Kyphoscoliosis as a cause of cardio-respiratory failure--pitfalls of diagnosis.
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Adult↗
Biomedical subjects
M A Branthwaite
Publications and source records attributed to M A Branthwaite.
The effect of intermittent positive-pressure hyperinflation in restrictive chest wall disease.
The effects of intermittent positive-pressure hyperinflation on lung volumes, arterial blood gas tensions and respiratory muscle performance have been assessed in 10 patients with severe scoliosis (vital capacity 22-43% predicted). Hyperinflation was carried out 2-3 times a day for 5 min aiming to double the spontaneous tidal volume with each delivered breath. A volume-preset, time-cycled device was used in 6 patients and a pressure-cycled, patient-triggered machine in 4 cases. Progressive reduction in lung volume was reversed by the volume-preset device which increased the mean vital capacity from 29 to 33% predicted after 3 months (p less than 0.05) with improvement maintained after 9 months. This machine offered a significant advantage over the pressure-cycled device which at maximum or near maximum inflation pressures produced less hyperinflation and had no beneficial effect on lung volumes. The improvement in vital capacity was correlated with the degree of hyperinflation (r = 0.65; p less than 0.05). The effect of hyperinflation on arterial blood gas tensions was transient and not accompanied by an increase in accessible alveolar volume.
Adult↗
Effect of protriptyline on ventilatory responses to hypercapnia and asphyxia in normal subjects.
A double-blind crossover study was undertaken to assess the effect of protriptyline on ventilatory responses in normal subjects. Seven subjects received in random order placebo, 10 mg and 20 mg protriptyline daily for 2 weeks. Measurements of hypercapnic ventilatory response (HCVR) and asphyxial hypoxic ventilatory response (HVR) were made before treatment, 6-8 h after the first dose, and after 2 weeks treatment. Mean HCVR and HVR following 10 mg and 20 mg protriptyline did not differ significantly from measurements on placebo, neither for the single dose study or after 2 weeks.
Adult↗
Intermittent positive pressure ventilation by nasal mask: technique and applications.
Intermittent positive pressure ventilation delivered non-invasively through a well-fitting nasal mask has been used to control nocturnal hypoventilation in three patients with severe, combined cardiorespiratory and skeletal disease. The advantages of this approach to domiciliary ventilation are described and the requirements of the ventilator are defined.
Adult↗
Getting a patient off the ventilator.
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Cardiovascular Diseases↗
Use of negative pressure ventilation to facilitate the return of spontaneous ventilation.
Negative pressure ventilation was used to facilitate the return of spontaneous ventilation in 10 patients with severe, chronic respiratory disability. All patients had required tracheal intubation and intermittent positive pressure ventilation, and conventional weaning techniques had failed. Details of the method are described. It permits extubation before spontaneous ventilation can be sustained indefinitely and thus assists the return of normal speech, sleep pattern and nutrition. The lack of flexible control of ventilatory variables and absence of access to the trachea for sputum clearance limit its widespread application.
Adolescent↗
Domiciliary ventilatory support: an analysis of outcome.
Prolonged ventilatory support has been used to treat 51 patients with respiratory failure secondary to skeletal (22) or neuromuscular (29) disease. Symptomatic relief was achieved in five patients with rapidly progressive neurological disease who died within 27 months. The remaining 46 patients, aged 11-69 years at presentation, have been followed for more than two and a half years. All but 10 were treated with negative pressure ventilation from the outset, intermittent positive pressure ventilation being used initially in the others and continued at home in three. Nocturnal negative pressure ventilation has been used at home by 39 patients. A permanent tracheostomy has been maintained in 14, to facilitate positive pressure ventilation in three and to circumvent upper airway obstruction during sleep in 11. Sustained improvement in symptoms and arterial blood gas tensions has been maintained, independence and the capacity for gainful employment have been regained in those of an appropriate age, and the incidence of subsequent hospital admissions has been low. Neither the mode of presentation nor the aetiology of the restrictive ventilatory defect influenced outcome in patients with stable or only slowly progressive primary disease.
Adolescent↗
Control of nocturnal hypoventilation by nasal intermittent positive pressure ventilation.
Ten patients with respiratory failure and nocturnal hypoventilation were treated for three to nine months by nasal intermittent positive pressure ventilation. Four patients had chronic obstructive lung disease (median FEV1 19% predicted) and six restrictive chest wall disorders (median FVC 25% predicted); eight of the patients also had cardiac failure. The median daytime arterial oxygen tension, measured before and after at least three months' treatment, increased from 6.2 (range 5.4-9.6) to 9.1 (7.1-9.8) kPa in those with restrictive disease (p less than 0.05), and from 6.0 (5.7-6.5) to 7.1 (6.3-7.7) kPa in the four with airflow limitation (NS). Median values for arterial carbon dioxide tension over the same time fell from 8.2 (range 6.7-9.8) to 6.5 (6.0-6.9) kPa in the group with restrictive disease (p less than 0.05) and from 8.2 (7.0-9.2) to 7.1 (4.9-7.7) kPa in those with airflow limitation (p less than 0.02). Total sleep time while patients were using nasal positive pressure ventilation varied from 155 to 379 (median 341) minutes, and included 4-26% rapid eye movement sleep (median 14%). The percentage of monitored time during the night in which the arterial oxygen saturation was less than 80% fell from a median (range) of 96 (3-100) to 4 (0-9) in the six patients with restrictive disease and from 100 (98-100) to 40 (2-51) in those with airflow limitation. There were no changes in spirometric values but exercise tolerance improved in all patients. The technique may prove an acceptable alternative to long term domiciliary oxygen therapy in selected patients.
Adult↗
Respiration during sleep in kyphoscoliosis.
Eleven subjects with non-paralytic and 10 with paralytic kyphoscoliosis and nine normal control subjects were studied during sleep. The Cobb angle of those with kyphoscoliosis varied from 60 degrees to 140 degrees (median 100 degrees) and the vital capacity varied from 17% to 56% (median 28%) of the value predicted on the basis of span. Recordings made during sleep included expired carbon dioxide tension at the nose, gas flow at the mouth, arterial oxygen saturation, chest wall movement, and the electroencephalogram, electro-oculogram, and electrocardiogram. In three subjects transcutaneous carbon dioxide tension was measured simultaneously. Patients with kyphoscoliosis hypoventilated during sleep, particularly in rapid eye movement sleep, resulting in a rise in end tidal and transcutaneous carbon dioxide tension, and a reduction in oxygen saturation to a degree not observed in normal subjects. Reduced chest wall movement was the major cause of these episodes, which were more frequent and occupied a greater proportion of sleep time in those with kyphoscoliosis than in normal subjects. Serious cardiac arrhythmias were rarely associated. It is concluded that disturbances of respiration during sleep occur in patients with kyphoscoliosis and that these may be important in the pathogenesis of cardiorespiratory failure.
Adolescent↗
Severity scoring in intensive care.
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Acute Disease↗
Management of respiratory failure complicating pregnancy in severe kyphoscoliosis: a new use for an old technique?
Six cases with paralytic or early onset kyphoscoliosis who developed cardiorespiratory complications during pregnancy are described. Four of these were treated during or immediately after pregnancy with negative pressure ventilation. The use and advantages of this type of treatment to relieve respiratory and cardiac failure related to pregnancy in patients with kyphoscoliosis is discussed.
Adolescent↗
Cardiorespiratory consequences of unfused idiopathic scoliosis.
A retrospective survey was carried out on approximately 800 scoliotic subjects attending a chest clinic over 25 years. One hundred thirty-one patients with unfused idiopathic scoliosis were identified and further consideration was restricted to 54 who were assessed at 30 years of age or older. Sex, age of onset of curvature, severity at the time of presentation, degree of dyspnoea, presence of independent cardiac or pulmonary disease and smoking habit were recorded. Measurements of lung function were compared with predicted figures calculated according to span, age and sex. Disabling dyspnoea or cardiorespiratory failure were associated with either scoliosis of early onset (curve first noticed before 5 years) or with independent cardiac or pulmonary disease. Only one of 28 patients with unfused idiopathic scoliosis of adolescent onset developed disabling dyspnoea in later life attributable solely to spinal deformity. A similar conclusion was drawn from a separate survey of mortality in 86 patients, 19 of whom suffered from idiopathic scoliosis. Cardiorespiratory failure attributable to the scoliosis was the cause of death of 11 patients, in 10 of whom the curve had first been noticed at less than 5 years of age whereas the onset was during early adolescence (11 years) in only one.
Age Factors↗
Effects of protriptyline on sleep related disturbances of breathing in restrictive chest wall disease.
The effects of protriptyline on sleep stage distribution and gas exchange have been assessed in eight patients with nocturnal hypoventilation secondary to restrictive chest wall disease. At a dose of 10-20 mg taken when they retired the total sleeping time was unaltered but the proportion of rapid eye movement (REM) sleep fell from 22% to 12% (p less than 0.05). The total time spent at an arterial oxygen saturation of less than 80% decreased (p less than 0.01) and the magnitude of the fall correlated with the reduction in REM sleep (r = 0.67, p less than 0.05). There was also a reduction in the maximum carbon dioxide tension reached during the night (p less than 0.01). The arterial oxygen tension measured diurnally increased (p less than 0.05) from a median of 8.0 kPa (60 mm Hg) to 9.0 kPa (67.5 mm Hg), but the carbon dioxide tension and base excess were unchanged. Anticholinergic side effects were experienced by most patients but did not limit treatment.
Adult↗
The intensive care of asthma.
Severe acute asthma is potentially lethal. Deaths occur in hospital unless patients are assessed accurately on admission, monitored regularly, and treated vigorously. Mechanical ventilation is required infrequently and is associated with additional hazards. The principles of management of the severely ill asthmatic are outlined here.
Adrenergic beta-Agonists↗
Use of prostacyclin and ultrafiltration in adult respiratory distress syndrome.
A primigravida with severe kyphoscoliosis developed cardio-respiratory failure in pregnancy. Cardiac arrest occurred 10 days after Caesarean section; gastric acid was aspirated then and was followed by the development of adult respiratory distress syndrome. Initial recovery, with clearing of peripheral oedema, was followed by a recurrence of respiratory distress associated with infection. Profound hypoxaemia and oliguria unresponsive to diuretics were relieved by the infusion of prostacyclin combined with fluid removal by ultrafiltration. This treatment may be of value in the management of respiratory distress syndrome when pulmonary oedema is the dominant feature.
Adult↗
Pleural surgery in patients with cystic fibrosis. A review of anaesthetic management.
Eighteen patients with advanced cystic fibrosis complicated by pneumothorax required either pleurectomy or abrasion pleurodesis. Anaesthesia with nitrous oxide and oxygen was combined with controlled ventilation to both lungs, usually through a double lumen endobronchial tube. Spontaneous ventilation was restored at the end of operation in all but a minority of cases. Ten of 25 procedures were uncomplicated and a further five were followed by non-respiratory events. Respiratory complications developed after the remaining ten procedures which were carried out on six patients, of whom only one survived for more than 6 months. All five fatal cases suffered a recurrence of pneumothorax within 6 months whereas three patients requiring re-operation 2-6 years later survived. The principles of management are discussed and it is concluded that the operative and anaesthetic hazards of thoracic surgery in this group of high-risk cases can be avoided successfully, except when re-operation is required within 6 months.
Adolescent↗
The effects of thoracic surgery.
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Anesthesia, General↗
Alveolar proteinosis: diagnosis and treatment over a 10-year period.
Ten years' experience of using bronchoalveolar lavage in the treatment of 10 patients with alveolar proteinosis is reported. The diagnosis was often missed. The interval between onset of symptoms and diagnosis varied from six weeks to six years (median 2 years), so that the start of treatment was often delayed. Some patients experienced severe progressive disability before they had treatment. Whole-lung lavage proved to be a safe, repeatable procedure which provided symptomatic, physiological, and radiological improvement and allowed all 10 patients treated to return to full-time employment.
Adult↗