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Anesthesia Management in a Crisponi Syndrome Patient Undergoing Tracheotomy Surgery.
Enes Celik1, Yusuf Ipek2, Osman Oguzhan Kursun2
1Department of Anesthesiology and Reanimation, TC Mardin Artuklu Universitesi Tıp Fakultesi, Mardin, Turkey.
Case Reports in Pediatrics
|July 21, 2025
Summary
Crisponi syndrome patients experience increased salivation and muscle contractions, leading to recurrent lung infections. Anesthesia is challenging due to potential convulsions, cyanosis, hyperthermia, and sudden death risks.
Area of Science:
- Medical Genetics
- Pediatric Medicine
- Anesthesiology
Background:
- Crisponi syndrome is a rare genetic disorder.
- It affects oropharyngeal muscles and autonomic function.
Observation:
- Patients exhibit increased salivation and oropharyngeal muscle contractions.
- Recurrent lung infections are common due to these issues.
Findings:
- Anesthesia management is complicated by convulsions during intubation/extubation.
- Autonomic dysfunction can cause cyanosis, hyperthermia, and cold-induced sweating.
- Hyperthermia may trigger rhabdomyolysis and disseminated intravascular coagulation.
Implications:
- Crisponi syndrome poses significant risks, including sudden death in children.
- Careful anesthetic planning is crucial for managing hyperthermia and muscular contractions.
- Understanding autonomic dysfunction is key to preventing fatal complications.
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