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Congenital scoliosis in a neonate: can a neonatologist ignore it?

The frequency of diagnosis of congenital scoliosis in the neonatal period is expected to rise given the increasing survival of high risk neonates in the surfactant era and their frequent exposure to x rays. Considering its significant long term implications a neonatologist cannot afford to ignore the diagnosis of congenital scoliosis in a neonate as close surveillance, early detection, and treatment may prevent/minimise the wide spectrum of potentially serious deformities that can affect the developing spine. The review provides general guidelines to help the neonatologists in counselling the parents and in planning the multidisciplinary follow up for management of congenital scoliosis.

Counseling↗

Pulmonary function in adolescent idiopathic scoliosis: a 25 year follow up after surgery or start of brace treatment.

BACKGROUND: Pulmonary function in patients with adolescent idiopathic scoliosis many years after posterior spinal surgery or brace treatment has not been documented. METHODS: A consecutive group of patients treated by posterior fusion or a brace at least 20 years previously was investigated. 90% attended a clinical follow up. Lung volumes were determined before treatment in 251 patients, 1.4 years after surgery in 141 patients, and 25 years after surgery or start of brace treatment in 110 patients. Vital capacity (VC) was calculated as percentage predicted according to height and age and the results were corrected for loss of height due to scoliosis. Scoliosis angles were measured and smoking habits were recorded. An age and sex matched control group was also examined with the same questionnaire and pulmonary function tests. RESULTS: VC increased from 67% predicted immediately before surgery to 73% (p<0.001) after surgery and to 84% (p<0.001) at the present follow up, mean change 10.8% (95% CI 9.5 to 12.1). In the brace treated patients VC increased from 77% predicted before treatment to 89% (p<0.001) 25 years after start of treatment, mean change 12.3% (95% CI 10.5 to 14.1). The mean Cobb angle at the present follow up study was 40 degrees in both surgically and brace treated patients. The present results of lung volumes did not correlate with pretreatment or post-treatment Cobb angles or smoking habits. CONCLUSIONS: Patients treated by posterior fusion or a brace gradually increase their pulmonary function up to 25 years after treatment. Smoking and curve size are not risk factors for reduced pulmonary function.

Adolescent↗

Cardiac and respiratory function before and after spinal fusion in adolescent idiopathic scoliosis.

Ten girls with adolescent idiopathic scoliosis were studied before and 17-23 months after spinal fusion. None had any cardiac or respiratory disease complicating the scoliosis. They underwent a range of resting lung function tests and a progressive exercise test. The mean angle of scoliosis decreased from 65.8 to 27.3 degrees after operation but the only significant physiological benefit detected in this study was a decrease in the submaximal minute ventilation. The physiological benefit of spinal fusion was therefore much less prominent than the anatomical improvement of the spinal curvature.

Adolescent↗

Mechanical inefficiency of the thoracic cage in scoliosis.

The mechanism of impairment of ventilatory function in idiopathic scoliosis has been studied in 23 children, all girls, and compared with 27 normal children and 24 normal young adult females. The vital capacity, FEV 1, gas transfer factor, and the maximum static expiratory airway pressure were all significantly reduced. total lung capacity and the maximum inspiratory pressure were lower than in the normal subjects, but the difference was not significant. Restriction of thoracic cage movement by a belt showed that the thorax in the children with scoliosis was as mobile as in the normal subjects. The results are explained in terms of the characteristic deformity in scoliosis which causes one hemi-thorax to become relatively smaller than the other. It is concluded that this causes an inherent mechanical inefficiency of ventilation which is likely to contribute to respiratory failure in these subjects.

Adolescent↗

Effect of bracing on respiratory mechanics in mild idiopathic scoliosis.

The use of a corrective orthopaedic brace is an established form of management for patients with progressive idiopathic scoliosis. Thirteen patients with mild idiopathic scoliosis were studied with and without the corrective brace applied. Lung volumes and the pattern of chest wall and abdominal movement were measured during quiet breathing. Transdiaphragmatic pressures were measured in six of the patients and upper ribcage movement in seven patients. Application of the brace resulted in a significant reduction in vital capacity (14%), functional residual capacity (22%), and total lung capacity (12%). There was no effect on respiratory rate or minute volume. In the erect position the pattern of chest wall movement was altered with a reduction in lower ribcage movement of 46% and abdominal wall of 39% and an increase in upper ribcage movement of 43%. These changes were greater in the supine position. There was at least a twofold increase in end inspiratory and end expiratory gastric pressures during tidal breathing, but oesophageal pressures were not affected by the brace. Transdiaphragmatic pressures showed a similar twofold increase, which implies a substantial increase in the work of breathing. In view of the doubts concerning the influence of bracing on the natural history of idiopathic scoliosis and the substantial functional effect of bracing on the respiratory system, it is suggested that the current practice of bracing in this condition needs to be reviewed.

Adolescent↗

Lung function in adult idiopathic scoliosis: a 20 year follow up.

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.

Adolescent↗

Pulmonary function before and after anterior spinal surgery in adult idiopathic scoliosis.

BACKGROUND: Little is known about the long term effects of anterior spinal surgery on pulmonary function in adult patients with idiopathic scoliosis. A study was therefore undertaken of pulmonary function before and after anterior spinal surgery in this group of patients. METHODS: Fourteen patients (12 women) of mean age 26.5 years (range 17-50, 10 > or = 20 years) were studied. All 14 patients underwent thoracotomy and anterior arthrodesis, and five also underwent posterior arthrodesis. Scoliosis curves and lung volumes were measured preoperatively and at a mean follow up of 32 months (range 14-49) after surgery. RESULTS: At long term follow up after surgery the mean (SD) Cobb angle of the thoracic curve improved from 59 degrees (25 degrees) to 39 degrees (18 degrees), a correction of 31%. Despite this there was a fall in mean forced vital capacity (FVC) of 0.21 litres (95% confidence interval 0.04 to 0.38). In the patients who underwent anterior surgery without posterior surgery (n = 9) there was a fall in mean FVC of 0.31 litres (95% confidence interval 0.12 to 0.50); mean forced expiratory volume in one second and total lung capacity were also reduced and there was an increase in mean residual volume. CONCLUSIONS: Forced vital capacity is reduced at long term follow up in adult patients with idiopathic scoliosis who undergo anterior spinal surgery. The fall in FVC is small and is unlikely to be of clinical significance in patients with reasonable lung function in whom surgery is planned for prevention of curve progression or improvement of cosmetic appearance and pain. However, surgical intervention should not be undertaken in an attempt to improve pulmonary function.

Adolescent↗

Computerized measurement and analysis of scoliosis: a more accurate representation of the shape of the curve.

A computer program was created to identify and accept spatial data regarding the location of the thoracic and lumbar vertebral bodies on scoliosis films. With this information, the spine can be mathematically reconstructed and a scoliotic angle calculated. There was a 0.968 positive correlation between the computer and manual methods of measuring scoliosis. The computer method was more reproducible with a standard deviation of only 1.3 degrees. Computerized measurement of scoliosis also provides better evaluation of the true shape of the curve.

Diagnosis, Computer-Assisted↗

The top view for analysis of scoliosis progression.

Using computerized spinal analysis, a new top view was developed that displays the spine as if the observer were above and looking down on the patient. Serial top views were obtained of 12 patients with idiopathic scoliosis. In five patients with clinically stable curves, the top views showed no change. One patient with an enlarging rib hump was seen on the top view to have progressive kyphosis but stable scoliosis. Six patients with progressive scoliosis all demonstrated collapse of the thoracic curve in the anteroposterior direction. Five of these six patients had associated lumbar curves. Three lumbar curves demonstrated collapse in the anteroposterior direction similar to the collapse of the thoracic curves, and the other two curves appeared elongated in the anteroposterior direction.

Adolescent↗

Radiation dose reduction in the evaluation of scoliosis: an application of digital radiography.

This report documents the clinical testing of scanning beam digital radiography as an imaging method in patients with scoliosis. This type of digital imaging requires a skin exposure of only 2.4 mR (0.619 microC/kg) per image, compared with the lowest possible posteroanterior screen-film exposure of 10 mR (2.58 microC/kg) at the chest and 60 mR (15.48 microC/kg) at the lumbar spine. Digital radiographic and screen-film images were obtained on multiple test objects and 273 patients. Scoliosis measurements using screen-film radiographs and digital radiographs were comparable to within a mean difference of 1 degrees at many different degrees of severity. The low-dose digital images were found to be useful and accurate for the detection and measurement of scoliosis after the first screen-film radiographs have excluded tumors and structural abnormalities.

Adolescent↗

Childhood scoliosis: MR imaging.

The spinal cords of 28 scoliosis patients between the ages of 1 month and 17 years were examined with magnetic resonance (MR) imaging. Complete visualization was obtained in all cases. In 15 patients (53%) neuropathologic abnormalities demonstrated by MR imaging significantly affected their clinical course, including tethered cords (n = 7), syringomyelia (n = 5), Arnold-Chiari I malformation (n = 4), spinal cord tumors (n = 2), Arnold-Chiari II malformation (n = 3), and diastematomyelia (n = 1). The advantages of MR imaging in the evaluation of the scoliotic spine in children include a high sensitivity for the occult conditions associated with scoliosis, good anatomic demonstration of the cord, and absence of bone artifacts. MR imaging is recommended as a primary imaging modality in scoliosis, following conventional radiography.

Arnold-Chiari Malformation↗

[Early postoperative complications after scoliosis surgery].

Several medical complications can occur after scoliosis surgery in children and adolescents. New surgical techniques have allowed greater degrees of scoliosis correction but have also increased the possibility of postsurgical deficit due to their greater aggressivity. We analyzed the early postsurgical complications of scoliosis surgery in a pediatric intensive care unit over a 10-year period. Seventy-six surgical procedures were performed. Of these, no complications occurred in 55 (73%). Chest X-ray revealed pulmonary atelectasia in 8 patients (10%) and pleural effusion in 7 patients (9%). Symptoms and signs of infection related to surgery were observed in only 6 children (8%). The absence of severe medical complications may be related to new surgical techniques and an experienced team.

Adolescent↗

Spinal ganglioneuroma mimicking adolescent idiopathic scoliosis.

The authors report a 12-year-old girl who presented with a 2-year progressive painless thoracic scoliosis. Her previous development had been normal with no significant past history of illness. MRI revealed a thoracic ganglioneuroma, which was confirmed by surgical pathology. Ganglioneuroma might cause progressive spinal deformity mimicking adolescent idiopathic scoliosis. A careful preoperative survey is mandatory for patients diagnosed with scoliosis with a rigid or rapidly progressive curve. The authors recommend a combination of neurosurgery and orthopedic spine surgery to treat this condition.

Child↗

Idiopathic scoliosis. Gas exchange and the age dependence of arterial blood gases.

The aims were to examine the gas exchange and arterial blood gas abnormalities among patients with scoliosis, and the correlation of these abnormalities with age and severity of deformity. Means among 51 patients were as follows: age 25.4 +/- 17.5 yr, angle of scoliosis 80.2 +/- 29.9 (SD), vital capacity 1.94 +/- 0.91 (SD) (i.e. 60.6 +/- 19.2% of predicted), PaO2 85.8 +/- 12.0 (SD), PaCO2 42.4 +/- 8.0, physiological dead space to tidal volume ratio 0.438 +/- 0.074 (SD), and alveolar-arterial oxygen difference breathing air 14.9 +/- 8.9 (SD). Statistically significant correlations were as follows: the PaCO2 and physiological dead space to tidal volume ratio increased with age, and the PaO2 and alveolar ventilation decreased with age. The PaO2, alveolar ventilation, and tidal volume were inversely related to the angle of scoliosis and directly related to the vital capacity, precent predicted vital capacity, and the compliance of the respiratory system. The physiological dead space to tidal volume ratio and the alveolar-arterial oxygen difference were inversely related to the vital capacity, percent predicted vital capacity, and the compliance of the respiratory system. PaCO2 was directly related to the elastance of the respiratory system. We conclude that ventilation-blood flow maldistribution as a result of deformity of the rib cage was the primary abnormality in gas exchange, and that with age there was progressive deterioration in gas exchange. The age-dependent increase in PaCO2 and decrease in alveolar ventilation were due to the increasing physiological dead space to tidal volume ratio and failure of a compensatory increase in ventilation.

Adolescent↗

An autopsy case of sudden death in a patient with idiopathic scoliosis.

A 38-year-old woman with idiopathic scoliosis (right convex thoracic scoliosis, 78 degrees; left convex lumbar curvature, 75 degrees) died suddenly. Forensic autopsy and histopathologic examination revealed chronic congestive oedema, numerous cavities and atrophic changes of heart. These changes, including both respiratory changes and biventricular failure caused by hypoplastic cardiac changes, were due to a deformed thoracic cage. This case illustrates that not only abnormalities of respiratory function and cor pulmonare, but also hypoplastic cardiac changes, may cause sudden death in a patient with untreated scoliosis.

Adult↗

Two siblings with familial idiopathic scoliosis with conjugate gaze palsy.

Familial idiopathic scoliosis with conjugate gaze palsy is a rare autosomal recessive disorder characterized by progressive scoliosis and congenital absence of conjugate horizontal eye movement. In this article, two siblings who had scoliosis and conjugate gaze palsy are presented regarding clinical and laboratory findings.

Adolescent↗

A comparison of radial artery blood pressure determination between the Vasotrac device and invasive arterial blood pressure monitoring in adolescents undergoing scoliosis surgery.

UNLABELLED: The Vasotrac device (Medwave, Arden Hills, MN) allows noninvasive measurement of arterial blood pressure (BP) and arterial waveform. We have previously demonstrated agreement between the Vasotrac and continuous intraarterial BP monitoring in children with a stable circulation after cardiac surgery in the cardiac intensive care unit. To assess this monitor during different physiologic conditions, we studied the Vasotrac in anesthetized adolescent children undergoing scoliosis surgery in the prone position, with or without controlled hypotension. Eleven children undergoing surgery for idiopathic scoliosis were enrolled in this study. The anesthetic consisted of primarily a nitrous oxide and narcotic technique with controlled hypotension obtained using IV labetalol. Data were analyzed using correlations, mean error, and Bland-Altman plots. Noninvasive BP measured by the Vasotrac correlated closely with intraarterial BP. Waveforms displayed by the two systems were qualitatively similar. Correlation between the two methods for systolic, diastolic and mean BP was r = 0.82, r = 0.83, and r = 0.90, respectively. We conclude that noninvasive BP measurement using the Vasotrac monitor enables near-continuous and reliable monitoring of BP during anesthesia in the prone position and pharmacologic-induced hypotension. IMPLICATIONS: The Vasotrac, a noninvasive arterial blood pressure monitor, performs well in the prone position in adolescent patients undergoing scoliosis surgery using a technique of mild-to-moderate induced hypotension.

Adolescent↗

[Studies of experimental scoliosis produced by electrical stimulation. With special reference to the histochemical properties of the muscle].

1) Back muscles on the right side of the rat were daily stimulated with electrical pulses through implanted electrodes. As a result, a scoliosis with convexity toward the non-stimulated side was produced. The cobb angle increased with time and became 18.6 +/- 9.9 degrees on average after 3 weeks. 2) On the stimulated side, type I fibers showed a significant increase in diameter. The area they occupied in the cross section, as well as the ratio of the area against that of type II fibers, were increased. These increases disappeared 3 weeks after the termination of the stimulation, whereas the scoliosis remained almost the same, maintaining an average angle of 17.2 +/- 7.4 degrees after 3 weeks. 3) From these results, the possibility was suggested that electrical stimulation is of use in treating idiopathic scoliosis.

Animals↗