Successful anesthetic and airway management in Coffin-Siris syndrome with congenital heart disease: Case report

Publication date: Available online 9 September 2016 Source:Egyptian Journal of Anaesthesia Author(s): Dilek Altun, Güray Demir, Asude Ayhan, Ayda Türköz Introduction Coffin-Siris Syndrome (CSS) is a rare congenital malformation syndrome characterized with mild to severe developmental and cognitive delay, coarse facial features, fifth digit aplasia or hypoplasia associated with ectodermal, constitutional and organ-related (cardiac/neurological/gastrointestinal/genitourinary…) anomalies. Here, we have reported a successful anesthetic and airway management in a case of 5-year old boy with CSS who underwent congenital heart surgery. Case report A 5-year old male child weighing 14kg, who was diagnosed as CSS underwent operation for the repair of partial atrioventricular septal defect and secundum atrial septal defect. This case report pertains to the successful anesthetic and airway management in the background of difficult airway and presence of various cardiac abnormalities. Although patient was anticipated to be difficult for intubation due to laryngomalacia, micrognathia, macroglossia, tracheal intubation was performed without any difficulty using fiber-optic laryngoscopy. At the end of the operation, the patient was transferred to the cardiovascular intensive care unit and was extubated when his spontaneous breathing was satisfactory 4h later after the operation without any complication. Results and discussion CSS often requires surgery and anesthetic interventio...
Source: Egyptian Journal of Anaesthesia - Category: Anesthesiology Source Type: research