Anesthetic management of a patient with Cowden syndrome

Cowden syndrome is a rare syndrome of chromosome abnormalities presenting with polyposis of digestive tracts, characteristic skin eruption and neuromuscular disorders. A 56-year-old male patient with Cowden syndrome underwent upper abdominal surgery under general anesthesia followed by post-operative epidural analgesia with buprenorphine. Proposed total gastrectomy was not performed because of massive invasion of carcinoma in the abdominal cavity and gastrojejunostomy was done instead. The anesthesia was satisfactory with inhalation of nitrous oxide and enflurane with intravenous vecuronium. Neuromuscular monitoring with electric twitch-responses of the hand showed normal patterns throughout the anesthesia. The recovery from anesthesia and neuromuscular blockade was prompt. Intermittent epidural buprenorphine, twice a day (0.2 mg of buprenorphine in 9 ml of normal saline for one time) was started just after the recovery of anesthesia and continued for four days. Delirium occurred two days after beginning epidural buprenorphine and disappeared three days after its discontinuation. The patient died 52 days after the operation from obstructive jaundice and sepsis. The delirium, therefore, seems to have been caused by buprenorphine possibly due to its impaired metabolism by the liver. Although we did not experience any abnormal neuromuscular reactions to vecuronium or anesthetic agents, it is important to perform preoperative neuromuscular examinations and peri-operative monitoring in the anesthetic management of a patient with this syndrome.     

Anesthetic management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome (BSS) is a hereditary hemorrhagic disease characterized by prolonged bleeding time due to abnormal platelet aggregation and giant platelets. Transfusion of platelet-rich plasma is the only treatment available for the hemorrhagic episodes in patients with this disease. A 20-year-old female with BBS was scheduled for sagittal osteotomy of the mandibular rami under general anesthesia. Anesthesia was induced with fentanyl and diazepam, and was maintained with nitrous oxide, fentanyl, and 0.5% enflurane. No exacerbation of the bleeding tendency was observed during or after the surgery. We consider that the use of halothane should be avoided in patients with BBS because it may inhibit the aggregation of platelets and prolong the bleeding time.     

Anesthetic management of a patient with hypertrophic cardiomyopathy using propofol, fentanyl and ketamine

A 59-year-old male with hypertrophic cardiomyopathy was scheduled for resection of a maxillary cyst. Metoprolol was discontinued the day before surgery. Thirty min before anesthesia, meperidine 35 mg was administered intramuscularly. After intravenous administration of midazolam 3 mg, a pulmonary catheter was inserted for monitoring hemodynamic parameters. Anesthesia was induced with propofol 75 mg, fentanyl 0.15 mg and ketamine 75 mg. Anesthesia was maintained with continuous infusion of propofol 5 mg.kg-1.h-1 and ketamine 1 mg.kg-1.h-1. Moreover, fentanyl was added as necessary during surgery. Blood pressure (BP), pulmonary arterial pressure (PA), systemic vascular resistance index (SVRI) and pulmonary vascular resistance index (PVRI) were measured using a pulmonary catheter during anesthesia. Since BP decreased after intubation, dopamine 3 micrograms.kg-1.min-1 was administered for 20 min. The hemodynamic state was stable during surgery. However, BP, PA, SVRI and PVRI increased temporally at extubation. His postoperative course was uneventful. In conclusion, total intravenous anesthesia with propofol, fentanyl and ketamine may be useful for anesthetic management of a patient with hypertrophic cardiomyopathy.     

Anesthetic management of a multiallergic patient scheduled for cholecystectomy

A 64-year-old woman was scheduled for cholecystectomy. Her past history revealed that serious anaphylactic reactions including generalized flushing and urticaria, severe hypotension and unconsciousness which occurred after eating crab four years ago. Puncture and/or intradermal skin test and subsequent lymphocyte stimulation test to several drugs commonly used in perioperative period were performed prior to anesthesia. Positive reactions to intravenous anesthetics and muscle relaxants, and negative reactions to inhalational and local anesthetics were found. Famotidine and ketotifen fumarate were given to prevent histamine release for four days before operation. After premedication with scopolamine, a catheter was inserted into epidural space at Th9-T10 level and 2% lidocaine 2 ml was administered initially into the epidural space. Anesthesia was induced with inhalation of nitrous oxide and oxygen, and deepened gradually by the increments of sevoflurane. Tracheal intubation was performed smoothly without adjunct muscle relaxant. Anesthesia was maintained with sevoflurane and epidural anesthesia with intermittent lidocaine administration. No adverse responses were noted at the time of iopamidol injection for intraoperative cholangiography. The anesthesia and postoperative course of this patient were uneventful.     

Anesthetic managements of a patient with multiple sclerosis using propofol

We experienced anesthetic management of a 45-yr-old female patient with a 12-yr history of multiple sclerosis who underwent orthopedic surgeries three times under general anesthesia. We chose rapid induction with propofol and maintained the anesthesia with nitrous oxide, oxygen, and sevoflurane. We monitored both core and peripheral temperatures to avoid the rapid increase of core temperature, which might worsen the symptoms of the disease. There is no other report of anesthesia using propofol as induction agent for a patient with multiple sclerosis. We succeeded in the satisfactory perioperative management of the patient.     

Anesthetic management of a Paget's disease patient complicated with Parkinson disease

Paget's disease is a metabolic disorder of unknown etiology characterized by excessively rapid remolding of bone. We report a case of Paget's disease complicated with Parkinson syndrome. A 69-year-old female patient was scheduled for ventriculo-peritoneostomy due to hydrocephalus. Her manifestations included disability to walk, slight deafness and muscular rigidity of limbs, without symptom of intracranial hypertension. After induction of anesthesia with thiopental and vecuronium, tracheal intubation with Macintosh laryngoscope was attempted but failed because mouth opening was restricted to only 3 cm. Again with Bullard laryngoscope, she was successfully intubated. During neurosurgical operation, the anesthesia was maintained with low concentration of isoflurane (under 0.3%), nitrous oxide and oxygen. The surgery was completed without adverse events such as disorder of autonomic nervous system. However 6-days after operation, ventilatory arrest occurred due to bronchial obstruction with sputum. Immediately, re-intubation was performed without any sequela and tracheostomy was also performed. In conclusion, as reported previously, intubation and postoperative pulmonary dysfunction should be carefully taken care of, and Bullard laryngoscope was useful for this patient.     

Anesthetic management of the trauma patient

The key to safe anesthesia of a trauma patient is preoperative stabilization. It minimizes the anesthesia risk and allows proper choices of anesthetic drugs. Adequate monitoring throughout anesthesia allows rapid and adequate therapeutic intervention. Proper control of upper airway and support of physiologic functions will enhance the chances of the patient to survive anesthesia.     

Anesthetic management of a patient with protein C deficiency associated with pulmonary thromboembolism

A patient with protein C deficiency associated with massive pulmonary embolism underwent open heart tromboembolectomy. The operation was successfully performed under cardiopulmonary bypass using a usual dose of heparin 3 mg.kg-1. The effect of heparin was successfully reversed by the administration of protamine sulfate 6 mg.kg-1. Perioperative administration of fresh frozen plasma or protein C concentrates might be necessary to manage hypercoagulability in a patient with protein C deficiency.     

Anesthetic management of a patient with Duchenne's muscular dystrophy undergoing radical repair for tetralogy of Fallot

This report describes a 13-month-old-girl with Duchenne's muscular dystrophy (DMD) who had radical repair for tetralogy of Fallot safely. Patients with DMD are considered to be at risk of malignant hyperthermia (MH). Drugs for induction and maintenance were chosen from a list of agents rarely associated with MH. To wash out the inhalation anesthetics from the equipment, oxygen was circulated continuously for 24 hours. Dantrolene sodium was kept readily available in case of MH occurrence. Differential diagnosis during surgery is difficult in term of the episodes of MH and complications of cardiac surgery, as cardiac surgery is also associated with tachycardia, tachyarrhythmias, metabolic asidosis and red colored urine, which are frequently accompanied by MH. Although increased levels of CK, GOT, LDH and myoglobin strongly support the diagnosis of MH, such evidence can only be confirmed after operation. Fortunately, these factors recovered to the normal range without treatment by dantrolene sodium. During the cardiac surgery, treatment of MH may be delayed due to its late confirmation.     

Anesthetic management of a patient with the allergic granulomatous angitis (Churg-Strauss syndrome)

We gave anesthesia 4 times to a patient (19-year-old female) with allergic granulomatous angitis (AGA). She had asthma, myopathy and detrimental side effect in her eyes of steroid therapy for AGA. Two of the 4 operations were emergency laparotomy for peritonitis due to colon perforation, and the other 2 operations were elective eye surgeries. General anesthesia was induced with thiamylal (1st operation) or midazolam (2nd-4th operation). Intraoperative anesthesia was maintained with N2O-O2-isoflurane combined with thoracic epidural anesthesia (1st operation) or N2O-O2-sevoflurane (2nd-4th operation). Asthmatic attack, which AGA accompanies frequently, did not occur during these anesthetic managements. The steroid therapy, which is a fundamental means to control the allergic syndrome, might suppress asthma. However, asthmatic attack and systemic vasculitis should be kept in mind in anesthetic management of AGA.     

Anesthetic management of a patient with renal cell carcinoma extending into the right atrium

A 60-year-old man with renal cell carcinoma extending through inferior vena cava into the right atrium was scheduled for the removal of the right kidney under general anesthesia and the cardiopulmonary bypass technique. In order to obtain a clear operative field and to minimize the risk for pulmonary embolism of necrotizing tumor, total circulatory arrest under profound hypothermia (20 degrees C) was performed. Anesthesia was maintained with high doses of fentanyl (62 micrograms.kg-1), midazolam and supplemented with enflurane. We attempted to prevent circulatory collapse due to acute pulmonary embolism by tumor fragments during operation. The body temperature of the patient was decreased down to 20 degrees C for protecting central nervous system with the minimal damage. No complications occurred during anesthesia and the post-operative period. For the safe anesthetic management of the patient such as our case, adequate monitoring of circulation and protection of central nervous system are essential.     

Anesthetic management for bronchoscopy in patients with congenital tracheomalacia

We experienced the anesthetic management for two patients with congenital tracheomalacia. Inhaled anesthetics are considered to worsen the respiratory condition of tracheomalacia because of its bronchodilating effect. Thus we tried awake intubation in one case, but it was difficult. In another case, we used slow induction with sevoflurane and the trachea was intubated smoothly. Inhaled anesthetics have possibility of worsening the degree of tracheomalacia and have been used very carefully. However, we considered that slow induction with inhaled anesthetics in children with congenital tracheomalacia is a safe and necessary technique, under careful observation of respiratory conditions.     

Acquired neuromyotonia in a patient with spinal epidural abscess

We report a case of acquired neuromyotonia in a patient with Staphylococcus aureus septicemia and a spinal epidural abscess. Autoantibodies to voltage-gated potassium channels, which are associated with acquired neuromyotonia, were present during the patient's acute illness but became undetectable on clinical recovery. The spinal epidural abscess may have triggered the production of these specific autoantibodies, resulting in clinically and electromyographically detectable neuromyotonia.     

Anesthetic management of a child with Lenz dysplasia associated with panhypopituitarism

We present a child with Lenz dysplasia associated with panhypopituitarism. Lenz dysplasia is characterized by small eyeball, small head, hydronephrosis, cleft lip and palate, and mental retardation. A 12 month-old boy with Lenz dysplasia was scheduled for plasty of the lip and basis of the nasal cavity under general anesthesia. We had to pay attention for airway management and hormone supplementation. Anesthesia was induced with sevoflurane and nitrous oxide in oxygen. Tracheal intubation was facilitated with vecuronium bromide. We had no difficulty in airway management. Since this patient could not release enough endogenous cortisol in response to the stress of surgery, we supplemented hydrocortisone after anesthesia induction. Urine output and serum electrolyte concentrations were carefully monitored during surgery because of the impaired ADH response. We encountered no complications in the anesthetic management of this patient.     

Long lasting epidural anesthesia for a morbidly obese patient

Blood pressure (BP) differences between two consecutive 24-h monitoring periods (P1, P2) were analyzed in 159 hospitalized pregnant women. Reproducibility index, or twice the standard deviation of differences between individual systolic and diastolic means, was better for 24-h (7.8 and 5.8) and daytime (8.7 and 6.3) than for nighttime (12.2 and 9.7) BP means. It did not depend on BP level or gestation week. Alert reaction to monitoring procedure increased BP only in the first 2 h of P1. The high overall reproducibility of ambulatory BP in pregnancy supports the expanding use of the technique also in this condition.