Anesthetic management for a patient with hyper-IgE syndrome

Hyper-IgE syndrome is a rare immunodeficiency disorder characterized by recurrent skin and pulmonary infections and extremely elevated serum levels of IgE. A 6-year-old girl with hyper-IgE syndrome underwent appendectomy. Anesthesia was induced with sevoflurane. After insertion of a laryngeal mask, anesthesia was maintained with nitrous oxide, sevoflurane, and oxygen. There were no perioperative complications related to anesthesia and surgery.     

Anesthetic management of a patient with Hallermann-Streiff syndrome

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

Anesthetic management of a patient with Brugada syndrome

Brugada syndrome is characterized by right bundle-branch block, ST elevation in leads V 1 through V 3 and normal QT interval. Ventricular fibrillation frequently occurs in patients with Brugada syndrome. There have been few reports of anesthetic management of Brugada patients. We managed a 47-year-old man with Brugada syndrome, who underwent hemilaminectomy under general anesthesia, without untoward cardiovascular events. Potential problems in anesthetic management of patients with Brugada syndrome are also discussed.     

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 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.     

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 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 Weill-Marchesani syndrome

Weill-Marchesani syndrome is characterized by short stature, brachydactylyl, myopia, microspherophakia, lens dislocation, glaucoma, joint stiffness, restricted articular movements and facial features. The anesthetic management of an 11-year-old-male patient with diagnosis of this syndrome is reported.     

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.