Anesthetic management of a pregnant patient with congenitally corrected transposition of the great arteries for emergency cesarean delivery

A 30-year-old woman at 35 weeks' gestation with congenitally corrected transposition of the great arteries (CCTGA), whose associated anomalies were VSD, ASD and pulmonary stenosis, underwent emergency cesarean delivery under general anesthesia. She had hypoxemia due to right-to-left shunt. We discussed with obstetricians and pediatric cardiologists about her at 25 weeks' gestation. Discussed points included: 1) delivery after 30 weeks' gestation, 2) her cardiac function and anomalies, 3) cesarean delivery under general anesthesia in case of emergency, 4) management of a sleeping baby after cesarean delivery by pediatricians. Cyanosis and heart failure were noted worsening during pregnancy. General anesthesia was induced by midazolam and fentanyl following cannulation of radial artery and right internal jugular vein for blood pressure and CVP monitoring. Anesthesia was maintained with sevoflurane and fentnyl. Dopamine and dobutamine were used to support blood pressure and cardiac function. After delivery, because of decreased CVP, lactated Ringer's solution was administrated. After operation, the patient was hemodynamically stable and trachea was extubated. Anesthetic management of the patient depends on the anatomical defects of CCTGA and associated conditions. Anesthesiologist should obtain information about a pregnant woman with CCTGA from cardiologists and obstetricians in early gestation to manage patient successfully.     

Cardiac arrest during emergency cesarean section due to peripartum cardiomyopathy--a case report

A 23-year-old woman at 35 weeks of gestation was scheduled for emergency cesarean section because of preeclampsia. Anesthesia was induced with thiopental 250 mg, and her trachea was intubated following administration of vecuronium 7 mg. Anesthesia was maintained with oxygen and sevoflurane (0.5%). Immediately after the start of the surgical procedure, she developed severe bradycardia and hypotension followed by cardiac arrest. She was resuscitated, and her heart beat resumed 30 min after the start of the resuscitation. Her babies were delivered without sequela during resuscitation. She was transferred to the intensive care unit of our university hospital. She was diagnosed as having peripartum cardiomyopathy from her history and echocardiography. She was discharged from the hospital with only slight disturbance of consciousness two months after surgery.     

Emergency microlaryngosurgery for a huge post-intubation laryngeal granuloma

A 55-year-old woman who had undergone esophagectomy 3 months earlier developed a huge post-intubation laryngeal granuloma. She was scheduled for emergency microlaryngosurgery. Anesthesia was induced with 8% sevoflurane in oxygen under spontaneous respiration. After confirming no airway compromise, the spontaneous respiration was obtunded by forced manual ventilation without using a muscle relaxant. The trachea was intubated easily with an endotracheal tube. Anesthesia was maintained with sevoflurane and nitrous oxide supplemented with intravenous fentanyl. The granuloma removed was 11 x 8 mm in diameter. There were no respiratory complications during and after anesthesia and surgery.     

Anesthetic management of a patient with May-Hegglin anomaly

The May-Hegglin anomaly is a rare hereditary disorder characterized by the presence of giant platelets and platelet deficiency. This report presents a successful management of a patient with May-Hegglin anomaly. A 5-year-old girl with May-Hegglin anomaly was scheduled for closure of fistulae of her ears. Although preoperative physical examination revealed platelet count of 8,000 mm-3, she had no bleeding tendency. No premedication was given. Anesthesia was induced with inhalational agents of a mixture of sevoflurane, nitrous oxide and oxygen. Neuromuscular blockade was achieved with vecuronium 0.1 mg.kg-1 and the trachea was gently intubated. Anesthesia was maintained with sevoflurane, nitrous oxide and oxygen. Five units of platelet were transfused during the operation. No bleeding tendency was observed perioperatively. It is important to plan the management of anesthesia for a patient with bleeding tendency.     

Insertion of a laryngeal mask airway before removal of a nasotracheal tube in a patient after anterior spine surgery

A 19-year-old man with cervical spondylosis (C4-C6) was scheduled for an anterior spine surgery. Anesthesia was induced with propofol and fentanyl, and nasotracheal intubation was performed without difficulty after vecuronium administration. Anesthesia was maintained with sevoflurane and nitrous oxide in oxygen supplemented with fentanyl. No complications were observed during the operation. After surgery, the patient's head and neck were stabilized by a halo-vest, and we attempted to minimize stress responses associated with tracheal extubation. While the patient was still deeply anesthetized, and the nasotracheal tube was in place, a laryngeal mask airway (LMA) was placed without difficulty. After confirming correct position of the LMA, the nasotracheal tube was removed without body movement or coughing. Removal of the LMA was safely performed after recovery of the patient's respiration and consciousness. We believe that the laryngeal mask airway is useful during emergence from anesthesia in the patient whose trachea is intubated nasally.     

Anesthetic management of a cesarean section in a patient with schizophrenia receiving a large amount of antipsychotic agents

We experienced perioperative management of a patient with schizophrenia who had been taking a large amount of antipsychotic agents until the cesarean section. A 31-year-old woman in 38th week gestation with schizophrenia underwent a cesarean section. Anesthesia was maintained with light plane of general anesthesia combined with epidural anesthesia. Perioperatively, the mother experienced no complications and the baby only developed slight generalized tremor and hypotonus for a short period after delivery.     

Total intravenous anesthesia for two patients complicated with myotonic dystrophy

Total intravenous anesthesia with propofol, fentanyl and ketamine (PFK) was given to two patients complicated with myotonic dystrophy. Case-1: A 42-year-old female underwent a hemithyroidectomy. Anesthesia was induced slowly with intravenous ketamine 20 mg and propofol 60 mg. Her tracheal intubation was performed smoothly without any muscle relaxants. Anesthesia was maintained with propofol infusion of 5 mg.kg-1.h-1, ketamine infusion of 0.3 mg.kg-1.h-1 and fentanyl 200 micrograms in total. She regained consciousness 20 minutes after the end of propofol infusion, and 15 minutes later, her trachea was extubated without any troubles. Case-2: A 41-year-old female underwent a removal of left parotid tumor. Anesthesia was induced slowly with ketamine 40 mg and propofol 100 mg intravenously. Anesthesia was maintained with propofol infusion of 5-10 mg.kg-1.h-1, ketamine infusion of 0.5 mg.kg-1.h-1 and fentanyl 350 micrograms in total. No muscle relaxant was used through the surgical procedure. Emergence from anesthesia was observed 10 minutes after the end of propofol infusion and her trachea was extubated. When a nasogastric tube was pulled out, her respiration stopped suddenly and she was intubated again only for two hours without any troubles. In both cases their serum CPK levels and rectal temperatures were very stable. PFK method would be a choice for patients with myotonic dystrophy.     

Anesthesia for a patient with Cockayne's syndrome

Cockayne's syndrome is a rare, inherited, autosomal recessive disorder, characterised by dwarfism and progressive physical and mental retardation. A case is described of a 6-year-old girl (height 75 cm, weight 5.0 kg) with Cockayne's syndrome who was scheduled for liver biopsy under general anesthesia. Anesthesia was induced using 5% sevoflurane combined with 67% nitrous oxide in oxygen. After the end-tidal sevoflurane concentration of 3.5% had been maintained for 10 minutes, laryngoscopy was attempted. The vocal cord was visualised and the trachea was intubated easily without muscle relaxant. Anesthesia was maintained with sevoflurane and nitrous oxide in oxygen. The procedure lasted for 13 minutes. The tracheal tube was removed uneventfully following the return of adequate spontaneous respiration and airway reflexes. There were no postoperative problems. Concerning Cockayne's syndrome, there have been some reports of difficult airway, laryngospasm, aspirations of gastric contents and others. It is important to prevent laryngospasm by keeping the adequate depth of anesthesia for endotracheal intubation.     

Three times of anesthetic management in a patient with myotonic dystrophy

A 38-year-old female, at 38-week gestation, was scheduled for cesarean section under epidural anesthesia. After the delivery, it was found that she had been diagnosed as myotonic dystrophy by the other physician and the neonate was a floppy infant indicating hereditary neuromuscular diseases. In her case, myotonic dystrophy had not been advanced and symptoms had been mild. We previously had given her general anesthesia for two times with nitrous oxide, isoflurane and vecuronium for her to undergo emergency operations, left salpingectomy under laparoscopy due to unruptured tubal pregnancy at 34 years of age and cesarean section due to liver function disorder indicating HELLP syndrome at 36 years of age. Although many problems have been described about the perioperative management in patients with myotonic dystrophy, she was safely managed for each operation.     

Case of emergent caesarean delivery in a patient with aplastic anemia complicated with pregnant induced hypertension

A 32-year-old pregnant woman diagnosed with aplastic anemia was admitted for emergent caesarean delivery of 26th week of the gestation due to PIH (pregnancy-induced hypertension) and NRFS (non-reassuring fetal status). After compensating platelets counts to 5.3x10(4) microl-1, general anesthesia was induced with propofol and rocuronium. Anesthesia was maintained with O2 and sevoflurane until delivery and with modified-NLA after delivery. She was additionally monitored with Vigileo/FloTrac system (Edwards Lifesciences, USA) and TOF-WATCH SX (Nihon Kohden, Tokyo). After 8 minutes of operation her baby was born with the 5-minute Apgar score of 5 and the UA-pH of 7.387. It was only 2 hours and 12 minutes that the baby was born after she was admitted. The baby was tracheally intubated and transferred to NICU. Blood loss during operation was 835 g and two units of RCC was transfused. Circulatory values were kept acceptable and neuromuscular blocking was completely reversed by sugammadex and extubated in the operating room. Bleeding tendency and atonic bleeding were not observed. She survived perioperative period and was to be treated for aplastic anemia. Her baby was discharged neurologically free. We should be ready to respond to anesthetic requirement for urgent cases of aplastic anemia.     

Anesthetic management in a patient with Goldenhar's syndrome using a perilaryngeal airway and a videolaryngoscope

A 5-year-old girl with Goldenhar's syndrome was scheduled for reconstruction of her left thumb. Because her preanesthetic X-p had revealed airway abnormality, difficult intubation was anticipated. After induction of anesthesia with thiamylal, the lungs were ventilated easily via a facemask. A perilaryngeal airway (PLA) was inserted after obtaining adequate depth of anesthesia with sevoflurane. Anesthesia was maintained with sevoflurane (2%) plus nitrous oxide (66%) under spontaneous ventilation. The operation was finished uneventfully. However, a re-operation was scheduled for postoperative hemorrhage on that day. In consideration of potential regurgitation of gastric content, endotracheal intubation was scheduled. Her vocal cord was confirmed with a videolaryngoscope, and she was intubated successfully. In conclusion, a PLA and a videolaryngoscope are useful for airway management of patients with Goldenhar's syndrome for whom difficulty in intubation is anticipated.     

Two cases of congenital airway obstruction managed with ex utero intrapartum treatment procedures: anesthetic implications

An ex utero intrapartum treatment (EXIT) procedure provides sufficient time to gain control of the potentially obstructed fetal upper airway while uterine placental circulation is maintained during cesarean section. We report 2 cases in which fetal congenital upper airway obstruction was managed without complications during EXIT procedures. We also discuss general considerations concerning the obstetric patient and the performance of intramuscular fetal anesthesia. Before the hysterotomy, sevoflurane at 1.5 minimum alveolar concentration was administered to assure sufficient uterine relaxation during EXIT. The 2 parturients remained hemodynamically stable during the procedure and uterine and placental perfusion was adequate. Nasotracheal intubation was possible in 1 fetus after a cervical mass was dissected. In the other, a tracheostomy was created. After the umbilical cord was clamped, the concentration of sevoflurane anesthetic gas was reduced and oxytocin and methylergometrine were administered to induce adequate uterine contractions within a few minutes. Both neonates survived the EXIT procedure with no complications.     

The use of potent inhalational agents for the ex- utero intrapartum treatment (exit) procedures: what concentrations?

The anesthetic management of a parturient undergoing ex-utero intrapartum treatment (EXIT) procedures for airway control of a newborn with a potentially life-threatening difficult airway is complex and often challenging. We herein report on the successful anesthetic management of the EXIT procedure in a 30-year-old primigravida carrying a fetus with large cervical lymphangioma. General anesthesia was maintained with sevoflurane 2%, combined with continuous infusion of nitroglycerine (TNG). Although the use of high concentrations of potent inhalational agents (to keep the uterus fully relaxed) is currently recommended we believe that the use of low concentrations of potent inhalational anesthetics with continuous infusion of TNG may be a safer anesthetic strategy for these operations.     

The impact of 3-dimensional ultrasonography on perinatal management of a large epignathus teratoma without ex utero intrapartum treatment

We report a case of epignathus teratoma diagnosed at 22 weeks of gestation in which 3-dimensional ultrasound (3DUS) was useful to plan perinatal management. A significant enlargement of the tumor, associated with polyhydramnios and preterm labor, was observed at 35 weeks of gestation. After amniotic fluid evacuation, 3DUS was performed in the presence of pediatricians, obstetricians, and otolaryngologists. Three-dimensional ultrasound revealed that great part of the tumor was located outside the fetal mouth and anterior to fetal mandible, suggesting that the newborn could breathe spontaneously by nasal via. A cesarean section with longitudinal hysterotomy was performed at 36 weeks followed by an immediate extirpation of the tumor and the intubation of the newborn. The management of this rare case illustrates that the ex utero intrapartum treatment (EXIT) procedure is not always necessary in this situation. Besides, the actual prenatal goal consists on carefully selecting fetuses with epignathus teratoma that will need the EXIT procedure from those that will not. Three-dimensional ultrasound and magnetic resonance imaging in association with 2DUS can be helpful in this prenatal selection.     

Perioperative management for cesarean section in a patient with pulmonary thromboembolism due to deep venous thrombosis

It has been estimated in Japan that Western-life style increases maternal mortality because of pulmonary thromboembolism (PTE). We report a 29-year-old primipara who suffered PTE due to deep venous thrombosis (DVT) in her 29th weeks' gestation. Except for slight tachypnea, she was relatively stable. Anticoagulation with heparin was started immediately. The retrievable inferior vena cava filter (IVC-F) was inserted. Four hours before surgery with discontinuation of heparin, the cesarean section was performed under general anesthesia. We used transesophageal echocardiography, a pulmonary artery catheter and end tidal CO2 monitoring for early detection and rapid management of recurrent PTE. She had no trouble during operation and her baby was born without serious symptoms. After recovery from anesthesia, she was admitted to the intensive care unit. Heparin was restarted after confirmation of hemostasis. On the 3rd postoperative day, we started thrombolytic therapy with urokinase which was tapered off during a week. Heparin was switched to warfarine gradually. On the 10th postoperative day, IVC-F could not be removed because of remaining DVT. She was discharged on daily warfarine. We experienced the perioperative management for cesarean section at 29 weeks' gestation following PTE due to DVT.     

A case of cardiac arrest at induction of anesthesia for postpartum hysterectomy

Although peripartum cardiac arrest is rare, the prognosis of the event may be worse than in nonpregnant patients because the events is often associated with pulmonary or amniotic embolisms. The following report is a case of cardiac arrest which occurred at the induction of anesthesia for the postpartum hysterectomy. A 31-year-old woman was treated with infusion of ritodrine chloride for preterm labor and placenta previa. Elective cesarean section was performed at 37 weeks' gestation for her delivery under epidural anesthesia. Following the operation, the total hysterectomy was scheduled due to the continuous massive bleeding. She rapidly developed ventricular tachycardia, following trachea intubation with propofol and suxamethonium. Since she was not resuscitated with cardiac massage, we started intravenous epinephrine, electronic cardioversion, the percutaneous cardiopulmonary support and intra-aortic balloon pumping with epinephrine and dopamine infusions leading to a successfully outcome. In this case, we believe that the combination of propofol and suxamethonium had the most impact on producing the cardiac arrest. Therefore, the case emphasizes the potential danger of using these combination for patients who have been treated with ritodrine.     

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 cesarean section in a patient with severe obesity

A 35-year-old pregnant woman (weight, 129.5 kg; height, 156 cm; 37 weeks of pregnancy) with a body mass index of 53 was scheduled for a cesarean section. It was thought that epidural or spinal anesthesia might result in complications due to her severe obesity. It was therefore decided to use general anesthesia following awake intubation. Her baby was delivered, and her Apgar scores at 1 and 5 minutes after delivery were 8 and 9 points, respectively. During surgery, she developed hypoxia due to upper shift of the diaphragm. After surgery, she was extubated after improvement of her oxygenation under spontaneous breathing. This case demonstrates that difficulties may be encountered during anesthetic management of a severely obese patient undergoing cesarean section.     

Open heart operation for a pregnant patient with Marfan syndrome

A 32-year-old woman with Marfan syndrome was diagnosed as annuloaortic ectasia and acute aortic dissection at 20 weeks of gestation. At 24 weeks of gestation, she got heart failure. Bentall's procedure was performed under cardiopulmonary bypass. Anesthesia was given using sevoflurane, midazolam and fentanyl. Normothermic high-flow high-pressure non-pulsatile perfusion was performed during cardiopulmonary bypass. We used dopamine, dobutamine, milrinone and nitroglycerin for weaning of cardiopulmonary bypass. Fetal heart rate was monitored, and was stable at 120 bpm throughout the operation. At 33 weeks of pregnancy a healthy baby was delivered by cesarian section.     

A case of laryngeal atresia (congenital high airway obstruction syndrome) with chromosome 5p deletion syndrome rescued by ex utero intrapartum treatment

The authors report a case of laryngeal atresia (congenital high airway obstruction syndrome [CHAOS]) that was diagnosed prenatally. The patient underwent successfully tracheostomy by ex utero intrapartum treatment (EXIT). The fetal ultrasonography and magnetic resonance imaging MRI showed a typical CHAOS pattern with expanded hyperechogenic lungs, inverted diaphragms, and a dilated trachea. Recently, 3 cases of prenatally diagnosed CHAOS were reported to be treated successfully by EXIT. The clinical manifestation and course of this case was not similar to these 3 cases. The 3 previous patients did not fare as well during gestation and were delivered earlier than that in our case. In our case, fetal hydrops was seen at 23 gestational weeks, but it gradually subsided and disappeared at 30 gestational weeks. The fetus was stable and well. After delivery at 39 weeks, the baby received respiratory assistance by ventilator assistance. After 3 days, she could breath well on her own. The patient also had chromosome 5p deletion syndrome and perineal groove. More experience in treating CHAOS cases with EXIT to fully estimate its clinical course and prognosis is needed.