Cases reported "Scoliosis"

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1/20. Combined spinal-epidural in the obstetric patient with Harrington rods assisted by ultrasonography.

    We describe a patient with severe scoliosis, which had been corrected partially with Harrington rods, who requested epidural analgesia for labour. With no palpable landmarks, the use of ultrasound enabled identification of the vertebral midline and allowed provision of regional anaesthesia.
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2/20. Unsuspected concentric tracheal rings in a 14-year-old with scoliosis.

    A 14-yr-old boy was first found to have tracheal stenosis when anaesthesia was induced for extensive scoliosis surgery in the prone position. There are no guidelines for airway management under these conditions. We describe how we managed the problem and suggest some useful modifications.
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3/20. Jet ventilation for anterior paediatric scoliosis surgery.

    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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4/20. Management of respiratory deterioration in a pregnant patient with severe kyphoscoliosis by non-invasive positive pressure ventilation.

    The problem of kyphoscoliosis in combination with pregnancy is uncommon and published cases are rare. Until now, little and controversial information on the outcome, optimal management and course of pregnancy in patients with kyphoscoliosis has been available. The majority of maternal deaths seem to be attributed to cardiorespiratory failure, while obstetric complications account for relatively few complications. We present the case of a 34-year old pregnant woman with congenital kyphoscoliosis and a forced vital capacity (FVC) of about one liter. A further deterioration of lung function was expected. In fact, severe limitations in exercise capacity (bed rest), fatigue and hypersomnolence, as well as a severe increase in pulmonary hypertension occurred during the second and third trimester. Nasal intermittent positive pressure ventilation (NIP-PV) with bilevel positive airway pressure (BiPAP) was started in the 20th week of gestation and adapted throughout pregnancy. Nasal BiPAP was well-tolerated and corrected exercise tolerance, fatigue and nocturnal oxygen desaturations. At 32 weeks of gestation, the patient was admitted for an elective Caesarean section under combined spinal-epidural anaesthesia with ongoing NIPPV, and delivered a healthy baby. Home nocturnal ventilatory support was continued as nocturnal episodic desaturations were also assessed during the postpartum period. At time of discharge, the patient's exercise capacity and lung function were nearly equal to levels before pregnancy. We conclude that pregnancy in selected kyphoscoliotic patients with severe limitations in lung function is relatively safe for both the mother and the child when NIPPV is used for overcoming respiratory deterioration and for preventing further cardiorespiratory failure.
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5/20. King syndrome: a genetically heterogenous phenotype due to congenital myopathies.

    We report on a patient with myopathy, kyphoscoliosis, joint contractures, and a facial appearance consistent with King syndrome. Unlike other reported cases, our patient had hyperextensible joints, normal stature, and pectus excavatum. The cardiac ventricles, aorta, and pulmonary artery were dilated. malignant hyperthermia did not occur under anaesthesia although there was a transient increase in CK levels. Muscle bulk and tone were significantly decreased but collagen and elastin fibres were normal. The variable clinical presentation of King syndrome suggests that the manifestations are caused by different congenital myopathies and in all cases there is probably an increased risk of malignant hyperthermia.
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6/20. Rocuronium for muscle relaxation in two children with Friedreich's ataxia.

    Friedreich's ataxia is a rare hereditary neurodegenerative disease caused by a defect in the gene that encodes a mitochondrial protein called frataxin. We report the use of rocuronium 0.6 mg kg(-1) in two adolescent girls with Friedreich's ataxia undergoing propofol-sufentanil-oxygen-air anaesthesia for spinal surgery. Neuromuscular transmission was monitored using acceleromyography, and onset and recovery times were recorded. The clinical duration of rocuronium was comparable to that of children without neuromuscular disease (25% recovery T(1)=44 and 24 min for patients 1 and 2 respectively).
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7/20. Anaesthesia for a patient with Leigh's syndrome undergoing surgery for scoliosis.

    Anaesthesia for patients with Leigh's syndrome has rarely been reported. Leigh's syndrome or subacute necrotizing encephalomyelopathy is a neurodegenerative disorder of infancy or childhood. Acute exacerbation with respiratory failure may accompany surgery and general anaesthesia. In this case report we describe the anaesthetic management of a 17 year old patient scheduled for spine surgery.
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8/20. Anaesthesia for caesarean section in spinal muscular atrophy type III.

    We describe the conduct of general anaesthesia for a patient with spinal muscular atrophy Type III (Kugelberg-Welander disease) undergoing elective caesarean section. Apart from a delayed return of skeletal muscle power following non-depolarising neuromuscular blockade the procedure was uneventful. We found no previously published reports of general anaesthesia for caesarean section in this condition in the English language literature. We review the available literature and discuss the potential anaesthetic problems in the management of obstetric patients with this degenerative neuromuscular disorder.
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9/20. Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation.

    BACKGROUND: spinal cord traction caused by a tight filum terminale may be considered a pathogenic mechanism involved in the development of syringomyelia, the Chiari malformation (type I) and scoliosis. Section of the filum terminale is proposed as a useful surgical approach in these conditions. methods: Between April 1993 and July 2003, a total of 20 patients (8 men and 12 women) with a mean age of 33.5 years underwent section of the filum terminale with or without opening of the dural sac through a standard sacrectomy. Eight patients suffered from scoliosis, 5 from syringomyelia, 2 from Chiari malformation and 5 with a combination of these conditions. FINDING: After section of the filum terminale, patients with syringomyelia showed an early clinical improvement of dysaesthesia, thermo-anaesthesia, hypo-aesthesia and walking difficulties. Rising of the medullary conus was also observed. In patients with scoliosis, back pain improved dramatically and a curve reduction was noticed, although progression of the curve was observed in one case. In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared. CONCLUSIONS: Section of the filum terminale is a useful strategy in the treatment of scoliosis, syringomyelia and the Chiari malformation, and offers a new aetiological basis for the understanding of these three disorders.
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10/20. scoliosis in Steinert syndrome: a case report.

    BACKGROUND CONTEXT: Steinert syndrome is described as an autosomal dominant condition characterized by progressive muscular wasting, myotonia, musculoskeletal manifestations and rare spinal defects. Little is reported about spinal deformity associated with this syndrome. PURPOSE: We present a patient with Steinert syndrome complicated by scoliosis. In the literature on muscular dystrophy, other than Duchenne, little mention is given to the problem of scoliosis in general and its treatment in particular. STUDY DESIGN: A case report of a patient with Steinert syndrome associated with thoracic scoliosis and hypokyphosis is presented. methods: A 17-year-old boy presented with King type II right thoracic scoliosis (T5-T11, Cobb angle of 40 degrees) and hypokyphosis--10 degrees. He was treated with posterior stabilization and instrumentation at level T3-L2 with a postoperative correction of the scoliotic curve to 20 degrees. Histopathologic examination of the muscles confirmed the diagnosis of Steinert myotonic dystrophy. RESULTS: At 30-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. CONCLUSIONS: scoliosis in Steinert syndrome shares the characteristic of an arthrogrypotic neuromuscular curve and demands the extensive soft tissue release for optimal surgical correction. Intraoperative observations included profound tissue bleeding, abnormally tough soft tissues and a difficult recovery from anaesthesia.
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