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Anesthesia considerations for patients with de Barsy syndrome
Elisabeth P Aponte1, Hugh M Smith, Bryan J Wanek
1Department of Anesthesiology, Mayo Clinic College of Medicine, 200 First St., SW, Rochester, MN 55905, USA.
Journal of Clinical Anesthesia
|November 9, 2010
Summary
Anesthesia for de Barsy syndrome patients, a rare condition with cutis laxa and progeria, appears relatively safe. Limited complications were observed during 35 anesthetics, suggesting careful management is feasible.
Area of Science:
- Medical Genetics
- Anesthesiology
- Rare Diseases
Background:
- De Barsy syndrome is a rare genetic disorder.
- Hallmark features include cutis laxa, progeria, and skeletal and eye abnormalities.
Purpose of the Study:
- To review anesthetic considerations for patients with de Barsy syndrome.
- To assess the safety of various anesthetic techniques in this population.
Main Methods:
- Retrospective chart review of two patients with de Barsy syndrome.
- Analysis of 35 anesthetics administered for diagnostic and surgical procedures.
- Data included patient demographics, comorbidities, and anesthetic management details.
Main Results:
- A diverse range of anesthetic agents and techniques were employed.
- Four instances of intraoperative hyperthermia and postoperative tachycardia were recorded.
- No other significant complications were noted during the anesthetics.
Conclusions:
- Anesthesia in de Barsy syndrome patients appears to be relatively safe.
- Observed hyperthermia episodes may resemble those in osteogenesis imperfecta and Costello syndrome.
- Further research is needed due to the rarity of the syndrome.
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