Management of a 10-month-old child with a rare combination of Bardet–Biedl syndrome and ano-rectal malformation undergoing anterior sagittal ano-rectoplasty

Anesthetic considerations for patients with Bardet–Biedl syndrome: a case series and review of the literature

“…Although most patients in this review underwent general anesthesia, there are no inherent contraindications for neuraxial and/or regional techniques related to BBS. Two patients in the literature review underwent neuraxial anesthesia without complication . Although three patients in this study had postoperative complications (PE POD 2 with subsequent death POD 6, acute congestive heart failure POD 3, and renal hematoma POD 6), none of these appear directly related to BBS.…”

“…A difficult airway was anticipated preoperatively in three published case reports as evidenced by physical exam or previously known difficult airway (6,7,11). All six published case reports describing airway management for patients with BBS recounted successful direct laryngoscopy; however, these authors reported the immediate availability of additional airway devices when difficulty was anticipated (7,10,11). Cardiac manifestations of BBS have previously been reported as congenital heart disease, ventricular hypertrophy, and cardiomyopathy (5,(17)(18)(19).…”

“…BBS manifestations often require effected patients to undergo multiple operations throughout their lifetimes. Disease manifestations of importance to anesthesiologists include: risk for difficult airway, cardiac abnormalities, renal impairment, obesity, and a range of intellectual capacities that can make perioperative care challenging . There is very limited literature describing the perioperative care of these complex patients.…”

“…A comprehensive literature search identified only seven isolated case reports in the English language describing anesthetic and/or airway management in patients diagnosed with BBS (Table 4) (6)(7)(8)(9)(10)(11)(12). In these individual case reports, difficult airways, airway abnormalities, and cardiac abnormalities were not infrequent.…”

“…Life expectancy in patients with BBS is significantly reduced and they often present for multiple surgical procedures requiring anesthesia care. Aside from very few isolated case reports , there is a void of literature describing the perioperative anesthetic management in this complex patient population. The 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 with special focus on BBS disease manifestations of interest to the anesthesiologist, namely, risk for difficult airway, cardiovascular abnormalities, renal impairment, obesity, and a range of intellectual capacities that can make perioperative care challenging.…”