Anesthesia and perioperative management for a patient with Ullrich syndrome undergoing surgery for scoliosis.

Ullrich syndrome is a rare congenital hypotonic-sclerotic muscular disorder in which affected children develop a slowly progressive scoliosis and contractures and limpness of joints. The disease causes increasingly invalidating contractures and hardening of the muscles of the neck and trunk. While this neuromuscular type of scoliosis is progressive, patients rarely attain the point of surgery due to their compromised general medical condition. This may explain the current lack of outcome data and the paucity of information on perioperative management for patients with Ullrich syndrome undergoing major surgery. The purpose of this report was therefore to describe our first experience with the perioperative and anesthetic management of a 15-year-old boy presenting with Ullrich syndrome and a secondary invalidating scoliosis. The specific challenges of this condition characterized by severe restrictive lung disease and a challenging airway abnormality are discussed.

Optimization of desflurane administration in morbidly obese patients: a comparison with sevoflurane using an 'inhalation bolus' technique.

BACKGROUND: The concept of an 'inhalation bolus' can be used to optimize inhaled drug administration. We investigated the depth of anaesthesia, haemodynamic stability, and recovery time in morbidly obese patients resulting from bispectral index (BIS)-guided sevoflurane or desflurane administration and BIS-triggered inhalation boluses of sevoflurane or desflurane combined with titration of remifentanil. METHODS: Fifty morbidly obese patients undergoing laparoscopic gastroplasty received either BIS-guided sevoflurane or desflurane anaesthesia in combination with a remifentanil target-controlled infusion. Intraoperative haemodynamic stability and BIS control were measured. Immediate recovery was recorded. RESULTS: Intraoperatively, the BIS was between 40 and 60 for a greater percentage of time in the sevoflurane (78 (13)% of case time) than in the desflurane patients (64 (14)% of case time), owing to too profound anaesthesia in the desflurane patients at the start of the procedure. However, fewer episodes of hypotension were found in the desflurane group, without the occurrence of more hypertensive episodes. During immediate recovery, eye opening, extubation, airway maintenance, and orientation occurred sooner in the desflurane group. CONCLUSIONS: Immediate recovery was significantly faster in the desflurane group. Overall hypnotic controllability measured by BIS was less accurate with desflurane. Overall haemodynamic controllability was better when using desflurane. Fewer episodes of hypotension were found in the desflurane group. The use of the inhalation bolus was found to be appropriate in both groups without causing severe haemodynamic side effects. Minimal BIS values were significantly lower after a desflurane bolus.