Anesthetic management of renal transplantation in a patient with familial dysautonomia

Familial dysautonomia is an inherited disorder characterized by autonomic and sensory nervous system neuropathy resulting in extremely labile blood pressure (severe hypertension followed by hypotension). As more patients with familial dysautonomia reach adulthood due to improved medical treatment, perioperative encounters of patients with familial dysautonomia will increase. This report is the first adult case to describe an anesthetic management of kidney transplantation for an adult familial dysautonomia patient. The clinical manifestations of this disease and rationale of our anesthetic management are discussed.     

Anesthetic management for a patient with renal arterio-venous fistula

Key words  anesthesia - renal arterio-venous fistula - vascular resistance     

Anesthetic management of a patient with narcolepsy

We report the anesthetic management of a narcoleptic patient performed using sevoflurane–remifentanil with bispectral index (BIS) monitoring. A 22-year-old man, who was diagnosed with narcolepsy at the age of 17, requested endoscopic sinus surgery, under general anesthesia, for chronic allergic rhinitis. On the morning of the day of operation, he took his daily dose of modafinil, used to control narcolepsy. Anesthesia was induced by 5% sevoflurane and maintained with sevoflurane and continuous infusion of remifentanil and 60% oxygen in conjunction with BIS monitoring. BIS values were between 47 and 58. Duration of surgery was 150 min. After surgery, the patient emerged from anesthesia within 10 min and was extubated. His recovery was uneventful. We found the use of BIS monitoring for titrating sevoflurane concentration in a narcoleptic patient is useful for preventing not only oversedation but also intraoperative awareness caused by the preoperative medication.     

Anesthetic management of a patient with tetra-amelia

A 52-year-old man with Burger disease was admitted for hemorrhoidectomy. He lost upper and lower limbs 19 years earlier. We could not monitor his blood pressure with noninvasive technique. Bilateral superficial temporal arteries were not palpable. In the operating room, the external jugular vein was cannulated and an electrocardilgram and oxygen saturation was monitored. Anesthesia was induced with spinal anesthesia of 0.5% hyperbaric bupivacaine. His carotid artery was palpable and heart rate, oxygen saturation and his consciousness were stable during operation. He had an uneventful intra- and post-operative course. Preoperative evaluation of surgery and monitoring may be needed in patients with tetra-amelia.     

Anesthetic management of a patient with oculopharyngodistal-myopathy

Oculopharyngodistal-myopathy (OPDM) is an autosomal dominant, heredofamilial myopathy accompanied with slowly progressive ptosis and extraocular palsy, and weakness of the masseter, facial, and bulbar muscles, as well as distal involvement of the limbs starting around 40 years of age or later. A 54-year-old female with OPDM underwent resection of the uterus for uterus body cancer. We speculated the patient might be at the risk of aspiration pneumonia, prolonged respiratory depression, and malignant hyperthermia, and chose spinal and epidural anesthesia. The operation was performed successfully and the patient was discharged uneventfully.     

Anesthetic management of renal transplantation for a 15-year-old girl with cerebral palsy

A 15-year-old girl with severe cerebral palsy underwent renal transplantation. It was difficult to anastomose blood vessels because her inferior limbs were contracted. The clamp time was 67 minites. After unclamping, blood pressure fell down from 120/60 to 80/50 mmHg, and CVP fell down from 6 to 3 mmHg. First flow of urine from the donor kidney was noticed 9 hours after unclamping. We regarded difficulty of the operation for cerebral palsy and insufficient infusion as the cause of the late first flow of urine. Two weeks later, there was enough urine flow, and renal function became better. Recently, it is thougt optimal to perform renal transplantations of children who have chronic renal insufficiency and end-stage renal disease. However, there are few reports of renal transplantations for children with cerebral palsy, and there is no guideline for them. Therefore, we anesthesiologists, also have to further examine anesthetic management for each case.     

Anesthetic management of the patient with a pacemaker

Anesthetic care depends on the patient underlying condition. Local anesthesia is preferable. Do not use heavy sedation. If you use GA avoid suxamethonium. If the PM was recently implanted, avoid N2O. During operation change to fixed-rate. Precautions for electrocautery. Post-operatively inform cardiologist to readjust the PM.     

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 congenital methemoglobinemia

A knowledge of congenital methemoglobinemia is essential to deliver a safe anesthetic to this group of patients. We report the case of a 33-year-old patient with congenital methemoglobinemia undergoing a gynecological procedure, and discuss the anesthetic implications. The etiology, pathophysiology, classification, diagnosis, clinical manifestations, anesthetic considerations, treatment options, and postoperative management are also discussed.     

Anesthetic management of a patient with severe hyponatremia

A 62-year-old woman was scheduled for an operation for ileus. Before the operation, we noticed severe hyponatremia (Na 117 mEq x l(-1)) probably due to dehydration. We corrected her hyponatremia slowly to avoid central pontine myelinolysis. Serum Na level increased to 131 mEq x l(-1) after surgery. She recovered from anesthesia without any neurologic problems.