1/6. Anaesthetic management of two paediatric patients with Hecht-Beals syndrome.We undertook the anaesthetic management of two children with Hecht-Beals syndrome for orthopaedic surgery under general anaesthesia. Both patients had arachnodactyly, kyphoscoliosis, and multiple congenital joint contractures, but limited mandible excursion was not obvious preoperatively in either, although mental retardation made it difficult for them to cooperate with mouth examination. They had no apparent difficulties with their mouths in daily activities. The anaesthesia records of one patient showed that intubation had been difficult in an earlier procedure. The other patient also had a history of difficult intubation, with slight tearing of the corners of her mouth during an intubation procedure. During slow induction of general anaesthesia with sevoflurane, face mask ventilation was easily performed. We attempted to visualize the larynx under anaesthesia with muscle relaxation, but we were unsuccessful because of the limited mouth opening. After several trials, blind oral intubations were fortunately successful in both patients. There were no postoperative problems with the airway.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/6. 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.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
3/6. 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).- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
4/6. 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.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
5/6. Acute airway obstruction, hypertension and kyphoscoliosis.An elderly woman developed acute respiratory obstruction after choking on a bolus of food. On rigid bronchoscopy no foreign body, or anatomical obstruction was seen, but airway obstruction recurred during emergence from general anaesthesia, and was thought to be functional in nature. A recurring airway obstruction followed, relieved by induction of anaesthesia and by sedation. This was subsequently demonstrated to be caused by a tortuous aorta which impinged intermittently on the anterior tracheal wall, as a result of kyphoscoliosis of the thoracic spine. We attribute the relief of airway obstruction by general anaesthesia to a reduction in arterial blood pressure.- - - - - - - - - - ranking = 0.75keywords = anaesthesia (Clic here for more details about this article) |
6/6. Extradural anaesthesia for caesarean section in achondroplasia.We describe the successful management of a 26-yr-old achondroplastic dwarf undergoing elective Caesarean section under extradural anaesthesia. The patient had marked thoracolumbar kyphoscoliosis and clinical features which suggested that tracheal intubation would prove difficult. Block sufficient for surgery required only 5 ml of 0.5% bupivacaine and, apart from an initial unilateral block and mild intraoperative hypotension, her perioperative course was uneventful.- - - - - - - - - - ranking = 1.25keywords = anaesthesia (Clic here for more details about this article) |