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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied 27 Duchenne muscular dystrophy patients having spinal fusion for scoliosis. One patient died intraoperatively of cardiac arrest; all others have done well with no instances of malignant hyperthermia, postoperative ventilatory system dependence, pneumothorax, persisting infection, neurologic damage, nonunion, or pain. The anesthetic management included primarily intravenous general anesthetics with minimal myocardial depressant effects, avoiding succinylcholine and inhalation agents. Preoperative cardiac studies aided anesthetic management intra-operatively. There was an almost universal sinus tachycardia. Holter monitoring defined 4 of 16 with ventricular premature beats, 4 of 16 with atrial premature beats, and no ventricular tachycardia or atrial flutter or fibrillation. Echocardiogram demonstrated mitral prolapse in 2 of 22, frequent abnormal systolic performance with abnormal shortening fraction less than 28% in 7 of 16, and reduced rate-corrected velocity of fiber shortening in 9 of 15. Afterload was elevated in 7 of 15. The mean forced vital capacity (FVC) preoperatively was 45.3 +/- 15.9% with continuing diminution to 28.7 +/- 14.9% at 3.3 +/- 2.2 years after surgery. The main benefit of surgical stabilization is the relative ease and comfort of wheelchair seating compared with those nonoperated patients who develop progressive deformity. We have not seen lasting improvement or stabilization in FVC following surgery as decreasing function is related primarily to muscle weakness.
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PMID:Spinal fusion in Duchenne muscular dystrophy: a multidisciplinary approach. 158 53

Malignant hyperthermia is a potentially fatal complication of general anesthesia that may occur with greater frequency in ptosis and strabismus surgery. The case of a two-year-old girl who suffered a malignant hyperthermia crisis during strabismus surgery is reported. The pathophysiology, clinical features, treatment and pre-anesthetic diagnosis are reviewed in detail.
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PMID:[Malignant hyperthermia in ophthalmologic surgery]. 307 76

Malignant hyperthermia is a potentially fatal complication of general anesthesia that may occur with greater frequency in some patients with ptosis or strabismus. The history of our knowledge of this recently described condition is outlined. The clinical features, pathophysiology, and differential diagnosis are reviewed in detail. A protocol for the treatment of the acute malignant hyperthermia crisis and guidelines for the management of susceptible patients are suggested. The medicolegal implications of the occurrence of such a crisis are discussed.
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PMID:Malignant hyperthermia. 635 12

Malignant hyperthermia, a relatively recently described entity, is a little-understood disease process usually manifesting as operative or postoperative hyperpyrexia in association with a hypermetabolic state. Specific therapy with procaine (Novocaine) and more recently with a muscle relaxant, dantrolene sodium (Dantrium), has shown itself to be life-saving, and currently diagnosis can be made by muscle biopsy in patients from affected pedigrees. Malignant hyperthermia is a risk in all general anesthetic procedures, particularly squint and ptosis repair, and may even be a consideration with local anesthesia.
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PMID:Malignant hyperthermia. Considerations for the ophthalmologist. 724 23

Malignant hyperthermia is an autosomal dominant disorder with variable expressivity that is caused by a membrane defect in the sarcolemma of myofibrils. A patient with strabismus (esotropia) had tachycardia and masseter muscle rigidity on exposure to succinylcholine chloride and halothane, but because of rapid recognition of the condition and discontinuation of the procedure, the potentially lethal complications of malignant hyperthermia did not develop. A serum creatine phosphokinase level showed a substantial increase above normal. Two weeks later, the patient underwent successful correction of the strabismus under general anesthesia, using morphine sulfate and thiopental sodium without complication. This condition is of interest to ophthalmologists because it occurs with increasing frequency in patients with strabismus and ptosis, and it may be triggered by certain local anesthetic agents often used by ophthalmologists.
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PMID:Suspected malignant hyperthermia in a strabismus patient. A case report. 725 98

The King-Denborough syndrome (KDS) is a congenital myopathy with musculoskeletal abnormalities, and definitely associated with susceptibility to malignant hyperthermia (MH). We present the first report in Japan concerning the management of a KDS patient. A 2-year-old boy was scheduled for cryptorchidopexy. He had some physical signs of KDS, e.g. pectus excavatum, low-set ears, malar hypoplasia, micrognathia, ptosis and down-slanting palpebral fissures. Moreover, his mother and maternal grand-uncle had medical history of MH. Therefore, he was diagnosed as KDS by pediatricians. All of the preoperative examinations, including serum creatine phosphokinase level, are normal. Previous 15 case-reports suggest that in KDS patients MH might be triggered by volatile anesthetics. To avoid the trigger, we maintained the general anesthesia with propofol, fentanyl and vecuronium. During perioperative period, his body temperature was 36.3-38.1 degrees C, and no symptom of MH was observed. It is rare that a diagnosis of KDS is made preoperatively since the MH-induction is involved in the criteria of this syndrome. In the anesthesia of patients who are suspected of KDS from their characteristic features or familial histories, an anesthesiologist should pay attention to prevent MH. The total intravenous anesthesia method appears useful for the management of KDS.
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PMID:[Anesthetic management of the King-Denborough syndrome]. 1134 52

We report on two boys aged 2 and 6 years-old respectively with dysmorphic face, ptosis, down-slanting palpebral fissures, hypertelorism, epicanthic folds, low-set ears, malar hypoplasia, micrognathia, high-arched palate, clinodactyly, palmar simian line, pectus excavatum, winging of the scapulae, lumbar lordosis and mild thoracic scoliosis who present congenital hypotonia, slightly delayed motor development, diffuse joint hyperextensibility and mild proximal weakness. The muscle biopsy revealed minimal but identifiable changes represented by size fiber variability, type I fiber predominance and atrophy, perimysial fibrous infiltration and some disarray of the intermyofibrillary network. These cases correspond to the first Brazilian reports of the King-Denborough syndrome and our objective is increasing the awareness of this disorder as these patients are predisposed to developing malignant hyperthermia.
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PMID:King-Denborough Syndrome: report of two Brazilian cases. 1236 41

Oculopharyngodistal-myopathy (OPDM) is an autosomal dominant, heredofamilial myopathy accompanied with slowly progressive ptosis and extraocular palsy, and weakness of the masseter, facial, and bulbar muscles, as well as distal involvement of the limbs starting around 40 years of age or later. A 54-year-old female with OPDM underwent resection of the uterus for uterus body cancer. We speculated the patient might be at the risk of aspiration pneumonia, prolonged respiratory depression, and malignant hyperthermia, and chose spinal and epidural anesthesia. The operation was performed successfully and the patient was discharged uneventfully.
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PMID:[Anesthetic management of a patient with oculopharyngodistal-myopathy]. 1264 70

An 18 month-old girl was diagnosed as ventricular septal defect (VSD) with mild aortic valve prolapse. She underwent a closure of VSD. Intra-and early postoperative course was uneventful. However, 20 hours after surgery, sudden bradycardia led to cardiac arrest and strong muscle rigidity was seen. Hyperkalemia and metabolic acidosis rapidly progressed and resuscitation was failed. Extracorporeal life support and continuous hemodialysis were initiated, but the patient died with multiple organ failure on 5th postoperative day. Her clinical course supported the diagnosis of delayed onset malignant hyperthermia. Histopathological findings of muscle biopsy were consistent with rhabdomyolysis, and immunopathological stains demonstrated changes as in a Duchenne type muscular dystrophy carrier. Delayed onset malignant hyperthermia is an extremely rare complication of general anesthesia. We should be aware of this lethal condition, which occurs with a certain time lag after surgery, especially when the patient has possible background of myopathy.
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PMID:[Delayed onset malignant hyperthermia after a closure of ventricular septal defect]. 1577 37

Native American myopathy (NAM) [OMIM 255995], a putative autosomal recessive disorder, was first reported in the Lumbee Indians of North Carolina. NAM features include congenital weakness and arthrogryposis, cleft palate, ptosis, short stature, kyphoscoliosis, talipes deformities, and susceptibility to malignant hyperthermia (MH) provoked by anesthesia. This report documents the phenotypic complexity and natural history of this rare congenital disorder in fourteen individuals with NAM. Findings include a previously unreported 36% mortality by age 18. Based on this study, our conservative estimate for prevalence of NAM within the Lumbee population is approximately 2:10,000; however, birth incidence remains unknown.
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PMID:Native American myopathy: congenital myopathy with cleft palate, skeletal anomalies, and susceptibility to malignant hyperthermia. 1855 14


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