LETTERS TO EDITOR
Year : 2022 | Volume
| Issue : 4 | Page : 519-520
Schwartz-Jampel syndrome is a high risk of difficult airway management rather than malignant hyperthermia
Ashraf Mohamed EL-Molla
Department of Anesthesiology, Misr University for science and Technology, Cairo, Egypt
Ashraf Mohamed EL-Molla
Department of Anesthesiology, Misr University for science and Technology, Cairo
Source of Support: None, Conflict of Interest: None
|Date of Submission||19-Feb-2022|
|Date of Acceptance||20-Feb-2022|
|Date of Web Publication||03-Sep-2022|
|How to cite this article:|
EL-Molla AM. Schwartz-Jampel syndrome is a high risk of difficult airway management rather than malignant hyperthermia. Saudi J Anaesth 2022;16:519-20
|How to cite this URL:|
EL-Molla AM. Schwartz-Jampel syndrome is a high risk of difficult airway management rather than malignant hyperthermia. Saudi J Anaesth [serial online] 2022 [cited 2022 Sep 29];16:519-20. Available from: https://www.saudija.org/text.asp?2022/16/4/519/355511
I read with interest the Letter to Editor, “Total intravenous anaesthesia in a child with Schwartz-Jampel syndrome”. However, I want to highlight the following considerations: First, the risk of malignant hyperthermia (MH) in association with Schwartz-Jamel syndrome (SJS) is not supported by scientific evidence. It is concluded that SJS patients have a risk of developing MH that is equivalent to that of the general population, and Godai stated that SJS is not related to MH. Second, spinal anesthesia may be an effective, safe and preferred anesthetic technique in patients with SJS and should be preceded by a neurological examination, radiological, and haematological investigation with special attention to the coagulation profile. Third, difficult airway management should always be expected in SJS patients, and difficult intubation may be due to microstomia, retro/micrognathia, jaw muscle rigidity, and short neck with limited mobility.,, Consequently, whatever the planned anesthetic technique, a full setup for difficult airway management including the presence of two experienced anaesthesiologists in the difficult paediatric airway, and the availability of fiberoptic and video- laryngoscopies should be ensured. In certain cases, the paediatric anaesthesiologist is likely to be present as a backup plan for the surgical airway. In summary, the consideration of spinal anaesthesia and difficult airway management have a priority in patients with SJS.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
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