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LETTERS TO EDITOR
Year : 2022  |  Volume : 16  |  Issue : 4  |  Page : 519-520

Schwartz-Jampel syndrome is a high risk of difficult airway management rather than malignant hyperthermia


Department of Anesthesiology, Misr University for science and Technology, Cairo, Egypt

Correspondence Address:
Ashraf Mohamed EL-Molla
Department of Anesthesiology, Misr University for science and Technology, Cairo
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sja.sja_163_22

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Date of Submission19-Feb-2022
Date of Acceptance20-Feb-2022
Date of Web Publication03-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



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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Palabıyık O. Total intravenous anesthesia in a child with Schwartz Jampel syndrome. Saudi J Anaesth 2022;16:137-8.  Back to cited text no. 1
    
2.
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.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Parness J, Bandschapp O, Girard T. The myotonias and susceptibility to malignant hyperthermia. Anesth Analg 2009;109:1054-64.  Back to cited text no. 3
    
4.
Godai K. Schwartz–Jampel syndrome is not related to malignant hyperthermia. JA Clin Rep 2017;3:32.  Back to cited text no. 4
    
5.
Shaalan O, Daoud M, EL-Molla A, Al-Otaibi R, Alatassi A. Spinal anesthesia in a patient with Schwartz–Jampel syndrome. JA Clin Rep 2020;6:51.  Back to cited text no. 5
    
6.
Ray S, Rubin AP. Case report: Anesthesia in a child with Schwartz-Jampel syndrome. Anesthesia 1994;49:600-2.  Back to cited text no. 6
    
7.
Kozanhan B, Saltalı AO. Syndromes with facial-extremity defects as major feature and anesthesia management. Genel Tıp Derg 2016;26:28-33.  Back to cited text no. 7
    
8.
de Oliveira Camacho FC, Lopes Amaral TM, de Barros Mourao JI. A successful anesthetic approach in a patient with Schwartz-Jampel syndrome. Saudi J Anaesth 2018;12:128-30.  Back to cited text no. 8
    




 

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