Dear Editor,
I read with interest the Letter to Editor, “Total intravenous anaesthesia in a child with Schwartz-Jampel syndrome”.[1] 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.[2] It is concluded that SJS patients have a risk of developing MH that is equivalent to that of the general population,[3] and Godai stated that SJS is not related to MH.[4] 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.[5] 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.[6,7,8] 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.[5] In summary, the consideration of spinal anaesthesia and difficult airway management have a priority in patients with SJS.
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References
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