Cases reported "Scoliosis"

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1/37. Surgical treatment of poliomyelitic scoliosis.

    Between 1968 and 1973 forty nine patients suffering from poliomyelitic scoliosis were treated surgically at the Rizzoli Institute. They were due to asymmetrical paralysis and contracture in the muscles of the trunk and limbs. Associated pathological conditions were found, such as pelvic obliquity, and vascular and trophic changes due to ganglionic lesions. The differing incidence and combination of these factors gave rise to various clinical types of spinal deformity. The average severity of curve was 39 degrees, the localisation was predominantly central, the average extent was ten vertebrae, and there was a marked predominance of right convexity (twenty nine out of thirty six). The rate of progression was maximum during puberty and almost negligible after bony maturity. It was greater in males and was unfavourably affected by the severity and asymmetrical distribution of the paralysis, by the early appearance of the disease, by high localisation of the deformity, and by the erect posture in patients who were ambulant. The most frequent visceral complications were in the respiratory system (ten patients with a deficit over 50%), followed by cardiac changes. Surgical treatment was adopted in patients with progressive curves over 60 degrees, because of the inevitable deterioration in their general condition and the tendency of the deformity to become fixed. Pre-operative correction by Halo-traction results (52% correction) than Risser plasters (38%). Posterior arthrodesis by Harrington's method was carried out in all the more recent cases (forty four). Post-operative plaster was maintained for eight months and then replaced by an orthopaedic corset. At bony maturity there was an averaged improvement of 35% in the angle of curvature, and an average improvement of 6% in vital capacity. The best corrections were obtained in patients under fourteen (42%), in dorso-lumbar scoliosis (40%) and in patients with curves above 100 degrees (38%). There was an average increase in height of 9.1 cms and a reduction in the gibbus of 3.4 cms. The complications included one traumatic pneumothorax, eight pseudarthroses, and breakage of the distraction rod in two cases resulting in complete relapse of the deformity. In six cases the upper hooks became loos and there were two cases of postoperative staphylococcal infection. In the distally sited curves our present policy is towards combining posterior arthrodesis with Dwyer's anterior interbody fusion.
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2/37. Anaesthetic implications of rigid spine syndrome.

    The perioperative management of a 14-year-old girl, suffering from the muscular disorder rigid spine syndrome, is presented. The anaesthetic implications with regard to possible difficult intubation, cardiac involvement, malignant hyperthermia, neuromuscular blocking agents, and postoperative recovery are discussed.
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3/37. scoliosis in proteus syndrome: case report.

    STUDY DESIGN: The case of patient with scoliosis based on a rare hamartomatous, proteus syndrome, is reported. OBJECTIVES: To present the characteristics of scoliosis associated with proteus syndrome, and to investigate the mechanisms that cause it. SUMMARY OF BACKGROUND DATA: proteus syndrome, a rare hamartomatous disorder first coined by Wiedemann, manifests many clinical morphologic abnormalities including scoliosis. The characteristics and cause of scoliosis in this syndrome are fully unknown. methods: A patient with proteus syndrome was followed from the age of 3 months to the age of 21 years. This patient received spinal corrective surgery for severe scoliosis. Detailed investigations of the scoliosis as well as the physical and imaging examinations were performed to characterize the scoliosis. RESULTS: Computed tomography showed exclusive asymmetric appearance of lumbar spine, hypertrophy of the only right facet joints, and pedicles at L1-L4, which accorded with the right-side hemihypertrophy of the patient's extremities. CONCLUSIONS: scoliosis with proteus syndrome seems to be based on hemihypertrophy, with no influence of mechanical stress.
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4/37. spinal cord injury in children.

    The spinal injured child has speical needs owing to the processes of physical, mental and social growth. goals of physical treatment programs include prevention of: genitourinary complications; contractures; pressure sores; long bone fractures, hip subluxation and dislocation; spinal deformity. Nonoperative treatment of spinal deformity employing external support should be initiated when the potential for spinal deformity exists. External support delays the development of spinal deformity, improves sitting balance and allows free upper extremity use. The overall treatment programs must consider altered body proportions, immaturity of strength and coordination. Case examples of children with spinal injury are presented above to illustrate specific problems stemming from immaturity of physical, cognitive, and social development. Spinal surgery can be a conservative measure in the growing child when there is radiologic evidence of progressive spinal deformity. Posterior spinal fusion with Harrington instrumentation and external support permits immediate return to vertical activity.
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5/37. chiropractic and pilates therapy for the treatment of adult scoliosis.

    OBJECTIVE: To describe the use of Pilates therapy and sacro-occipital technique in the management of a 39-year-old woman with scoliosis who had undergone spinal fusion many years earlier. Clinical Features: The patient had progressive severe low back pain that had worsened over the years after her surgery and had prevented her from activities such as carrying her son or equipment necessary for her job as a photographer. Intervention and Outcome: The patient was provided a series of Pilates exercises used to overcome her chronic habituation and muscle weakness. Although this therapy went on for some time, she did begin to stabilize and increase physical activity. At present, she is no longer limited in her physical activity, although she still exhibits some symptoms from her scoliosis. CONCLUSION: The addition of Pilates therapy can be useful to care for patients with chronic low back pain and deconditioning.
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6/37. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years.

    INTRODUCTION: The entity of hindbrain herniation without myelodysplasia in the very young child has been poorly described. A retrospective analysis of children diagnosed with Chiari I malformation (CM I) before their sixth birthday is presented. methods: Since 1985, 31 children with CM I (0.3-5.8) years of age have been diagnosed at University of iowa hospitals and Clinics. Their records were reviewed for presenting symptoms, signs, radiographic findings, treatment, complications, and outcome. RESULTS: The average age at diagnosis was 3.3 years. Sixteen patients were under age 3. Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache or neck pain (23%), sensory disturbance (6%), weakness (3%), and other (10%). Sixty-nine percent of children under age 3 had abnormal oropharyngeal function. Three patients under age 3 (19%) had undergone fundoplication and/or gastrostomy before diagnosis of CM I. Common physical findings included abnormal tendon reflexes (68%), scoliosis (26%), abnormal gag reflex (13%), and normal examination (13%). vocal cord dysfunction (26%, all under age 3) and syringohydromyelia (52%) were also seen. Twenty-five patients were treated surgically at our institution with posterior fossa decompression, duraplasty, and cerebellar tonsillar shrinkage. Three patients were lost to follow-up. Ninety-one percent of patients reported improved symptomatology at last follow-up (mean: 3.9 years). Three patients required reoperation for recurrence of symptoms. syringomyelia improved in all patients. scoliosis resolved in 2 of 8 patients, improved in 5, and stabilized in 1. There was no permanent morbidity from surgery. DISCUSSION: We show that children with Chiari I abnormality are very likely to present with oropharyngeal dysfunction if under age 3, and either scoliosis or headache or neck pain worsened by valsalva if age 3 to 5. These symptoms are very likely to improve after Chiari decompression, which can be done with low morbidity. CONCLUSIONS: Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause.
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7/37. Hemimetameric segmental shift: a case series and review.

    STUDY DESIGN: A case series of patients with hemimetameric shift. OBJECTIVES: To describe the radiographic and physical findings as well as treatment provided to a subset of patients with congenital scoliosis. SUMMARY OF BACKGROUND DATA: Hemimetameric segmental displacement, or hemimetameric shift, is a clinical entity defined by two contralateral hemivertebrae separated by at least one normal vertebra. Although the entity is briefly described in textbooks, there are no published series to date. methods: From 1974 to 2000, 186 cases of congenital scoliosis were identified from two referral centers. Of these, 27 cases (15%) of hemimetameric shift were identified. medical records and radiographs were reviewed, as well as magnetic resonance imaging when available. RESULTS: There were an average of 2.9 hemivertebrae per patient (range 2-6) with the following distribution: T1-T6 (29), T7-T11 (28), T12-L1 (10), L2-L4 (11), and L5 (1). The average curve magnitude at presentation was 28 degrees (range 9 degrees -55 degrees). Nine patients required surgery-most commonly with involvement of the thoracolumbar or lumbosacral junction. Eleven patients also had associated anomalies to include klippel-feil syndrome (3), goldenhar syndrome (2), imperforate anus (2), tracheoesophageal fistula (2), and a single kidney (2). There was only one patient who had an abnormal magnetic resonance imaging (1 out of 17; 6%). CONCLUSIONS: Hemimetameric shift is a common finding in congenital scoliosis. Hemivertebrae are most commonly found in the thoracic spine; however, surgical intervention is most commonly observed when the caudal hemivertebrae is located from the thoracolumbar to lumbosacral junction. The incidence of abnormal magnetic resonance imaging findings is low (6%).
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8/37. Acquired thoracic scoliosis following minimally invasive repair of pectus excavatum.

    The minimally invasive pectus excavatum repair as described by Nuss et al. is rapidly gaining acceptance as an effective method of repair of severe pectus excavatum deformities in the pediatric population. It potentially offers several advantages over previous techniques. The incidence of major complications of the procedure has been reduced by recent modifications including utilization of video-assisted thoracoscopy during placement of the Lorenz pectus bar as well as utilizing the pectus bar stabilizer that provides more rigid fixation of the strut. We report two cases of acquired thoracic scoliosis following minimally invasive repair of severe pectus excavatum deformity. This particular complication has not been reported in previous literature and warrants concern. In both cases the thoracic scoliosis slowly improved with physical therapy and range-of-motion exercises.
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9/37. chiropractic management of ehlers-danlos syndrome: a report of two cases.

    OBJECTIVE: To discuss 2 patients with ehlers-danlos syndrome seeking chiropractic evaluation and management of their disabling musculoskeletal pain and associated disorders. CLINICAL FEATURES: Two disabled patients diagnosed with Ehlers-Danlos syndrome had spinal pain, including neck and back pain, headache, and extremity pain. Commonalities among these 2 cases included abnormal spinal curvatures (kyphosis and scoliosis), joint hypermobility, and tissue fragility. One patient had postsurgical thoracolumbar spinal fusion (T11-sacrum) for scoliosis and osteoporosis. The other patient had moderate anterior head translation. INTERVENTION AND OUTCOME: Both patients were treated with mechanical force and manually assisted spinal adjustments delivered to various spinal segments and extremities utilizing an Activator II Adjusting Instrument and Activator methods chiropractic Technique. patients were also given postural advice, stabilization exercises, and postural corrective exercises, as indicated in chiropractic biophysics Technique protocols. Both patients were able to reduce pain and anti-inflammatory medication usage in association with chiropractic care. Significant improvement in self-reported pain and disability as measured by visual analog score, Oswestry Low-Back Disability Index, and neck pain Disability Index were reported, and objective improvements in physical examination and spinal alignment were also observed following chiropractic care. Despite these improvements, work disability status remained unchanged in both patients. CONCLUSION: chiropractic care may be of benefit to some patients with connective tissue disorders, including ehlers-danlos syndrome. Low-force chiropractic adjusting techniques may be a preferred technique of choice in patients with tissue fragility, offering clinicians a viable alternative to traditional chiropractic care in attempting to minimize risks and/or side effects associated with spinal manipulation. Psychosocial issues, including patient desire to return to work, were important factors in work disability status and perceived outcome.
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10/37. anesthesia for corrective spinal surgery in a patient with Leigh's disease.

    We report a case of anesthesia for posterior spinal fusion in a woman with Leigh's disease. This is a syndrome with a heterogeneous phenotype including ocular signs, motor signs, and respiratory disorder. It is associated with defects in the enzymes of the mitochondrial respiratory chain and central neural degeneration. anesthesia is associated with worsening of the respiratory symptoms. Our patient underwent major spinal surgery as a palliative procedure. Her postoperative course was complicated by acute lung injury and sepsis. She ultimately failed a prolonged respiratory wean. Serial magnetic resonance imaging revealed a rapidly progressive necrosis of her brain stem and cervical spinal cord consistent with activation of her underlying Leigh's disease. This is the first report of spinal surgery in this patient group. It is also the first radiological demonstration of Leigh's disease reactivation in the postoperative period. anesthesia and surgery are hazardous in this patient population, and respiratory symptoms make this a high-risk group. Surgery should only be undertaken with caution and after frank consent. Early postoperative imaging is recommended if there are respiratory complications. No drug prophylaxis has been shown to alter disease activation. IMPLICATIONS: patients suffering from Leigh's disease are at high risk of serious postoperative respiratory morbidity. We present a case that demonstrates delayed respiratory complications and link this postoperative adverse outcome to aggressive reactivation of the underlying neurodegenerative condition.
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