Anesthetic management of a patient with congenital diaphragmatic eventration

Congenital diaphragmatic eventration is uncommon in adults and is caused by paralysis, aplasia or atrophy of the muscular fibers of the diaphragm. It may cause severe dyspnea, orthopnea and hypoxia in adult patients. Most symptomatic patients may be managed efficiently without the need for surgical correction, although any event that leads to an increase in intra-abdominal pressure puts them at the risk of spontaneous diaphragmatic rupture. This case report presents the successful anesthetic management of an adult female with congenital diaphragmatic eventration undergoing diagnostic laparoscopy and hysteroscopy using a total intravenous anesthesia technique. Essential steps to prevent any rise in intrathoracic and intra-abdominal pressures along with care to minimize intragastric volume were taken.     

Anesthetic management of a patient with non-bullous congenital ichthyosiform erythroderma

Non-bullous congenital ichthyosiform erythroderma is a disorder in the cornification of the skin, histopathologically characterized by hyperkeratosis. Previous reports mentioned that the fixation of endotracheal tubes and ECG electrodes to the skin was likely to be infirm in the patients with this disorder, and that the patients' body temperatures were easily affected by the environment. A 3-year-old girl with non-bullous congenital ichthyosiform erythroderma underwent two operations separately under general anesthesia. We used Hollister Skin Gel to fix the endotracheal tube. This gel helped prevent the irritation associated with the application and removal of adhesives. The patient became severely hypothermic during the first operation. Fortunately, the intraoperative body temperature could be maintained at a normal level during the second operation with the use of a forced-air warming system. Careful perioperative consideration is required for the fixation of endotracheal tubes and the management of body temperature in patients with non-bullous congenital ichthyosiform erythroderma.     

Anesthetic management of a patient with deficiency of congenital coagulation factor XII

A 63-year-old man with deficiency of congenital coagulation factor XII, was referred to our hospital for an operation of the hypopharynx cancer. The day before surgery under general anesthesia, fresh frozen plasma was administered to him with good response of APTT shortening from 397.0 to 36.4 seconds. During operation, bleeding tendency was controlled and fresh frozen plasma was administered. Total blood loss during the operation was 255 ml. During post-operative period, APTT was kept at an adequate level. The post-operative course was uneventful and he was discharged after the radiation and an adjuvant therapy.     

Anesthetic management of a patient with congenital plasminogen activator inhibitor-1 deficiency

Congenital plasminogen activator inhibitor-1 (PAI-1) deficiency is an extremely rare disorder characterized by a bleeding diathesis due to hyperfibrinolysis as a result of decreased PAI-1 activity. A 21-year-old male with congenital PAI-1 deficiency underwent wisdom teeth extraction of the mandible under general anesthesia using propofol, nitrous oxide, sevoflurane, fentanyl, and vecuronium. No complications including prolonged bleeding and rebleeding after the operation were observed because hemostatic management was successful by using intravenous tranexamic acid.     

Anesthetic management of the adult patient with congenital heart disease

As the number of CHD repairs in adults continues to increase, these operations will be performed in a wider variety of institutions and systems. Unfortunately, not all of these centers will have an optimal environment for correcting CHD in adults. This type of surgery is best accomplished in a facility specifically designed for treating adults with CHD. Optimal care of these patients is provided by cardiologists who are trained and experienced in pediatric and adult cardiology, by surgeons who are trained and experienced in treating CHD, and by anesthesiologists who are experienced in caring for adults with CHD. Whatever the setting, cardiac anesthesiologists involved in these cases must be thoroughly aware of the anesthetic implications for the unique pathophysiology of each patient, and they must not rely on their "usual" expectations of either true pediatric CHD or acquired adult heart disease.     

Anesthetic management of a patient with congenital myopathy complicated with severely impaired pulmonary function

A 17-year-old man, who had received a diagnosis of congenital myopathy, was scheduled for superficial temporal artery to middle cerebral artery anastomosis procedure. Preoperative respiratory examinations showed the breathing capacity of 450 ml because of a scoilosis, deformity of a thorax and severe muscular atrophy. Anesthesia was maintained with propofol, fentanyl, remifentanil and vecuronium to avoid malignant hyperthermia (MH). Endotracheal intubation was performed with a gum elastic bougie for difficult airway management (DAM). After intubation, we checked the positioning of the tracheal tube by a chest X ray and bronchofiber findings. During perioperative period, no symptom of MH and respiratory dysfunction was noticed. In the anesthesia for patients with congenital myopathy, deterioration of respiratoy function, prevention for MH, and possibility of DAM should be considered.     

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 for a patient with mental retardation and unexamined complex congenital heart disease

An 18-year old female with mental retardation and unexamined complex congenital heart disease received dental care under general anesthesia. Anesthesia was induced and maintained successfully without any significant hemodynamic changes with inhalation of nitrous oxide, oxygen (FIO2 0.25-0.3) and sevoflurane after a heavy premedication (morphine 10 mg, scopolamine 0.3 mg and midazolam 5 mg i.m.). After induction of anesthesia, cardiac anomaly was diagnosed by transesophageal echocardiography as TGA, VSD, PS, and operation was completed without any problem. Two points are considered important in this case; first, to appropriately estimate preoperative cardiac function and second, to adequately manage anesthesia to avoid any hemodynamic fluctuation.     

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.