Anesthesia considerations for patients with de Barsy syndrome.
OBJECTIVE
To determine anesthetic considerations for patients with de Barsy syndrome, a rare complex whose hallmark findings include cutis laxa, progeria, and multiple orthopedic and ophthalmologic abnormalities.


METHODS
Retrospective chart review.


METHODS
Medical center.


METHODS
A search of Mayo Clinic medical records from 1968 to 2007 identified two patients with de Barsy syndrome who underwent a combined total of 35 anesthetics for diagnostic and surgical procedures. Data collected included: age, gender, ASA physical status, relevant comorbidities, surgical procedures, airway management, vascular access, monitoring, anesthetic induction, maintenance, and other observations.


RESULTS
A wide range of anesthetics and techniques were used. Apart from 4 episodes of intraoperative hyperthermia and postoperative tachycardia, no complications were noted. These episodes may be similar to the nonmalignant hyperthermia reported in osteogenesis imperfecta and Costello syndrome patients.


CONCLUSIONS
While the safety of any anesthetic technique cannot be established or extrapolated from a small series, given the extreme rarity of the syndrome, these cases suggest the relative safety of anesthesia in de Barsy syndrome patients.
Department of Anesthesiology, Mayo Clinic College of Medicine, 200 First St., SW, Rochester, MN 55905, USA.
Elisabeth P
EP
Aponte
Hugh M
HM
Smith
Bryan J
BJ
Wanek
Steven R
SR
Rettke
D000758:Anesthesia / Q000009:adverse effects / Q000379:methods*; D000777:Anesthetics / Q000008:administration & dosage* / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D003318:Corneal Opacity / Q000503:physiopathology / Q000601:surgery; D003483:Cutis Laxa / Q000503:physiopathology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000503:physiopathology / Q000601:surgery; D007431:Intraoperative Complications / Q000453:epidemiology; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies
D000777:Anesthetics