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SPA Newsletter.
Out and About the ASAControversies in Malignant Hyperthermia, Cases from the MH HotlineModerated and reviewed by: Ronald S. Litman, DOThe panel "Controversies in Malignant Hyperthermia, Cases from the MH Hotline" featured a number of cases of pediatric anesthesia. Moderated by this author, the panel included Henry Rosenberg, MD, Richard Kaplan, MD, Charles Watson, MD, and Joe Tobin, MD. Dr. Rosenberg, who was one of the co-founders of MHAUS in 1981 and has been a malignant hyperthermia (MH) hotline consultant for over 20 years, led off the presentation by describing the organization and functions of the Malignant Hyperthermia Association of the United States (MHAUS). The emergency hotline telephone number is 1-800-MH-HYPER (644-9737), and the number for general non-urgent questions and consultations is 1-800-98-MHAUS (986-4287). MHAUS is a not-for-profit organization formed in 1981. Its Board of Directors consists of laypersons and professionals. A group of knowledgeable physicians makes up the Professional Advisory Council, which sets the policies and procedures for perioperative MH management. The MH hotline is managed by 26 volunteer physicians that provide around-the-clock consultation for known or suspected MH cases. Its subsidiary organizations include the Neuroleptic Malignant Syndrome Information Service (NMSIS) and the North American Malignant Hyperthermia Registry (NAMHR), which collates and analyzes data on patients with suspected or proven episodes of malignant hyperthermia. The NAMHR is directed by Dr. Barb Brandom at Children's Hospital of Pittsburgh. Major supporters of MHAUS and its subsidiaries include Proctor & Gamble Pharmaceuticals, ASA, AANA, and individuals and anesthesia departments. Dr. Rosenberg characterized MHAUS as a not-for-profit organization dedicated to reducing the morbidity & mortality of MH and other heat-related disorders by improving medical care related to MH, providing support information for patients, and improving the scientific understanding and research related to MH and other kinds of heat-related syndromes. He described the various MHAUS services, which include informational brochures, hotline consultation, web site information, a quarterly newsletter ("The Communicator"), publication of treatment protocols, procedure manuals, in-service materials, small grants and awards, and educational presentations. Dr. Watson took the podium next and described the hotline process and the MHAUS quality improvement (QI) program. Dr. Watson is Chairman of the Anesthesia Department at Bridgeport Hospital in Connecticut, and Co-Director of the surgical intensive care unit there. He has been a hotline consultant for MHAUS for over 20 years, and is Chair of the MH hotline Q/I committee. Dr. Watson discussed the function of the MH hotline, which serves as a voluntary crisis hotline for medical staff on the front line. The hotline consultants provide expertise regarding a rare problem. Trained nurses and physicians are available around-the-clock to answer urgent calls and general questions about management of suspected or proven MH cases. Hotline consultants are "on-call" for two consecutive weeks, two to four times per year. New consultants are matched with an experienced consultant and mentored during the first year of service. Dr. Watson described the MHAUS Q/I process, which consists of a quarterly review of advice given by hotline consultants. Although individual management styles and opinions differ, overall advice is consistently good. To conform to HIPPA guidelines, patient information is noted but not shared without patient permission. These preliminary discussions were followed by the presentation of previous hotline cases and opinions from the experts on the most appropriate management. During and following each case, the audience was encouraged to ask questions and offer opinions. The first case was submitted and discussed by Dr. Richard Kaplan, Professor of Anesthesiology and Pediatrics at Children's National Medical Center in Washington, DC. Dr. Kaplan, who has also served as a hotline consultant for over 20 years, presented the case of a 10 y.o., 40-kg, healthy female that underwent ptosis surgery. Following premedication with oral midazolam, induction of general anesthesia with sevoflurane and N2O, and endotracheal intubation without muscle relaxants, the surgery progressed uneventfully until one hour later when the child developed PVCs along with hypoxia and hypercarbia. Despite an increase in minute ventilation, hypercarbia persisted, and the child's temperature began to rise. The sevoflurane was discontinued and the propofol was begun. While waiting for the results of an arterial blood gas, the patient was noted to develop generalized muscle rigidity, which triggered the provisional diagnosis of MH and administration of dantrolene. Soon thereafter, the patient developed bradycardia and cardiac arrest, which required over one hour of cardiac resuscitation. Although a sinus rhythm was then established, the child developed rhabdomyolysis and cerebral edema, leading to her death several days after the episode. Dr. Kaplan emphasized that fulminant, acute MH, although rare, remains a cause of perioperative mortality, despite optimal prevention and treatment strategies. He also discussed the role of the anesthesiologist in helping to diagnose MH after the patient's death, and appropriate resources for counseling the family. Dr. Joseph R. Tobin, Professor of Anesthesiology at Wake Forest University in Winston-Salem, North Carolina led a discussion on the relationship between heat stroke, exercise-induced rhabdomyolysis, and MH susceptibility. Dr Tobin, a hotline consultant for more than 2 years, presented a series of cases of patients with heat stroke who were also proven to be MH susceptible. A discussion ensued on the current anesthetic management of patients with a history of heat stroke or exercise-induced rhabdomyolysis. Additional case discussions for the panel included a woman that developed masseter muscle rigidity during rapid-sequence induction of general anesthesia for an emergency C-section, guidelines for ambulatory surgery centers and MH susceptible patients, and management of high fever in critically ill patients. Table of Contents
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