1/5. Total intravenous anaesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta.osteogenesis imperfecta is a genetically determined rare disease of the connective tissue, associated with abnormalities of type 1 collagen. The primary bone lesion is the lack of normal ossification of the endochondrial bone. patients with osteogenesis imperfecta present several problems for anaesthetists. They have a tendency to develop malignant or non-malignant hyperthermia. During laryngoscopy and tracheal intubation, the mandible, teeth and cervical spine may be fractured or injured, and mucosal bruising or bleeding may occur. Renal or ureteral stones are common. The main problems are thus with airway control and intubation, and the risk of anaesthetic agents triggering malignant hyperthermia. We describe the successful anaesthetic management of a patient with osteogenesis imperfecta, undergoing nephrolithotomy and ureterolithotomy with total intravenous anaesthesia including propofol, remifentanil and cisatracurium, using an intubating laryngeal mask.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/5. Lacticacidosis after short-term infusion of propofol for anaesthesia in a child with osteogenesis imperfecta.We describe the case of a 7-year-old boy with osteogenesis imperfecta, who underwent anaesthesia with propofol-fentanyl-nitrous oxide-suxamethonium for orthopaedic surgery of a distal femur fracture. He developed lacticacidosis after short-term propofol infusion (150 min, mean infusion rate 13.5 mg.kg-1.h-1) associated with a prolonged recovery time without serious haemodynamic changes. The highest lactate concentration was 9.2 mmol.l-1 at 160 min after discontinuation of propofol. There was no significant increase in body temperature. The boy fully recovered.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
3/5. Anaesthesia in a parturient with osteogenesis imperfecta.A 20-yr-old primigravida at 38 weeks gestation with premature rupture of membranes and evidence of fetal distress required urgent Caesarean section. The diagnosis of osteogenesis imperfecta had been made 4 days earlier. The patient refused a regional anaesthetic. Difficulties in the administration of general anaesthesia to patients with osteogenesis imperfecta were recognized and managed accordingly. These problems are discussed and the importance of early antenatal assessment of such patients by the department of anaesthesia is emphasized.- - - - - - - - - - ranking = 0.4keywords = anaesthesia (Clic here for more details about this article) |
4/5. Occurrence of malignant hyperpyrexia in a patient with osteogenesis imperfecta.Probable malignant hyperpyrexia (MH) developed and was successfully treated in a 20-yr-old man during anaesthesia for reduction of a fractured mandible. The sister of this patient had died after an anaesthetic at the age of 14 yr, but malignant hyperpyrexia was not suspected. Subsequent enquiries revealed that the patient and his sister both had osteogenesis imperfecta. This case illustrates the infrequently reported association of malignant hyperpyrexia with osteogenesis imperfecta, and the difficulties in obtaining an adequate personal and family history of previous anaesthetics.- - - - - - - - - - ranking = 0.2keywords = anaesthesia (Clic here for more details about this article) |
5/5. osteogenesis imperfecta and malignant hyperthermia. Is there a relationship?We describe a patient with osteogenesis imperfecta who developed tachycardia, metabolic and respiratory acidosis (pH 7.14, PCO2 8.4 kPa, BE -8.5 mmol.l-1) and hyperthermia up to 40 degrees C during anaesthesia with barbiturates, fentanyl, pancuronium, and nitrous oxide. malignant hyperthermia was suspected and the patient treated accordingly. Two years later the in-vitro contracture test for malignant hyperthermia was completely normal. We conclude that hypermetabolism in patients with osteogenesis imperfecta is due to unknown mechanisms other than malignant hyperthermia.- - - - - - - - - - ranking = 0.2keywords = anaesthesia (Clic here for more details about this article) |