A review of allergic granulomatous angitis (AGA) (Churg-Strauss syndrome)

We reported clinical features of 11 patients with Churg-Strauss syndrome (CSS). Subjects included 2 males and 9 females with a mean age of 56.3 year-old. The age of the onset of bronchial asthma was high, and most of the patients had been suffered from frequent and severe asthmatic attacks during the period preceding the vasculitis. At the onset of the disease, patients showed a variety of vasculitic symptoms, including fever and neuropathy. Peripheral blood eosinophilia was universally seen. Three patients died of heart failure or central nerve involvement. Vasculitis ameliorated in 8 cases following steroid treatment. Discontinuing maintenance therapy with low-dose steroid resulted in a relapse in only one case. The significance and indication for long-term maintenance therapy should be further clarified. Based on these findings and previous publications, the clinical characteristics of CSS in Japan are discussed.     

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 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 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.     

Familial granulomatous arthritis (Blau syndrome) with granulomatous renal lesions

PURPOSE: To identify the BIGH3 gene mutation in 10 unrelated Japanese individuals with granular corneal dystrophy. METHODS: Genomic DNA was obtained from each patient's leukocytes. Exons 4 and 12 of the BIGH3 gene were amplified by polymerase chain reaction and were directly sequenced. RESULTS: Nine of these patients were found to have the R124H mutation, whereas only one had the R555W mutation. Slit-lamp examination showed that the granular corneal dystrophy associated with each mutation is different. CONCLUSIONS: These results, together with our previous findings, show that the classic form of granular corneal dystrophy associated with the R555W mutation is rare in Japanese patients, whereas granular corneal dystrophy accompanied by amyloid deposits and associated with the R124H mutation, Avellino corneal dystrophy, is more common. Direct examination may be insufficient in the proper diagnosis of corneal dystrophy, and BIGH3 mutation analysis may be required.     

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 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 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 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 of Seckel syndrome: a case report

Seckel syndrome is a rare syndrome of chromosome aberration, in which bird-headed dwarfism, microcephalus and other minor deformities are recognized. A 24-year-old male patient with Seckel syndrome underwent both abdominal and orthopaedic surgeries in 1 year under general anesthesia. The first operation was an emergent operation under preshock state and enterostomy was performed. The second was arthrodesis of the hip joint. Before the second operation, laryngeal CT, tomography and fiberscopy revealed stenosis just below his vocal cord. During the second operation, the anesthesia was unsatisfactory with inhalation of nitrous oxide and sevoflurane and intravenous vecuronium, because of intraoperative abnormal hypertension. But the recovery from the anesthesia was prompt. Although we experienced no difficulty in intubation except for intraoperative abnormal hypertension, preoperative laryngeal and renal examinations are necessary in the anesthetic management of this syndrome.     

Pneumonia caused by granulomatous Pneumocystis carinii in a patient with the acquired immunodeficiency syndrome

A 54-year-old man was admitted to the hospital because of fever and general fatigue. A chest roentgenogram on admission showed lobular opacities and ill-defined opacities in both lower lobes. The pneumonia was successfully treated with antibiotics. The acquired immunodeficiency syndrome was diagnosed because ELISA and PCR tests for antibodies to the human immunodeficiency virus were positive and the CD 4+ lymphocyte count was 39 per cubic millimeter. Examination of bronchoalveolar lavage fluid revealed no Pneumocystis carinii. Trimethoprim and sulfamethoxazole were given prophylactically, but were withdrawn because of a rash. The patient began to receive aerosolized pentamindine and was discharged. On the next day, he was readmitted to the hospital because of a high fever. A chest roentgenogram showed diffuse miliary opacities. Chest CT scan also showed diffuse small nodular opacities in both lungs. Examination of a transbronchial biopsy specimen revealed well-defined, noncaseating granulomas with pneumocystis organisms in their centers. Cultures for tuberculosis and fungi were all negative. We diagnosed granulomatous pneumonia caused by Pneumocystis carinii, which is an atypical manifestation of Pneumocystis carinii pneumonia. The patient died of sepsis and cardiac tamponade. Microscopically, the lung tissue was found to have foamy intra-alveolar exdates, which is a typical histological feature of Pneumocystis carinii pneumonia.     

Peripartum management of a patient with Isaacs' syndrome

PURPOSE: To describe the peripartum management of a patient with Isaacs' syndrome with specific reference to the anaesthetic implications of the disease process. Associated medical problems included obesity, pregnancy induced hypertension and a difficult airway. CLINICAL FEATURES: This 30-yr-old gravida V para 0 woman presented to the anaesthesia consultation clinic at 37-wk gestation to discuss pain relief options for labour and delivery. She had a history of Isaacs' syndrome (a peripheral motor neuron disorder), congenital heart disease (ASD and VSD), treated Hashimotos thyroiditis, obesity and a family history of haemachromatosis. On the day of consultation, she was hypertensive and peripheral oedema was noted. Her urine showed trace protein. Four days later, she presented to the labour suite and her cervix was 9 cm dilated. An epidural anaesthetic was given without difficulty and she had an uneventful labour and delivery course. There were no subsequent neurological complications. CONCLUSION: Isaacs' syndrome is an extremely rare peripheral motor neuron disorder. This patient was successfully managed with epidural analgesia for labour and delivered a healthy child with no congenital anomalies.     

Anesthetic management for a patient with pheochromocytoma using magnesium sulfate and epidural block

A 54-year-old woman with pheochromocytoma was anesthetized using magnesium sulfate infusion. Anesthesia was induced with thiopental and fentanyl, and maintained with 0.5% sevoflurane in nitrous oxide/oxygen and 60 mg.hr-1 of epidurally infused lidocaine. Muscle relaxation was achieved with intermittent administration of vecuronium under neuromuscular monitoring. Magnesium 2 g was infused prior to the tracheal intubation and followed by continuous infusion of 2 g.hr-1. These anesthetic techniques made hemodynamic state stable, and no additional vasoactive agents were needed. However, the infusion rate of magnesium was reduced to a half because of PQ interval prolongation. Magnesium is believed to be useful for anesthetic management for patients with pheochromocytoma, but the proper dose and the way of administration should be further investigated for their safe clinical use.     

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.     

Tongue involvement in a patient with granulomatous myositis

A 64-year-old woman had difficulty swallowing and talking, weakness of the tongue, and progressive muscle weakness that was proven to be caused by a granulomatous myopathy. This case resembled a motor neuron disease, indicating that granulomatous myopathy should be considered in patients with similar presentations, since it is a treatable condition with a more benign prognosis.