Anesthetic Management of a Pediatric Patient With Bardet-Biedl Syndrome: A Case Report

Abstract

Bardet-Biedl syndrome is a rare autosomal recessive genetic disorder that affects many organ systems. In cases of Bardet-Biedl syndrome, since the risk of developing inspiratory, cardiovascular, and metabolic problems is high, endotracheal intubation and anesthesia management are difficult. In this report, we present our experience in the management of anesthesia during endoscopic sinus surgery that was performed for the first time on a 6-year-old pediatric patient diagnosed with Bardet-Biedl syndrome.

Bardet Biedl Syndrome: Insights for the Oral Healthcare Provider

“…In case of BBS patients requiring operation under a general anaesthetic, such as a facial bone fracture repair or tumour/cyst excision involving the jaws, features such as dental anomalies, high arched palate, facial dysmorphism and morbid obesity may present with significant airway difficulties or impede mask ventilation [2,43]. Presence of a bifid epiglottis has been reported to further complicate establishment of a secure airway, wherein use of fibre optic laryngoscopy or video laryngoscopy could prove vital [2].…”

“…Gencol [43] emphasized on the fact that ciliary dysfunction leads to thickened airway secretions, thereby making it difficult for the patient to clear the airway after Extubation. They suggested that maintenance of good intra operative hydration would circumvent this problem.…”