Cases reported "Kyphosis"

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1/9. Multicore myopathy: respiratory failure and paraspinal muscle contractures are important complications.

    Three ambulant males with multicore myopathy, a rare congenital myopathy, are reported with nocturnal hypoventilation progressing to respiratory failure at the age of 9, 13, and 21 years. Deterioration in these individuals occurred over several months without any precipitating event. patients had clinical evidence of nocturnal hypoventilation with hypoxaemia and hypercapnia. Forced vital capacity was significantly reduced (20 to 43% of predicted level). These parameters improved on institution of overnight ventilation using a BiPAP pressure support ventilator with face mask or nasal pillows with O2 saturation maintained above 90% overnight and an increase in forced vital capacity by as much as 100% (0.3 to 0.6 litres). This was matched by a symptomatic and functional improvement. Also present in these patients and not previously reported is the association of multicore myopathy with paraspinal contractures which produce a characteristic scoliosis described as a 'side-sliding' spine. This may be improved by spinal bracing or surgery.
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ranking = 1
keywords = vital capacity, capacity
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2/9. Managing ventilatory insufficiency and failure in a patient with kyphoscoliosis: a case study.

    This article reports a case study of 'Sue', a 37 year old female who was transferred to a metropolitan hospital's intensive care unit in acute respiratory failure secondary to severe kyphoscoliosis (KS). KS is defined as a deformity of the spine involving both lateral displacement (scoliosis) and anteroposterior angulation (kyphosis). Over time, this anatomical distortion results in ventilatory insufficiency due to muscle weakness. Sue displayed a restrictive lung pattern, evidenced by a decreased vital capacity and tidal volume with severe nocturnal dyspnoea, resulting in raised carbon dioxide levels in arterial blood and decreased oxygenation. This paper reviews Sue's progress throughout her hospitalisation and examines the key issues involved in her care. Particular attention is given to specific problems encountered on the acute care ward related to oxygen delivery, tracheostomy care, non-invasive ventilation and rehabilitation. The paper highlights the increased acuity of respiratory ward patients who require the use of substantial technological support to optimise their management. nurses working in these wards need specialised knowledge, excellent patient communication ability and well-developed technical skills. The trend is to treat patients with respiratory failure, either chronic or acute, on wards rather than in critical care units' which has promoted the development of a specialised role in respiratory nursing.
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ranking = 0.50000461616879
keywords = vital capacity, capacity, volume
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3/9. Management of respiratory deterioration in a pregnant patient with severe kyphoscoliosis by non-invasive positive pressure ventilation.

    The problem of kyphoscoliosis in combination with pregnancy is uncommon and published cases are rare. Until now, little and controversial information on the outcome, optimal management and course of pregnancy in patients with kyphoscoliosis has been available. The majority of maternal deaths seem to be attributed to cardiorespiratory failure, while obstetric complications account for relatively few complications. We present the case of a 34-year old pregnant woman with congenital kyphoscoliosis and a forced vital capacity (FVC) of about one liter. A further deterioration of lung function was expected. In fact, severe limitations in exercise capacity (bed rest), fatigue and hypersomnolence, as well as a severe increase in pulmonary hypertension occurred during the second and third trimester. Nasal intermittent positive pressure ventilation (NIP-PV) with bilevel positive airway pressure (BiPAP) was started in the 20th week of gestation and adapted throughout pregnancy. Nasal BiPAP was well-tolerated and corrected exercise tolerance, fatigue and nocturnal oxygen desaturations. At 32 weeks of gestation, the patient was admitted for an elective Caesarean section under combined spinal-epidural anaesthesia with ongoing NIPPV, and delivered a healthy baby. Home nocturnal ventilatory support was continued as nocturnal episodic desaturations were also assessed during the postpartum period. At time of discharge, the patient's exercise capacity and lung function were nearly equal to levels before pregnancy. We conclude that pregnancy in selected kyphoscoliotic patients with severe limitations in lung function is relatively safe for both the mother and the child when NIPPV is used for overcoming respiratory deterioration and for preventing further cardiorespiratory failure.
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ranking = 0.50561772772555
keywords = vital capacity, capacity
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4/9. All pedicle screw fixation technique in correcting severe kyphoscoliosis in an osteogenesis imperfecta patient: a case report.

    Spinal deformity in patients with osteogenesis imperfecta demonstrates a high prevalence. The surgical treatment of this problem had numerous difficulties, which included breakage of bone, dislodgement of implants, and late loss of correction. We reported the correction of severe kyphoscoliosis in a patient with osteogenesis imperfecta using 3-rod all pedicle screw fixation technique. In this case, the 2 main goals of spinal operation, stabilization and correction of spinal curvatures, were achieved. The Cobb angles of scoliosis and thoracic kyphosis were corrected from 110 degrees to 68 degrees and from 107 degrees to 39 degrees, respectively. One and a half years after the operation, spinal radiographs showed no loss of correction, either on coronal or sagittal planes. The predicted forced vital capacity, predicted forced expiratory volume in 1 second and vital capacity of the lung of the patient had improved 2-fold. The usage of pedicle screw, with its conical core and cylindrical thread design, and 3-rod technique in fixation, together with cyclic intravenous bisphosphonate administration and halo-gravity traction preoperatively, contributed to the success in this case.
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ranking = 1.0000046161688
keywords = vital capacity, capacity, volume
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5/9. Dysphagia as a manifestation of occult hypoxemia. The role of oximetry during meal times.

    A 25-year-old woman with severe kyphoscoliosis reported a six-month history of increasing dysphagia to both liquids and solids. A barium swallow and esophageal motility studies showed no significant gastrointestinal abnormalities. Trials of antispasmodic agents were unsuccessful in relieving her symptoms. Pulmonary function tests showed a severe restrictive ventilatory defect (vital capacity = 0.67 L) with adequate oxygenation and alveolar ventilation as reflected by arterial blood gas testing during quiet wakefulness. However, continuous noninvasive oximetry demonstrated desaturation to 85 percent while eating. These transient episodes of desaturation were abolished by the administration of supplemental oxygen delivered by nasal prongs. Following the prescription of supplemental oxygen, dysphagia resolved immediately, with weight gain following over several weeks. We conclude that dysphagia may be a presenting feature of hypoxemia. This case report draws attention to the usefulness of continuous noninvasive monitoring of oxygenation and the clinical importance of at least some transient hypoxemic events.
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ranking = 0.5
keywords = vital capacity, capacity
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6/9. Cervico-oculo-acousticus syndrome with pregnancy.

    Cervico-oculo-acousticus syndrome is a very rare entity with only 21 cases described in the world literature. A study of a case of this syndrome with pregnancy is presented. This primigravida, in addition to the classical triad of the syndrome, had other dysmorphic features such as short stature (141 cm), and marked thoracic kyphosis with restricted ventilatory capacity. Her physical activity during pregnancy was guided according to her respiratory status. In spite of marked thoracic kyphosis the cephalo-pelvic relationship was found to be adequate for a vaginal delivery. A healthy male baby weighing 3.8 kg was delivered by an outlet forceps at term. Puerperium was uneventful. The child was clinically normal at the age of 2 years.
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ranking = 0.0028088638627768
keywords = capacity
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7/9. Compressive myelopathy associated with type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome).

    spinal cord compression with resultant myelopathy is a frequent occurrence in patients with mucopolysaccharidoses. Etiological factors include developmental abnormalities of the cervical spine and infiltration of the dura mater by the accumulated products of mucopolyssacharide metabolism. Compression at the thoracolumbar junction is rare, but was found in a child with the characteristic physical and biochemical stigmata of the Maroteaux-Lamy syndrome (mucopolysaccharidosis vi). An anterolateral approach to remove the compressing bony elements resulted in symptomatic improvement. Careful radiological evaluation is required so that all surgical options can be considered. patients with metabolic storage diseases and the capacity for normal intellectual function warrant aggressive surgical care to optimize neurological function.
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ranking = 0.0028088638627768
keywords = capacity
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8/9. Kyphoscoliosis and bronchial torsion.

    STUDY OBJECTIVE: Severe idiopathic scoliosis is associated with respiratory failure. This usually is secondary to restrictive airway disease and reduced vital capacity. patients may also suffer from an increase in airway resistance when severe kyphoscoliosis is present. SETTING: Three patients (two of whom required assisted ventilation) with varying degrees of kyphoscoliosis presented with moderate to severe breathing difficulties. INTERVENTION: Bronchoscopic examination of these patients showed evidence of torsion with secondary obstruction of the central airways. RESULTS: The airway obstruction was notable for its slit-like appearance, for the normality of the mucosa at the site of the obstruction, for the relative ease through which an instrument could traverse the obstruction, and once the retained secretions had been cleared, for the preservation of normal anatomy of the distal airways. The insertion of metal prostheses to stent the areas of obstruction prompted an impressive improvement in respiratory status, radiologic findings, and spirometric criteria in each case. Improvement has been maintained over a maximum follow-up period of 4 years. CONCLUSION: Severe kyphoscoliosis can lead to bronchial torsion and obstruction of the central airways. patients should be assessed by bronchoscopy to exclude this deformity or any other cause of obstruction. The use of a metal endobronchial stent has been effective in both the immediate and long-term period.
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ranking = 0.5
keywords = vital capacity, capacity
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9/9. Single-lung transplantation in a patient with cystic fibrosis and an asymmetric thorax.

    We report metachronous single-lung transplantation for cystic fibrosis after contralateral pneumonectomy. Kyphoscoliosis and mediastinal shift required careful donor-lung sizing with computed tomography and was not dependent on typical parameters. Severe reperfusion injury was treated with nitric oxide, C1-esterase inhibitor, and continuous venovenous hemodialysis. The patient was extubated on the fifth postoperative day and is alive and well. We conclude that single-lung transplantation after contralateral pneumonectomy for patients with cystic fibrosis and an asymmetric chest and evident lung volume mismatch may be an acceptable functional therapeutic option.
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ranking = 4.6161687921115E-6
keywords = volume
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