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LETTER TO EDITOR
Year : 2012  |  Volume : 6  |  Issue : 1  |  Page : 86-87

Malignant hyperthermia-like episode in a child with Larsen syndrome


Department of Anaesthesiology, Kasturba Medical College, Manipal University, Manipal, Karnataka, India

Correspondence Address:
Madagondapalli S Nataraj
Department of Anaesthesiology, Kasturba Medical College, Manipal University, Manipal - 576 014, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-354X.93076

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Date of Web Publication21-Feb-2012
 


How to cite this article:
Kulkarni M, Nataraj MS, Sarah C. Malignant hyperthermia-like episode in a child with Larsen syndrome. Saudi J Anaesth 2012;6:86-7

How to cite this URL:
Kulkarni M, Nataraj MS, Sarah C. Malignant hyperthermia-like episode in a child with Larsen syndrome. Saudi J Anaesth [serial online] 2012 [cited 2019 Dec 9];6:86-7. Available from: http://www.saudija.org/text.asp?2012/6/1/86/93076

Sir,

Larsen syndrome was first described by Dr. Loren J. Larsen in 1950 as a syndrome with multiple joint dislocations, ligamentous laxity, club foot, dysmorphic facies, and depressed nasal bridge. [1] Patients with this syndrome require repeated anesthetics. Because of the various musculoskeletal abnormalities in these patients, they are at increased risk during perioperative period. [2] We present a case of malignant hyperthermia (MH)-like episode in a child with Larsen syndrome undergoing surgery for deformity correction.

A 4-year-old boy weighing 15 kg was scheduled to undergo corrective surgery under general anesthesia. The child had multiple musculoskeletal abnormalities and dysmorphic facies with depressed nasal bridge, suggestive of Larsen syndrome.

After premedication with oral midazolam, anesthesia was induced with sevoflurane in oxygen. He was paralyzed with vecuronium 1.5 mg intravenous (IV). In view of the possibility of cervical spine instability in Larsen syndrome, laryngoscopy and intubation were done with manual inline stabilization. Thirty minutes through surgery, endtidal CO2 (EtCO2 ) had increased to 55 mmHg. Bilateral equal air entry into lungs was reconfirmed and ventilatory settings were re-adjusted to increase the minute ventilation. The EtCO2 did not decrease with the ventilatory changes and had increased to 60 mmHg in another 15 min. The nasopharyngeal temperature had increased to 39.1°C and the heart rate was 160 beats/min in spite of adequate depth of anesthesia and analgesia. Tepid sponging with cold water was given. In view of the raising temperature, EtCO2 and heart rate with underlying musculoskeletal abnormality, a possibility of MH was considered. Blood gas analysis showed respiratory and metabolic acidosis. Immediately, nitrous oxide and sevoflurane were discontinued and the breathing circuit was changed. Further maintenance of anesthesia was done with IV propofol infusion. Supportive management with cold IV saline drip, tepid sponging, and hyperventilation was continued. In the next half an hour, the temperature, heart rate, and EtCO2 were almost normalized. IV hydrocortisone 25 mg was given. After the end of surgery, residual neuromuscular blockade was reversed and because the child was awake and stable, trachea was extubated and observed in the intensive care unit. The next 24 h were uneventful.

This child with Larsen syndrome had intraoperative clinical features suggestive of MH. Although dantrolene was not used because of immediate nonavailability, the signs subsided with supportive management. Although not all myopathic patients are MH susceptible, the presence of a known or suspected myopathy should alert the anesthesiologist to the increased risk for MH or conditions that may mimic MH. [3] Based on the MH clinical grading scale, established in 1994 by an MH expert panel, [4] our patients scored 38 points; 15 for respiratory acidosis, 10 for rapid rise in temperature, 3 for inappropriate sinus tachycardia, and 10 for arterial pH<7.25. A score of 38 on this scale was suggestive of "very likely of MH." But this score was underestimated in our patient because the response to dantrolene was not checked and creatinine kinase levels were not estimated.

Preoperative evaluation of these patients should consist of a thorough assessment of the respiratory, cardiovascular, neurologic, and musculoskeletal systems to avoid unanticipated complications.

 
  References Top

1.Larsen LJ, Schotstaedt ER, Bost FC. Multiple congenital dislocations associated with characteristic facial abnormality. J Pediatr 1950;37:574-81.  Back to cited text no. 1
    
2.Strazis KP, Fox AW. Malignant hyperthermia: A review of published cases. Anesth Analg 1993;77:297-304.  Back to cited text no. 2
[PUBMED]    
3.Brandom BW, Gronert GA. Malignant hyperthermia. In: Motoyama EK, Davis PJ, eds. Smith's anesthesia for infants and children, 6 th Ed. St Louis: C.V. Mosby; 1996. p. 809-26.  Back to cited text no. 3
    
4.Larach MG, Localio R, Allen GC. A clinical grading scale to predict malignant hyperthermia susceptibility. Anesthesiology 1994;80:771-9.  Back to cited text no. 4
    



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