Barbara, David W.

SummaryBackgroundBardet–Biedl syndrome (BBS) is a rare genetic condition with manifestations that can impact anesthetic and perioperative care. There is a void of literature describing the perioperative anesthetic management in this complex patient population.ObjectivesThe purpose of this retrospective series was to describe the perioperative care of patients diagnosed with BBS at a large academic tertiary referral center with experience in caring for these patients.MethodsAll patients receiving anesthesia during the time between July 10, 1997 and Jan 1, 2015 were identified. Anesthetic and perioperative records were reviewed in detail for demographic, echocardiographic, preoperative, intraoperative, and postoperative data.ResultsWe identified 12 patients with BBS undergoing 40 anesthetics at our institution. The study identified a high risk for difficult airway with need for advanced airway techniques (67% of patient's ≥18 years of age required either awake fiberoptic or video laryngoscopy techniques), cardiac abnormalities, renal impairment, morbid obesity, and intellectual disability as the main syndrome manifestations of interest to the anesthesiologist. No patient had perioperative complications directly related to BBS; however, each underwent thorough perioperative evaluation with emphasis on the systemic comorbidities associated with the syndrome. This report illustrates that patients with BBS can safely undergo anesthesia, but a detailed and often multidisciplinary preoperative evaluation is prudent.

Abstract Supplemental Digital Content is available in the text.

Abstract Supplemental Digital Content is available in the text.

SummaryObjectivesTo describe the experience of a single, tertiary care institution in the care of patients with Fontan physiology undergoing anesthesia for noncardiac surgery.BackgroundThe Fontan procedure was developed in 1971 to palliate patients with univentricular cardiac physiology leading to long‐term survival of these patients, who may now present as adults for noncardiac surgery.MethodsWe retrospectively reviewed the medical records of Fontan patients 16 years and older who underwent general anesthesia for noncardiac surgery at Mayo Clinic in Rochester, Minnesota. Preoperative data, perioperative course, intraoperative and postoperative hemodynamic, pulmonary, cardiovascular, and renal complications were described.ResultsThirty‐nine general anesthetics were administered to 31 patients for noncardiac surgery after Fontan palliation. Perioperative complications occurred in 12 of the 39 (31%) noncardiac surgeries, and there was one postoperative death that occurred on day 13 after ventral hernia repair. The two patients who had complications that did not resolve (long‐term dialysis and death) had ejection fractions well below the mean for the group (22% and 28%).ConclusionIt may be more appropriate for Fontan patients to undergo anesthesia for noncardiac surgery in a tertiary institution, particularly patients with an ejection fraction of <30%. Intraoperative arterial blood pressure monitoring and overnight admission are likely appropriate for most cases.