Medical Journal of Dr. D.Y. Patil Vidyapeeth

CASE REPORT
Year
: 2014  |  Volume : 7  |  Issue : 3  |  Page : 381--383

Difficulties in management of malignant hyperthermia in a rural set up of a developing country


Debashis Saha1, Arunima Chaudhuri2, Sumanta Ghosh Maulik3, Shankar Ray1,  
1 Department of Anaesthesiology, Murshidabad Medical College, Burdwan, India
2 Department of Anaesthesiology, Physiology, BMCH, Burdwan, India
3 Department of Anaesthesiology, Anaesthesiology, BMCH, Burdwan, India

Correspondence Address:
Arunima Chaudhuri
Krishnasayar South, Borehat, Burdwan - 713 102
India

Abstract

Lack of availability of dantrolene sodium causes difficulty in management of malignant hyperthermia in different parts of India. We hereby report the case of a seven-year-old girl child who developed malignant hyperthermia and expired in spite of all sincere efforts. In conclusion, it is essential to have a comprehensive knowledge of the disease (MH) for a successful anesthetic management and dantrolene sodium should be available in all parts of India.



How to cite this article:
Saha D, Chaudhuri A, Maulik SG, Ray S. Difficulties in management of malignant hyperthermia in a rural set up of a developing country.Med J DY Patil Univ 2014;7:381-383


How to cite this URL:
Saha D, Chaudhuri A, Maulik SG, Ray S. Difficulties in management of malignant hyperthermia in a rural set up of a developing country. Med J DY Patil Univ [serial online] 2014 [cited 2020 Feb 26 ];7:381-383
Available from: http://www.mjdrdypu.org/text.asp?2014/7/3/381/128993


Full Text

 Introduction



Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases such as halothane, sevoflurane, desflurane and the depolarizing muscle relaxant succinylcholine, and rarely, in humans, to stresses such as vigorous exercise and heat. The incidence of MH episodes during anesthesia is between 1 : 5,000 and 1 : 50,000-100,000 anesthesias. [1],[2],[3] All ethnic groups are affected, in all parts of the world. The highest incidence is in young people. Genetically, MH is an autosomal dominant condition. [1],[2]

The pathophysiology of MH involves altered sarcoplasmic reticulum calcium channel gating kinetics. [2] The high levels of calcium in sarcoplasmic reticulum sets in aerobic metabolism, glycolysis and neutralization of hydrogen ions and hydrolysis of high energy phosphate compound leading to acidosis, rigidity, altered permeability, hyperkalemia and rise in temperature. The first sign of MH under anesthesia is increase in end tidal CO 2 exponentially. Inheritance of MH is probably mutation of two to three genes and the pattern of inheritance may vary from dominant to recessive (RYR1 and CACNAIS). [3] Mutations in RYR1 (ryanodine receptor) are also responsible for central core disease (CCD), a myopathy that can be associated with a positive halothane contracture test. The incidence of malignant hyperthermia in India may not be as low as was thought previously. Dantrolene, the treatment of choice is now available in New Delhi, and should be made available in other cities also. If an MH crisis is suspected in the operating room, all triggering anesthetic agents are stopped. Early aggressive management of MH, strongly suspected on clinical basis could change outcome of a patient. [3],[4]

 Case Report



An active healthy seven-year-old girl weighing 18 kg was scheduled to undergo interpositional gap arthroplasty for bony ankylosis of both temporo-mandibular joint (TMJ) under general anesthesia. On pre-anesthetic examination, there was a history of trauma in early childhood and restricted mouth opening (<0.5 cm with crowding of lower anterior teeth), Mallampatti grade-IV. All routine hematological and biochemical investigations were found to be normal. Radiographic investigation included posterior-anterior view; OPG and CT-scan confirmed bony ankylosis of both TMJ and elongation of coronoid process bilaterally. Intubation was planned under sedation with the patient breathing spontaneously with an alternative back-up plan of tracheostomy. During preoperative visit, a good rapport was established with the patient and written informed consent was obtained from her parents. On the day of surgery the patient had received tablet diazepam 2.5 mg orally and injection glycopyrrolate 0.1 mg i.m. one hour earlier. In the operating room, an intravenous line was secured and monitors (spO 2 , NIBP, ECG, and ETCO 2 ) attached. Oropharynx was anesthetized by lignocaine (gargling and nebulizing). The patient was sedated with inj. Propofol 40 mg + 1 mg midazolam. The superior laryngeal nerve was blocked with lignocaine. Patient was pre-oxygenated with 100% O 2 for 5 min. Then blind nasal intubation was performed with 4.5 mm flexometallic tube (first attempt failed but succeeded in second attempt). Atracurium was given and IPPV was started with oxygen and nitrous oxide with halothane. After one hour it was noted that the EtCO 2 was being maintained at 47-48 mm Hg in spite of slight hyperventilation. Suspecting some problem to have developed in the breathing circuit, it was changed, but the EtCO 2 continued to be maintained at this level. The patient's pulse rate rose gradually to 130 beats per minutes and then suddenly to 160 beats per minutes. Atracurium required more frequently than usual. The pulse rate, temperature ETCO 2 all went up. Nasopharyngeal temperature increased to 108°F. A provisional diagnosis of MH was made, halothane discontinued and the circuit was changed. Cold hydrotherapy was started. The patient was ventilated O 2 : N 2 O 50 : 50 and the surgery completed as early as possible. ABG was done at FiO 2 0.5, showing pH - 7.1, P CO2 -74 mm Hg, PaO 2 - 250 mm Hg, BE - 10, HCO 3 - 23 mEq/L, serum Na-144 mEq/L, K - 6.8 mEq/L. Blood samples were sent to the laboratory for estimating creatinine phosphokinase (CPK), CPK (MB) and lactic dehydrogenase (LDH). Patient was catheterized. Myoglobin was present in urine. Inj. hydrocortisone and NaHCO 3 were given as required intravenously, hyperventilation was continued to correct respiratory acidosis and dextrose insulin drip given. The patient was shifted to ICU for ventilatory support. Midazolam injection was used for sedation. After 4 h of ventilation the patients suddenly developed supraventricular tachycardia and expired. The treatment of choice for MH, dantrolene sodium, is not available in West Bengal.

 Discussion



A clinical grading scale for MH Raw score was developed in 1994. Larch et al. described the scale for definitive diagnostic indicator of MH. [5]

The total score in the present reported case was 56, MH rank 6 [Table 1] and [Table 2]. Microscopic examination of the biopsy was suggestive of MH.

Punj J et al, in 2001 from All India Institute of Medical Sciences reported a case of malignant hyperthermia in a 21-one year old male from Nepal. MH was triggered by isoflurane. They reported the non-availability of the drug dantrolene. The patient developed diffuse bleeding from all sites after half an hour after being shifted to ICU. A diagnosis of diffuse intravascular coagulation (DIC) was made. Twelve hours after the initial episode, patient suffered a cardiac arrest, and despite all resuscitative measures, could not be revived. [6]{Table 1}{Table 2}

Saxena K et al, in 2007 from New Delhi, reported case of a 28-year-old female. On pre-anesthetic evaluation, there was no significant finding except restricted mouth opening and a swelling over the mandible. On attempting laryngoscopy the patient was found to have masseteric spasm following administration of succinylcholine. After one hour it was noted that the EtCO 2 was being maintained at 47-48 mm Hg in spite of slight hyperventilation. A provisional diagnosis of malignant hyperthermia was made and the patient was treated accordingly. The patient was shifted to the ICU, where she was electively ventilated for the next 24 h. The patient was reversed and extubated the next day and was kept under observation for the next five days during which she remained normal and was discharged on the eighth day. Dantrolene sodium could not be obtained anywhere. [7],[8] The second case reported by Saxena K occurred approximately in 2010. The patient was managed appropriately. In this instance, intravenous Dantrolene was used. The patient recovered without any sequelae. [9]

In 2012, Jain G et al. from Moradabad, reported a case of endosulfan poisoning, admitted in a state of altered consciousness with vomiting and seizure. Caffeine halothane contraction test confirmed it to be malignant hyperthermia (MH). Patient was immediately shifted to synchronized intermittent mandatory ventilation (100% FiO 2 , RR ~20/min). Dantrolene infusion was started (2.5 mg/kg over 10 min). He was extubated on the 4 th day after admission with no other complications. [10]

 Conclusion



In conclusion, it is essential to have a comprehensive knowledge of the disease (MH) for a successful anesthetic management and dantrolene sodium should be available in all parts of India.

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