Anesthetic management for patients with Brugada syndrome

Brugada syndrome should not be neglected in terms of anesthetic management because its perioperative autonomic imbalance may cause ventricular fibrillation and sudden cardiac arrest. Diagnosis of Brugada syndrome is easily made by unique electrocardiographic pattern of right bundle branch block and ST segment elevation in the right precordial leads. Thus the number of patients with Brugada syndrome for anesthetic management tends to increase. We review current concept of anesthetic management for patients with Brugada syndrome including fourteen cases in our institution, two out of which developed VF during operation.     

Anesthetic management of a patient with Hallermann-Streiff syndrome

Key words  airway management - difficult intubation - Hallermann-Streiff syndrome     

Anesthetic management of a patient with West syndrome

A 21-year-old female with West syndrome was scheduled for resection of hordeolum. She had an episode of convulsion at three months of age, and was diagnosed as having West syndrome at one year of age. She had epileptic seizures twice a week in spite of administration of phenytoin, clonazepam and sodium valproate. These drugs had been administered till the morning of the surgery. After premedication with atropine 0.25 mg, anesthesia was induced with propofol (12-->10-->8 mg.kg-1.h-1). The tracheal intubation was performed with vecuronium 0.1 mg.kg-1 and anesthesia was maintained with continuous infusion of propofol 6-8 mg.kg-1.h-1 and local infiltration with 1.0% lidocaine 5 ml. We administered phenytoin to prevent epileptic seizures during the surgery. No epileptic seizures occurred perioperatively. We conclude that propofol may be useful for a patient with West syndrome, and we should be careful not to lower the threshold for convulsion during the perioperative period.     

Anesthetic management of a patient with Freeman-Sheldon syndrome

We describe a case of Freeman-Sheldon syndrome that presented some problems for anesthetic management. A 2-yr-old girl required orthopedic surgery for the bilateral lower extremities. Anesthesia was induced via a mask with oxygen (2 l.min-1), nitrous oxide (4 l.min-1) and sevoflurane (approximately 5%). Tracheal intubation by direct laryngoscopy was successfully achieved. Combined caudal epidural block was, however, avoided because spina bifida occulta was suspected. Spina bifida occulta was revealed postoperatively by X-ray. For anesthetic management of a patient with Freeman-Sheldon syndrome, the spine should be evaluated preoperatively when performing epidural/spinal anesthesia.     

Anesthetic management of a patient with Mulvihill-Smith syndrome

Mulvihill-Smith syndrome is a rare disease that belongs to progeroid syndromes. This syndrome is characterized by a senile face with an underdeveloped lower half, short stature, microcephaly, multiple pigmented nevi, immunodeficiency, hearing loss, and high-pitched voice. We report anesthetic management of a 27-year-old woman, 138 cm and 27 kg, with this syndrome, who underwent removal of mandibular cyst, partial resection of tongue and keratoplasty. Anesthesia was induced with fentanyl, propofol and vecuronium. There was difficulty in maintaining adequate ventilation with a face mask for children, and we used a mask for infants. Her Cormack grade was rated 3 but her trachea could be intubated assisted by BURP procedure. Anesthesia was maintained with sevoflurane, nitrous oxide and oxygen supplemented with fentanyl. The changes of blood pressure during anesthesia were extraordinary, suggesting the presence of advanced arteriosclerosis. The postoperative course was uneventful, with stable hemodynamics, and the patient was discharged from the hospital on 9th postoperative day. Anesthesia for Mulvihill-Smith syndrome should be performed with caution for the potential risk of difficult airway and unstable hemodynamics.     

Anesthetic management of a patient with Swyer-James syndrome

Swyer-James syndrome (SJS) shows the constellation of radiographic findings of a small, hyper lucent lung, with an ipsilateral, diminished peripheral vasculature, air trapping, and a lack of peripheral fill on bronchography. We report a case of 70-year-old woman with SJS who underwent pulmonary resection of the normal side lung for lung tumor. Because of this syndrome, we could predict the hypoxia during one-lung ventilation. In fact, about ten minutes after beginning of one-lung ventilation, Sp(O2) decreased from 100% to 90%. As we could not improve the hypoxia in spite of increasing FI(O2), O2 administration to the operating side lung was started. Sp(O2) recovered after O2 administration. For anesthetic management of a patient with SJS in the normal-side-lung, it is essential to prevent the hypoxia during one-lung ventilation.     

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.     

Anesthetic management of a patient with Young-Simpson syndrome

Young-Simpson syndrome (YSS) is a rare malformation syndrome characterized peculiar facies, congenital hypothyroidism, congenital heart disease, and postnatal growth deficiency. A 3-year-old boy with YSS underwent tracheostomy for respiratory failure under general anesthesia using sevoflurane, nitrous oxide and oxygen. Although he was assumed to be difficult for intubation due to micrognathia and macroglossia, tracheal intubation was done without difficulty. No complications were observed during the 40 minutes of the operation.     

Anesthetic management of a patient with Alport-leiomyomatosis syndrome

We report the anesthetic management of esophagectomy for a patient with Alport-leiomyomatosis syndrome. A 23-year-old woman complained of dysphagia and severe chest pain. Her chest X-ray, computed tomography (CT), and magnetic resonance imaging (MRI) showed an enlarged esophagus, in contact with the trachea, heart, aorta, and large vessels. She frequently experienced severe asthma attacks. Because various risks in both respiration and circulation, especially in anesthesia induction, were of concern, her right femoral vessels were exposed, for the emergency use of percutaneous cardiopulmonary support (PCPS), prior to anesthesia induction. Anesthesia was induced and maintained with propofol, fentanyl, and vecuronium. Esophagectomy was performed uneventfully and no severe events were seen in anesthesia management. Alportleiomyomatosis syndrome is a very rare disease. When we are involved in the anesthetic management of a patient with this disease, evaluation of the influence of the enlarged esophagus on both respiration and circulation, and careful preparation for emergence, are very important.