1/134. Severe progressive osteoporotic spine deformity with cardiopulmonary impairment in a young patient. A case report.STUDY DESIGN: This report describes a young patient with a rapidly progressive kyphosis caused by collapse of a severely osteoporotic thoracolumbar spine, which led to impairment of cardiopulmonary function. OBJECTIVES: To highlight the treatment strategy, difficulty of diagnosis, operative stabilization, and outcome. SUMMARY OF BACKGROUND DATE: Little is known about natural history, treatment options, and results of this condition. methods: The magnitude of bone loss was measured by dual-energy x-ray absorptiometry, and the deformity was visualized by computed tomography and magnetic resonance imaging. Laboratory investigations also were performed before and during halotraction in an attempt to establish a diagnosis. These data constituted the preoperation information required to assess later results of medical and surgical intervention. RESULTS: An extensive evaluation of possible underlying etiologies failed to identify a specific etiology. Before and during halotraction, bone mineral substitutes were given, partially correcting the bone mineral content as measured on repeated dual-energy x-ray absorptiometry scans. In addition, the thoracic kyphosis was partially corrected, from 100 degrees to 70 degrees Cobb's angle. Subsequently, a combined anterior and posterior stabilization was performed from C7 to S1 using a vascularized fibula graft, a double Isola rod system (AcroMed, Cleveland, OH), and a carbonate apatite cancellous bone cement to reinforce the pedicle screws. At follow-up assessment 40 months surgery, the patient was asymptomatic and fully mobilized, with radiographs showing complete incorporation of the grafts and no loosening of the fixation device. CONCLUSIONS: The diagnostic and therapeutic difficulties of progressive spine deformity caused by severe osteoporosis in young patients emphasizes the importance of a thoroughly planned treatment strategy. Halotraction is recommended to stop progression of the deformity, or even partially correct it, and to allow time to search for the diagnosis and bone mineral substitution. Surgical treatment using vascularized fibular strut grafts and a strong fixation device was successful. Biocompatible carbonated apatite cancellous bone cement was successfully used to reinforce pedicle screw fixation.- - - - - - - - - - ranking = 1keywords = deformity (Clic here for more details about this article) |
2/134. The importance of an intact abdominal musculature mechanism in maintaining spinal sagittal balance. Case illustration in prune-belly syndrome.STUDY DESIGN: A rare case of thoracic hypokyphotic deformity secondary to prune-belly syndrome is presented. OBJECTIVES: To discuss the role of an intact abdominal musculature mechanism in maintaining spinal sagittal balance, and to present a case illustration of prune-belly syndrome. SUMMARY OF BACKGROUND DATA: There has been an ongoing debate concerning the integrity of the abdominal musculature unit in maintaining spinal support and stability. It is now believed that intra-abdominal pressure hitherto generated plays an important role in the stabilization of the spine. Congenital aplasia of the abdominal musculature, termed prune-belly syndrome, might therefore result in the loss of spinal function and stability. The literature also is reviewed for the incidence of spinal deformities related to this condition. methods: A unique case of prune-belly syndrome in a 33-year-old man with congenital aplasia of the abdominal musculature is presented. RESULTS: The patient exhibited loss of the spinal sagittal balance, with resultant development of a thoracic hypokyphotic deformity and thoracolumbar scoliosis. CONCLUSIONS: scoliosis appears to be the most commonly reported spinal deformity. Unequal compressive forces on the vertebral endplates may be the proposed mechanism for the spinal deformities. Compensatory lumbar paraspinal overactivity resulting from the inability to generate normal intra-abdominal pressures because of a deficient abdominal wall musculature mechanism seems to be the plausible explanation for the observed thoracic hypokyphatic deformity.- - - - - - - - - - ranking = 0.57142857142857keywords = deformity (Clic here for more details about this article) |
3/134. Cor pulmonale presenting in a patient with congenital kyphoscoliosis following intercontinental air travel.We present the case of a 59-year-old man with congenital kyphoscoliosis who developed cor pulmonale for the first time following intercontinental air travel. Prolonged exposure to the low partial pressure of oxygen in the cabin of the aircraft led to pulmonary hypertension and right heart failure. The case highlights the potential for long-haul air travel to cause decompensation in patients with thoracic deformity and apparently stable cardiorespiratory function. It also emphasises the need for patients and their medical attendants to carefully consider the potential health implications of the hypoxic atmosphere in pressurised aircraft.- - - - - - - - - - ranking = 0.14285714285714keywords = deformity (Clic here for more details about this article) |
4/134. Multilevel thoracic pedicle aplasia causing congenital thoracic kyphosis: case report.OBJECTIVE AND IMPORTANCE: Congenital thoracic kyphosis is a rare cause of treatable myelopathy. Multilevel thoracic pedicle aplasia as a cause of this deformity has not been previously reported in the literature. We report a case and describe the surgical management and outcome. CLINICAL PRESENTATION: A 14-year-old boy presented to us with a 4-month history of back pain and slowly progressive spastic paraparesis. Radiographic studies revealed thoracic kyphosis and bilateral aplasia of the pedicles of T4-T8. INTERVENTION: The patient underwent surgical treatment via a posterior approach for decompression of T4-T8, followed by arthrodesis from T2 to T12, using a hook claw construct with multiple points of fixation and autologous bone grafting. CONCLUSION: Congenital vertebral anomalies may be clinically occult, and delayed presentation may occur in adolescence or adulthood. Aplasia of multiple thoracic pedicles can produce kyphotic deformities with neurological compromise. A posterior approach with multiple points of segmental instrumentation can be effective in treating kyphotic deformities that are flexible and of moderate severity (<75 degrees).- - - - - - - - - - ranking = 0.14285714285714keywords = deformity (Clic here for more details about this article) |
5/134. Progressive rotational dislocation in kyphoscoliotic deformities: presentation and treatment.STUDY DESIGN: Progressive rotational dislocation of the spine has been described as the most serious evolutive risk of kyphoscoliosis. A retrospective chart review was conducted on 11 patients with this deformity. OBJECTIVES: To delineate the clinical and radiologic characteristics of this entity to facilitate early diagnosis and treatment. The outcome after treatment was analyzed to point out the rationale for appropriate treatment. methods: The characteristic radiologic feature was a short sharp angled kyphosis (average 112) at the junction of two lordoscoliotic curvatures. The etiology of the spinal deformity was neurofibromatosis in four patients and various dysplastic conditions in seven patients. Two patients had congenital vertebral defects. Structural weakness of the bone was therefore a basic feature. Neurologic impairment was identified in three patients (one complete, two incomplete). Four patients had a nonunion after a previous attempt at spinal fusion: two after a combined anterior and posterior fusion with an anterior approach from the convexity and two after a posterior fusion alone. All patients underwent complete circumferential stabilization through anterior strut-grafting and posterior fusion. An anterior approach from the concavity was performed systematically with tibial strut grafts inserted in a palisade fashion. Preoperative correction of the deformity was performed by progressive controlled elongation in a Stagnara elongation cast. Cotrel-Dubousset instrumentation was used in two patients, Harrington instrumentation was used in two patients, and cast immobilization alone was used in seven patients. RESULTS: The average follow-up period was 5 years 5 months. All but one patient achieved successful spinal fusion. Loss of correction at the latest follow-up evaluation was less than 3 degrees in nine patients. The two patients with incomplete neurologic deficits were improved, but the patient with the complete deficit remained unchanged after surgery. CONCLUSIONS: awareness of the possibility of a progressive rotational dislocation in dystrophic forms of kyphoscoliosis should allow for an early diagnosis and stabilization. The percentage of patients having a neurologic deficit in this series was significantly less important than in the initial report. Early anterior strut grafting from the concavity of the scoliotic curvature and posterior fusion is recommended.- - - - - - - - - - ranking = 0.42857142857143keywords = deformity (Clic here for more details about this article) |
6/134. Delayed paraplegia caused by the gradual collapse of an infected vertebra.A case of delayed paraplegia caused by a gradual and progressive collapse of a vertebra after healing of pyogenic spondylitis is reported. A 73-year-old man was treated for a hematogenously seeded pyogenic spondylitis of the first lumbar vertebra. magnetic resonance imaging showed a high signal intensity of the involved vertebra and adjacent discs and a paravertebral abscess without disc space narrowing. Eight months after healing of the infection, the patient had muscle weakness and paresthesia of the lower extremities, which gradually increased. The plane radiographs revealed a kyphotic deformity of 36 degrees with a collapse of the first lumbar vertebra.- - - - - - - - - - ranking = 0.14285714285714keywords = deformity (Clic here for more details about this article) |
7/134. Surgical management of spinal involvement in children and adolescents with Gaucher's disease.Gaucher's disease is an uncommon hereditary glycolipid storage disorder characterized by the accumulation of glucocerebroside in the lysosomes of macrophages of the reticuloendothelial system. Skeletal manifestations are variable in severity and typically involve the long bones. Vertebral involvement is less well characterized, particularly in children and adolescents. We report on the surgical management of spinal involvement in four children and adolescents with Gaucher's disease; two for kyphotic deformity and two for kyphotic deformity associated with neurologic compromise. We recommend anterior spinal release with fusion and posterior spinal fusion with segmental instrumentation in cases of kyphotic deformity. In cases of spinal cord compromise at the apex of the kyphotic deformity with retropulsion of involved bone, anterior decompression also should be performed. Routine surveillance for spinal deformity in patients with Gaucher's disease is necessary to allow early intervention before the development of severe deformity and neurologic compromise.- - - - - - - - - - ranking = 0.85714285714286keywords = deformity (Clic here for more details about this article) |
8/134. Evaluating congenital spine deformities for intraspinal anomalies with magnetic resonance imaging.SUMMARY: The incidence of intraspinal abnormalities associated with congenital spinal anomalies as detected by magnetic resonance imaging (MRI) is becoming better defined. In this study, 41 nonrandomized children with congenital spinal deformities (excluding myelomeningocele) who underwent complete MR evaluation were reviewed. Of the 41 congenital spinal deformities, 37 demonstrated congenital scoliosis, with failure of formation in 19, failure of segmentation in 4, and mixed defects in 14. The remaining four deformities were cases of congenital kyphosis. Thirteen patients with congenital spine anomalies were noted to have intraspinal abnormalities identified by MRI: tethered cord in 12 patients, syringomyelia in 3 patients, and diastematomyelia in 5 patients. Of the 12 patients with tethered cord, 2 patients had neurologic deficits. Urorectal anomaly was one of the most common associated findings (15%). Considering an incidence of intraspinal anomalies of 31% and as clinical manifestations may not be initially detectable, MRI is recommended in patients with congenital spinal deformity as part of the initial evaluation even in the absence of clinical findings.- - - - - - - - - - ranking = 0.14285714285714keywords = deformity (Clic here for more details about this article) |
9/134. The association of neurofibromatosis 1 and spinal deformity with primary hyperparathyroidism and osteomalacia: might melatonin have a role?A 35-year-old woman with neurofibromatosis 1 and thoracic kyphoscoliosis had incomplete paraplegia. She had a history of hyperparathyroidism due to a parathyroid adenoma which had been excised 4 years previously. Plain radiographs of the spine revealed kyphoscoliosis from the third to sixth thoracic vertebrae. kyphosis and scoliosis angles were 86 degrees and 28 degrees, respectively. Radiographs of the skull and hands showed radiological changes suggestive of hyperparathyroidism. Laboratory tests showed low-normal serum calcium, hypophosphatemia, elevated serum alkaline phosphatase, and low serum 25-hydroxyvitamin D. Retrospective review of the patient's laboratory data showed that she had osteomalacia at the time of diagnosis of primary hyperparathyroidism. The patient had been treated by anterior and posterior decompression and fusion with posterior instrumentation through a single posterior approach. The postoperative kyphosis and scoliosis angles were 30 degrees and 12 degrees, respectively. Neurological recovery and spinal fusion had been achieved. osteomalacia responded well to vitamin d therapy. This is the first case of coexisting neurofibromatosis 1, primary hyperparathyroidism due to parathyroid adenoma and osteomalacia to be reported in the literature. The osteomalacia in this patient could be related to primary hyperparathyroidism, and not to neurofibromatosis 1. A drop in melatonin level after parathyroidectomy may have been the cause of spinal curvature progression in this patient.- - - - - - - - - - ranking = 0.57143248501926keywords = deformity, hand (Clic here for more details about this article) |
10/134. Spinal lordosis with marked opisthotonus secondary to dystonia musculorum deformans: case report with surgical management.STUDY DESIGN: A case report of severe spinal lordosis with marked opisthotonus and retrocollis secondary to dystonia musculorum deformans is presented. OBJECTIVE: To describe a case of dystonia musculorum deformans with progressive spinal lordosis and its surgical treatment. SUMMARY OF BACKGROUND DATA: Four patients with correction of coronal spinal deformity associated with dystonia musculorum deformans have been reported in the literature. No reports of sagittal spinal deformity treated with surgical instrumentation and fusion were found. methods: A retrospective chart and radiographic review of a single case was conducted. RESULTS: Orthotic management and pharmacologic therapy with botulinum toxin injections were unsuccessful in controlling the deformity. Severe spinal lordosis (170 degrees ) from occiput to sacrum was corrected surgically, allowing an upright posture. CONCLUSION: dystonia musculorum deformans is a rare condition resulting in coronal or sagittal plane deformities. When other treatment methods are unsuccessful, surgical instrumentation and arthrodesis may correct the deformity and improve function.- - - - - - - - - - ranking = 0.57142857142857keywords = deformity (Clic here for more details about this article) |
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