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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scoliosis can lead to respiratory failure and premature death. Alveolar hypoventilation is a dominant cause and artificial ventilation at home (AVH) is probably the treatment of choice. It has been suggested that long-term domiciliary oxygen therapy (LTO) is of little value because of the worsening of hypercapnia. We analyzed survival and predictors of death among 80 patients with scoliosis and other severe thoracic spine deformities receiving LTO for chronic hypoxia. The survival rate was higher in patients under the age of 65 (p = 0.01) and in patients without concomitant pulmonary or airways disease. Likewise, the survival rate was higher in patients with a PaCO2 of greater than 7.4 kPa than in patients with a lesser degree of hypoventilation and hypercapnia (p less than 0.05). The risk of developing life-threatening hypercapnia during well-controlled LTO appeared to be small. In younger patients without complicating disease, long-term survival was achieved with LTO, but with time, an increasing proportion of the patients changed to AVH, with or without LTO.
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PMID:Survival of patients with severe thoracic spine deformities receiving domiciliary oxygen therapy. 162 46

Respiratory function was assessed in 20 patients with idiopathic scoliosis undergoing spinal surgery (median age, 15 years; range, 11-34 years; median preoperative vital capacity, 67%; range, 28-109% predicted). Ten patients underwent anterior spinal surgery through a thoracotomy incision, seven of whom had posterior spinal surgery as a second-stage procedure. The other ten had posterior spinal surgery as their initial operation. Postoperatively, three patients had clinical evidence of respiratory complications. Daytime oxygen saturation was reduced throughout the first postoperative week, with no significant difference between anterior and posterior spinal surgery. Hypercapnia was unusual and generally mild. Vital capacity was reduced significantly 1 week after both anterior and posterior spinal surgery (P less than 0.05). The median vital capacity 1 week after anterior spinal surgery was 45% of preoperative values compared with 78% after posterior surgery (P less than 0.05). Inspiratory muscle strength, as assessed by sniff mouth pressure, was 56% of preoperative values 1 week after anterior spinal surgery (P less than 0.05) and 85% after posterior spinal surgery (not significant). Vital capacity, but not sniff mouth pressure, remained significantly reduced 6 weeks after surgery. Oxygen saturation should be monitored noninvasively during the first week after both anterior and posterior spinal surgery even in patients at low risk of developing respiratory complications.
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PMID:Pulmonary function after spinal surgery for idiopathic scoliosis. 162 5

Severe idiopathic scoliosis may lead to respiratory failure, which can be treated by assisted ventilation. Twenty four patients with surgically untreated idiopathic scoliosis who had been examined in 1968 were re-examined in 1988 to assess changes in lung function and risk factors for respiratory failure. The patients were aged 15-67 years in 1968 and had a scoliotic angle of 10-190 degrees and a vital capacity of 1.0-6.0 litres. Spirometric values and scoliotic angles were determined in 1968 and 1988, and arterial blood gas tensions in 1988. The decline in spirometric values over the 20 years was of the same magnitude as the predicted decline due to aging. Arterial blood gas tensions in 1988 were strongly correlated with the scoliotic angles and spirometric indices recorded in 1968. Hypoxaemia and hypercapnia was seen in four patients in 1988 (then aged 43-67 years) and these were the four patients who had a vital capacity below 43% predicted in 1968. The remaining 20 patients had blood gas values within normal limits. Two further patients had died from respiratory failure before 1988, so a total of six patients had developed respiratory failure. In a multiple logistic analysis vital capacity expressed as % predicted in 1968 was the strongest predictor of the development of respiratory failure, followed by the scoliotic angle. Respiratory failure occurred only in patients who had a vital capacity below 45% predicted in 1968 and an angle greater than 110 degrees. Thus respiratory failure develops in adults with scoliosis with a large angle and a low vital capacity when normal aging reduces the ventilatory capacity further. Such individuals merit close follow up.
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PMID:Lung function in adult idiopathic scoliosis: a 20 year follow up. 187 34

Adolescents with mild, asymptomatic scoliosis (thoracic curvature less than 35 degrees) may have little or no impairment of resting lung volumes. Progression to more severe disease may, however, be accompanied by lung restriction, impaired exercise tolerance, and respiratory failure with CO2 retention. We wished to see whether adolescents with mild scoliosis and minimally abnormal resting pulmonary mechanics had impairment of their responses to hypercapnia, hypoxia, and progressive cycle exercise. Forty-four adolescents with idiopathic scoliosis were studied. The mean forced vital capacity (FVC), expressed as a percentage of the predicted value, was 94.3 +/- 2.2 (SE). The mean ventilatory response to hypercapnia (2.57 +/- 0.24 L/min/mm Hg) was within the normal range but was achieved with a tidal volume response (1.87 +/- .17% vital capacity [VC]/mm Hg) that was significantly lower than that previously reported in healthy young adults. Ventilatory responses to exercise were also within the normal range, the mean dyspnea index (VE-max/maximal voluntary ventilation) = 0.92 +/- 0.04. However, at a ventilation of 30 L/min, the tidal volume was 0.38 +/- 0.01% FVC, which was considerably lower than predicted. The tidal volume response to hypoxia was also abnormally low, the mean response being 0.52 +/- 0.059% VC/% decrease in arterial O2 saturation. These findings indicated that, even when scoliosis is asymptomatic and associated with minimal impairment of resting pulmonary function, abnormal patterns of ventilation occur during exercise or in response to chemical stimuli.
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PMID:Ventilatory patterns during hypoxia, hypercapnia, and exercise in adolescents with mild scoliosis. 370 36

In early phases of neuromuscular disease, patients are either free of respiratory symptoms or have exertional dyspnea not explained by obvious obstructive or restrictive lung disease. Physical examination may be negative because generalized muscle weakness does not correlate with the degree of respiratory muscle involvement. When the diaphragm is involved, one may detect the absence of outward excursion during inspiration or even paradoxic inward inspiratory movement of the abdomen on one side. A substantial loss of respiratory muscle strength is typically accompanied by little or no change in spirometry or arterial blood gas composition. Other characteristics are moderate loss of maximal voluntary ventilation and an increase in residual volume, yet PImax and PEmax may be as low as 50% of the predicted value. In more advanced neuromuscular disease, patients may have severe symptoms if the onset is acute or subacute; however, patients with chronic advanced generalized muscle weakness do not exercise and, therefore, may not be breathless. Many patients with advanced neuromuscular disease present with daytime somnolence as a manifestation of a sleep-related breathing disorder. Physical examination may reveal generalized muscle weakness and difficulty with speech or swallowing. Signs specific to respiratory involvement include tachypnea, use of neck inspiratory muscles and abdominal expiratory muscles, and loss of chest-abdomen synchrony. Sometimes paradoxic bilateral inward movement of the abdomen with inspiration is overt. Patients may be unable to cough effectively, have scoliosis, and lack a gag reflex. At this advanced stage, PImax and PEmax are lower than 50% of the predicted value, and the vital capacity is reduced. Maximal voluntary ventilation increases, and residual volume increases further. Patients may not yet exhibit CO2 retention during the day and may even have a low PaCO3. A sleep study may reveal significant hypopneas with severe desaturation and hypercapnia, especially during REM sleep. It is important to be aware that overt ventilatory failure can occur abruptly and that measurement of arterial blood gas composition is not a reliable indicator of this danger. Therefore, it is critically important to heed clinical phenomena, such as increasing dyspnea and tachypnea, and symptoms of sleep disturbance, such as morning headache and daytime somnolence. Physicians should make serial measurements of VC and respiratory muscle strength in patients considered to be at risk for further deterioration.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Assessment of ventilatory function in patients with neuromuscular disease. 786 89

Pregnancy often poses a risk to patients with neuromuscular and skeletal disorders when these affect the respiratory muscles or the rib cage. The outlook is determined both by the severity of the underlying condition and the physiological changes during pregnancy. Patients with a vital capacity of less than 1 to 1.5 litres, hypercapnia, severe scoliosis, diaphragm weakness or pulmonary hypertension before pregnancy are particularly at risk. Pregnancy may adversely affect the conducting airways, respiratory pump and gas exchange in the lungs. Close monitoring of high risk patients during pregnancy is required and either a termination of pregnancy or mechanical respiratory support may be indicated if ventilatory or cardiac failure develops.
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PMID:Pregnancy in neuromuscular and skeletal disorders. 808 20

A 48-year-old man was referred to our hospital because of hypoxemia (PaO2 = 43 mmHg), hypercapnia (PaCO2 = 70 mmHg), complete atrio-ventricular block, and heart failure. He also had limitation of spine flexion, scoliosis, deformity of the rib cage, and constriction of the ankle joints, complicated by cor pulmonale. These findings were compatible with rigid spine syndrome. To avoid progressive pulmonary hypertension and hypoxemia, nasal BiPAP and home oxygen therapy (0.5 liters/minute) were begun. Rigid spine syndrome is clinically characterized by limitation of spine flexion, and the limitation of thoracic movement often causes severe constrictive respiratory dysfunction. This syndrome should be considered when evaluating patients who have both thoracic deformity, especially scoliosis, and respiratory failure.
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PMID:[Rigid spine syndrome associated with marked hypoxemia and hypercapnia]. 875 23

A 13-year-old boy with juvenile-onset acid alpha glucosidase deficiency was reported. Proximal muscle weakness including respiratory muscles and scoliosis progressed since nine year of age. He developed nocturnal dyspnea and daytime somnolence at age 13. His arterial blood gas analysis showed hypoxemia (PO2 54.1 mmHg) and hypercapnia (PCO2 72.3 mmHg), and spirometry showed significantly decreased vital capacity (% VC 21%). He was treated with nocturnal NIPPV employing a device for delivering bilevel positive airway pressure (Bi-PAP). Nocturnal dyspnea and daytime somnolence rapidly disappeared with nocturnal ventilatory support. Daytime arterial PO2 and PCO2 improved after the therapy, namely 74.8 mmHg and 64.1 mmHg respectively. We conclude that NIPPV is a noninvasive and effective therapy for respiratory failure in patients with chronic progressive neuromuscular disorder including acid alpha glucosidase deficiency.
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PMID:[Chronic respiratory failure in a case with juvenile-onset acid alpha-glucosidase deficiency; successful therapy with nasal intermittent positive pressure ventilation (NIPPV)]. 898 97

The purpose of the lung is intrapulmonary gas exchange. The circulatory system delivers the respiratory gases to the tissue. The ventilatory pump however is responsible for the circulation of air between the lungs and the ambient atmosphere. Due to better diffusing capabilities, hypercapnia always is a result of pump failure and little dependent on the lung. Ventilatory failure, either compensated with an increased demand on the muscles or decompensated with an additional increase in pCO2, should be separated from lung failure where primarily oxygen exchange is involved. Decompensated hypercapnic ventilatory failure is then the indication for intermittent mechanical ventilation. The pCO2, either arterial or transcutaneously registered together with the noninvasive evaluation of the mouth occlusion pressures during tidal breathing and during a maximal inspiratory effort, define well the severity of ventilatory failure. In acute on chronic ventilatory failure, noninvasive mechanical ventilation in three randomised and controlled studies resulted in a better survival compared to intubation. To fulfil certain weaning criteria is no longer required in difficult to wean patients, as a transfer from invasive to noninvasive mechanical ventilation can be performed if only cooperativity is preserved together with a minimal capacity of spontaneous breathing. Weaning will thereafter occur by progressive relief from intermittent noninvasive ventilation. 2300 difficult to wean patients in Germany should profit from this approach. Chronic ventilatory failure as a result of neuromuscular disease or scoliosis of the thoracic spine are the classical indications. COPD and myasthenia gravis are under discussion as indications for intermittent mechanical ventilation with an increasing tendency to ventilate. Epidemiological data however can only be roughly estimated due to the heterogeneity of indication and selection of the patients.
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PMID:[Epidemiology and diagnosis of intermittent self-ventilation]. 923 70

In kyphoscoliosis restrictive ventilatory defect occurs. In idiopathic scoliosis vital capacity failure is significantly correlated with Cobb angle, vertebral rotation, and thoracic lordosis. Maximum voluntary ventilation is the most affected measurement. Forced expiratory volume in 1 second is reduced. Residual volume remains longtime normal. Hypoxemia due to decrease of diffusing capacity occurs, with initially reflex hyperventilation hypocapnia, and secondary hypercapnia. Pulmonary hypertension and cor pulmonale is related to hypoventilation and hypoxia. The lung situated on the concave side of the scoliosis curve shows a more functional derangement. Ventilatory pattern consists of low tidal volume and high respiratory rate with increase of ventilatory work. Scoliosis that appears in the earlier stage of the life has the worst respiratory prognosis (before 5 years of age) with impairement of lung and thoracic growth. To stimulate pulmonary and thoracic growth, intermittent ventilatory assistance by pressure preset ventilator should be performed as soon as possible and pursued up to 8 years of age, at least, more if necessity. In over 60 degrees angle idiopathic scoliosis, respiratory failure appears after 40 to 50 years of age. Non invasive ventilatory assistance with preset pressure ventilator by oral way in moderate cases and nocturnal nasal ventilation by volume ventilator or inspiratory assistance ventilator, in the most severe cases are efficient. In very severe and acute respiratory insufficiency (scoliosis over 90 degrees) ventilation by intubation then tractheostomy may be required. Earlier orthopedic management and surgical procedure to correct and stabilize spinal deformities is the best to prevent respiratory insufficiency. For scoliosis below 60 degrees, post operative pulmonary complications are very low, with no requirement of post operative ventilatory support. In very severe respiratory insufficiency treatment of respiratory failure precedes, and follows, orthotic treatment and surgical procedures; it shouldle pursued afterwards.
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PMID:[Respiratory problems in severe scoliosis]. 1043 94


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