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we report on the anesthetic management using opioid - free method of a patient with steinert syndrome ( myotonic dystrophy , md ) , autosomal dominant dystrophy which is characterized by consistent contracture of muscle following stimulation .
a myotonic crisis can be induced by numerous factors including hypothermia , shivering , and mechanical or electrical stimulation . in patients with md , hypersensitivity to anesthetic drugs , especially muscle relaxants and opioids ,
may complicate postoperative management . if opioids are employed ( systemic or neuraxial ) , then icu care and continuous pulse oximetry must be considered given the high risk for respiratory depression and aspiration .
patients with md present high sensitivity to the usual anesthetics such as volatile and muscle relaxants ( both depolarizing and nondepolarizing ) .
increased susceptibility to intra- and postoperative complications , it is recommended to introduce opioid - free anesthesia ( ofa ) , for example , with use of dexmedetomidine ( dex ) .
this is a new method of conducting general anesthesia without opioids and is based on concept of multimodal approach to pain management .
a 31-year - old male patient ( 183  cm , 69  kg ) was scheduled for laparoscopic operation of cholecystectomy .
the patient received intravenously ( iv ) : propofol in a dose of 250  mg followed by continuous infusion , rocuronium in a dose of 20  mg , and dex in a loading dose of 0.6  g / kg over 10  minutes followed by continuous infusion of dose of 0.2  g / kg / hour .
the patient exited the operating theatre in a good medical state , with vitals within normal limits and fully regained consciousness .
dex is effective and safe for moderately painful procedures in patients with the elevated risk of respiratory and cardiovascular failure .
we report on the anesthetic management using opioid - free method of a patient with steinert syndrome ( myotonic dystrophy , md ) , autosomal dominant dystrophy which is characterized by consistent contracture of muscle following stimulation .
an abnormal nucleotide sequence on chromosome-19 causes prolonged stimulation of the actin  myosin complex due to a larger sodium current , causing delayed relaxation of contracted muscle .
the estimated incidence is 1 in every 8000 births , with an estimated prevalence of between 2.1 and 14.3 cases per 100,000 inhabitants .
providing a successful anesthesia to patients suffering from steinert syndrome ( md ) constitutes a serious challenge .
these difficulties are an implication of multiple disorders which may be related to respiratory , cardiac , and central nervous systems . a myotonic crisis can be induced by numerous factors including hypothermia , shivering , and mechanical or electrical stimulation . in patients with md , hypersensitivity to anesthetic drugs , especially muscle relaxants and opioids ,
patients may present poor outcomes related to the following complications : loss of airway secondary to medication - induced respiratory depression , aspiration of stomach contents , and sudden death that is usually secondary to cardiac conduction delays and dysrhythmias .
myotonia is described as muscle contraction ( voluntary or otherwise ) with abnormal , prolonged relaxation .
triggers for myotonia include certain medications , potassium , hypothermia , shivering , or any mechanical or electrical stimulus .
dystrophia myotonica ( dm , myotonic dystrophy ) patients are exquisitely sensitive to the respiratory depressant effects of anesthetic medications .
postoperative pain control should be managed with non - steroid anti - inflammatory drugs ( nsaids ) , regional techniques using local anesthetics , and acetaminophen when possible . if opioids are employed ( systemic or neuraxial ) ,
then icu care and continuous pulse oximetry must be considered given the high risk for respiratory depression and aspiration .
weakness of the pharyngeal muscles and a delayed gastric emptying time predispose dm patients to aspiration .
respiratory muscle weakness predisposes md patients to restrictive lung disease with concurrent dyspnea and ineffective cough .
dm subjects have heightened central nervous system sensitivity to sedatives , anxiolytics , and analgesics , further impeding ventilatory drive and airway protection .
it is not uncommon for dm patients to have a history of hypoxia , dyspnea , sleep apnea requiring continuous positive airway pressure ( cpap ) , or marked ventilatory muscle weakness necessitating ventilation support , dm patients can be exquisitely sensitive to the respiratory depressant effects of commonly used premedications ( e.g. , opioids and benzodiazepines ) .
patients with md present high sensitivity to the usual anesthetics such as volatile and muscle relaxants ( both depolarizing and nondepolarizing ) .
therefore , a choice of adequate drug with minimal risk of complication remains a serious problem .
the exquisite sensitivity of dm patients to the respiratory depressant effects of opioids ( systemic or neuraxial ) can equate to fatal outcomes in the postoperative period .
the most common route of opioid administration that places dm patients at high risk for respiratory depression is intravenous , yet there is a case report that details respiratory depression following a small dose of epidural morphine as well . due to hypersensitivity to opioids and
increased susceptibility to intra- and postoperative complications , it is recommended to introduce opioid - free anesthesia ( ofa ) , for example , with use of dexmedetomidine ( dex ) .
this is a new method of conducting general anesthesia without opioids and is based on concept of multimodal approach to pain management .
the drugs used instead of opioids for intraoperative analgesia include clonidine , dex , ketamine , and continuous intravenous infusion of lidocaine .
this method was supposed to maintain stability of vital parameters , and prevent undesired events with irreversible effects .
therefore , keep the operating room and table warm so that the patient will be better able to maintain a normal body temperature . using the warmed iv fluids as well as forced - air blankets during surgery
if a muscle relaxant is needed , then a nondepolarizing agent with a short recovery index should be chosen ( e.g. , rocuronium , cis - atracurium ) .
dm patients are no more susceptible to the development of malignant hyperthermia than the rest of the general population .
a 31-year - old male patient ( 183  cm , 69  kg ) was scheduled for laparoscopic operation of cholecystectomy .
the patient received intravenously : propofol in a dose of 250  mg followed by continuous infusion , rocuronium in a dose of 20  mg , and dex in a loading dose of 0.6  g / kg over 10  minutes followed by continuous infusion of dose of 0.2  g / kg / hour . during the intubation , cricoid pressure was maintained .
except hemodynamic parameters , patient 's body temperature was monitored carefully and corrected if needed .
all the time patient was warmed by air - blanket . at the end of surgery , 200  mg of sugammadex
the patient exited the operating theatre in a good medical state , with vitals within normal limits and fully regained consciousness .
ethical approval for this case was not necessary because one of the indications for ofa is hypersensitivity to opioids .
this is the first case of opioid - free general anesthesia using dex in patient with steinert syndrome described in the medical literature .
the successful use of dex as adjunct to regional anesthesia was described in a 53-year - old woman with md for a total abdominal hysterectomy by yoshino et al .
combined spinal and epidural block was used in this patient and dex was used for sedation during surgery .
airway obstruction was observed after the initial administration of dex at 2  g / kg , therefore authors concluded that dex was proved to be useful in this case ; however , use of the drug should be carefully started at a low initial dose in patients with md .
we used dose of 0.6  g / kg and did not observed airway complications .
there are several reports on safe use of dex for anesthesia in other muscular diseases .
rozmiarek et al reported successful use of a combination of dex and ketamine to provide sedation and analgesia in a 21-year - old patient with duchenne muscular dystrophy ( dmd ) undergoing bone marrow aspiration and biopsy .
kako et al used 2 different doses of dex for sedation during muscle biopsy in patients with dmd : 1.0 or 0.5  g / kg was administered as a loading dose over 3  minutes followed by a continuous infusion of 1.0 or 0.5  g / kg / hour .
the regional anesthesia in dm patients is preferred if possible ( thoracic epidural even for cholecystectomy ) ; however , in most described cases of general anesthesia of patients with md , general anesthesia was performed using continuous infusion of propofol .
however , remifentanil has been reported to induce hyperalgesia causing difficulties in postoperative pain management , especially after intra - abdominal surgery .
we decided to perform ofa , in which we have great experience because of bariatric surgery .
dex is effective and safe for moderately painful procedures in patients with elevated risk of respiratory and cardiovascular failure .
abstractintroduction : we report on the anesthetic management using opioid - free method of a patient with steinert syndrome ( myotonic dystrophy , md ) , autosomal dominant dystrophy which is characterized by consistent contracture of muscle following stimulation . 
 a myotonic crisis can be induced by numerous factors including hypothermia , shivering , and mechanical or electrical stimulation . in patients with md , hypersensitivity to anesthetic drugs , especially muscle relaxants and opioids , 
 may complicate postoperative management . if opioids are employed ( systemic or neuraxial ) , then icu care and continuous pulse oximetry must be considered given the high risk for respiratory depression and aspiration . 
 patients with md present high sensitivity to the usual anesthetics such as volatile and muscle relaxants ( both depolarizing and nondepolarizing ) . 
 opioids may induce muscle rigidity in this type of md . 
 therefore , omitting opioids is recommended . due to hypersensitivity to opioids and 
 increased susceptibility to intra- and postoperative complications , it is recommended to introduce opioid - free anesthesia ( ofa ) , for example , with use of dexmedetomidine ( dex ) . 
 this is a new method of conducting general anesthesia without opioids and is based on concept of multimodal approach to pain management.methods:a 31-year - old male patient ( 183  cm , 69  kg ) was scheduled for laparoscopic operation of cholecystectomy . 
 the patient received intravenously ( iv ) : propofol in a dose of 250  mg followed by continuous infusion , rocuronium in a dose of 20  mg , and dex in a loading dose of 0.6  g / kg over 10  minutes followed by continuous infusion of dose of 0.2  g / kg / hour.results : the course of anesthesia and postoperative period were uneventful . 
 the patient exited the operating theatre in a good medical state , with vitals within normal limits and fully regained consciousness.conclusion:dex is effective and safe for moderately painful procedures in patients with the elevated risk of respiratory and cardiovascular failure . 
 this substance provides adequate analgesia level during surgeries of patients suffering from md .