Anaesthesia Management in a Child with Rubinstein - Taybi Syndrome Scheduled for Punctoplsty

Robinstein - Taybi Syndrome (RTS) is a rare chromosomalmutation, which was first described by Robinstein and Taybi in 1963. Leading cause of death are respiratory complications and congenital heart disease. Anaesthesia management of these patients because of a number of body systems involvement, such as heart and respiratory systems, and probability of difficult intubation due to craniofacial abnormalities are very challenging for anaesthesiologists (1). This patient was a 3 - year - old child weighting 11 kg with a height of 80 cm and came to the Operating Room (OR) for punctoplsty because of punctual atresia. He couldn’t speak and walk and a neurologist diagnosed his disorder after his mental and growth retardation. Microstomia, macroglossia, and slight micrognathia were observed during physical examinations. Drug history was negative. Chest X - ray, electrocardiography, and echocardiography were normal. Lab tests were in a normal range. He was very anxious and refused to eat premedication drugs, such as ketamine or midazolam for tranquility. He didn’t allow for insertion of intravenous cannulation and he tightly held his father. His father wore operating room special cloths and came with him to the OR. Because of these problems and probability of difficult intubation, it was decided to use anaesthesia with sevoflurane for inhalation induction. The researchers checked monitoring and provided difficult intubation cart and then said to his father to put on a mask over his face. Sevoflurane dial was increased step by step until 8%. After the patient became deep, he was separated from his father and his father went out of the OR. Monitoring was initiated with pulse oximetry, capnography, non - invasive blood pressure examination, and electrocardiography.