1/5. Fatal pulmonary haemorrhage during anaesthesia for bronchial artery embolization in cystic fibrosis.Three children with cystic fibrosis (CF) had significant pulmonary haemorrhage during anaesthetic induction prior to bronchial artery embolization (BAE). Haemorrhage was associated with rapid clinical deterioration and subsequent early death. We believe that the stresses associated with intermittent positive pressure ventilation (IPPV) were the most likely precipitant to rebleeding and that the inability to clear blood through coughing was also an important factor leading to deterioration. Intermittent positive pressure ventilation should be avoided when possible in children with CF with recent significant pulmonary haemorrhage.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/5. Complete staged palliation of hypoplastic left heart syndrome in a child with cystic fibrosis.In recent years, the palliative treatment of hypoplastic left heart syndrome (HLHS) with a three-staged surgical repair has gained widespread acceptance in north america and elsewhere and has significantly improved the life-expectancy of these children. We report on a child in whom the diagnosis of cystic fibrosis (CF) was made shortly after the first-stage palliation (modified Norwood procedure), and in which surgical palliation was successfully completed with second- (bidirectional Glenn) and third-stage (fenestrated Fontan) procedures. During this period, the child suffered several CF-related complications, i.e. repeated respiratory failure, meconium ileus and coagulation abnormalities, which required adjustment of the anaesthesia, surgical and intensive care management. Considering the trend to perform surgical corrections of major cardiac defects in the early neonatal period, before concomitant genetic diseases are discovered, paediatric cardiac anaesthesiologists, surgeons and intensivists may be confronted with more cases of open heart surgery in infants with CF in the future.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
3/5. Successful pregnancy and delivery in a young woman with cystic fibrosis and gestational diabetes.We describe a 23-year-old Delta F508 homozygote cystic fibrosis primigravida. At the onset of gestation, she had mild to moderate pulmonary involvement, exocrine pancreatic insufficiency, focal biliary cirrhosis, satisfactory nutritional status and normal fasting and post-prandial glucose blood levels. At 29 weeks, she developed polyhydramnion and gestational diabetes. At 37 weeks, she was delivered of a live 2,980 g boy by caesarean section under epidural anaesthesia. insulin was subsequently discontinued and her pulmonary function improved spontaneously. Neither maternal nor neonatal health problems were observed during the 3-month follow-up.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
4/5. Caesarean section in a patient with cystic fibrosis.Severely impaired pulmonary function due to cystic fibrosis is associated with a poor pregnancy outcome. A 19-year-old primigravida with cystic fibrosis had a forced expiratory volume in one second 34% of predicted and a low body mass index of 17.1 kg/m(2) before pregnancy. During pregnancy she required several admissions to hospital for deteriorating pulmonary function due to infection. She also developed gestational diabetes and required overnight feeding via a gastrostomy tube throughout pregnancy to improve her nutritional status. With close medical attention, pulmonary function tests remained stable. A joint decision was made to deliver the baby by caesarean section at 33 weeks' gestation as this was considered optimal time for mother and baby and to avoid potential catastrophic pulmonary deterioration later in pregnancy. A combined spinal-epidural technique provided adequate anaesthesia. Pulmonary function tests were performed in theatre before and after the spinal injection, as well as two and five hours postoperatively. Despite a spinal anaesthetic block to T5 bilaterally, lung function tests remained stable. An epidural infusion provided good postoperative analgesia. The outcome was successful for mother and fetus.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |
5/5. Caesarean section in a parturient with respiratory failure caused by cystic fibrosis.We describe a 27-year-old primigravida suffering from cystic fibrosis. Her chest was colonised with burkholderia cepacia and she was in respiratory failure for which she required constant nasal intermittent positive pressure ventilation. In view of her rapid deterioration, Caesarean section was performed under epidural anaesthesia at 25 weeks gestation. A live 790-g boy was delivered. Post-operatively she made steady progress for 5 days although still requiring nasal ventilatory support. Thereafter she developed pneumonia and required tracheal intubation and ventilation on the eighth day. Her increasing hypoxaemia and pulmonary hypertension failed to respond to any therapy including inhaled nitric oxide and she died on the tenth postoperative day.- - - - - - - - - - ranking = 0.25keywords = anaesthesia (Clic here for more details about this article) |