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Case Reports
. 2025 Jul 11:2025:7470643.
doi: 10.1155/crpe/7470643. eCollection 2025.

Anesthesia Management in a Crisponi Syndrome Patient Undergoing Tracheotomy Surgery

Enes Celik  1 , Yusuf Ipek  2 , Osman Oguzhan Kursun  2 , Mehmet Nur Talay  3 , Hakan Akelma  1
Affiliations
Case Reports

Anesthesia Management in a Crisponi Syndrome Patient Undergoing Tracheotomy Surgery

Enes Celik et al. Case Rep Pediatr. .

Abstract

Increased salivation and contractions of the oropharyngeal muscles are frequently observed in Crisponi syndrome. This causes frequent recurrent lung infections. Anesthesia management can be challenging due to the frequent convulsions that occur during the intubation and extubation of the patient and subsequent cyanosis and hyperthermia attacks. Cold-induced sweating attacks may also occur due to the low operating room temperature. Hyperthermia attacks can lead to rhabdomyolysis and disseminated intravascular coagulation. Sudden deaths may occur in children with Crisponi syndrome. Hyperthermia, paroxysmal muscular contractions and trismus due to autonomic dysfunction are held responsible for sudden deaths.

Keywords: airway management; anesthesia; hyperthermia.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Face of a typical Crisponi syndrome patient, characterized by full cheeks.
Figure 2
Figure 2
Edematous appearance of the airway and epiglottis on videolaryngoscopy.
See this image and copyright information in PMC

References

    1. Dagoneau N., Bellais S., Blanchet P., et al. Mutations in Cytokine Receptor-Like Factor 1 (CRLF1) Account for Both Crisponi and Cold-Induced Sweating Syndromes. The American Journal of Human Genetics . 2007;80(5):966–970. doi: 10.1086/513608. - DOI - PMC - PubMed
    1. Onesimo R., Sforza E., Palermo F., et al. Feeding and Nutritional Key Features of Crisponi/Cold-Induced Sweating Syndrome. Genes . 2024;15(9):p. 1109. doi: 10.3390/genes15091109. - DOI - PMC - PubMed
    1. Hakan N., Aydın M., Zenciroğlu A., et al. A Rare Clinical Condition that Confusing With Neonatal Tetanus: Criponi Syndrome: Case Report. Turkiye Klinikleri J Pediatr . 2012;21:31–33.
    1. Allary C., Caruselli M., Fabre A., audic F., Michel F. General Anesthesia for Crisponi Syndrome: Case Report. Brazilian Journal of Anesthesiology (English Edition) . 2020;70(3):299–301. doi: 10.1016/j.bjane.2020.04.010. - DOI - PMC - PubMed
    1. Artilheiro V., Portela F., Reis A. T. Anesthesia for Stüve-Wiedemann Syndrome: A Rare Adult Patient Case Report. Journal of Applied Genetics . 2020;61(4):571–573. doi: 10.1007/s13353-020-00581-5. - DOI - PubMed

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