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Effects of anesthetic agents on
patients with muscle disorders
Servizio di Anestesia e Rianimazione
Ospedale di Faenza(RA)
• i.v anesthetics do not cause
Increased sensitivity to iv drugs
– Bourke T, Zuck D. Thiopentone in dystrophia myotonica. Br J Anaesth 1957;
– Dundee JW. Thiopentone in dystrophia myotonica. Anesth Analg 1952;
hyperkalemic periodic paralysis
– Egan TJ, Discavage WJ. Hyperkalemic periodic paralysis. Pediatrics 1959;
– , Atkinson RS, Rushman GB, Davies NJH. Lee’s synopsis of anaesthesia.
11th ed. Oxford, UK: Butterworth-Heinemann, 1993; 419-40.
56 year male with ophthalmoplegia who required only 75 mg of
thiopentone for loss of eyelid reflex
– (James RH. Thiopentone and ophthalmoplegia plus [letter]. Anaesthesia
Charcot-Marie-Tooth syndrome (CMT)
– Hirota K, Muraoka M, Sugihara K, et al. Anesthetic experience of CharcotMarie-Tooth disease. Jpn J Anesth 1988; 37:207-10.
Kotani,N,Hirota K,Anzawa N,Takamura,K, Sakai T,Matsuki
A..Motor and Sensory Disability Has a Strong Relationship to
Induction Dose of Thiopental in Patients with the Hypertropic
Variety of Charcot-Marie-Tooth Syndrome.Anesth Analg 1996;
• Mean Induction dose of thiopental:
• MID in the control patients: 3.7 ± 1.1 mg/kg
(mean ± SD).
• The 95% confidence interval of the MID in
control normal patients : 2.5–4.9 mg/kg.
• In 11 patients (No. 1–11), an MID of 0.83–
2.4 mg/kg proved to be sufficient. The
remaining 9 patients (No. 12–20) required
2.5–5.0 mg/kg as the MID.
Relationship bertween minimun induction doses of
thiopental and motor and sensory disability scores of
20 patients with CMT disease(Kotani,et a.Motor and Sensory Disability Has a
Strong Relationship to Induction Dose of Thiopental in Patients with the Hypertropic Variety of CharcotMarie-Tooth Syndrome.Anesth Analg 1996; 82:182-6..
Stackhouse R, Chelmow D, Dattel BJ: Anesthetic
complications in a pregnant patient with nemaline
myopathy. Anesth Analg 79:1195, 1994
GA for C/S
the patient received sodium citrate (30 mL per os), was preoxygenated for 4 min, and had
a modified rapid-sequence induction of anesthesia performed using thiopental (320
mg: 4 mg/kg) and vecuronium (10 mg) while applying cricoid pressure. The
patient was ventilated with isoflurane (1%) in oxygen until twitch response was
eliminated (4 min postinduction).
The first attempt at laryngoscopy with a Mac 3 blade failed. A second
laryngoscopy with a Miller 2 blade was unsuccessful because it was not long enough
to lift the epiglottis. Ventilation was reinstituted after rapid oxygen desaturation from 98% to
80%, and it continued until oxygen saturation returned to 95%. Copious oral secretions
were suctioned. Laryngoscopy(3) with a Mac 4 blade was performed
twice and provided visualization only of the arytenoids. Nonetheless,
the endotracheal tube was passed anterior to the arytenoids and placement verified by
bilateral breath sounds, capnography, and increasing oxygen saturations. The patient was
hemodynamically stable throughout this period. Frothy secretions were suctioned from the
endotracheal tube. The pH of these secretions was measured and found to be neutral.
Surgery then proceeded without incident. Induction to delivery time was approximately 30
min; however, uterine incision to delivery time was within normal limits. An infant boy was
delivered with Apgar scores of 1 at 1 min, 4 at 5 min and 9 at 10 min. The results of an
umbilical venous blood gas test were pH 7.09, PCO2 86, PO2 13, HCO3 25.1, and a base
deficit of -5.2, indicating an acute respiratory acidosis. The newborn was intubated and
ventilated for 20 min until the respiratory acidosis was resolved and the anesthetic vapors
exhaled. No further resuscitative measures were required……………………..
• Speedy H. Exaggerated physiological responses to propofol in
myotonic dystrophy. Br J Anaesth 1990; 64:110-2.
– <1 mg /kg of propofol was sufficient to induce hypnosis and permit
• Gottschalk A,Heiman-Patterson T, deQuevedo R II;, Quinn
PD..General Anesthesia for a Patient with Centronuclear
(Myotubular) Myopathy.Anesthesiology 89:1018-20, 1998
– induced with propofol and maintained with a propofol infusion,
nitrous oxide, and oxygen. No additional intravenous medications
were administered at any time, except for ketorolac 1 h before
– Bilateral coronoidectomies with bilateral arthroplasty with eminectomies
were performed without difficulty. The surgical sites were infiltrated with
bupivacaine by the surgeon. The patient awoke and was transported to
the postanesthesia care unit while breathing spontaneously. After
uneventful extubation, she was placed on nasal bilevel pressureassisted ventilation at her usual settings.
Sasuga M, Matsukawa T, Ookawa I, Tamaki F,
Masamune T, Kumazawa T. Anesthetic management
of three patients with myotonic dystrophy in a family
Masui. 2004; 53:269-72.
Case-1: A 24-year-old woman was admitted because of pressing hydramnion. She
was treated by ritodrine hydrochlorides leading to rhabdomyolysis, and she was
diagnosed as myotonic dystrophy. She underwent cesarean section because of
urgent premature birth. The surgery was performed with spinal anesthesia using
tetracaine. Case-2: A 1-year-old boy, the son of Case 1, underwent orchiopexy. He
showed respiratory distress at birth and needed respiratory support for 140 days. The
surgery was performed under general anesthesia combined with caudal anesthesia.
Anesthesia was induced with nitrous oxide-oxygen-sevoflurane. He was intubated
without muscle relaxants. Since he recovered consciousness soon after the surgery,
he was extubated and returned to the ward. Case-3: A 30-year-old woman,
the sister of Case 1, underwent tonsillectomy. At the age of
27 she underwent salpingectomy under general anesthesia
with nitrous oxide-oxygen-halothane, after which she was
diagnosed as myotonic dystrophy. She was anesthetized
with propofol and fentanyl. Because severity of the myotonic dystrophy
varies among the patients, the strategy for anesthesia should be planned on each
patient. Generally speaking, regional anesthesia including spinal and epidural
anesthesia is preferable
Propofol in a case of myotonic dystrophy
(Tzabar, Y.; Marshall, R.; Russell, S. H.Myotonic dystrophy and target–controlled
propofol infusions . Br. J. Anaesth. 1995; 74:108.
23–yr–old, 63–kg Caucasian male
admitted for operative fixation of a fractured mandible and suffering from myotonic
TCI set at 2 µg/ ml and increased in steps of 2 µg/ml Alfentanil 1 mg was
administered when verbal contact with the patient was lost.:when the target
concentration reached 12 mg/ ml and the patient’s jaw was relaxed, laryngoscopy was
performed and nasotracheal intubation easily accomplished .
No neuromuscular blocker was used at any time.,but N2O was added intraoperatively.
Anaesthesia maintained with a TCI of 6 µg/ml which was reduced to 5 µg/ ml
after 30 min and 4 µg /ml at 60 min. Total operative time was 75 min.
Towards the end of the procedure the target concentration was set to 0 mg/ ml so that
the reduction in calculated propofol concentration could be followed.
The patient did not commence spontaneous respiration until the calculated propofol
concentration decreased to less than 2 µ g/ ml. Respiration was slow and shallow
initially but improved with time. There were no signs of awakening or response to voice
even when the predicted propofol concentration was less than 1 µ g/ ml. He was
observed for a further 30 min with no change
He was then transferred to the intensive care unit where he was observed while still
breathing spontaneously through the tracheal tube, attached to a T–piece. Arterial
oxygen saturation and end–tidal carbon dioxide remained satisfactory throughout.
Approximately 1 h after the end of surgery, he began to show signs of awakening and
extubated his own trachea. His subsequent postoperative course was uneventful and
the following morning he was discharged to the general ward.
Bennun M,Goldstein B,Finkelstein Y,Jedeikin
R.Continuous propofol anaesthesia for patients with
myotonic dystrophy.Clinical Investigation. Br. J.
Anaesth. 2000; 85:407-409
13 patients with myotonic dystrophy scheduled for oropharyngeal and neck
surgery, The median age was 21 yr (range: 11–42 yr).
enrolled in an open prospective study.
able to function adequately, with good physical mobility and without
no history of systemic disease and none was taking medication.
Physical examination showed wasting of thenar and masseteric muscles.
Preoperative haematological and biochemical investigations, ECG,
echocardiograpy and lung function tests were normal.
. No pre-anaesthetic medication was administered.
Anaesthesia was induced with fentanyl 0.05 mg, propofol 2.5
mg kg-1 and atracurium 0.5 mg kg-1. After intubation of the trachea, the
patients' lungs were ventilated with 70% N2O in oxygen and anaesthesia
was maintained with a continuous infusion of propofol 6 mg kg-1
h-1, bolus fentanyl 2 µ g kg-1 and incremental doses of atracurium 0.2 mg
kg-1. After patients had regained consciousness and four equal `train of
four' twitches were observed, the trachea was extubated.
Results from Bennun
The mean (SD) duration
of anaesthesia was 104 (44) min(
– Operations: pharyngeal flap for correction of velopharyngeal incompetence (8 pts)
pharyngeal flap and tonsillectomy (t2 pts)
endoscopic surgery of the sinuses (FESS) for pansinusitis (1)
cervical lymph node biopsy (1)
total thyroidectomy for papillary carcinoma of thyroid (1).
mean (SD) recovery time was 12 (11.5) min. T
Ephedrine was not required in any case.
The mean postoperative SpO2 (97 (2.9)) did not differ from the preoperative (98 (1)) values.
The median hospital admission was 2 days (range: 1–4 days).
There was no difference between mean preoperative and postoperative tidal volumes (334
(119) ml versus 330 (122) ml) (P=0.97).
However, there was a significant decrease in mean postoperative vital
capacity (965 (349) ml) from the preoperative value (1664 (566) ml)
There were no perioperative respiratory or cardiac complications and bronchial secretions were
not a problem.
Only two patients complained of nausea and vomiting, but no treatment was required. Similarly,
muscular hypertonia and shivering were not observed.
Johnson GW,Chadwick S,Eadsforth P, Hartopp
Ist 53–yr–old man with moderate myotonia dystrophica, ischaemic heart disease
(including myocardial infarction 1 yr previously) and marked peripheral vascular
disease. He presented with an acute on chronic ischaemic leg, requiring urgent
exploration of his femoral artery.
Temazepam 10 mg was administered orally 1 h before operation. After
preoxygenation, anaesthesia was induced with fentanyl 250 mg, propofol 50 mg (the
effect noted), followed 1 min later by another 25 mg. A propofol infusion was then
given at a rate of 6 mg kg-1 h-1 for 15 min, then 4 mg kg-1 h-1 for another 10 min
and finally 2 mg kg-1 h-1 for the remainder of the operation. Atracurium 20 mg was
also given. No other sedative or analgesic drugs were used. The patient underwent
femoral embolectomy without any major problems.
The propofol infusion was discontinued 55 min after induction. Within 3 min his
respiratory efforts were adequate and the trachea was extubated. However, although
respiration was satisfactory and the airway was well maintained, it was a further
65 min before verbal contact was made with the patient.
The immediate postoperative period was uneventful but on day 7 he began to
develop increasing weakness, including bulbar weakness, and he died on day 14.
Johnson GW,Chadwick S,Eadsforth P, Hartopp
The second case was a 27–yr–old female with moderately severe myotonia dystrophica
causing cataracts, marked muscle weakness, slurred speech and swallowing difficulties.
However, she was mobile and had no respiratory or cardiovascular system involvement. She
was admitted for laparoscopic cholecystectomy.
The patient was premedicated with temazepam 10 mg, and heparin 5000 u. was
administered s.c. After preoxygenation, fentanyl 100 mg was given and the effect observed.
Three 25–mg increments of propofol were given at 1–min intervals to assess the patient's
sensitivity to propofol. When it was evident that the patient was not sensitive, propofol 125 mg
was given followed by atracurium 15 mg. Anaesthesia was maintained with 0.5–1.0% isoflurane
and nitrous oxide in oxygen. The operation and anaesthetic proceeded uneventfully with a
further 100 mg of fentanyl and 10 mg of atracurium being required. Morphine 10 mg was
administered at the end of operation. The patient made a rapid recovery and was fit to leave
theatre recovery 35 min after the anaesthetic.
The third case was female with moderate myotonia undergoing total abdominal
hysterectomy. She had mild muscle weakness with no other problems (she had only been
diagnosed because of family screening.) After premedication with temazepam 10 mg,
anaesthesia was induced with alfentanil 2 mg followed by propofol 140 mg given slowly.
Intubation was performed without formal paralysis and anaesthesia was maintained with a
propofol infusion of 10 mg kg-1 h-1 followed by 8 mg kg-1 h-1 followed by 6 mg kg-1 h-1 , and
an infusion of alfentanil 1.5 mg h-1. The operation lasted 40 min and the patient rapidly
regained consciousness. Her trachea was extubated within 5 min of termination of the infusions.
She made an uneventful recovery and was fit to go to the ward within 45 min.
Myotonia caused by propofol
• generalized myotonic state was also reported after the i.v.
administration of propofol.
– Bouly A, Nathan N, Feiss P. Propofol in myotonic dystrophy.
Anaesthesia 1991; 46:705.
• Regional myotonia:
– Kinney MAO, Harrison BA.Propofol-Induced Myotonia in Myotonic
Dystrophy .Anesth Analg 1996; 83:665–6
• A 38-yr-old male, ASA physical status II, with myotonic
dystrophy presented for removal of orthopedic hardware from
the lumbar spine. Midazolam, 1.0 mg, and fentanyl, 100 mg,
were given intravenously. General anesthesia was induced with
200 mg propofol (2.5 mg/kg). Immediately after the propofol,
the patient described pain in his right arm causing flexion of his
right arm. After loss of consciousness, a sustained contracture
occurred in the right arm and lasted approximately 20 s before
spontaneously relaxing. Anesthesia, emergence, and recovery
• Ketamine has been used as and adjunct to caudal
anesthesia in a 2 years old child
– Shiraishi M,Minami K, Kadaya T.A Safe Anesthetic Method
Using Caudal Block and Ketamine for the Child with
conngenital Myotonic Dystrophy. Anesthesia & Analgesia
• TIVA(ket/fent/ propofol) for laparoscopic
cholecystectomy for cholelithiasis.
– Kushikata T, Yatsu Y, Kubota T, Matsuki A.[Total
intravenous anesthesia with propofol, ketamine, and fentanyl
(PFK) for a patient with mitochondrial myopathy. Masui.
An obese female presenting for coronary bypass grafting
known carrier of central core disease and therefore MH susceptible;
After ensuring full precautions to prevent and treat MH were in place, she was
premedicated with i.m. morphine 15 mg and metoclopramide 10 mg and oral lorazepam 3 mg, 1
h before surgery.
Anaesthesia was induced with midazolam 7 mg, fentanyl 20 micrg/ k and
pancuronium 16 mg.
Anaesthesia was maintained using a propofol infusion at 300-400 mg/ h.
Other infusions: dopamine , glyceryl trinitrate ,aprotinin 2 000 000 U was given at sternotomy,
followed by another 2 000 000 U during bypass. Anticoagulation was achieved with heparin
(total dose 45 000 IU).
The patient was cooled to 32C on cardiopulmonary bypass and the aortic cross-clamp time was
After bypass, the residual effects of heparin were reversed with protamine 375 mg.
4 units of platelets and 2 units of fresh frozen plasma were transfused perioperatively. The
decision to transfuse blood products was made on account of increased microvascular
bleeding. No allogenic blood was transfused at any time. After surgery, the patient was
transferred to the intensive care unit and her trachea was extubated after 12 h. Postoperative
recovery was uneventful. This patient did not show any sign of muscle weakness at any time in
the postoperative period and was discharged from hospital after 8 days. Lung function tests and
determination of serum CK concentration were not repeated.
• patient severely compromised by MELAS syndrome
– 20-yr-old man presenting for a right cochlear implant had a medical
history significant for MELAS syndrome. Between the ages of 1 and 5 yr,
the only possible abnormality was delayed speech development. At the
age of 7 yr, he was diagnosed with hearing loss. He began having
episodes of dizziness and bilateral leg weakness at the age of 14 yr.
Two years later, he experienced a generalized seizure. His continued
neurologic deterioration led to his present condition of blindness,
deafness, dementia, and severe muscle wasting.
• anesthetized successfully by a combination of
– Thompson VA,Wahr JA..Anesthetic Considerations in Patients
Presenting with Mitochondrial Myopathy, Encephalopathy, Lactic
Acidosis, and Stroke-Like Episodes (MELAS) Syndrome
Anesth Analg 1997; 85:1404-6.
Uchiyama Y, Miyamoto E, Yamada M, Murao K, Nakao S, Shingu
K.Anesthesia for laparoscopic cryptorchidpexy in a patient with
congenital myopathy.Masui. 2003;52:1107-9.
– 18-month-old, 11.3-kg, male patient, who had received a diagnosis of CM,
was scheduled for the laparoscopic cryptorchidpexy. Anesthesia was
induced with propofol and fentanyl, and the trachea was intubated without
muscle relaxants. An epidural catheter was inserted via the sacral hiatus, the
tip of which was located at the second lumbar level for a purpose of
obtaining not only pain relief but also muscle relaxation. Anesthesia was
maintained with propofol, nitrous oxide and fentanyl, combined with epidural
anesthesia. The anesthetic course was uneventful with enough pain relief
and good muscle relaxation.
• Sasuga M, Matsukawa T, Ookawa I, Tamaki F,
Masamune T, Kumazawa T. Anesthetic management of
three patients with myotonic dystrophy in a family
Masui. 2004; 53:269-72.
– I pt.anesth for tonsillectomy with propofol/fentanyl(
Ay B, Gercek A, Dogan VI, Kiyan G, Gogus YF.
Pyloromyotomy in a patient with paramyotonia
congenita.Anesth Analg. 2004; 98:68-9,
A 2-mo-old infant with paramyotonia congenita was scheduled for
pyloromyotomy and repair of inguinal hernia. Diagnosis of paramyotonia
congenita was done with positive family history, myotonia at eyelids,
provocation by cold, and electromyogram analysis. Anesthesia was induced
via face mask with sevoflurane at 4 minimum alveolar
anesthetic concentration in oxygen. Tracheal intubation was
attempted without a neuromuscular relaxant. Anesthesia was
maintained with sevoflurane at 0.5 minimum
alveolar anesthetic concentration in oxygen and
remifentanil infusion at a rate of 0.2 micro g. kg(1). min(-1). After discontinuation of sevoflurane and remifentanil, the
patient was awake and had full recovery of muscle activity. IMPLICATIONS:
The literature concerning general anesthesia in paramyotonic patients is
limited. We report a case of paramyotonia congenita in a 2-mo-old male
infant undergoing surgery for pyloric stenosis and inguinal hernia after an
Balanced anesthesia for emergency surgery in a
patient with myotonic dystrophy
Marsh D F, Scott R C,Russell SH, Hirsch NP. Anaesthesia in myotonic dystrophy Br. J. Anaesth. 199
A 37–yr–old women presented for surgery with a diagnosis of
myotonic dystrophy. ….. Previous anaesthesia for minor elective
gynaecological procedures had been uneventful. On examination she
was obese and had prominent upper incisors with micrognathia.
The patient was scheduled for appendicectomy and no
premedication was given. Full monitoring was applied in the
anaesthetic room and after preoxygenation for 5 min, anaesthesia was
induced with alfentanil 0.5 mg mg followed by propofol 140
mg, with the application of cricoid pressure. OTI was achieved
with the aid of a gum elastic bougie. Anaesthesia w;as maintained with
isoflurane and 70% nitrous oxide in oxygen and the lungs were
ventilated mechanically, without neuromuscular block. At the end of
surgery, adequate spontaneous ventilation returned rapidly and the
patient was transferred to the intensive care unit where an infusion of
fentanyl was commenced. The trachea was extubated after return of
consciousness and the patient made an uneventful recovery.
Problems of this patient
Marsh D F, Scott R C,Russell SH, Hirsch NP. Anaesthesia in myotonic dystrophy Br. J.
Anaesth. 1994; 73:124 .
a potentially difficult patient with regard to tracheal intubation
e additional risk of aspiration of stomach contents after induction of
avoidance of suxamethonium is mandatory in patients with myotonic
dystrophy and thus a conventional rapid sequence induction could not be
It was thought that a regional technique would have been a suitable
alternative, but was not an option because of patient refusal.
An awake intubation was considered but places the patient at risk from
Inhalation induction might cause early upper airway obstruction because of
her pharyngeal muscle weakness and obesity.
Induction and intubation using alfentanil and propofol alone without the need
for neuromuscular block have been described recently , but have not been
reported previously in a patient with myotonic dystrophy. The new non–
depolarizing neuromuscular blocker, rocuronium, provides rapid onset of good
intubating conditions comparable with suxamethonium and may be a suitable
alternative when freely available.
We consider that the intubating conditions obtained with this technique were
excellent and may be considered as an alternative to conventional rapid
sequence induction in patients in whom suxamethonium is contraindicated.
patient affected by the the King-Denborough syndrome (KDS)a rare
disorder that is associated with myopathy, susceptibility to malignant
hyperthermia (MH) as well as congenital skeletal and facial anomalies
• 24-yr-old primiparous woman with a diagnosis of KDS and a history of
previous MH reaction (at age 2). Her KDS resulted in chronic
respiratory failure,with a permanent tracheostomy and
overnight ventilatory support for the previous two years.
She had three admissions during her pregnancy, one for pneumonia and
two for preterm labour. Labour was induced at 37 weeks. Her labour was
managed in the operating room where a "clean" anesthesia machine was
ready. Cooling aids and a MH emergency kit were immediately available.
Intravenous access, an arterial line and a lumbar epidural catheter were
inserted before induction of labour. Ropivacaine 0.08% + fentanyl 2
microg/ml were used for patient-controlled epidural analgesia. After 6.5 hr
of labour the patient required ventilation. An outlet forceps was performed
for delivery. Postpartum, she was ventilated overnight in the intensive care
– Habib AS, Millar S, Deballi P 3rd, Muir HA. Anesthetic management of a
ventilator-dependent parturient with the King-Denborough syndrome.Can J
Anaesth. 2003 ;50:589-9.
• 1)Muscular system:
– respiratory.;intra/postop !!response to
– Measurement of the pvO2 during aerobic forearm
exercise provides an easily performed screening test that
detects impaired O2 use and the severity of oxidative
impairment in patients with mitochondrial myopathy and
– Respiratory function is frequently compromised before surgery
– patients are at a higher risk of post-operative respiratory complications.
After surgery, the patient with Duchenne’s muscular dystrophy must be monitored closely for evidence
of pulmonary dysfunction and retention of pulmonary secretions. Vigorous respiratory therapy and
ventilatory support may be necessary.
– A history of chest infections and apnoea should be sought .
– Pulmonary flow volume loops, a chest X‑ray and blood gases are
indicated preoperatively in these patients
– Respiratory depressants should be avoided with pre-medication as
respiratory responses to hypoxaemia and hypercapnia are impaired .
– Patients with progressive bulbar muscle involvement are especially
predisposed to aspiration :to reduce risk:
• reduce gastric volume and acid secretion
• keep the gastric pH >2.5 with the use of adequate (but not prolonged) fasting
• nasogastric and percutaneous gastrostomy tube (aspiration of in case of
• histamine‑2 receptor antagonists
• proton pump inhibitors
• non‑particulate antacids
• cardiomyopathies (dilated and hypertrophic)
– Dodds T M, Haney M F,Appleton F MManagement ofperipartum CHF using
continuous AV hemofiltration in a patuient with myotonic
Case report of a woman in the third trimester of pregnancy with CHF from myotonic
dystrophy-related cardiomyopathy; CAVH was used for the treatment of
refractory CHF in preparation for operative delivery.
pre‑excitation syndromes (Wolf-Parkinson‑White)
conduction defects (heart blocks)
Mitral valve prolapse(myotonia)
– A 12‑lead ECG should be performed preop.
– further work‑up using echocardiograph
• There is increased risk of sudden death from conduction
4:Liver & Metabolic
• Liver function and hepatic mitochondrial redox potentials can be
measured by arterial /ven ketone body ratios, calculated by the
ratio of acetoacetate to 3‑hydroxybutyrate
• . If the liver and kidneys are affected, this might lead to altered
pharmacokinetics and pharmacodynamics of any drugs
---------careful drug dose titration!!
• Metabolic dysfunction due to liver involvement result in altered
glucose, lactate and protein metabolism should be avoided an
good hydration maintained
• Glucose management can be challengig requiring frequent
assessment In the newborn glucose represents the sole
energy supply to the heart
• In children, preoperative hypoglycaernia shoul be avoided by
the i.v. infusion of glucose containing fluids or
glucose‑containing solutions up to 3 h p induction
• The prevalence of diabetes is higher patients with
5:Liver & Metabolic
• Patients often have elevated serum lactate ev Although a
raised lactic acid concentration not considered to be a
specific test, it points to t diagnosis especially when
significantly elevated [5 Lactic acidosis is usually worsened
by stress .
• In mitochondrial myopathies, high lactate production m
contribute to the decreased muscle function observ by
inhibiting the production of interleukin‑6
• Any increases in metabolic stress that may provoke worsen
lactic acidosis should be avoided.
• Adequate oxygenation and minimization of oxygen demand
stable cardiovascular fiinctions, maintenance of norm serum
glucose levels, and acid‑base and electrolyt balance help to
minimize acidosis .
• Communication may be a problem in
these patients due to sensory deprivation
• The presence a parent or guardian during
induction is beneficial
Monitoring should matched to the clinical state and the surgery
performed A basic minimum would be continuous electrocardio
graph and pulse rate, non‑invasive blood pressure pulse
oximetry, capnography and temperature monitoring;ABG
Temperature monitoring with a nasopharyngeal probe is
essential to main tain normothermia All i.v. fluids and epidural
local anaesthetic solutions should be warmed to body temp.
It is advisable to avoid Ringer's lactate to prevent an
exacerbation of lactic acidosis Maintaining normothermia (core
and surface), normoglycaemia and avoiding metabolic stress
are important in the perioperative management of these
In cardiac bypass, normothermia during the bypass avoids
mitochondrial stress from hypothermia .
LMA>ET?:anesth depth lighter,less or no muscle relaxants
General recommendations :2
• Aerobic metabolism is already dysfunctional and any increases
in basic metabolic rate (such as Perioperative shivering)
should be prevented
• Any increases in O2 consumption should be minimized by
maintaining normal body temperature and adequate surgical
anaesthesia (either general or regional) should be provided
• A cardiovascularly stable anaesthetic is recommended .
• Any anaesthetic agents that depress cardiovascular function
should be avoided. During spontaneous breathing, all oploids
and sedative‑hypnotics should be carefully titrated due to the
decreased ventilatory response to hypoxia and hypercarbia .
• Effective perioperative analgesia will avoid increases in
metabolism, provide comfort and allow early ambulation .
• In spontaneously breathing patients, the cautious use of
oploids is advised as oploids may further impair regulation of
breathing and lead to a respiratory acidosis (in addition to any
underlying metabolic acidosis
• A multimodal analgesic approach should be used involving
the use of oploid administration (via i.v. infusion or
patient‑controlled analgesia), non‑steroidal
anti‑inflammatory drugs and the use of local anaesthesia at
wound sites, peripheral nerves, regional plexuses and
• In labour and delivery in women with mitochondrial
myopathies, the management should be individualized
according to the severity of the disease and by
multidisciplinary consensus. Epidural analgesia reduces
stress and work associated with labour and reduces oxygen
demand during labour . Patients with documented increased
lactate concentrations at rest and exercise are best
managed with elective Caesarean sections as soon as fetal
lung maturity has been confirmed under regional
anaesthesia to prevent life‑threatening lactic acidosis during
• Butler MG,Hayes BG, Hathaway M,Begleiter
M L., Specific genetic diseases at risk for
ABSTRACT: We reviewed of a number of genetic diseases known or at risk
for sedation or anesthesia complications. Some of these conditions are
relatively common (e.g., Down's syndrome) whereas others are rare or
present with multiple congenital anomalies that have an impact on health
care delivery. We listed complications, recommended presedation
evaluations, and included checklist items to assist the health care provider
administering sedation and anesthesia. A better recognition and awareness
of risk factors associated with specific genetic diseases should lessen the
likelihood of complications during these procedures.
• Wappler F. Current aspects of anesthesia in neuromuscular
Anasthesiol Intensivmed Notfallmed Schmerzther. 2003
Jul;38(7):4959. Review. German. No abstract available.
Bischoff P. [Which diagnostics are essential in patients with
Anasthesiol Intensivmed Notfallmed Schmerzther. 2003
Jul;38(7):48891. Review. German. No abstract available.
Rosenkranz T. [Myopathieswhat does the anesthetist need
Anasthesiol Intensivmed Notfallmed Schmerzther. 2003
Jul;38(7):4838. Review. German. No abstract available.
Wappler F, Schulte am Esch J. Anaesthesia in patients with
neuromuscular diseasesnew concepts for old problems?
Anasthesiol Intensivmed Notfallmed Schmerzther. 2003
Jul;38(7):4701. English, German. No abstract available.
• uropean Journal of Anaesthesiology 2004;
• Mitochondrial myopathies and
• E. A. Shipton, D. 0. Prosser
• University of Otago, Christchurch School of Medicine
and Health Sciences, Department of Anaesthesia,
• Christchurch, New Zealand