Cases reported "Kyphosis"

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1/6. Cord monitoring changes and segmental vessel ligation in the "at risk" cord during anterior spinal deformity surgery.

    STUDY DESIGN: Retrospective analysis of all cases of anterior spinal deformity surgery that had intraoperative spinal cord monitoring (somatosensory-evoked potentials, SSEPs). OBJECTIVES: The prime purpose of this study was to determine the incidence of significant SSEP changes in patients undergoing anterior spinal deformity surgery. A secondary objective was to ascertain if patients with "cords at risk" were more likely to produce significant intraoperative SSEP changes and what proportion of these changes resulted in postoperative neurological deficit. SUMMARY OF BACKGROUND DATA: There is anecdotal evidence to suggest that patients with intraspinal abnormalities are at greater risk of postoperative neurological deficit after spinal deformity surgery. To date, there have been no studies detailing this risk and how it relates to the type of surgery performed. This is a question of increasing relevance with the modern trend towards more anterior scoliosis correction and instrumentation. Recent reports have suggested a low incidence of neurological complication with anterior deformity procedures. There is controversy as to whether SSEP monitoring is required for these anterior procedures and whether soft clamping of segmental vessels before their division is a necessary precaution. METHOD: This study is a chart review of all patients who had an anterior deformity operation between 1990 and 2001. Those patients who had a complete data set (preoperative magnetic resonance imaging scan, patient and procedural documentation, and adequate intraoperative SSEP traces) were included in this study. A significant SSEP change was correlated with the type of procedure performed, whether that patient had a "cord at risk" (CAR) and the degree of postoperative neurological deficit if present. RESULTS: During the study period, 871 patients underwent elective anterior spinal deformity surgery. Ninety five (11%) patients had intraspinal abnormalities on magnetic resonance imaging. From this group, 27 (3%) were termed CAR. Twenty six (3%) patients had significant change in the intraoperative SSEP monitoring. Seventeen (2% total) occurred in the CAR group and nine (1% of total) in the normal cord group. There were five patients (0.6%) with significant postoperative neurological deficits, four (0.5%) in the CAR group, and one (0.1%) in the normal cord group. These patients had also demonstrated changes in their SSEPs. The sensitivity of SSEP monitoring for the whole series was 100%, specificity 97.5%, the positive predictive value was 19% and the negative predictive value was 100%. The CAR group was significantly more likely to have significant SSEP changes during any operation and was more likely to have postoperative paresis. CONCLUSION: patients with identified cords at risk should undergo spinal cord monitoring (SSEP) if they undergo anterior spinal deformity surgery. Soft clamping of segmental vessels is indicated with cord monitoring to prevent the risk of postoperative neurological sequelae.
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2/6. Gowers intrasyringal hemorrhage. Case report and review of the literature.

    The concept of hemorrhage in a preexisting syringomyelic cavity was first described by Gowers in 1904. Since its first description only 13 cases have been reported. The aims of this report are to describe a new case, bring this entity to wider attention, and summarize the existing literature on the subject. This 36-year-old woman presented with progressive gait disturbance and unsteadiness. physical examination revealed incomplete quadriparesis, predominantly on the left side, and hypesthesia below C-7. magnetic resonance imaging revealed hematomyelia characterized by a heterogeneous hyperintense signal within the central cervical cord. A liquefied well-limited hematoma was evacuated. The postoperative course was uneventful; a near-complete recovery was observed at the 7-year follow-up examination. Most cases of intrasyringal hemorrhage (ISH) have occurred in syringomyelic cavities associated with scoliosis or Chiari malformation Type I. Although there is no specific clinical picture associated with this entity, it can be characterized by three neurological forms: 1) sudden onset or rapid development of signs and symptoms, 2) acute worsening of symptoms that may improve but leaving greater neurological dysfunction than before the previous episode, and 3) ISH may initiate progressive deterioration in a patient with known syringomyelia. Intrasyringal bleeding is most probably caused by a sudden dilation of the syringomyelic cavity, which may provoke rupture of the intrasyringal vessels by an acute distension of the accompanying strands. magnetic resonance imaging is the most accurate diagnostic modality, and recognition of ISH can lead to early, safe, and efficient surgical treatment.
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3/6. Disappearing bone disease: a case report and review of the literature.

    Disappearing bone disease is a rare condition manifested by massive osteolysis. Its etiology is unknown. Histologically, involved bones show a nonmalignant proliferation of thin-walled vessels. The mechanism of bone absorption remains unclear. No treatment has proven effective in arresting the disease. Cases in which there is extraosseous involvement have a much poorer prognosis.
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4/6. Anterior exposure of the cervicothoracic spine using a combined cervical and thoracic approach.

    Anterior stabilization of severe structural deformities of the spine, especially kyphoscoliosis, at the cervicothoracic level is a difficult surgical procedure. A new combined procedure including anterolateral cervical and posterior transpleural transthoracic approaches to the cervicothoracic spine provided adequate-exposure from the third cervical to the ninth thoracic vertebra and at the same time gave access to the great vessels in the event that control of these became necessary. The procedure has been employed in six patients with severe cervicothoracic kyphoscoliosis. Despite the magnitude of the combined cervical and transthoracic exposure, the patients withstood the procedure well and there were no complications. The fusion was successful in all six patients. The kyphotic deformities were corrected by an average of 26 degrees and the scoliotic curves, by an average of 17 degrees.
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5/6. Restrictive dermopathy: report and review.

    Restrictive dermopathy (RD) is a lethal autosomal recessive genodermatosis (MIM No. 275210) in which tautness of the skin causes fetal akinesia or hypokinesia deformation sequence (FADS). polyhydramnios with reduced fetal movements is followed by premature delivery at around 31 weeks gestation. Manifestations include a tightly adherent, thin, translucent skin with prominent vessels, typical facial changes, generalized joint contractures, enlarged fontanelles, dysplasia of clavicles, respiratory insufficiency, and an enlarged placenta with short umbilical cord. Histologic abnormalities of the skin include thin dermis with paucity and hypoplasia of the appendages and abnormally arranged collagen bundles. Elastic fibers are nearly missing. The subcutaneous fat is slightly increased. These skin findings usually appear after 22 or 24 weeks of gestation, which is why prenatal diagnosis with skin biopsy may fail. This disease is easily differentiated from other congenital FADS, such as Pena-Shokeir syndrome, COFS syndrome, Parana hard-skin syndrome, etc. We report on an affected boy of consanguineous parents and 30 previous cases are reviewed.
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6/6. Major intraoperative neurologic deficits in pediatric and adult spinal deformity patients. incidence and etiology at one institution.

    STUDY DESIGN: A retrospective study of 1,090 patients undergoing corrective spinal deformity surgery for scoliosis (n = 920), kyphosis (n = 77), or a combination of the two (n = 93) at one institution. OBJECTIVES: To ascertain the etiologies and incidence of neurologic deficits occurring at the time of surgery. SUMMARY OF BACKGROUND DATA: Potential etiologies of intraoperative neurologic deficits include cord compression, overdistraction, purely vascular, or a combination. methods: The study group included only patients with useful function of their lower extremities and normal bowel and bladder control, and patients whose surgeries were in spinal cord territory as opposed to purely cauda equina territory. RESULTS: There were four major neurologic deficits that occurred during surgery. Three of the four deficits were purely vascular in etiology. The fourth may have had a vascular and mechanical etiology. All four patients had anterior and posterior surgery with harvesting of the unilateral convex segmental vessels, and each had a component of hyperkyphosis, as well as intraoperative controlled hypotension. All four patients showed marked improvement of motor weakness with time. CONCLUSIONS: Significant risk factors were combined anterior and posterior surgery (P = 0.009) and hyperkyphosis (P = 0.0006).
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