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

After describing the clinical and functional effects of pathological changes in the thoracic cage ("mechanical syndrome" and "neuromuscular syndrome"), the authors discuss the disorders of respiratory function observed in a group of patients with poliomyelitic scoliosis. The aim was to establish the type and nature of these changes with a view to clarifying certain pathogenetic aspects. Two factors suggest that the altered geometry of the thoracic cage in scoliosis is responsible for the mechanical inefficiency of the thoracopulmonary apparatus, namely: 1. changes in respiratory function, since there was a strict correlation between the severity of the scoliosis and the degree of change in certain indices of respiratory function (vital capacity, maximum expiratory volume per second, maximum ventilation per minute); 2. changes in the regulation of ventilation, indicated by: a) good correlation between the ventilatory response to CO2 and the severity of the anatomical lesion; b) a reduction in the occlusion pressure, which is regarded as the pressure available to produce ventilation.
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PMID:Changes in respiratory function in disorders of the thoracic cage. With special reference to the ventilatory mechanism and the regulation in scoliosis. 688 93

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

After primary repair of myelomeningoceles or lipomyelomeningoceles, late progressive neurologic deterioration commonly occurs due to a treatable cause. In our experience many of these patients have a tethered cord. With early untethering, most patients are stabilized and a significant percent of the patients show improvement in their clinical status. Of 341 tethered cord releases done from 1981 to 1988, we report on 153 patients reoperated upon following primary repair. One hundred were performed after primary closure of a myelomeningocele and 53 after repair of a lipomyelomeningocele. The average age of the patients with a myelomeningocele was 6 years old, and for the spinal lipoma patients, 8 years old. The presenting symptoms were similar; weakness, deterioration in gait, scoliosis, orthopedic deformities, and urinary incontinence represented the most common complaints. All 153 patients were noted to have a tethered cord at operation. Additional pathology (dermoid tumors, hydromyelia, tight filum and diastematomyelia) was present in 30% of the cases. With the use of the CO2 laser for dissection, all but 10 patients could be untethered. Follow-up over an average of 4 years revealed 93% of the patients with a myelomeningocele had stabilization or improvement of their presenting complaints, and 7% had progression of their presenting complaints. All of the lipomyelomeningocele patients had either stabilization or improvement of their presenting complaints. There were no mortalities. Close follow-up and early treatment of this patient population is indicated. With release of the cord a significant portion of the population will have relief or improvement of their presenting complaints.
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PMID:Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome. 839 48

Tracheomalacia is a condition characterized by weakness of the tracheal walls and supporting cartilage. It may be congenital, occurring in association with other lesions, such as vascular ring, tracheoesophageal fistula, dyschondroplasia, congenital cysts or tumors, or it may be acquired, most commonly as a result of protracted endotracheal intubation and less often of irradiation, trauma or neoplasm. We present a case of acquired tracheomalacia in a 15-year-old boy, a victim of Proteus syndrome (hemihypertrophy, subcutaneous tumors, and macrodactyly), who had congenital scoliosis with secondary chronic restrictive pulmonary disease, for which he was admitted to our hospital for surgical correction of the spine. Unfortunately, his conditions, particularly of respiratory, worsened after the operation, which called for an emergent tracheostomy. During the procedure, high airway pressure and CO2 retention developed, and an extreme narrowing of the tracheal lumen was revealed by fiberoptic bronchoscopy. Acquired tracheomalacia was diagnosed. The probable mechanisms, diagnostic tests, and treatments of tracheomalacia are hereunder discussed.
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PMID:Acquired tracheomalacia--a case report. 908 53

In adolescent idiopathic thoracic scoliosis (ITS) working capacity may be reduced during exercise. Despite concern about its usefulness, bracing is still being used in ITS. Thus the effects of bracing on exercise performance need to be examined. We studied six females, ages 12-15 years who had mild ITS (Cobb angle range 20-35 degrees). Pulmonary volumes, maximal voluntary ventilation (MVV), breathing pattern, the lowest (most negative in sign) pleural pressure during sniff maneuver (Pplsn), and pleural pressure swings (Pplsw) were measured first. Then, Pplsw, O2 uptake (VO2), CO2 output (VCO2), heart rate (HR) at rest and during progressive incremental exercise on a cycling ergometer (10 watts/min) were recorded. The exercise test was performed under control conditions without bracing (C) and after 7 days of bracing with the braced on (B). Dyspnea was measured by a modified Borg scale. At rest, bracing mildly affected total lung capacity and forced vital capacity (p < 0.03 for both) but not breathing pattern, Pplsn, or Pplsw (%Pplsn), a measure of respiratory effort. Furthermore, bracing did not consistently affect maximum work rate (WRmax). In both B and C VO2 was below (< 70%) the predicted value, VE was below (< 45%) MVV, and HR reserve was < 15 beats/min, indicating some cardiovascular deconditioning. On the other hand, respiratory frequency (Rf) increased more in B than in C (p < 0.03). In addition, Pplsw, Pplsw (%Pplsn), and Pplsw (%Pplsn)/VT, an index of neuroventilatory dissociation (NVD) of the respiratory pump, were greater in B (p < 0.03 for all). At a similar work rate, the Borg rating score was greater with bracing on than off, and the difference (delta Borg) tended to relate to concurrent changes in Pplsw (%Pplsn)/VT (r2 = 0.71; p < 0.07). We conclude that bracing affects respiratory effort, NVD, and dyspnea score during progressive exercise. These effects are consistent with increased lung elastance. Diminished exercise tolerance in patients with mild ITS probably reflects impaired physical fitness but is not affected by bracing. Training programs proposed for this subset of patients to increase peripheral muscle performance might also consider NVD of the respiratory pump.
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PMID:Short-term effects of bracing on exercise performance in mild idiopathic thoracic scoliosis. 927 Sep 87

Spinal braces such as the Soft Boston Orthosis (SBO) help stabilize scoliosis and improve sitting, positioning, and head control in individuals with cerebral palsy. However, their impact on pulmonary mechanics in this population has not been studied. We examined the effect of a Soft Boston Orthosis on the pulmonary mechanics and gas exchange in 12 children and young adults (5-23 years of age) with severe cerebral palsy. Pulmonary resistance, compliance, tidal volume, minute ventilation, work of breathing, oxygen saturation, and end-tidal CO2 tension were measured with the subjects seated both with and without the orthosis and in the supine position without the orthosis. There were no significant differences in the measured parameters when comparing subjects with and without their orthoses in the sitting or in the supine position. As would be expected in individuals with severe cerebral palsy, pulmonary resistance was increased (7.33 cm H2O/L/s) and compliance was decreased (0.12 L/cm H2O) compared to reported normal values. Work of breathing was greatest in the sitting position without the orthosis (1.2 dynes/cm), suggesting that the improved positioning achieved with the orthosis may decrease the work of breathing. We conclude that the application of a Soft Boston Orthosis does not impact negatively on pulmonary mechanics and gas exchange in young people with severe cerebral palsy.
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PMID:Effect of a Soft Boston Orthosis on pulmonary mechanics in severe cerebral palsy. 1040 51

A 12-year-old boy with Duchenne muscular dystrophy underwent posterior spinal fusion for progressive scoliosis. Preoperative evaluation was focused on respiratory function as well as cardiac function, which revealed markedly reduced respiratory reserve (FVC 0.77 l, %FVC 25.9%, FEV1.0 0.48 l, %FEV1.0 62%) and well-preserved biventricular function. A possible association between malignant hyperthermia and Duchenne muscular dystrophy has been documented. Thus anesthesia was administered without triggering agents. Propofol and fentanyl were used for induction and maintenance of anesthesia, and the patient was ventilated with O2-nitrous oxide mixture. The anesthesia machine, prepared by using a disposable circuit and fresh CO2-absorbent and disconnecting the vaporizers, was flushed with O2 at a rate of 10 l.min-1 for 20 minutes before use. A small dose of vecuronium was administered while monitoring the train-of-four ratio. The bispectral index (BIS) was utilized to optimize the depth of anesthesia so that the wake-up test could be performed promptly on surgeon's request while avoiding the intraoperative awareness. The BIS was helpful in continuously assessing the wakening process. BIS increased from 40's to 80's in 15 minutes after discontinuation of propofol and nitrous oxide during the test. The patient was kept under close observation postoperatively without any sign of malignant hyperthermia.
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PMID:[Application of bispectral index (BIS) monitoring to anesthetic management of posterior spinal fusion in a patient with Duchenne muscular dystrophy]. 1216 84

Neuromuscular and chest wall disorders are individually uncommon but together form an important group of conditions that can lead to chronic ventilatory failure. This is best recognised in scoliosis, kyphosis, following a thoracoplasty, in muscular dystrophies, such as Duchenne muscular dystrophy (DMD), and myotonic dystrophy, after poliomyelitis and with motor neurone disease (amyotrophic lateral sclerosis). If bulbar function is impaired, tracheostomy ventilation may be required, but in other situations, noninvasive ventilation is preferable. Positive pressure techniques using nasal and face masks are usually the first choice, but negative pressure ventilation is an alternative. There are no randomised-controlled trials regarding the indications for initiating noninvasive ventilation, but this is usually provided if there are symptoms due to nocturnal hypoventilation or right heart failure in the presence of a raised carbon dioxide tension in arterial blood (Pa,CO2) either at night or, more usually, in the daytime as well. There is no evidence that "prophylactic" ventilatory support is of benefit if this is provided before ventilatory failure has appeared. Careful selection of patients is required, especially in the presence of progressive neuromuscular disorders such as DMD and motor neurone disease. There are no randomised-controlled trials concerning the outcome of noninvasive ventilation in these conditions, but studies have shown an improved quality of life, physical activity and haemodynamics, normalisation of blood gases and slight improvement in other physiological measures, such as the vital capacity and maximal mouth pressures. Survival in chest wall disorders is approximately 90% at 1 yr and 80% at 5 yrs, and similar figures have been obtained in nonprogressive neuromuscular conditions. If, however, the underlying disorder is deteriorating, particularly if it involves the bulbar muscles, it may limit survival despite the provision of adequate noninvasive ventilatory support.
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PMID:Noninvasive ventilation for chest wall and neuromuscular disorders. 1508 69

Differences between bilevel ventilators used for noninvasive intermittent positive pressure ventilation (NIPPV) have been demonstrated during bench testing. However, there are no clinical studies comparing these machines. The authors have previously shown that the Quantum pressure support ventilator and Sullivan variable positive airway pressure II ST differ in performance during bench testing. To examine the clinical significance of this, these two machines were compared in the overnight treatment of subjects with chronic respiratory failure. Ten clinically-stable subjects with thoracic scoliosis were recruited. The subjects were already established on NIPPV, but none were using either of the ventilators to be tested. After familiarisation, the patients used the two ventilators in random order on consecutive nights. Peripheral oxygen saturation and transcutaneous carbon dioxide tension (Pt,CO2) were measured continuously, and sleep was recorded using polysomnography. There were no significant differences in arterial oxygen saturation, Pt,CO2 or sleep duration and quality between the two nights. Despite previously illustrated variation in laboratory performance, no differences were seen between the two ventilators when comparing overnight gas exchange and sleep in vivo. Further study is required to evaluate the significance of the differences found during bench testing in the clinical setting.
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PMID:An overnight comparison of two ventilators used in the treatment of chronic respiratory failure. 1241 87

Single-lung anaesthesia for thoracotomy is usually achieved with endobronchial intubation, a double-lumen tube or an endobronchial blocker. High-frequency jet ventilation (HFJV) is seldom described for thoracotomy in children, although it is used for both laryngology procedures in the operating room and as a ventilation mode in intensive care. HFJV was used in three children, aged 10-12 years, who presented for scoliosis correction involving thoracotomy. The jet ventilation catheter was passed through a tracheal tube to reduce the risk of outflow obstruction and allow a smooth conversion to intermittent positive-pressure ventilation when required. Mean airway pressures measured at the tip of the HFJV catheter were at or below 4 cmH2O. Surgical opening of the nondependent lung pleura resulted in sufficient collapse of the pulmonary parenchyma with the patient in the lateral decubitus position for the surgical procedure. Arterial blood gas analyses performed during thoracotomy were within normal limits, with no CO2 retention. HFJV is an alternative ventilation strategy for thoracotomy in children because of its unique ability to deliver small tidal volumes at low mean airway pressures via a narrow catheter.
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PMID:Jet ventilation for anterior paediatric scoliosis surgery. 1247 11


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