1/51. Suspected recurrence of malignant hyperthermia after post-extubation shivering in the intensive care unit, 18 h after tonsillectomy.A 25-yr-old man, subsequently shown to be malignant hyperthermia (MH) susceptible by in vitro contracture testing, developed MH during anaesthesia for tonsillectomy. Prompt treatment, including dantrolene, led to rapid resolution of the metabolic crisis. Eighteen hours later the patient's trachea was extubated in the ICU, when he had been stable and apyrexial overnight. Twenty minutes after extubation, an episode of shivering was followed by the onset of tachycardia, hypertension, tachypnoea and a rapid increase in temperature. recurrence of MH was suspected and the patient was given another dose of dantrolene with good clinical effect. shivering in this patient may have been an indicator or a causative factor of recurrence of MH.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/51. Identification of a novel mutation in the ryanodine receptor gene (RYR1) in a malignant hyperthermia Italian family.malignant hyperthermia (MH) is an inherited autosomal dominant pharmacogenetic disorder and is one of the main causes of death subsequent to anaesthesia. Around 50% of affected families are linked to the ryanodine receptor (RYR1) gene. To date, 19 mutations have been identified in the coding region of this gene and appear to be associated with the MH-susceptible phenotype. Here we report the identification by two independent methods of a novel mutation associated with the MH-susceptible phenotype in the RYR1 gene: the 6488G-->C transversion, resulting in the replacement of the Arg2163 with a proline residue.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
3/51. Suxamethonium-induced rhabdomyolysis in a healthy middle-aged man.A 43-year-old man developed rhabdomyolysis after uvulo- palatopharyngoplasty. After induction with thiopentone and suxamethonium the anesthesia was maintained with halothane. The patient responded to treatment and made an uneventful recovery. In earlier reports of rhabdomyolysis after general anaesthesia with halothane and suxamethonium almost all the patients had malignant hyperthermia (MH) or muscular dystrophy. About 50% of malignant hyperthermia patients carry a mutation in the RYR1 gene. Our patient did not have mutations in the four MH-associated genes tested, but the total amount of different mutations is by now about twenty. Therefore, despite these negative tests rhabdomyolysis may be a sign of subclinical malignant hyperthermia which cannot be ruled out by our investigations. This rare case of rhabdomyolysis in a healthy man suggests careful monitoring of the patient when-ever suxamethonium is used.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
4/51. Fatal malignant hyperthermia--delayed onset and atypical course.A case of malignant hyperthermia (mh) in a 27-year-old man is described. In a first anaesthesia using isoflurane and succinylcholine, the end-tidal CO(2) rose from 39 to 49 mmHg 2.75 h post-intubation and the body temperature rose to 39.8 degrees C 14 h post-intubation but was normal again the next day. In a second anaesthesia using the same medication, the maximal end-tidal CO(2) was 44 mmHg and the body temperature rose to 39 degrees C after 9 h. After 4 days, the fever rose to 40 degrees C, and to 42 degrees C when death occurred 10 days after the second anaesthesia. Masseter spasms or muscle rigidity were never present. According to the death certificate, death was due to multi-organ failure from sepsis. At autopsy, the skeletal muscles were pale and oedematous. histology demonstrated focal necroses in the skeletal muscles, shock kidneys with myoglobin excretion and myoglobin clots in small blood vessels of the lungs. Hence, the postmortem diagnosis "malignant hyperthermia" was established but accusations of medical maltreatment were rejected because of the atypical and protracted clinical course and because uncharacteristic signs of malignant hyperthermia were attributable to the clinically suspected sepsis.- - - - - - - - - - ranking = 3keywords = anaesthesia (Clic here for more details about this article) |
5/51. The diagnostic dilemma of intraoperative hyperpyrexia in a malaria endemic area.malignant hyperthermia (MH) is a serious and feared complication of general anaesthesia. The diagnosis of MH may pose a diagnostic dilemma, as its two most common signs tachycardia and hyperthermia are also common features of infections, transfusion and drug reactions. We report three cases of hyperthermia occurring during anaesthesia posing diagnostic dilemma in which two of the cases appear to be due to malaria. It is therefore suggested that all patients undergoing anaesthesia in a malaria endemic area should be investigated and treated for malaria if indicated preoperatively even when asymptomatic to prevent such diagnostic confusion.- - - - - - - - - - ranking = 3keywords = anaesthesia (Clic here for more details about this article) |
6/51. 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.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
7/51. malignant hyperthermia.malignant hyperthermia refers to covert myopathies, which do not affect the individual during daily life activities, but may lead to life-threatening tachycardia, rigor, labile blood pressure and most importantly high-grade temperature when exposed to general anaesthesia. This conditions is mimicked by thyroid storm, neuroleptic malignant syndrome, phaeochromocytoma and sepsis. We present a presumptive case of malignant hyperthermia.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
8/51. Sevoflurane-induced malignant hyperthermia during cardiopulmonary bypass and moderate hypothermia.A 56-year old man was admitted for elective mitral valve repair and coronary artery bypass surgery due to mitral valve leakage and unstable angina. After induction of anaesthesia he developed a combined metabolic and respiratory acidosis. Different diagnosis were considered and we decided to treat the patient with dantrolene due to suspicion of malignant hyperthermia (MH). The patient received one dose of dantrolene 2,5 mg/kg during cardiopulmonary bypass (CPB) and a second dose of dantrolene 2,5 mg/kg during weaning from CPB. The first arterial blood gas sample taken in the intensive care unit showed relapse of the acidosis and we administered an infusion of 150 mg dantrolene over 3 hours. The patient gradually recovered without sequel and MH was verified by muscle biopsy testing.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
9/51. McArdle's disease and anaesthesia: case reports. review of potential problems and association with malignant hyperthermia.BACKGROUND: McArdle's disease of isolated deficiency in glycogen degradation in skeletal muscles has the potential of creating perioperative anaesthesiological problems; such as hypoglycaemia, rhabdomyolysis, myoglobinuria, acute renal failure and possibly malignant hyperthermia. methods: Eight patients with McArdle's disease were asked about previous surgery, anaesthesia and perioperative problems, and available hospital records were reviewed. Existing literature was reviewed for reports on McArdle's disease and anaesthesia. RESULTS: The eight patients had 35 anaesthesias (23 general anaesthesias, three regional anaesthesias and nine local anaesthesias). Perioperative problems of a non-specific nature were mentioned in three cases of general anaesthesia: two with postoperative nausea/vomiting, and one with an episode of tachycardia and low blood pressure. Three patients were tested for malignant hyperthermia (MH) using the in vitro contracture test (IVCT); two of them with a positive result. The literature search revealed seven case reports of McArdle's disease and anaesthesia. Apart from one report of hyperthermia, pulmonary oedema and rhabdomyolysis; probably not associated with MH, no problems were encountered from the literature search. CONCLUSION: McArdle's disease does not seem to cause severe perioperative problems in routine anaesthetic care. However, measures for preventing muscle ischaemia and rhabdomyolysis should be kept in mind, as well as the potential for these patients to develop postoperative fatigue, myoglobinuria and renal failure. Although no clinical association with malignant hyperthermia has been established, many of these patients can have a positive in vitro contracture test, and simple prophylactic measures, as with malignant hyperthermia, may be recommended if otherwise not contraindicated.- - - - - - - - - - ranking = 12keywords = anaesthesia (Clic here for more details about this article) |
10/51. Transient talipes equinus deformity of bilateral lower limbs following malignant hyperthermia: a case report and review of literature.malignant hyperthermia (MH) is a potentially fatal disease triggered by general anaesthesia. Four cases of compartment syndrome complicating MH have been reported. We report here a case of transient talipes equinus deformity of bilateral lower limbs, a condition similar to compartment syndrome, following MH in a previously healthy pediatric patient. MRI revealed high intensity in the bilateral soleus muscles but not in the gastrocnemius muscles. We discuss the possible mechanisms of the deformity of the lower limbs after the MH crisis.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
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