Anesthetic management of a patient with MERRF syndrome

There are several specific considerations regarding anesthesia in patients with mitochondrial disease. We describe the successful administration of a combined general and epidural anesthesia with sevoflurane maintenance in a patient with myoclonic epilepsy with ragged red fibers (MERRF syndrome) scheduled for surgical treatment of bilateral clubfoot.     

Anesthetic management of a patient with Freeman-Sheldon syndrome

The Freeman-Sheldon syndrome (FSS) is rare congenital myopathy and dysplasia. The musculoskeletal and soft-tissue manifestations of FSS often require orthopedic and plastic reconstructive surgery. We report a case of 19-month-old girl with FSS.     

Laser catheter ablation of simulated ventricular tachycardia

Catheter-directed laser injury of the left ventricular endocardium for ablation of ventricular tachycardia was studied in a canine model of simulated ventricular tachycardia. Bipolar plunge electrodes were placed at thoracotomy into the left ventricular endocardium in nine anesthetized dogs. Ventricular tachycardia was simulated by pacing at 200 beats per minute. After four days of recovery, catheter-directed neodymium:yttrium-aluminum-garnet laser injury was produced at the site of earliest recorded electrical activation during pacing tachycardia as detected by endocardial catheter mapping. Immediately after laser injury and again five days after injury, pacing tachycardia was attempted. Failure to pace after laser injury was defined as successful arrhythmia ablation. In 3/9 (33%) experiments, the laser-injured tissue surrounded the tachycardia source (pacing wires), and pacing-simulated ventricular tachycardia was prevented. When the laser injury did not involve the tachycardia source, 6/9 animals, due to limitations of the mapping system, pacing was not prevented. One animal developed sustained ventricular tachycardia during laser injury, with conversion to sinus rhythm by lidocaine. One animal, without recognized ventricular arrhythmia, died five days after laser injury. No unusual findings were noted at autopsy. These preliminary data suggest that catheter-directed laser-induced injury can ablate arrhythmia sources. Further studies are indicated in a more physiologic model, and the safety and risks of the procedure need further evaluation.     

Anesthetic management of a patient with progressive supranuclear palsy

A 74-year-old woman with progressive supranuclear palsy (PSP) was scheduled for laryngotracheal separation surgery. Her neck showed severe backward tilt as a symptom of PSP. Magnetic resonance imaging (MRI) showed a significant airway stenosis due to the neck deformity. In the operating room, awake orotracheal intubation failed because of the neck deformity and airway stenosis. Therefore, tracheotomy was performed for airway management. General anesthesia was induced and maintained with sevoflurane (1.0%–2.5%) and fentanyl (total, 200 μg). Vecuronium (total, 5 mg) was used as a muscle relaxant. Monitoring of the train-of—four ratio in the ulnar nerve was impossible because of contracture of the fingers. Patients with PSP may have some serious associated deformities, and specific management, especially for the airway, may be necessary for general anesthesia.     

Anesthetic management for subtotal gastrectomy in a patient with paramyotonia congenita

We performed anesthesia for a subtotal gastrectomy in a 70-year-old female patient with paramyotonia congenita (PC). She had been diagnosed with PC at the age of 47 years by electromyogram analysis. Several points to consider have been revealed regarding the management of anesthesia in patients with PC. In this patient, anesthesia was safely maintained using sevoflurane and nitrous oxide together with concomitant epidural anesthesia using mepivacaine. Efforts should be made to prevent perioperative attacks of muscle weakness when planning anesthesia for patients with this kind of disorder. Specifically, refraining from the use of muscle relaxants, care with regard to the composition of infusion fluids during operations, and the maintenance of body temperature are required for anesthesia. In addition, postoperative pain management using a continuous epidural block proved to be a useful method.     

Anesthetic implications of a pediatric patient with stiff skin syndrome: A case report

Stiff skin syndrome is a rare genetic disorder that is present in infancy or early childhood. It is characterized by hard, inflexible skin and limited joint mobility making anesthetic management of these patients challenging. Their limited neck flexibility and chest wall rigidity make intubation and mask ventilation difficult. Intraoperative positioning can be challenging due to joint contractures and potential entrapment peripheral neuropathy. Even though peripheral intravenous access can be relatively easy, central venous cannulation may be problematic due to the hard skin overlying the entry sites. Our case report details the anesthetic management and considerations of a pediatric patient with stiff skin syndrome.     

Position dependent ventricular tachycardia in two children with peripherally inserted central catheters (PICCs)

We report two cases of ventricular tachycardia (VT) in children following the insertion of peripherally inserted central catheters (PICCs). These children had additional procedures requiring turning into the left lateral position after PICC insertion. In both cases sustained VT occurred after turning and flexion of the arm with the PICC. VT was terminated in both cases by withdrawing the catheter.     

Surgery for ventricular tachycardia and left ventricular aneurysm provides arrhythmia control

Objectives. Report long-term freedom from ventricular tachycardia (VT), survival, and causes of death in patients with left ventricular aneurysm and VT, who underwent a combined procedure for VT and surgical ventricular restoration (SVR). Design. The primary outcome measures VT, survival, and cause of death, were ascertained by review of patients’ records, interrogation of implanted cardioverter-defibrillators and use of national registers. Results. Mean follow-up was 5.2 years. Overall survival was 62% at 5 years and 51% at 9 years. Freedom from spontaneous VT was 89%. In 32 patients who were non-inducible at postoperative testing, there was no occurrence of VT during a mean follow-up of 6.0 years. Causes of death were cardiac in 17 patients, and non-cardiac in 6 patients. No patient died from ventricular arrhythmia. Conclusions. Direct surgery for VT combined with SVR resulted in a very low risk of late recurrence of VT and good long-term survival. Implantation of a cardioverter-defibrillator can safely be withheld in patients who are non-inducible on postoperative programmed electrical stimulation.     

Anesthetic management of a patient with right ventricular myxoma]

Right ventricular myxoma in a 79 year old male, whose pulmonary trunk was obstructed by the myxoma, was reported. Myxoma of the heart is rare, and especially right ventricular myxoma is rare. Myxomas of the heart have been reported by thirty-seven authors, but right ventricular myxoma has been reported by only one author. Anesthesia for the removal of right ventricular myxoma must be carried out carefully, because some critical troubles may happen during anesthesia for the resection of the right ventricular myxoma. Particularly, occlusion of the pulmonary artery is the most dangerous complication.     

Anesthetic management of a patient with myotonic dystrophy for a Nissen fundoplication and gastrostomy

A 16-month-old baby with myotonic dystrophy underwent an open Nissen fundoplication and gastrostomy insertion under general anesthesia with an epidural. Postoperative care was managed on the pediatric intensive care unit for the first 6 h. She was then discharged to the ward, where she continued to make an uncomplicated recovery. Other anesthetic management that has been used in children with myotonic dystrophy is discussed.     

Anesthetic management of bidirectional cavopulmonary shunt in a patient with pulmonary atresia with intact ventricular septum associated with sinusoidal communications

Pulmonary atresia with intact ventricular septum (PAIVS) is sometimes associated with coronary artery anomalies, including right ventricle (RV)-to-coronary artery fistulas (sinusoidal communications), coronary artery stenoses, and coronary artery occlusions. In some cases, the coronary circulation depends entirely or partly on the desaturated systemic venous blood supply from the RV. Under these circumstances, decompression of the RV can result in fatal myocardial ischemia. A 6-month-old boy, diagnosed with PAIVS associated with sinusoidal communications, underwent a bidirectional cavopulmonary shunt procedure under venoarterial cardiopulmonary bypass (CPB). During CPB, to prevent RV decompression, we maintained right atrial pressure above 5 mmHg and used a pump perfusion rate of 30%–40% of the calculated value based on body surface area. Although electrocardiography showed slight ST depression and bradycardia, myocardial contractility after weaning from CPB was adequate to maintain the circulation with the administration of dobutamine and atrial pacing. In patients with PAIVS and RV-dependent coronary circulation, it is important to maintain coronary artery perfusion throughout the period of anesthesia.