fractures. Radiographic outcomes were assessed using plain radiographs, and computed tomographic scans. Clinical outcomes were assessed using the Oswestry Disability Index (ODI) and questionnaire about buttock pain. RESULTS: The median follow-up period was 33 months (range, 13-54 months). Radiographic fusion across the lumbosacral junction was obtained in all 13 patients. The average pre- and post-operative ODI scores were 40.0 and 17.5, respectively. The questionnaire for buttock pain revealed the following: 9 patients (69%) perceived improvement; 3 patients (23%) reported no change; and 1 patient (7.6%) had aggravation of pain. Two patients complained of prominence of the iliac hardware. The complications included one violation of the greater sciatic notch and one deep wound infection. CONCLUSION: Iliac screw fixation is a safe and valuable technique that provides added structural support to S1 screws in long-segment spinal fusions. Iliac screw fixation is an extensive surgical procedure with potential complications, but high success rates can be achieved when it is performed systematically and in appropriately selected patients.
Buttocks ; Congenital Abnormalities ; Follow-Up Studies ; Humans ; Pseudarthrosis ; Surveys and Questionnaires ; Spinal Fusion ; Spondylolisthesis ; Wound Infection