Abstract
Ullrich congenital muscular dystrophy (UCMD) is a severe form of progressive congenital muscular dystrophy, and it presents with severe hypotonia, recurrent respiratory infections, respiratory failure, and problematic airway management secondary to congenital anatomical abnormalities and high aspiration risk. Pierre Robin sequence (PRS) is a rare congenital syndrome presenting at birth with a triad of micrognathia, glossoptosis, and chronic upper airway obstruction leading to known difficult airway management and opioid sensitivity. Perioperative management of both UCMD and PRS requires careful consideration of airway management, choice of anesthetic agents, and potential involvement of respiratory complications; each condition presents its own unique challenges. This report discusses the successful, complete anesthetic management of a patient with collagen 12 (COL12A1)-variant UCMD and concurrent PRS.