【要約】 三叉神経傷害による持続的な痛みをもつ患者が多い一方で, メカニズムの複雑さのために適切な診断や治療は困難である. 近年, 脊髄後角において, 末梢神経障害によりミクログリアの活性化やIFN-γ発現量の上昇を引き起こすことが報告されている. そこで眼窩下神経障害モデルラットを用いて, 口腔顔面領域における神経障害性疼痛へのIFN-γの関与を調べることを目的とした. ラットの左側眼窩下神経 (infraorbital nerve) を半結紮したIONIラットおよび眼窩下神経の剖出だけを行ったshamラットを作製した. 神経結紮前と結紮3日目に両ラットの三叉神経第2枝領域 (V2) に機械刺激を加え, 逃避反射閾値 (HWT) を測定した. また, 両ラットの三叉神経脊髄路核尾側亜核 (Vc) において, Iba1とIFN-γの陽性発現様式を免疫組織学的に検索した. さらに, 3日間, IFN-γまたはvehicleをラットのくも膜下腔内に持続投与し, HWTの変化を調べた. IONIのV2への機械刺激に対する神経結紮側のHWTは, 結紮3日目において有意に低下したが, shamラットでは変化は認められなかった. また, ラットの髄腔内にIFN-γを持続投与することにより, HWTの有意な低下が認められた. 神経結紮3日目のIONIラットの結紮側Vc表層において, ミクログリアのマーカーであるIba1の有意な増加が認められ, 増加したIba1上にIFN-γ受容体の発現が認められた. 以上より, IFN-γはVc領域で活性化されたミクログリアから放出され, 眼窩下神経障害により口腔顔面領域での痛覚過敏を引き起こす可能性が示唆された.

  A 20-year-old woman with adult glycogen storage disease (GSD) type 0, a congenital glycogen synthase deficiency, underwent a velopharyngeal closure for velopharyngeal insufficiency following a palatoplasty. The patient had been diagnosed as having GSD type 0a because of hypoglycemia and a short stature at the age of one year. When she underwent a palatoplasty at the age of 3 years, however, she was suspected of having GSD type 0b because she also exhibited intraoperative metabolic acidosis, hypoglycemia, a high body temperature, and an elevation in postoperative creatine phosphokinase levels during surgery. When she underwent closure of a palate fistula at the age of 12 years, no such symptoms were observed during total intravenous anesthesia (TIVA) using propofol. For the presently reported procedure, therefore, we planned to use TIVA with propofol and remifentanil and without a muscle relaxant.

  A solution containing 2.5% dextrose and 1% dextrose was infused during anesthesia. Her intraoperative body temperature ranged from 36.5°C to 37.2°C, and the velopharyngeal closure was completed without any adverse events.

  GSD type 0 can potentiate the occurrence of several problems, such as hypoglycemia, muscle weakness, myoglobinuria, and myocardial damage. In the present case, therefore, we infused a solution containing glucose to avoid hypoglycemia and performed TIVA without a muscle relaxant to avoid serial complications caused by GSD type 0.

  Cardio-facio-cutaneous (CFC) syndrome is a RASopathy, a family of syndromes that includes Noonan and Costello syndromes and presents with overlapping phenotypic similarities. The major features of CFC syndrome include a characteristic craniofacial dysmorphology, congenital heart disease, dermatologic abnormalities, seizures, and intellectual disability. Musculoskeletal findings, such as muscle weakness and decreased muscle mass, are also observed in all RASopathies but are particularly prominent in CFC syndrome. We performed dental treatment under general anesthesia in an 8-year-boy with CFC syndrome. The boy was 120 cm tall, weighed 22 kg, and had a history of epileptic seizures and micrognathia. No abnormalities were noted in a cardiac evaluation including echocardiogram and electrocardiogram. However, respiratory muscle weakness leading to profound postoperative respiratory depression following the administration of general anesthetic, sedative, and opioid drugs was a major concern because of the presence of CFC syndrome. Anesthesia was induced by the inhalation of sevoflurane, N₂O, and oxygen. Although mask ventilation and intubation were difficult to perform, success was eventually achieved using a nasal airway and the McGRATH^TM MAC laryngoscope (McGRATH). Tracheal intubation was performed using sevoflurane and remifentanil without the use of a muscle relaxant. Anesthesia was maintained with desflurane and continuous intravenous remifentanil. Respiratory depression and epileptic seizures did not occur postoperatively. In patients with CFC syndrome who have muscle weakness, a rapid offset of desflurane may be effective to prevent respiratory depression after general anesthesia. The present case suggested that a preoperative cardiac evaluation, airway management, and the selection of an anesthetic agent to prevent postoperative respiratory depression are important for performing anesthesia safely in patients with CFC syndrome.

  Marshall-Smith syndrome (MSS) is an autosomal dominant inheritance disease characterized by hypoplasia of the middle face, sensory deafness, a tendency for bone fractures, and intellectual disability. For anesthetic management, difficult airway management because of mandibular hypoplasia has been reported. The removal of two mesiodens was planned in a ten-year-old boy with MSS. He had a history of bone fractures, mild intellectual disability, and the facial characteristics of MSS. A CT scan showed the partial obstruction of the upper airway and mandibular hypoplasia. Prior to entry into the operational room, midazolam was injected intravenously for sedation. General anesthesia was induced with propofol, fentanyl, sevoflurane and rocuronium. A nasal airway was effective for airway management, and a McGRATH^® MAC was used for intubation through the nasal cavity. General anesthesia was maintained with total intravenous anesthesia (TIVA), consisting of propofol and fentanyl. The operation was completed in 30 minutes, and no signs of agitation were observed during the emergence period. There were no significant changes in Spo₂ or other parameters throughout the anesthetic management. Leg protectors were used to prevent bone fractures throughout the preoperative period. An assessment of the airway using a CT scan was useful for predicting the difficulty of airway management and intubation. A nasal/oral airway and video laryngeal scope are strongly recommended for the induction of anesthesia in patients with MSS. Since TIVA can enable a calm emergence from anesthesia, it is likely to contribute to the prevention of bone fractures.

  We report our experience providing general anesthesia to a patient with thalidomide embryopathy. A 59-year-old man with thalidomide embryopathy, weighing 60 kg, was scheduled for dental treatment under general anesthesia because of trismus caused by microstomia. In thalidomide embryopathy, various congenital defects caused by thalidomide exposure are often accompanied by functional and structural orofacial impairments. Degenerative cervical vertebra changes, e.g., block vertebra or osteophytes, can also be seen in patients with this condition. For this reason, we considered the likelihood of technical difficulties with mask ventilation and tracheal intubation during general anesthesia. After readying the difficult airway cart and emergency cricothyrotomy kit and inducing anesthesia with propofol, we used a bronchoscope to perform nasotracheal intubation. Because of the patient’s restricted head tilt and laryngopharyngeal airway space stenosis caused by osteophytes in the epiglottal area, the position of the glottis was difficult to confirm. After multiple attempts to insert the bronchoscope tip, we succeeded in intubating the patient. Anesthesia was maintained using room air, oxygen, propofol, and remifentanil. Because of the likelihood of reintubation brought on by the high risk of post-procedure airway obstruction or difficulty in maintaining the airway, a tube exchanger was left in place while extubating the patient. Afterwards, the tube exchanger was removed once the patient’s respiration had stabilized. To obtain satisfactory airway management during general anesthesia in thalidomide embryopathy patients, an adequate pre-procedure assessment and preparation for the possibility of mask ventilation and intubation difficulties are essential.

  Long QT syndrome is characterized by a prolongation of the QT interval and a morphological change in the T wave on an electrocardiogram. Prolongation of the QT interval is associated with serious arrhythmia and may cause syncope and sudden death. A 13-year-old male with a prolonged corrected QT interval (QTc) on a preoperative electrocardiogram was scheduled to undergo the extractation of a supernumerary tooth under general anesthesia.

  Although he had no remarkable past medical history, his QTc was prolonged to 514 ms on a 12-lead electrocardiogram. There was no history of syncope or sudden death in his family. The QTc at the time of his entrance into the operation room was 483 ms. During surgery, his QTc was continuously monitored in addition to the usual intraoperative monitoring. After the rapid introduction of anesthesia with remifentanil and propofol, the anesthesia was maintained with remifentanil and sevoflurane in oxygen and air. No significant change in the QTc was observed at the time of tracheal intubation accompanied by sympathetic stimulation. There was also no significant change in the QTc after the administration of atropine for bradycardia. However, when 2% lidocaine containing adrenaline was used for local anesthesia, the QTc gradually became prolonged reaching a maximum of 507 ms. In patients with a prolonged QT interval on a preoperative electrocardiogram, careful attention may be needed during the use of local anesthetics containing adrenaline. In addition, continuous monitoring of the QTc during surgery may be useful for the prevention of serious arrhythmias.

  Multiple myeloma (MM) is a neoplastic proliferation of plasma cells characterized by the production of a large amount of abnormal immunoglobulin. Complications include renal impairment, hyperviscosity syndrome, amyloidosis, hypercalcemia, fractures, anemia, hemorrhagic tendency, and susceptibility to infection. We report the general anesthetic management of a patient with multiple myeloma undergoing a sequestrectomy for the treatment of medication-related osteonecrosis of the jaw (MRONJ).

  An 85-year-old woman (weight, 47.7 kg ; height, 148 cm) had a 4-year history of multiple myeloma. She had multiple myeloma-related complications including moderate renal impairment, anemia, and susceptibility to infection, and had received several immuno-pharmacotherapies. To prevent multiple myeloma-related destructive bony lesions, she was medicated with intravenous zoledronic acid for 14 months, which led to the development of MRONJ. She was scheduled to undergo a mandibular sequestrectomy under general anesthesia. Anesthesia was induced with propofol and remifentanil, and a lower dosage of rocuronium (20 mg) was administered incrementally under neuromuscular monitoring before tracheal intubation. The anesthesia was maintained with desflurane and remifentanil. To avoid acute kidney failure, low-dose carperitide was administered continuously. Because the effect of rocuronium was prolonged until the end of the surgery, sugammadex was administered to reverse the effect. Low-dose unfractionated heparin was injected subcutaneously to prevent deep vein thrombosis, which could have been induced by multiple myeloma-related hyperviscosity syndrome.

  Because bisphosphonates are the standard of care for the prevention of multiple myeloma-related destructive bone disease, the number of patients requiring surgical treatment for MRONJ is likely to increase. Since the symptoms of multiple myeloma vary among patients, appropriate perioperative management for each complication is required.

  Paroxysmal kinesigenic choreoathetosis (PKC) is a type of seizure disorder in which involuntary abnormal movements lasting a short time are triggered by a sudden, voluntary movement. Such seizures could be extremely dangerous if they were to occur during dental treatment. Here, a good outcome after tooth extraction under conscious sedation is reported for a patient with PKC-like seizures.

  The patient was a 34-year-old woman. At the age of 21 years, her involuntary movement seizures had begun to occur more frequently. Her electroencephalogram was normal, and her symptoms abated after the prescription of oral carbamazepine. Accordingly, she was diagnosed as having PKC-like seizures. Her presently treated illness was periodontitis of the lower left wisdom tooth. Taking into account the patient’s dental anxiety and the risk of a seizure, she was referred to our hospital for the administration of general anesthesia during the procedure. However, since the patient’s seizures had abated and the tooth extraction was considered to be a minorly invasive procedure, a decision was made to perform the extraction under intravenous sedation. Anesthesia was induced with midazolam and propofol, and the patient was sedated to a level of 3-4 on the Ramsay Sedation Scale (RSS) ; at this level, the patient was spoken to and the inside of her mouth was touched, triggering an immediate seizure. After the seizure disappeared, treatment was recommenced under conscious sedation at RSS 2. No further seizures occurred, and the surgical procedure was completed without incident.

  Since PKC occurs when a subject experiences excessive strain or fear, anesthesia was initially induced to a sedation level of RSS 3-4 in the present patient. However, speaking to the patient and touching her, even at this level of sedation, triggered a seizure. By continuing to perform the procedure under conscious sedation, allowing the patient to respond when spoken to, the procedure was completed safely.

  This report describes an adult patient with mental retardation and congenital heart disease who underwent a tooth extraction under intravenous sedation. The patient, an 18-year-old female, visited our hospital to undergo an orthodontics-related tooth extractions. She had been diagnosed as having tetralogy of Fallot soon after her birth and had undergone several surgical treatments including bidirectional Glenn procedures and a Fontan procedure, etc., for the treatment of corrected transposition of great arteries, ventricular septal defects, and pulmonary atresia prior to the age of 11 years. She had been cooperative with the treatments despite her mental retardation. She frequently had episodes of hypoxia caused by crying spells during her everyday life. She had undergone the extraction of bilateral upper first premolars and the right lower first premolar under local anesthesia but had cried excessively during the treatment. As a result, her Spo₂ had decreased markedly. The left lower first premolar was completely impacted and required extraction under intravenous sedation to reduce the invasiveness and to alleviate the patient’s physical and mental stress. Despite the fact that a Fontan circulation had been established, her daily Spo₂ was relatively low because of veno-venous collateral circulation and a persistent left superior vena cava. Episodes of hypoxia triggered by a low pulmonary blood flow caused by an increase in pulmonary vascular resistance or a decrease in systemic vascular resistance were thus a concern. Midazolam and pentazocine was administered at the start of sedation. Propofol was administered during the operation.

  In cases such as this one, episodes of hypoxia should be prevented. Intravenous sedation was found to be effective for maintaining an optimal hemodynamic condition, as it enabled increases in the central venous pressure to be avoided, compared with general anesthesia. Intravenous sedation also reduced anesthetics-induced vasodilatation and did not stimulate the sympathetic nervous system due to a need for intubation and extubation.

  In patients with single ventricle heart disease, non-cardiac surgery using the Glenn procedure can be performed to alleviate cyanosis prior to repair using the Fontan procedure. We performed general anesthesia management during lip repair and palatoplasty in a patient with left ventricular hypoplasia following a Glenn procedure.

  The patient underwent lip repair at an age of 1 year and 2 months and a palatoplasty at the age of 1 year and 10 months. During the preoperative examinations, hemorrhagic diathesis, hypoxemia, and secondary polycythemia were observed.

  General anesthesia was induced using midazolam, fentanyl, and remifentanil and was maintained using fentanyl and sevoflurane in oxygen (Fio₂=0.47). Low blood pressure was treated with the administration of an α1 receptor stimulant or volume expansion with infusion to prevent an increase in pulmonary vascular resistance and to maintain the pulmonary blood flow. In addition, pulmonary blood flow was maintained by increased oxygen administration and mild hyperventilation, inhibiting any deterioration in oxygenation through the maintenance of blood flow. After the palatoplasty, hypoxemia and intraoral bleeding were observed, and reintubation was performed.

  Patients who are scheduled to undergo a palatoplasty have a high risk of bleeding because the wound surface remains in front of the palate. In addition, the risk of bleeding increases in patients with cyanosis because of the use of antithrombotic drugs, the increase in collateral blood flow, and the presence of secondary polycythemia.

  In conclusion, the risks of increased pulmonary vascular resistance, hypoxemia, and increased bleeding must be considered for safe anesthesia during a palatoplasty in patients with cyanotic heart disease.

  Stellate ganglion block can be effective for neuropathic pain with sympathetically dependent pain in the orofacial area. A paratracheal approach for stellate ganglion block has been recommended as the safest route because of its technical simplicity. We herein report a rare complication of total spinal anesthesia following the inadvertent subarachnoid injection of a local anesthetic during a stellate ganglion block.

  A-55-year old female received stellate ganglion blocks for the relief of neuropathic pain in the right lower molar area. Immediately after the sixth stellate ganglion block with 6 ml of 1% lidocaine, the patient suddenly lost consciousness and her breathing stopped completely. We soon initiated cardiopulmonary resuscitation using a bag valve mask connected to an oxygen tank. Her electrocardiogram (ECG) was normal, with a heart rate of 62 beats/min, a blood pressure of 140/84 mmHg, and a pulse oximetry of 74%. The pulse oximetry value quickly recovered to 99%. Approximately 10 minutes later, the patient was able to open her eyes slowly when her name was called. Spontaneous respiration appeared, and her consciousness had nearly recovered 13 minutes later. Approximately 90 minutes later, her general condition had almost recovered. She did not complain of a headache.

  The clinical findings strongly suggested the occurrence of total spinal anesthesia following the inadvertent subarachnoid injection of lidocaine. The possible cause of the spread of the local anesthetic into the subarachnoid space is discussed in the present report. This case suggests that the preparation of a CPR kit should be required when performing stellate ganglion block treatment.

  Multiple system atrophy is a component of spinocerebellar degeneration. Multiple system atrophy is a progressive neurodegenerative disorder characterized by a combination of symptoms affecting both the autonomic nervous system and movement. Symptoms of autonomic failure that may be seen in patients with multiple system atrophy include fainting. We herein report a patient with multiple system atrophy who transiently lost consciousness as a result of postprandial hypotension while in the waiting room of general dentistry.

  A 60-year-old man with multiple system atrophy complaining of dental pain and requiring oral care visited our dental clinic. While the patient was in the waiting room, a dental hygienist connected him to a monitor. His blood pressure then decreased to 41/31 mmHg. An emergency call was immediately placed, and the patient’s vital signs were examined. Although the electrocardiogram findings were normal, the patient transiently lost consciousness. His blood pressure was 43/34 mmHg, his heart rate was 95 beats/min, and his pulse oximetry level was 95%. His blood sugar level was 105 mg/dl. A bolus dose of 2 mg of ephedrine was administered intravenously, indirectly stimulating the sympathetic nervous system and causing the patient’s blood pressure to increase to 78/38 mmHg. Approximately 3 minutes later, the patient showed a slight reaction when his name was called.

  The clinical findings strongly suggested postprandial hypotension. When performing dental treatments for patients with multiple system atrophy, caution regarding hypotension and severe autonomic nervous failure is needed. Additionally, preparation for emergency resuscitation is absolutely necessary.

  Considerable progress in community-based integrated care systems based on the principle of “supporting people so that they may live according to their own values in their local communities” has been made. At present, these systems target not only the elderly, disabled people, and children, but also individuals who have difficulty living within their community. However, very few studies have demonstrated how the range of support could be expanded through cooperation amongst various professionals or the role of dentistry in such systems.

  Several studies have implied that the role of dentistry is limited to oral health. However, dentists could play not only oral health but also other roles in integrated care systems. Dentists are skilled at detecting oral abnormalities, which can be helpful for detecting cases of child abuse and other problem cases. Physicians are necessarily regarded as key figures in multidisciplinary collaborations. However, dentists are similar to physicians in their medical capacity and can also act as intermediaries between medical and welfare professionals. Importantly, among dentists who can serve as a bridge between physicians and other professionals, dental anesthesiologist may contribute the most.

  Over the last few years, the authors have actively promoted the inclusion of dentists in integrated care systems. In their daily practice, dental anesthesiologists manage many different professionals to form an organic team and act behind the scenes to achieve the team’s healthcare goals. These tasks enable dental anesthesiologists to maximize their abilities as coordinators of multidisciplinary collaborations.

  In this article, these characteristics of dental anesthesiologists are first reaffirmed. Thereafter, the means by which dental anesthesiologists can use their special skills to fulfill their ideal role as dentists in integrated care systems will be discussed.