A 21-year-old white male with
Rubinstein-Taybi syndrome (RTS) underwent emergency
laparotomy and
volvulus reduction for a strangulated
ileus.
Low blood pressure, rapid heart rate and dusky skin color indicated that he was in a
hypovolemic and/or
septic shock state. Communication with the patient was impossible because of severe
mental retardation,
deafness, and
blindness, and he was quite combative and agitated. Because of an urgent situation and in anticipation of a great risk of regurgitation, no
sedatives or
anesthetics were used for induction of
anesthesia. A large dose of
vecuronium with a priming principle technique was the only agent used for endotracheal intubation. Vigorous fluid replacement and appropriate
catecholamine therapy were required for the perioperative management and recovery from the
shock state. Any cardiac episodes which have been reported in patients with RTS, such as supra-ventricular or ventricular
arrhythmia, did not occur throughout the
perioperative period. Lack of communication with the patient was an obstacle in the
postoperative care, such as respiratory management or the estimation for the timing of extubation. In conclusion, the preparations for a possibly difficult airway and the possible occurrence of
arrhythmia were thought to be prudent for the management of the patient with RTS.