Zusammenfassung
Störungen der Muskulatur und des peripheren Nervensystems werden als neuromuskuläre
Erkrankungen (NME) bezeichnet. Bedeutsam für die Anästhesie sind diese Erkrankungen,
da sie einerseits sehr unterschiedliche Störungen verursachen können und andererseits
mit teilweise hohen Risiken bei Allgemeinanästhesien behaftet sind. Probleme entstehen
vorallem durch den Einfluss von Succinylcholin und volatilen Anästhetika, aber auch
durch Opiate, nichtdepolarisierende Muskelrelaxanzien und intravenöse Anästhetika.
Bei Narkosen auftretende Probleme lassen sich als Maligne Hyperthermie, Maligne Hyperthermie-ähnliche
Symptome und primäre oder sekundäre krankheitsbedingte Veränderungen klassifizieren.
Diese Störungen können vorallem zu respiratorischen und kardialen Einschränkungen,
autonomen Störungen sowie hypothermen und hyperthermen Reaktionen führen. Das perioperative
Management muss daher sorgfältig auf den jeweiligen Patienten abgestimmt werden um
eine größtmögliche Sicherheit zu gewährleisten. Zu den obligaten Voruntersuchungen
gehören EKG, Echokardiographie, Lungenfunktionsuntersuchungen einschließlich arterieller
Blutgasuntersuchung, Röntgen-Thorax, Neuro-Status und erweiterten Laboruntersuchungen
(z. B. CK und Myoglobin). Bei der Prämedikation muss auf atemdepressive Medikamente
verzichtet werden. Regionale Narkoseverfahren lassen sich insbesondere bei respiratorischen
und kardialen Einschränkungen vorteilhaft einsetzen. Bei Allgemeinanästhesien sollten
alle erlaubten Medikamente in der geringstmöglichen Dosierung gegeben werden. Auf
volatile Anästhetika sollten bei den meisten Erkrankungen verzichtet werden, Succinylcholin
ist grundsätzlich kontraindiziert (Ausnahme: Myasthenia gravis). Neben dem üblichen
Monitoring sollte die Indikation zur invasiven Blutdruckmessung grosszügig gestellt
werden, die zudem engmaschige Blutgasanalysen erleichtert. Relaxometrie und Temperaturmessung
sind obligat. Postoperativ sollte vor allem auf Normothermie und einen ausgeglichenen
Elektrolyt- und Säure-Basen-Haushalt geachtet werden. Eine Verlegung aus dem überwachten
Bereich darf erst bei stabiler respiratorischer Funktion erfolgen.
Abstract
Disorders of skeletal muscle and peripheral nervous system are collectively called
neuromuscular disorders (NMD). Important for anesthesia is that these disorders show
various symptoms and have a high risk during general anesthesia. Especially administration
of succinylcholine and volatile anaesthetics may cause problems. Under special circumstances
opioids, nondepolarising muscle relaxants and intravenous anaesthetics can interfere
with this kind of disorder, too. Complications during and after anaesthesia may result
in malignant hyperthermia, malignant hyperthermia-like reactions and primary or secondary
changes relating to the underlying NMD. These include cardiac and respiratory problems,
dysautonomia as well as hypothermia or hyperthermia. Thus the perioperative management
must be determined individually to assure the best possible safety for each patient.
Preoperative examination such as ECG, echocardiography, respiratory function test
including arterial blood-gas analysis, x-ray of the thorax, neurological status, and
extended serum chemistry (such as CK and myoglobin) needs to be done. For premedication
no drugs suppressing respiratory function should be administered. Regional anesthesia
should be used whenever possible, especially in patients with respiratory and cardiac
problems. The dosage of all recommended drugs should be as low as possible. Volatile
anaesthetics should not be administered in the majority of NMD and succinylcholine
is contraindicated, with the exception of myasthenia gravis. Additionally to the usual
intraoperative monitoring, the invasive measurement of blood pressure allows frequent
blood-gas analysis. It is obligate to monitor neuromuscular function and body temperature.
During recovery special attention should be paid to maintain normal body temperature
and electrolytes and acid-base status. The discharge of the patient from the recovery
area to the normal ward should be performed only after respiratory function is normalized.
Schlüsselwörter
Myopathie - Maligne Hyperthermie - Narkosekomplikation - Perioperatives Management
Key words
Myopathy - Malignant hyperthermia - Anaesthetic risk - Perioperative treatment
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Dr. Christoph P. Baur
Universitätsklinik für Anästhesiologie, Universität Ulm
Steinhövelstraße 9
89070 Ulm
Email: christoph.baur@medizin.uni-ulm.de