Oral Management of Steinert's Disease and Role of Anxiolysis.

BACKGROUND: Myotonic dystrophy type I (DM1) is a genetic autosomal dominant disorder; malignant hyperthermia is a possible complication. It may occur following administration of some halogenated general anesthetics, muscle relaxants, or surgical stress. AIM: The purpose of this case report is to evaluate the dental management of patients with Steinert's disease. CASE REPORT: The patient needed dental extraction. A locore-gional paraperiosteal anesthesia was performed using bupiva-caine without vasoconstrictor and sedation with nitrous oxide. The syndesmotomy of the elements 3.1, 4.1, and 4.2 was executed. The elements were dislocated through a straight lever and avulsed with an appropriate clamp. The socket was courted, washing with saline solution, inserting a fibrin sponge, and applying sutures (silk 3-0). CONCLUSION: Dental treatment of the patient with Steinert's dystrophy must be carried out under a hospital environment and the use of local anesthetic without vasoconstrictor and with use of nitrous oxide; anxiolysis is recommended. CLINICAL SIGNIFICANCE: This case report describes the precautions to perform oral surgery in patients with Steinert's disease and emphasizes the role of anxiolysis to avoid episodes of malignant hyperthermia.

Perioperative thermoregulation and heat balance.

Core body temperature is normally tightly regulated to within a few tenths of a degree. The major thermoregulatory defences in humans are sweating, arteriovenous shunt vasoconstriction, and shivering. The core temperature triggering each response defines its activation threshold. General anaesthetics greatly impair thermoregulation, synchronously reducing the thresholds for vasoconstriction and shivering. Neuraxial anaesthesia also impairs central thermoregulatory control, and prevents vasoconstriction and shivering in blocked areas. Consequently, unwarmed anaesthetised patients become hypothermic, typically by 1-2°C. Hypothermia results initially from an internal redistribution of body heat from the core to the periphery, followed by heat loss exceeding metabolic heat production. Complications of perioperative hypothermia include coagulopathy and increased transfusion requirement, surgical site infection, delayed drug metabolism, prolonged recovery, shivering, and thermal discomfort. Body temperature can be reliably measured in the oesophagus, nasopharynx, mouth, and bladder. The standard-of-care is to monitor core temperature and to maintain normothermia during general and neuraxial anaesthesia.

[Malignant hyperthermia - problem in dental surgery. An introductory report]. / Hipertermia zlosliwa - problem w chirurgii stomatologicznej. Doniesienie wstepne.

Malignant hyperthermia is a genetic defect of uncontrolled hypermetabolic skeletal muscle response to anesthetic triggering drugs. Some congenital myopathies are regarded as risk increasing factors. The use of volatile anaesthetics or suxamethonium (succinylcholine) in patients who are predisposed to malignant hyperthermia leads to an increase in Ca2+ release from sarcoplasmic reticulum, which in turn causes a set of biochemical and clinical symptoms, which can be a cause of death, if dantrolene is not administered adequately. The aim of the study was to draw attention to the problem of malignant hyperthermia, which is hardly ever described in Polish literature, and requires the necessity of intensifying the cooperation between the dentist and specialists from other medical fields. The origin of the article was a case of congenital myopathy with recognized malignant hyperthermia in an 18-year-old patient, in whom surgical extraction of teeth was indicated. The course of diagnostics and treatment showed once more that contemporary medicine is in need of holistic approach, and in consequence, promising and effective cooperation of many specialists.

[Fulminant MH crisis during the ninth general anaesthesia]. / Kasuistik: Fulminante Maligne Hyperthermie - Untypischer Verlauf während der 9. Allgemeinanästhesie.

Malignant hyperthermia (MH) is a rare, often life-threatening complication of general anaesthesia. The timely diagnosis of an MH crisis in onset, as a prerequisite for successful therapy, can be difficult for the anaesthetist because of the few and non-specific early symptoms. This is even more so in patients in whom anaesthesia with MH trigger substances has already been performed in the past without any particular complications so leading to a false sense of security with regard to MH sensitivity. The case presented is a healthy young man with a congenital cleft lip, jaw and palette who developed a fulminant MH crisis during his ninth general anaesthesia. Post-operative research into the course of the previous anaesthesias revealed signs of MH crises which however proceeded abortively and were therefore unnoticed. In the case presented, the diagnosis was additionally complicated by the untypical course of the early symptoms. Tachycardia which in 80 % of cases is described as the first symptom of an MH crisis in onset, was at first completely absent and was only moderately pronounced in the full clinical picture of MH. On the other hand, a steady increase in body temperature, a cardinal symptom which usually appears later, was registered early. The suspected diagnosis of MH was then finally confirmed in the fourth hour after start of anaesthesia on the basis of the repeatedly increased end expiratory CO(2) levels. These could not otherwise be explained although several respiratory corrections were performed. Despite immediate MH specific therapy, the crisis developed in the following hour into the full clinical picture: maximum temperature of 41.4 degrees C, end expiratory CO(2) 100mm Hg, consumptive coagulopathy, acute renal failure and shock (systolic blood pressure < 50mm Hg, heart rate 115/minute). After 2 hours of specific intensive therapy, the patient was finally stabilized and transfer to the intensive care unit was possible. 24 hours after the event, the patient was could be extubated without any complications and 2 days later, he was transferred to the normal ward. The intra-operative diagnosis of MH was confirmed 3 days later by means of genetic analysis. Two mutations of the RYR1 gene were identified.

[Anesthetic management of a patient with mental disabilities as well as the past history of heat stroke and neuroleptic malignant syndrome].

A 22-year-old man with mental disabilities with the history of neuroleptic malignant syndrome and heat stroke was scheduled for dental treatment under general anesthesia. Heat stroke and neuroleptic malignant syndrome are related to malignant hyperthermia. We suggested the patient and family to undergo preoperative screening tests for malignant hyperthermia susceptibility, but they rejected. We selected slow induction using nitrous oxide, oxygen and sevoflurane to prevent excitement and anxiety for placing a catheter in a peripheral vein. We were very cautious in primary symptoms of malignant hyperthermia, i.e., tachycardia, increased end-tidal carbon dioxide, and rigidity of masseter. In the perioperative period, no complications occurred.

Succinylcholine-induced masseter muscle rigidity during bronchoscopic removal of a tracheal foreign body.

Masseter muscle rigidity during general anesthesia is considered an early warning sign of a possible episode of malignant hyperthermia. The decision whether to continue or discontinue the procedure depends on the urgency of the surgery and severity of masseter muscle rigidity. Here, we describe a case of severe masseter muscle rigidity (jaw of steel) after succinylcholine (Sch) administration during general anesthetic management for rigid bronchoscopic removal of a tracheal foreign body. Anesthesia was continued uneventfully with propofol infusion while all facilities were available to detect and treat malignant hyperthermia.

Continuous infusion propofol general anesthesia for dental treatment in patients with progressive muscular dystrophy.

Progressive muscular dystrophy may produce abnormal reactions to several drugs. There is no consensus of opinion regarding the continuous infusion of propofol in patients with progressive muscular dystrophy. We successfully treated 2 patients with progressive muscular dystrophy who were anesthetized with a continuous infusion of propofol. In case 1, a 19-year-old, 59-kg man with Becker muscular dystrophy and mental retardation was scheduled for dental treatment under general anesthesia. General anesthesia was maintained by a continuous infusion of 6-10 mg/kg propofol per hour and an inhalational mixture of 67% nitrous oxide and 33% oxygen. No complications were observed during or after the operation. In case 2, a 5-year-old, 11-kg boy with Fukuyama type congenital muscular dystrophy and slight mental retardation was scheduled for dental treatment under general anesthesia. General anesthesia was maintained with a continuous infusion of 6-12 mg/kg propofol per hour and an inhalational mixture of 0.5-1.5% sevoflurane in 67% nitrous oxide and 33% oxygen. No complications were observed during or after the operation. It is speculated that a continuous infusion of propofol in progressive muscular dystrophy does not cause malignant hyperthermia because serum levels of creatine phosphokinase and myoglobin decreased after our anesthetic management. Furthermore, our observations suggest that sevoflurane may have some advantages in patients with progressive type muscular dystrophies other than Duchenne muscular dystrophy and Becker muscular dystrophy. In conclusion, our cases suggest that a continuous infusion of propofol for the patients with progressive muscular dystrophy is a safe component of our anesthetic strategy.

Guidelines for the use of local anesthetics in the dental treatment of patients who are susceptible to malignant hyperthermia.

There is controversy regarding the safe use of local anesthetics in patients who are susceptible to malignant hyperthermia undergoing dental treatment. This article reviews the literature for reports of malignant hyperthermia reactions under local and general anesthesia, and suggests a protocol for the management of these patients in the dental office.

[Anesthetic management of a child with Schwartz-Jampel syndrome].

A 6-year-old child with Schwartz-Jampel syndrome (SJS) underwent tenotomy of bilateral lower limbs under general anesthesia. Patient with SJS has problems such as difficulty of intubation owing to microstomia and jaw muscle rigidity, and is susceptible to malignant hyperthermia by using volatile inhalation anesthetics. In this case, we used a laryngeal mask for airway management and anesthesia was maintained with inhalation of nitrous oxide and continuous i.v. infusion of propofol with caudal block, and his clinical course was uneventful.

[Anesthetic management for a patient with Charcot-Marie-Tooth disease using propofol and nitrous oxide].

A 65-year-old woman who had been diagnosed as having Charcot-Marie-Tooth disease (CMTD) 8 years ago and was scheduled to undergo excision of a spinal tumor in the prone position. General anesthesia using propofol was selected as the anesthetic method in order to avoid possible occurrence of malignant hyperthermia due to inhalation anesthetics. The patient was given 80 mg of propofol for anesthetic induction, and then propofol was infused at a rate of 4-5 mg.kg-1.h-1 with intermittent administration of fentanyl (total dose of 0.25 mg) for anesthetic maintenance. Vecuronium 4 mg was injected for intratracheal intubation, and then vecuronium 1 mg was injected at 50 min intervals. The operation proceeded uneventfully. The necessary time for anesthesia was over 460 minutes. There was no delay in wakening, and the patient experienced no problems in the postoperative course. Intravenous anesthesia using propofol is thought to be a safe and effective method of anesthesia for patients with CMTD.

Malignant hyperthermia in the oral and maxillofacial surgery patient: an update.

Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases, such as halothane, sevoflurane, desflurane, the depolarizing muscle relaxant succinylcholine, and, rarely in humans, to stresses, such as vigorous exercise and heat. The syndrome is likely to be fatal if untreated. Early recognition of the signs of MH provides the clinical diagnostic clues. Diagnostic testing relies on assessing the in vitro contracture response of biopsied muscle to halothane, caffeine, and other drugs. Dantrolene sodium is a specific antagonist of the pathophysiologic changes of MH and should be available wherever general anesthesia is administered. The prevention and treatment of acute episodes of this disorder is of paramount importance to the oral and maxillofacial surgeon. The management of such patients in the oral and maxillofacial surgery setting and the recent advances in the field of MH are presented.

Congenital insensitivity to pain with anhydrosis: report of a family case.

Congenital Insensitivity to pain with anhydrosis (CIPA) is a rare inherited disease. It is classified as hereditary sensory and autonomic neuropathy type IV. Pain insensitivity and autonomic deficits are present, but touch and pressure sensitivity are unimpaired. Mental retardation is usually present. We report a family case of a 5 years old girl and 2 years old boy with congenital insensitivity to pain, while discussing the clinical features and the anesthetic strategy of such patients. Patients with Congenital Insensitivity to Pain with anhydrosis may undergo surgery because of susceptibility to trauma due to absence of pain. The clinical features may intrinsically possess anesthetic challenges.

Propofol anesthesia in the malignant hyperthermia susceptible patient.

The anesthetic technique chosen for a malignant hyperthermia (MH) susceptible patient should include drugs that do not trigger MH, while providing stress-free conditions. This case report describes a MH susceptible patient who was successfully induced and maintained with propofol for third molar extractions while under general anesthesia. Based on this case report, and the other relative few in the literature, it appears unlikely that propofol will trigger an episode of MH. Propofol provides the anesthetist with an alternative for inducing MH susceptible patients, but continued experience is necessary to document its safety and efficacy in these patients.

Malignant hyperpyrexia: successful management following oral surgery.

Malignant hyperpyrexia (M.H.) is a rare, important and frequently fatal disease provoked by inhalational anaesthetic agents and other drugs. It is manifested most frequently by tachycardia, a rise in core body temperature, muscle stiffness and is associated with profound metabolic effects. It may be transmitted in a predictable familial dominant fashion, but sporadic cases in whom this transference cannot be demonstrated do occur. Susceptibility may be revealed on questioning regarding previous anaesthetic exposure. One such unexpected but successfsully treated case of M.H. is described in detail and the current management and investigation is discussed.

Dantrolene sodium in the management of patients at risk from malignant hyperthermia.

Malignant hyperthermia is a dominantly inherited, usually subclinical, disease that occurs in individuals who have an underlying muscular disorder and connotes the gravest possible consequences. When it occurs, it is usually during the use of muscle relaxants in anesthesia and potent anesthetic agents such as halothane. Patients at risk must be identified through careful history and screening procedures; however, a patient susceptible to this condition may have had general anesthesia in the past without complications. A careful monitoring regimen must be established for the procedure and some means of cooling the patient must be ready in case pyrexia occurs. Dantrolene sodium is currently the preferred drug for prevention of the syndrome and may be valuable for its treatment.

Malignant hyperthermia in a patient receiving nitrous oxide.

A patient who had malignant hyperthermia that apparently was triggered by nitrous oxide anesthesia even though he was premedicated with dantrolene sodium was successfully managed on a separate occasion using dantrolene without nitrous oxide.

Preoperative determination of susceptibility to malignant hyperthermia.

A careful individual and family history is of utmost importance in evaluating MH susceptibility. Patients having myopathy with or without a significant family history of MH must be suspected of having the condition. The initial evaluation should include routine blood studies and CPK determinations in addition to an electrocardiogram. Full evaluation differentiating rigid from nonrigid types must include a muscle biopsy for microscopic and contraction studies. Platelet ATP depletion with exposure to halothane shows promise as a prescreening test. Susceptible patients can be given both general and local anesthetics if the surgeon and support personnel have a thorough understanding of MH and if proper precautions have been taken and preparation of facilities has been made.

[The hereditary motor-sensory neuropathy Charcot-Marie-Tooth disease: anesthesiologic management--case report with literature review]. / Die hereditäre motorischsensible Neuropathie: Charcot-Marie-Tooth-Erkrankung. Anästhesiologisches Management--ein Fallbericht und eine Literaturübersicht.

A 53-year-old woman diagnosed as having hereditary motor-sensory neuropathy Charcot-Marie-Tooth (CMT) disease Type 2, underwent inguinal hernia surgery. In this patient CMT disease was manifested as distal muscle weakness and wasting. Anaesthetic experience with patients who have CMT disease is limited. Association to malignant hyperthermia is very unlikely although there is one case report that shows that there could be a relationship. We describe a total intravenous anaesthesia (TIVA) protocol with propofol and alfentanil without any muscle relaxants after fiberoptic intubation. The patient made an uneventful recovery and was discharged from the hospital on the fourth postoperative day. TIVA was a safe technique in this patient and should be considered as an alternative for patients presenting with CMT disease.