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

Peer review under responsibility of Egyptian Society of Anesthesiologists.

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.

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 4 h later after the operation without any complication.

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.

Thinking that the practicing anesthetist needs to have appropriate knowledge for this entity and the equipment for managing difficult airway should readily be available. One of these patients which successfully managed without any complication was described in this brief report.

Keywords:

• Coffin-Siris syndrome
• Congenital heart disease
• Laryngomalacia
• Difficult airway

Notes

Peer review under responsibility of Egyptian Society of Anesthesiologists.