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

Altun, Dilek; Demir, Güray; Ayhan, Asude; Türköz, Ayda

doi:10.1016/j.egja.2016.08.012

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

Authors

10.1016/j.egja.2016.08.012

Abstract

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 14 kg, 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.
Results and discussion
CSS often requires surgery and anesthetic intervention. The abnormal facial and airway as well as mental related features may lead intubation difficult, potentially due to short neck, large tongue and lips, poor dentition and poor communication.