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Congenital spine deformities: scoliosis, kyphosis, and lordosis.

By definition, congenital spine deformities-scoliosis, kyphosis, and lordosis-are due to abnormal vertebral development. Thus, affected children tend to have a curvature noted much earlier in life than typical patients with idiopathic scoliosis. This early development of the deformity has resulted in a tendency for the young child with congenital deformities to receive less than optimal care. These curves must not be allowed to progress. In many cases, early fusion is necessary, which is preferable to allowing severe curves to develop.

Humans↗

Management of neuromuscular scoliosis.

Neuromuscular scoliosis is classified as a neuropathic or myopathic type. Cerebral palsy is the most common form of neuropathic type, and Duchenne's muscular dystrophy best characterizes the principles and recommended treatment for the myopathic type. Nonoperative measures rarely fully control a progressive scoliosis. Careful preoperative planning and surgery can achieve a well-balanced spine over a level pelvis with a good functional result.

Adolescent↗

[Lateral gaze palsy and progressive scoliosis in 4 Tunisian families].

In 1975, Sharpe and Silversides described a neurological entity in a Chinese family. Clinical picture was characterized by paralysis of horizontal gaze, pendular nystagmus and progressive scoliosis. To date, 43 cases have been reported. The pathogenesis remains unclear. The Authors report four Tunisian families with 12 affected individuals. The age of patients ranges from 6 to 34 Years. All examined patients have complete lateral gaze palsy, pendular nystagmus and progressive scoliosis. Blood routine tests, cerebrospinal fluid (CSF), evoked potentials, electromyography (EMG), muscle biopsy, CT scan and cerebral MRI were normal. Autosomal recessive (AR) mode of inheritance is the most probable pattern.

Adolescent↗

Diffusion chamber cultures of muscle from patients with scoliosis.

Explants from paraspinal muscles of patients with scoliosis were cultured in Millipore diffusion chambers placed in peritoneal cavities of mice. Three and four weeks of incubation produced a predominantly myogenic growth. Cell counts done on paraffin sections and on dissociated cells from the chambers showed that explants from the concavity of the spinal curvature grew better than those from the convexity. Since cells from injured muscles, when transplanted or cultured, are known to grow better than those from healthy control tissue, these findings support our previous morphologic observations that in idiopathic scoliosis concave muscles are more involved by the disease process. Secondary subculture of the chamber contents by conventional methods produced myotubes within one to two weeks. The relative ease of diffusion chamber muscle culture should make the method useful to laboratories that are not specifically equipped for tissue culture work.

Adolescent↗

[Pelvis obliquity, hip excentration and scoliosis in a population of 120 polyhandicaped adults. Descriptive study].

UNLABELLED: The objective of this study is to give objective data about neuro-orthopaedic status of the population of polyhandicapped patients for improving both our medical and surgical practices. MATERIAL AND METHOD: It was a multicentre, cross-sectional study to describe the population of polyhandicapped patients over 15 years of age. Each person was provided with clinical examination and X rays to detect scoliosis, pelvis obliquity and hip excentration (subluxation or dislocation) and other factors associated with polyhandicap. Polyhandicap was defined as a severe handicap linking physical and mental disability and responsible for extreme restriction of autonomy. RESULTS: One hundred and twelve persons were studied. Mean age was 27.2 years. Etiology of the handicap was neonatal asphyxia (38.3% of patients). 69.0% of patients had no possibility to turn over in lying position. Orthopaedic status was serious : 75.9% scoliosis, 65.2% pelvis obliquity (75% left and 25% right), 19.1% hip dislocation. General condition was precarious : one patient in two had a weight judged lower than normal weight, 32.5% had bedsores, 45.2% suffered from hip pain and only 13.6% received treatment against pain. DISCUSSION AND CONCLUSION: The description of general and neuro-orthopaedic status of these 120 people with polyhandicap cannot leave us indifferent. Although life expectancy in this population was lower than in normal populations, we are faced with life process and no with accompanying the terminally ill. We urgently need to review preventive care in terms of quality and quantity as well as surgical proposals for treating spasticity in children and adults.

Adolescent↗

Congenital muscular dystrophy with severe infantile scoliosis.

A girl with congenital muscular dystrophy with severe scoliosis from birth was presented. No positive family history was obtainable. She developed muscle hypotonia and weakness, and feeding difficulty during the neonatal period. Her developmental milestones were delayed; she learned to walk at the age of 2 years when she walked with a "waddling gait" and stood up with Gowers' maneuver. On physical examination at 2 years old, she had mild proximal dominant muscle weakness and atrophy, and severe scoliosis with a Cobb's angle of 74 degrees but no joint contractures in the extremities. Creatine kinase was slightly elevated. Biopsied muscle showed myopathic changes, including variation in fiber size, moderate fibrous tissue proliferation, some necrotic and regenerating fibers and type 1 fiber predominance, consistent with those seen in chronic progressive muscular dystrophy.

Biopsy↗

[Flumazenil and peroperative awakening in surgery of scoliosis].

Motor and sensory function must be assessed during surgery of scoliosis so as to avoid possible damage to the spinal cord. The intraoperative awakening by a specific benzodiazepine antagonist, flumazenil, was studied prospectively in 20 patients (mean age 17 years) undergoing surgery for severe scoliosis. Premedication consisted in 0.02 mg.kg-1 atropine and 0.15 mg.kg-1 midazolam. Anaesthesia was induced with a mean dose of 0.42 +/- 0.1 mg.kg-1 midazolam, 1.6 +/- 0.6 micrograms.kg-1 fentanyl and 0.1 mg.kg-1 pancuronium. Maintenance was obtained with a continuous infusion of 0.22 +/- 0.1 mg.kg-1.h-1 midazolam, 66% nitrous oxide in oxygen, and fentanyl (1.6 +/- 0.5 micrograms.kg-1.h-1). Nitrous oxide and midazolam were respectively stopped 10 and 1 min before giving the antagonist (5 micrograms.kg-1 flumazenil) if required (17 out of the 20 patients). Eye opening occurred a mean 42 +/- 32 s after giving the antagonist. At this time, there was a significant increase in mean arterial blood pressure (+ 11 mmHg) and heart rate (+ 7 b.min-1). Thiopentone, 66% nitrous oxide in oxygen and 0.5% halothane were given to re-induce and maintain anaesthesia for completion of the procedure. The day following surgery, 19 patients were unable to remember the period of intraoperative awakening. One patient, although remembering the episode, did not experience any pain or any other disagreement in relation to it. Two patients were given a second dose of flumazenil at extubation so as to improve the quality of their recovery.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Postpneumonectomy syndrome and pre-existing thoracic scoliosis.

We report a case of postpneumonectomy syndrome in a 75-year-old man operated on for right lung cancer 18 months previously. The patient had a pre-existing severe thoracic scoliosis. Treatment involved positioning of an expandable silastic prosthesis in the postpneumonectomy cavity. A favorable outcome was observed. We think that a pre-existing scoliosis could be considered as a potentially predisposing factor to the development of the syndrome.

Aged↗

Atypical idiopathic scoliosis in childhood.

Scoliosis, common in preadolescent females is a finding which warrants investigation in any other age group or if the clinical or neuroimaging features are atypical even in the preadolescent female. The cause of scoliosis are numerous but include several important conditions some of which are treatable and some of which are genetic. A rational approach to sequencing the neuroimaging studies to be used is based on what the clinical suspicions are and the results of the initial studies.

Child, Preschool↗

Neuromuscular scoliosis: diagnostic and therapeutic considerations.

Neuromuscular scoliosis in a child presents as a diagnostic and therapeutic dilemma to the pediatric neurologist, the pediatric neurological surgeon, and the pediatric orthopaedist. Because of its progressive nature, it tends to be refractory to conservative management and requires active intervention. Neuromuscular scoliosis is a symptom, and the diseases leading to the deformity must be addressed together with the spinal abnormality. The framework of the diagnosis and management is addressed in this article.

Case Management↗

Pathomechanics and management of scoliosis in Friedreich ataxia patients: preliminary report.

The preliminary results based on a one year study on the evolution and management of scoliosis are presented. Twenty-one patients were followed in the Neuro-Muscular Disease Clinic at Ste-Justine Hospital where standardized spinal radiographs were taken periodically with the Scoliosis Chariot and the Throne. The short period of observation as well as the relatively small number of Friedreich ataxia patients followed requires that these results and the following remarks be interpreted with caution. Pathomechanics -- Between the age groups I (5 - 10 years) and II (10 - 15 years), a substantial increase in the Cobb values occurs. Associated with it, an increase was observed in the thoracic and thoracolumbar projected surface area indices. The relative rotation between the thoracic and lumbar segments was presumed to be the cause of the sudden increase in the Cobb measurements. For the non-ambulatory patients, a decrease in the lumbar lordosis towards a thoraco-lumbar kyphosis as well as a sudden increase in the sacral angle and a drop in the lumbo-sacral angle were associated with the seated posture assumed by the patient. Management -- Prevention of the progression of established curves was our main objective. Careful examination of the spine, depending on the age of the child, in our preliminary study, stimulated early orthopaedic treatment in any curve of 20 degrees or more. There was always concern for curves of 30 degrees or more. In the growing child, bracing was recommended. In the older child, the curve was usually stable after sixteen years of age. Surgery was usually attempted in curves over 40 degrees in the growing child. The same curve was usually stable after the growth period. For the non-ambulatory patients, the present study suggested the prescription of a molded seat with the following characteristics: i) a posterior lumbar support, ii) low thoracic lateral supports and iii) a slight inclination of the seating system. This was presumed to be beneficial in maintaining stability of the spine. Presently, an evaluation of such a device is under investigation.

Adolescent↗

Scoliosis assessment in Friedreich's ataxia by means of intrinsic parameters.

Spinal deformities have been evaluated in a five year retrospective study where 28 boys and 25 girls all having Friedreich's ataxia were on the average assessed once a year in a multidisciplinary clinic. Scoliosis seems somewhat more progressive in girls than in boys and more severe in non-ambulatory than ambulatory patients. Some of those scolioses are very progressive reaching 60 degrees to 100 degrees Cobb angle values requiring spinal surgery while other progress less rapidly and do well on their own. In addition it was shown that the intrinsic geometric spine curve parameters namely that of curvature and torsion are a powerful diagnostic tool in the assessment of evolutive scoliosis.

Adolescent↗

Analysis of nutritional parameters in idiopathic scoliosis patients after major spinal surgery.

OBJECTIVE: The aim of the study is to investigate the evolution of nutritional parameters after major spinal surgery in patients with idiopathic scoliosis. METHODS: This retrospective study included 31 patients with a mean age of 18 y, diagnosed with idiopathic scoliosis. The following variables were analyzed: demographic, surgical (type, number of fused segments, duration, and blood loss), nutritional assessment (proteins, albumin, prealbumin, transferrin, lymphocytes, and body mass index), and duration of hospitalization at different time points. Statistical analyses were performed with the SPSS 6.1 software. RESULTS: Before surgery, nutritional status was normal in all patients. At 24-48 h after surgery, statistically significant decrease with respect to preoperative values was recorded for all the parameters studied: proteins (P < 0.001), albumin (P < 0.001), prealbumin (P < 0.01), transferrin (P < 0.001), and lymphocytes (P < 0.001). CONCLUSION: Our results showed a significant postoperative decrease in the nutritional parameters analyzed in a previously well-nourished population considered to be at low risk for nutritional depletion.

Adolescent↗

Intra-operative monitoring in scoliosis surgery with multi-pulse cortical stimuli and desflurane anesthesia.

STUDY DESIGN: Prospective, observational study. SETTING: Country General Hospital, Singapore. OBJECTIVE: Intraoperative monitoring (IOM) with motor-evoked potentials (MEPs) assesses the integrity of cortical spinal tracts during scoliosis surgery. MEPs are sensitive to the effects of inhalational anesthetic agents. We evaluate the use of desflurane in combination with multipulse cortical stimulation in this study. METHODS: In all, 10 consecutive neurologically normal subjects underwent scoliosis surgery with desflurane anesthesia (0.5 maximum alveolar concentration) and five pulse cortical stimulation (250 Hz) from two stimulators in parallel configuration, delivering a maximum intensity of 160 mA. RESULTS: Consistent MEPs were obtained from the abductor hallucis and tibialis anterior in nine of ten and five of five of subjects, respectively. Baseline coefficients of variations were below 16% for both muscles. CONCLUSION: This combination of anesthetic and stimulation protocols is efficacious for IOM during spinal cord surgery. Our findings support the use of desflurane for successful acquisition of MEPs during scoliois surgery as an alternative anesthetic regime.

Adolescent↗

Magnetic resonance evaluation of idiopathic scoliosis: a prospective study.

The objective of the present study was to determine the incidence of unsuspected intraspinal pathology and to assess the value of atypical clinical features as predictors of these intraspinal pathologies, in patients with idiopathic scoliosis. Twenty-five consecutive patients (13 boys, 12 girls) with idiopathic scoliosis were prospectively evaluated with MRI. Magnetic resonance imaging detected intraspinal pathology in seven patients (28%), which included syringohydromyelia with Chiari I malformation (n = 5), and syringomyelia and dumb-bell neurofibromas in one patient each, respectively. Dural ectasia was also present in five patients. Atypical features, described in the literature as pointers to intraspinal pathologies such as the age < 11 years at presentation, presence of pain, hyperkyphosis, severe curves and the presence of the left thoracic or thoracolumbar curves, were seen to be equally distributed between the two groups (those with and without intraspinal pathologies), thus raising doubts about the importance of these features.

Adolescent↗

[Surgical treatment of infantile and juvenile scoliosis--a retrospective analysis of 21 patients].

PROBLEM: We carried out a retrospective analysis of 21 patients with infantile or juvenile scoliosis (9 boys, 12 girls) who underwent surgical treatment between 1985 and 1997. 13 patients underwent primary dorsal instrumentation without fusion (growing-rod group). In contrast, 8 patients received already in this young age an instrumented spondylodesis (fusion-group). PATIENTS AND METHODS: The initial examination was performed at an age of 6 years (median; range 29 years), and first surgery was performed at an age of 8 years (median, range 310 years). The patients have been followed-up clinically and radiologically at an age of 16 years (median; range 1021 years). RESULTS: The preoperative measurement showed no significant differences between both groups (thoracic Cobb angle 77 degrees versus 66 degrees [median], lumbar Cobb angle 40 degrees versus 51 degrees [median]). At follow-up we measured significantly better results in the fusion-group than in the growing-rod group (thoracic Cobb angle 24 degrees versus 60 degrees [median; p < 0.01]), lumbar Cobb angle 21 degrees versus 34 degrees [median, p < 0.05]). The fusion-group also showed significantly better results with respect to the parameters (median values given): duration of hospital stay (14 versus 150 days), number of operations per patient (2 versus 3) and number of complications (0 versus 26). CONCLUSION: Based on the results of this small patient group we no longer see an indication to treat infantile and juvenile scoliosis by dorsal instrumentation without fusion. In contrast, we find the principle of short instrumented anterior fusion to be more promising.

Adolescent↗

[Long-term results of posterior correction and fusion of scoliosis using the Cotrel-Dubousset instrumentation].

AIM: Radiographic analysis of long-term results following CDI. METHODS: Retrospective analysis of radiographs of 43 patients with idiopathic right thoracic scoliosis with an average follow-up of 82 months. Extensive radiographic analysis with special regard to curve correction in the frontal and sagittal planes. RESULTS: The average Cobb-angle of the preoperative primary curve was 61.6 degrees (min.: 40 degrees, max.: 84 degrees ), the correction postoperatively was 51.1 % representing 29.9 degrees. Due to the loss of correction of 7.1 degrees, the overall outcome was 39.9 % by the time of follow-up. More than 90 % of the loss of correction occurred within the first 2 years. The lumbar secondary curve correction was 47.2 % (preop.: 37.5 degrees; postop.: 19.8 degrees ). Average fusion length was 11 segments, fusion usually ended two vertebrae below the end vertebra. Translation of the apex was corrected by 38.8 % (preop.: 4.9 cm; postop.: 3.0 cm), tilt of the last instrumented vertebra by 44.2 % (preop.: 18.2 degrees; postop.: 10.16 degrees ). The amount of derotation was negligible (preop.: 24.1 degrees; postop.: 22.6 degrees ). The readjustment of a preoperative pathologic sagittal profile, meaning a thoracic hypokyphosis, was successful in 12 out of 15 cases. Blood loss, duration of operation and complications were documented. CONCLUSION: CD-Instrumentation in scoliosis surgery offers a long-lasting suffcient correction of the fronal and a good correction of the sagittal plane. Stability of correction is achieved 2 years after operation.

Adolescent↗

[Anthropometric 3D-body scanning in idiopathic scoliosis].

PURPOSE: A new, non-invasive method of 3D-measurement is presented which allows the spatial recording of the entire body surface in scoliotic deformities. The application of the system is examined to raise automatically anthropometric data of patients with scoliosis. METHOD: 32 patients with idiopathic scoliosis were examined (average age 15.3 years, 25 girls and 7 boys, Cobb angles between 11 and 72 degrees). The whole body recording is carried out with a 3D laser scanner. During the measuring process the patient is standing in a frame. Within the measuring time of 15 seconds the body surface is registered by lasers and four cameras. The measured values are converted to a digital 3D model. The resolution is up to 1 mm. On the digital 3D model an automatic calculation of defined anthropometric parameters were carried out. Each patient was measured twice. RESULTS: In all patients a virtual 3D model with a high surface accuracy was obtained. Was the model the typical body asymmetries in scoliotic deformities were visible. The automatic calculation shows a mean deviation of the second measurements between 0.23 and 0.71 cm. The reproducibility depended on the type of the measured parameters. CONCLUSIONS: The laser scanning system allows a rapid, touchless and accurate 3D measurement of the whole body in scoliotic deformities. To determine anthropometric parameters the reproducibility of the automatic calculation is sufficient in most parameters.

Adolescent↗