|Year : 2007 | Volume
| Issue : 1 | Page : 47-49
Anaesthetic challenges in a patient with mitochondrial cytopathy undergoing surgery
DN Dhananjay1, Shyam Sundar2, Kurian P Thomas2, Lakshmi Kumar3, Suresh G Nair4
1 D.A., Lecturer, Department of Anesthesia, Amrita Institute of Medical Sciences and Research Center, Kochi- 682 026, Kerala, India
2 D.A., DNB, Asst. Prof, Department of Anesthesia, Amrita Institute of Medical Sciences and Research Center, Kochi- 682 026, Kerala, India
3 M.D., Asso. Prof, Department of Anesthesia, Amrita Institute of Medical Sciences and Research Center, Kochi- 682 026, Kerala, India
4 M.D.,Prof.andHead, Department of Anesthesia, Amrita Institute of Medical Sciences and Research Center, Kochi- 682 026, Kerala, India
|Date of Acceptance||25-Jan-2007|
|Date of Web Publication||20-Mar-2010|
Kurian P Thomas
D.A., DNB, Asst. Prof, Department of Anesthesia, Amrita Institute of Medical Sciences and Research Center, Kochi- 682 026, Kerala
Source of Support: None, Conflict of Interest: None
This article will highlight certain basic aspects in the management of a case of mitochondrial cytopathy that is unknown to most of us. Clinical condition of the patient is the most important aspect in the management, as the patients with mild disease may be at lower risk for complications while those with severe disease are at a higher risk. We managed a 45year old lady posted for incisional hernia repair
Keywords: Mitochondrial cytopathy, Anaesthesia, Complications
|How to cite this article:|
Dhananjay D N, Sundar S, Thomas KP, Kumar L, Nair SG. Anaesthetic challenges in a patient with mitochondrial cytopathy undergoing surgery. Indian J Anaesth 2007;51:47-9
|How to cite this URL:|
Dhananjay D N, Sundar S, Thomas KP, Kumar L, Nair SG. Anaesthetic challenges in a patient with mitochondrial cytopathy undergoing surgery. Indian J Anaesth [serial online] 2007 [cited 2020 Apr 2];51:47-9. Available from: http://www.ijaweb.org/text.asp?2007/51/1/47/61115
| Introduction|| |
Luft was first to report the case of mitochondrial encephalomyopathy in the year 1962. But the term was first used by Shapira in 1977 to describe cases with complex multisystem disease with structurally and/or functionally abnormal mitochondria in brain or muscles.  Though regarded as rare clinical entities, in the last few years due to heightened awareness and research, mitochondrial abnormalities have been attributed in many neurological disorders. Because of its varied presentation, clinical correlation with the investigations is needed to label it as a mitochondrial disorder.
| Case report|| |
A 45-year-old female, presented with complaints of swelling on the anterior abdominal wall of eighteen months duration. She had urinary incontinence for the past four years, which was precipitated by coughing, vomiting and sneezing. It was diagnosed as stress incontinence. She was practicing pelvic floor exercises.
She had undergone LSCS in the year 1983, 1993 and 1996 under uneventful General anaesthesia.
The patient had been diagnosed to have Mitochondrial cytopathy in the year 1997. The incidental diagnosis was made to confirm the same condition in her children who were investigated for recurrent seizures.
On examination the patient was 71 kg, 141 cm in height, had a pulse rate of 85/ minute and a blood pressure of 130/80 mmHg. She had no neurological deficits or muscle weakness. She had problem with the vision, which was attributed to pigmentary retinopathy. The systemic examination was normal.
On investigation, haemoglobin, creatinine and electrolyte estimations were normal. Her blood sugar was elevated, which was attributed to the steroids she was on. Preoperative serum lactic acid was estimated to be 1.36 mmol/L. An electrocardiogram and chest X-ray were normal. Abdominal ultrasound showed moderate hepatomegaly with grade I fatty infiltration.
In view of the mitochondrial cytopathy it was decided to expose the patient to the minimal possible anaesthetics and avoid polypharmacy. A combined spinal and epidural anaesthesia was planned.
The procedure was explained to the patient. She was kept nil by mouth for 8 hours and premedicated with Ranitidine 150 mg orally. In the anaesthesia room, an intravenous access was secured with a 16G cannula in her left hand under local anaesthesia. After routine monitors were applied, patient was placed in left lateral position and under aseptic precaution local anaesthetic was injected at L1/L2 using a 26 G needle. A 20G epidural catheter was secured at a depth of 8 cm using 18G Tuhoy needle. Test dose of 3ml of 2%lignocaine with 1:200000 adrenaline was injected. We then proceeded to achieve a subarachnoid block at L3/L4 using a 25 G spinal needle with 3.5 ml of 0.5% Bupivacaine heavy. Block was checked to be achieved up to the level of T6.
Intraoperatively sedation was avoided. Fluids were managed by avoiding Ringers lactate.
Post-operatively the patient was monitored in the recovery room for return of good muscle power, new neurological problems, respiratory difficulties and any cardiac arrhythmias. Serum lactic acid levels was repeated and found to be 2.1 mmol/L.
| Discussion|| |
The mitochondrial cytopathies are a clinically and biochemically heterogeneous group of disorders characterized by abnormalities that involve either a mitochondrial or a nuclear gene.  Mitochondrial cytopathies can be divided into two main categories. In the first category, the respiratory complexes in the respiratory chain are affected as in MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke) and Kearns-Sayre syndromes. ,,, In the second category, lipid metabolism is affected either due to a defect in the transferred fatty acids across the mitochondrial membrane which is caused by carnitine deficiency or due to a defect of B-oxidation of fatty acids. In both cases, the energy production from lipid metabolism will be insufficient. 
Patients with mitochondrial cytopathies may have mitochondrial DNA mutations.  This will cause impaired electron transport chain function resulting in decreased ATP production and formation of damaging free radicals. These reactive oxygen species have also been implicated in mitochondrial cytopathies.  It is possible that anaesthetics affect patients with mitochondrial disease by causing further decrease in energy supplies. 
In reviewing a limited number of reports available, describing adverse events and outcomes in patients with mitochondrial diseases following surgery and anaesthesia, a number of inferences can be made. However these does not define the limits for the safe use of any anaesthetic in these cases.
Patients with mitochondrial cytopathies, on average, are "sicker" and hence are prone perioperatively for new neurological problems such as strokes, worsening of the overall neurological status, respiratory difficulties, seizures, cardiac arrhythmias, prolonged coma and death. Risk for perioperative pneumonia increases because of low muscle tone, weakness of the muscle that protects the airway  and relatively poor ventilatory function.
Although published reports indicate a possible association between malignant hyperthermia and mitochondrial myopathies,  volatile anesthetics have been used uneventfully in several case reports. ,
Some patients with Kearn-Sayres are at risk for severe heart electrical conduction blocks. Isoflurane may be a preferred inhalational agent as opposed to Halothane, because it causes fewer disturbances in heart rhythms.
Patients with mitochondrial cytopathy may have a high resting lactate levels.  Hence ringer's lactate is avoided as an intravenous solution.
Though it has been suggested that mitochondrial myopathies does not involve the neuromuscular junction,  increased sensitivity to different nondepolarizing neuromuscular blockers  and succinylcholine  has been demonstrated..
Although intrathecal and epidural anaesthesia appears to be safe in patients with mitochondrial cytopathies, , it should be used with extra caution because of the possible deleterious effects on blood pressure and respiratory function.
| Conclusion|| |
Taking all modes of anaesthesia into consideration we feel that the optimum precautions to decrease the risk of complications in these cases are
- Fasting not more than 8hours at a stretch.
- Avoiding the depolarizing neuro muscular blocker and judicious use of non-depolarizing neuro muscular blocker.
- Avoiding the drugs that effect mitochondrial function like Propofol, Benzodiazepines and Halothane.
- Avoidance of Ringers Lactate.
- Maintenance of normothermia and normoglycemia.
- Monitoring of Acid base balance.
- Heightened level of suspicion for complications such as pneumonia.
- Early intervention in case of respiratory deterioration.
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