LETTERS TO EDITOR
Year : 2018 | Volume
| Issue : 3 | Page : 498-499
Schwartz–Jampel syndrome: Is risk of malignant hyperthermia the same as that of the general population?
Michael Olusegun Ayeko
King Faisal Specialist Hospital and Research Centre, Riyadh 12713, Saudi Arabia
Dr. Michael Olusegun Ayeko
King Faisal Specialist Hospital and Research Centre, Zahrawi Street, Al Maather, Riyadh 12713
Source of Support: None, Conflict of Interest: None
|Date of Web Publication||2-Jul-2018|
|How to cite this article:|
Ayeko MO. Schwartz–Jampel syndrome: Is risk of malignant hyperthermia the same as that of the general population?. Saudi J Anaesth 2018;12:498-9
|How to cite this URL:|
Ayeko MO. Schwartz–Jampel syndrome: Is risk of malignant hyperthermia the same as that of the general population?. Saudi J Anaesth [serial online] 2018 [cited 2020 Oct 25];12:498-9. Available from: https://www.saudija.org/text.asp?2018/12/3/498/235778
I read with interest the case report, “A successful anesthetic approach in a patient with Schwartz–Jampel syndrome (SJS)” by de Oliveira Camacho et al. However, I wish to point out that their conclusion that patients with SJS are at increased risk of malignant hyperthermia(MH) is not supported by the scientific evidence. Similar to the previous authors reporting anesthetic management of SJS cases, they based their conclusion on the case report by Seay and Zifer published in 1978. Seay and Zifer described a 23-month-old patient with SJS booked for cleft palate repair whose temperature rose by 1.5°C after induction of general anesthesia with ketamine (40 mg intramuscular [im]), nitrous oxide, atropine (0.2 mg im), and curare (1.5 mg intravenous). Her pulse rate also increased from 140 to 200 beats/min. Her temperature returned to preoperative values within 30 min without any treatment.
It is noteworthy that the patient was not exposed to suxamethonium and any volatile anesthetic agent, known MH-triggering agents. An in vitro muscle contracture test was not done afterward, although it is considered by some authorities not to be fully sensitive in children aged <10 years. It should also be noted that Parness et al. after carrying out a detailed review of the physiology of muscle excitability and excitation–contraction coupling, as well as the pathophysiology of MH and the myotonias, and the clinical literature on which the claims of MH susceptibility are based concluded that SJS patients have a risk of developing MH that is equivalent to that of the general population. Indeed, this is supported by the case report of a boy who had multiple anesthetics with sevoflurane with no evidence of MH. The authors however recommended that suxamethonium should be avoided in patients with SJS because of the risk of causing dangerous hyperkalemia.
In summary, the association of SJS, a rare chondrodystrophic myotonic syndrome, with MH in the literature is based on one case report in which no triggering anesthetic agents were administered to the patient. Recent scientific evidence suggests that no such association exists. Suxamethonium is however contraindicated in these patients.
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| References|| |
de Oliveira Camacho FC, Lopes Amaral TM, de Barros Mourão JI. A successful anesthetic approach in a patient with Schwartz-Jampel syndrome. Saudi J Anaesth 2018;12:128-30.
Seay AR, Ziter FA. Malignant hyperpyrexia in a patient with Schwartz-Jampel syndrome. J Pediatr 1978;93:83-4.
Gupta PK, Hopkins PM. Diagnosis and management of malignant hyperthermia. BJA Educ 2017;17:249-54.
Parness J, Bandschapp O, Girard T. The myotonias and susceptibility to malignant hyperthermia. Anesth Analg 2009;109:1054-64.