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.

Postoperative residual curarization with cisatracurium and rocuronium infusions.

BACKGROUND AND OBJECTIVE: Monitoring of neuromuscular blockade still often relies on clinical judgement. Moreover, there are substantial national differences in the use of agents to 'reverse' their effects. We investigated the recovery characteristics and incidence of postoperative residual curarization after cisatracurium and rocuronium infusions for long duration interventions without systematic antagonism. METHODS: In 30 patients undergoing major surgery, we measured infusion dose requirements for rocuronium and cisatracurium during propofol anaesthesia. Infusions were discontinued at the beginning of surgical closure; spontaneous recovery of neuromuscular function was awaited in both groups. Neostigmine (50 microg kg(-1)) was administered only when a patient started to wake without a train-of-four ratio (TOF) of 0.9. RESULTS: In the cisatracurium and rocuronium groups, four (27%) and one (7%) patients, respectively, had a TOF ratio > or = 0.9 at the end of surgery. The TOF ratio in each group at that time was 51 +/- 32% for cisatracurium and 47 +/- 31% for rocuronium (P = 0.78). Six patients (40%) in the cisatracurium group and seven (47%) in the rocuronium group required neostigmine. The TOF ratio at the time of reversal was 63 +/- 7% for cisatracurium and 40 +/- 19% for rocuronium (P = 0.01). The time interval between the end of surgery and a TOF ratio of 0.9 was 10 +/- 9 min for cisatracurium and 18 +/- 13 min for rocuronium (P = n.s.). CONCLUSIONS: Patients receiving a cisatracurium or rocuronium infusion have a high incidence of postoperative residual curarization when the block is not antagonized. When 'reversal' is not attempted, cisatracurium seems to be safer than rocuronium.

Effect of nitric oxide predilution on inhaled nitrogen dioxide concentrations.

We examined the possibility that predilution of a concentrated nitric oxide (NO) source with nitrogen, before contact with oxygen, can reduce the inspired nitrogen dioxide (NO2) concentration during administration of nitric oxide. A Manley Blease and a Siemens Servo 900 C ventilator delivered 10, 20, 40, 60 and 80 parts per million (ppm) NO using an NO source of 1000, 400 and 200 ppm. With the Manley Blease system, predilution from 1000 to 200 ppm NO reduced the inhaled NO2 concentration from 0.14 to 0.05 ppm (p < 0.01) at 10 ppm inhaled NO, and from 1.20 to 1.00 ppm (p < 0.01) at 40 ppm inhaled NO. With the Siemens Servo 900 C ventilator, inspiratory NO2 concentrations decreased from 0.21 to 0.11 ppm (p < 0.01) at 10 ppm inhaled NO, and from 1.49 to 1.16 ppm (p < 0.01) at 40 ppm NO. Predilution from 1000 to 400 ppm NO reduced the inspired NO2 concentrations by < 3% using either ventilator when the inspirated NO concentration was 80 ppm. Predilution of NO with nitrogen significantly reduced the inspired NO2 concentrations for nitric oxide concentrations between 10 and 40 ppm, but offered no clinically relevant advantage at higher NO concentrations.

In vitro evaluation of the effect of profound haemodilution with hydroxyethyl starch 6%, modified fluid gelatin 4% and dextran 40 10% on coagulation profile measured by thromboelastography.

Synthetic colloids have been implicated as a cause of coagulopathy when administered in large quantities. The effect of profound haemodilution (50%) on coagulation profile was measured in vitro by thromboelastography. Blood samples were taken from 11 ASA grade 1 patients prior to induction of anaesthesia for elective surgery. Each sample was simultaneously tested in four different preparations: undiluted blood (control sample); blood diluted with hydroxyethyl starch 6%; blood diluted with modified fluid gelatin 4%; blood diluted with dextran 40 10%. There was a significant decrease in reaction time in the preparations treated with hydroxyethyl starch 6% and modified fluid gelatin 4%, reflecting activation of initial fibrin formation. A significant increase in clot formation time was noted in the hydroxyethyl starch 6%-treated preparations. There was also a significant decrease in clot formation rate and maximum amplitude in the hydroxyethyl starch 6% group. Clot formation time, clot formation rate and maximum amplitude did not change in the modified fluid gelatin 4% group. Profound haemodilution with dextran 40 10% exerted extreme effects on the measured variables, very often resulting in a straight line on the thromboelastography profile.

Limited intentional normovolemic hemodilution: ST-segment changes and use of homologous blood products in patients with left main coronary artery stenosis.

OBJECTIVE: To assess and compare the effects of limited intentional normovolemic hemodilution (LINH) on ST-segment changes and to evaluate the need for homologous blood products. DESIGN: Prospective, randomized study. SETTING: University hospital. PARTICIPANTS: Seventy-one patients with left main stenosis scheduled for semi-urgent coronary artery bypass grafting. INTERVENTIONS: Patients in group A (n = 39) underwent LINH during the prebypass period until a hematocrit of 34% was obtained. Simultaneously, succinyl-linked gelatin was infused. In group B (n = 32), no hemodilution was performed. Mean arterial pressure and central venous pressure were kept as constant as possible. During the postbypass period, autologous blood was retransfused. The need for homologous blood products was noted intraoperatively and postoperatively. MEASUREMENTS AND MAIN RESULTS: ST-segment analysis of lead II and chest lead was continuously performed in all patients. An ST-segment change was defined as a decrease from baseline of 1.0 mm (-0.1 mV). The appearance and degree of ST-segment depression were comparable in both groups (group A: 7 patients -0.1 mV, 1 patient -0.2 mV; group B: 5 patients -0.1 mV; 3 patients -0.2 mV). In group A, ST-segment depression occurred during and after the blood exchange. However, the mean duration of the ST-segment depression (group A: 33 +/- 18 minutes; group B: 20 +/- 10 minutes) was comparable between groups. In group A, a mean of 750 mL +/- 245 mL of blood was obtained. Total blood loss was significantly higher in group B (p < 0.052); 25 patients in group A (64%) and 12 patients in group B (38%) did not require homologous blood products (p < 0.03). Intraoperatively, only the need for packed red cells was greater in group B (p < 0.04). Postoperatively, the use of homologous blood products is higher than intraoperatively (p < 0.02). CONCLUSIONS: LINH performed in patients with left main stenosis, scheduled for semi-urgent coronary bypass, is not associated with increases in frequency, degree, or duration of ST-segment changes. This procedure allowed a reduction in the number of patients who received homologous blood products.

Evaluation of the anticoagulant properties of aprotinin in vitro.

The interaction of aprotinin with normal coagulation was studied in blood samples obtained from 10 healthy subjects. Each sample was simultaneously tested in four different preparations: NaCl-treated blood: 0.03 mL 0.9% NaCl in 0.33 mL blood; aprotinin treated blood: 0.33 mL blood+aprotinin in 0.03 mL in aliquots to obtain a final blood concentration of respectively 50 KIU mL-1; 100 KIU mL-1 and 200 KIU mL-1. The coagulation process was analysed by thromboelastography. R-time, reflecting intrinsic coagulation, increased in a dose dependent manner between NaCl-treated and aprotinin-treated blood. These findings suggest a dose dependent impairment of intrinsic coagulation by aprotinin.

Rapidly progressive pulmonary artery hypertension and end-stage liver disease.

Pulmonary hypertension is a recognized but unusual complication of liver disease. It can complicate the perioperative course of liver transplantation. Mild to moderate pulmonary hypertension is generally well tolerated during the procedure and does not appear to contribute to mortality. Since the pulmonary vascular disease may progress rapidly, it may have advanced to the point of irreversibility at the time of surgery. So, patients with known moderate pulmonary hypertension should have pulmonary arterial catheterisation immediately prior to transplantation. If pulmonary artery hypertension has become severe, then a preoperative trial of vasodilators is warranted. If this fails, the procedure should be cancelled. We present a patient with alcoholic liver cirrhosis in whom a rapidly progressive pulmonary hypertension made liver transplantation impossible.

Doxacurium block is not influenced by age.

STUDY OBJECTIVE: To determine the influence of aging on the efficacy and safety of doxacurium. DESIGN: Open, randomized, phase III study. SETTING: Inpatient ophthalmic surgery clinic at a university medical center. PATIENTS: 30 elderly patients (65 years or older) compared with a control group of 30 younger patients (18 to 64 years). INTERVENTIONS: An elective ophthalmological surgical intervention of more than 2 hours' expected duration with general anesthesia with isoflurane. MEASUREMENTS AND MAIN RESULTS: Neuromuscular function after a bolus dose of doxacurium was monitored electromyographically. No significant difference was observed in maximum block achieved or onset time. Clinical duration of neuromuscular block was similar in both groups. Hemodynamic changes were clinically unimportant. CONCLUSION: The use of doxacurium in elderly patients is possible with no need for dose adjustment. Doxacurium might be a good choice for patients with cardiac disease who are scheduled for long surgical procedures.