Continuous infusion propofol general anesthesia for dental treatment in patients with progressive muscular dystrophy

Progressive muscular dystrophy may produce abnormal reactions to several drugs. There is no consensus of opinion regarding the continuous infusion of propofol in patients with progressive muscular dystrophy. We successfully treated 2 patients with progressive muscular dystrophy who were anesthetized with a continuous infusion of propofol. In case 1, a 19-year-old, 59-kg man with Becker muscular dystrophy and mental retardation was scheduled for dental treatment under general anesthesia. General anesthesia was maintained by a continuous infusion of 6-10 mg/kg propofol per hour and an inhalational mixture of 67% nitrous oxide and 33% oxygen. No complications were observed during or after the operation. In case 2, a 5-year-old, 11-kg boy with Fukuyama type congenital muscular dystrophy and slight mental retardation was scheduled for dental treatment under general anesthesia. General anesthesia was maintained with a continuous infusion of 6-12 mg/kg propofol per hour and an inhalational mixture of 0.5-1.5% sevoflurane in 67% nitrous oxide and 33% oxygen. No complications were observed during or after the operation. It is speculated that a continuous infusion of propofol in progressive muscular dystrophy does not cause malignant hyperthermia because serum levels of creatine phosphokinase and myoglobin decreased after our anesthetic management. Furthermore, our observations suggest that sevoflurane may have some advantages in patients with progressive type muscular dystrophies other than Duchenne muscular dystrophy and Becker muscular dystrophy. In conclusion, our cases suggest that a continuous infusion of propofol for the patients with progressive muscular dystrophy is a safe component of our anesthetic strategy.     

Propofol anesthesia for a patient in the terminal stage of Fukuyama type congenital muscular dystrophy

A 31-year-old tracheostomized woman weighing 32.5 kg with Fukuyama type congenital muscular dystrophy in terminal stage was scheduled for dilatation of the tracheostoma, removal of the tracheal granulomas and reconstruction of gastrostoma. Anesthesia was induced and maintained with continuous propofol infusion and intermittent fentanyl. Muscle relaxant was not necessary because of generalized severe muscular atrophy. There was no hemodynamic derangement during the surgery and emergence from the anesthesia was rapid. There were no postoperative complications related to anesthesia and surgery.     

Femoral nerve blocks

Peripheral nerve blocks can provide high-quality anesthesia and analgesia after unilateral lower-extremity surgery. Lower-extremity nerve blocks, though underused, have significant advantages to central neuraxial techniques, especially in the ambulatory setting. Femoral nerve blocks are easy to perform, have few side effects, and a low incidence of complications. Despite these advantages, thorough knowledge of the pertinent anatomy is required to consistently perform these blocks successfully. This article reviews relevant femoral nerve anatomy. Techniques for single injection, as well as continuous blockade of the femoral nerve, are discussed. Also considered in this article is the efficacy of femoral nerve blocks and some of the shown improvements in patient outcome. This review also focuses on improved catheter technology and the accessibility of novel disposable infusion pumps that have enabled the use of continuous femoral nerve blocks on a larger scale. Copyright 2003, Elsevier Science (USA). All rights reserved.     

Tipps and Tricks - Anesthesiologic management in total knee replacement

Total knee replacement improves mobility and quality of life in gonarthrosis. In 2004 more than 60.000 total knee arthroplasties were performed in Germany. Because of severe postoperative pain which can interfere with functional outcome and impair hospital discharge, effective postoperative analgesia is not only important for patient satisfaction but also for economic reasons. For the 1990(th) years femoral nerve blocks have become more popular leads back to lower risk of complications and equal quality of analgesia compared to neuroaxial blockades. The prevention of "tourniquet pain" as well as treatment of hemodynamic and respiratory failure in the context of pulmonary embolic events and tourniquet deflation is part of anesthesiologic challenge.     

Anesthetic management of a patient with Becker muscular dystrophy

We experienced anesthetic management of a patient with Becker muscular dystrophy. He had advanced dilated cardiomyopathy and high serum CK in the preoperative examinations. Anesthesia was planned to avoid triggering malignant hyperthermia or rhabdomyolysis and hemodynamic changes. Propofol, remifentanil and a minimum dose of rocuronium bromide were used for anesthetic induction and maintainance. Arterial pressure, cardiac output and stroke volume variation were monitored by Flotrac sensor. There were no adverse events observed during the anesthetic management. In conclusion, total intravenous anesthesia with the administration of rocuronium and circulatory monitoring by Flotrac sensor could be safe and efficient for anesthetic management of patients with Becker muscular dystrophy.     

Peripheral nerve blocks in patients with Ehlers-Danlos syndrome,hypermobility type: a report of 2 cases

Ehlers-Danlos syndrome is an inherited disorder of collagen production that results in multiorgan dysfunction. Patients with hypermobility type display skin hyperextensibility and joint laxity, which can result in chronic joint instability, dislocation, peripheral neuropathy, and severe musculoskeletal pain. A bleeding diathesis can be found in all subtypes of varying severity despite a normal coagulation profile. There have also been reports of resistance to local anesthetics in these patients. Several sources advise against the use of regional anesthesia in these patients citing the 2 previous features. There have been reports of successful neuraxial anesthesia, but few concerning peripheral nerve blocks, none of which describe nerves of the lower extremity. This report describes 2 cases of successful peripheral regional anesthesia in the lower extremity. In case 1, a 16-year-old adolescent girl with hypermobility type presented for osteochondral grafting of tibiotalar joint lesions. She underwent a popliteal sciatic (with continuous catheter) and femoral nerve block under ultrasound guidance. She proceeded to surgery and tolerated the procedure under regional block and intravenous sedation. She did not require any analgesics for the following 15 hours. In case 2, an 18-year-old woman with hypermobility type presented for medial patellofemoral ligament reconstruction for chronic patella instability. She underwent a saphenous nerve block above the knee with analgesia in the distribution of the saphenous nerve lasting for approximately 18 hours. There were no complications in either case. Prohibitions against peripheral nerve blocks in patients with Ehlers-Danlos syndrome, hypermobility type, appear unwarranted.     

Inadvertent life-threatening total spinal anesthesia following caudal block in a preschool child underwent urologic surgery: A rare case report

Introduction and importanceCaudal block is considered to be safe and provide optimal analgesia for pediatric patients undergoing sub-umbilical operations. It overcomes opioid-related side effects, particularly the dangers associated with respiratory depression in small children.Case presentationA 5-year-old male underwent uneventful hypospadias surgery under general endotracheal anesthesia. Caudal block planned to be administered postoperatively for postoperative analgesia then performed after palpation of sacral cornu with 8 ml of 0.25% bupivacaine. A few minutes later, the patient became apneic, heart rate, blood pressure, and oxygen saturation dropped abruptly—immediate resuscitation with ventilatory support, fluid bolus, and atropine administration. After a minute patients' vital signs returned to the normal range then 2 h later patient started to breathe spontaneously and consciousness is regained. After close follow-up for 24 h in the post-anesthesia care unit patient was discharged to the pediatric ward then discharged to home without any neurologic sequelae after 3 days.Clinical discussionTotal spinal anesthesia in a very infrequent incident during central neuraxial blocks, especially in the pediatrics population where a caudal block is usually performed. Manifestation of this event can be detected by loss of consciousness, cessation of respiratory effort, hemodynamic instability, and dilated pupils. Delayed treatment can result in cardiopulmonary arrest.ConclusionUnanticipated total spinal anesthesia following central neuraxial blocks can potentially cause severe adverse consequences. Preventive modalities must be employed to avoid this incident. Early recognition and instant management should be instituted to avoid dangerous complications following the total spinal blockade.     

Ultrasound-guided combined femoral nerve and lateral femoral cutaneous nerve blocks for femur neck fracture surgery--case report

We report two cases of elderly patients who underwent ultrasound-guided combined femoral nerve and lateral femoral cutaneous nerve blocks for osteosynthesis of femur neck fracture. In both cases, neuraxial anesthesia was contraindicated due to coagulopathy, and severe restrictive ventilatory disorder was observed. The femoral nerve and lateral femoral cutaneous nerve blocks were performed with 20ml of 0.375% ropivacaine under ultrasonographic visualization using a high frequency linear probe. Ultrasonographic visualization was useful to identify the needle tip and to observe the spread of local anesthetics. Supplemental local infiltration of anesthetics and intravenous low-dose sedative drugs were administered during surgery. The perioperative course was uneventful in both cases. This combined block technique could be a choice for osteosynthesis of femur neck fracture especially in high-risk patients.     

Regional techniques as an adjunct to general anesthesia for pediatric extremity and spine surgery

The purpose of this review is to evaluate the safety of regional anesthesia techniques performed for postoperative analgesia in anesthetized children. Pediatric regional anesthesia techniques, such as nerve blocks and neuraxial injections of either local anesthetics or narcotics, can potentially reduce postoperative pain for all children undergoing surgery. However, children may react differently to anesthesia than adults, and they usually cannot tolerate the administration of regional anesthesia unless they are under general anesthesia.During a 5-year period (1999-2004) at the Shriners Hospitals for Children Northern California, 2236 regional anesthetic procedures were performed in 1809 patients. All of the regional procedures were performed with patients under general anesthesia. Ninety-one percent (1641) of patients were for orthopaedic extremity or spine surgeries. Patients ranged from 2 months to 20 years old, with 65% (1169) between the ages of 6 months and 12 years. One thousand eleven procedures were lower extremity blocks, 646 were upper extremity blocks, and 579 were neuraxial injections. Four hundred fifty-four peripheral nerve blocks were performed in patients aged 3 years or younger. Two self-limiting complications possibly related to peripheral nerve blocks were noted. No complications were noted in patients who received neuraxial injections. This retrospective review indicates that regional anesthesia techniques performed 'under general anesthesia have a low rate of complications in children. A prospective trial is recommended to establish the efficacy and safety of this practice.     

Guidelines and alternatives for neuraxial anesthesia and venous thromboembolism prophylaxis in major orthopedic surgery

Neuraxial anesthesia during major orthopedic surgery, combined with venous thromboembolism prophylaxis, is generally safe and well tolerated, with potential benefits over general anesthesia. The risk of spinal/epidural hematoma, a rare but very serious complication, can be minimized by careful patient selection and attention to anesthetic technique. This risk is further reduced with the use of peripheral nerve blocks in place of neuraxial anesthesia.     

Anesthesia with sevoflurane for tonsillectomy in a boy with Duchenne muscular dystrophy

A 6-year-old boy with Duchenne muscular dystrophy (DMD) and foreseen difficult tracheal intubation underwent tonsillectomy under general inhaled anesthesia with sevoflurane. No neuromuscular blockers were administered and no perioperative complications emerged. In spite of advances in genetic diagnosis there continue to be patients with DMD because of spontaneous mutation of the dystrophin gene. Late detection leaves them vulnerable to administration of drugs like succinylcholine that can trigger fatal reactions involving hyperpotassemia, rhabdomyolysis, and malignant hyperthermia. Total intravenous anesthesia seems the best way to provide general anesthesia for a patient with DMD. Inhaled anesthesia is an alternative. Although halogenated agents can lead to rhabdomyolysis and malignant hyperthermia, the frequency seems low if we bear in mind that the use of sevoflurane is widespread in pediatrics. In this case sevoflurane induction facilitated safe tracheal intubation.     

Regional anesthesia for orthopedic procedures: What orthopedic surgeons need to know

Regional anesthesia is an integral component of successful orthopedic surgery. Neuraxial anesthesia is commonly used for surgical anesthesia while peripheral nerve blocks are often used for postoperative analgesia. Patient evaluation for regional anesthesia should include neurological, pulmonary, cardiovascular, and hematological assessments. Neuraxial blocks include spinal, epidural, and combined spinal epidural. Upper extremity peripheral nerve blocks include interscalene, supraclavicular, infraclavicular, and axillary. Lower extremity peripheral nerve blocks include femoral nerve block, saphenous nerve block, sciatic nerve block, iPACK block, ankle block and lumbar plexus block. The choice of regional anesthesia is a unanimous decision made by the surgeon, the anesthesiologist, and the patient based on a risk-benefit assessment. The choice of the regional block depends on patient cooperation, patient positing, operative structures, operative manipulation, tourniquet use and the impact of post-operative motor blockade on initiation of physical therapy. Regional anesthesia is safe but has an inherent risk of failure and a relatively low incidence of complications such as local anesthetic systemic toxicity (LAST), nerve injury, falls, hematoma, infection and allergic reactions. Ultrasound should be used for regional anesthesia procedures to improve the efficacy and minimize complications. LAST treatment guidelines and rescue medications (intralipid) should be readily available during the regional anesthesia administration.     

Anesthetic management for a patient of myotonic dystrophy with pheochromocytoma

We experienced an anesthetic management for a patient of myotonic dystrophy with pheochromocytoma. Much attention is required to manage myotonic dystrophy on surgical manipulation. This disease interacts with anesthetic drugs. It may cause prolongation of drug action used during anesthesia compared with the usual case. It also may cause dangerous interactions such as severe arrhythmia and malignant hyperthermia. That is why we were faced with serious limitation in choosing anesthetic and adjuvant drugs. At the same time, the case of pheochromocytoma must be handled with scrupulous care. Pheochromocytoma causes severe hypertension and sometimes tachycardia leading to intracranial hemorrhage or adrenaline-induced severe hypovolemia. Besides, laparoscopic operation was scheduled to resect the pheochromocytoma. This operation demanded the anesthetic management with artificial ventilation. It must be difficult to cope with these conditions by limited number of drugs. This time, we managed this case by epidural anesthesia with propofol and nitrous oxide without opioid and muscular relaxant. Though, this patient was not fully awake from anesthesia and could not take enough breaths on his own. We extended the period of spontaneous breathing with careful check whether the patient has resumed spontaneous breathing. It took us fourteen days till extubation.     

Neurologic complications of central neuraxial blocks

Central neuraxial blocks, which are associated with a low incidence of complications, are safe. When complications do occur, however, the resulting morbidity and mortality is considerable. The reported incidence of complications in all series is under 4 per 10000 patients, but given the absence of formal registries and notification procedures, which have legal implications, the real rate of occurrence of these rare events is uncertain. We searched the literature through PubMed and the Cochrane Plus Library for a 5-year period, using the search terms epidural anesthesia AND safety, spinal anesthesia AND safety, complications AND epidural anesthesia, complications AND spinal anesthesia, neurologic complications AND epidural anesthesia, and neurologic complications AND spinal anesthesia. Neuraxial injury after a central blockade may be the result of anatomical and/or physiological lesions affecting the spinal cord, spinal nerves, nerve roots, or blood supply. The pathophysiology of neuraxial injury may be related to mechanical, ischemic, or neurotoxic damage or any combination. When a complication occurs, factors related to the technique will have interacted with pre-existing patient-related conditions. Various scientific societies have published guidelines for managing the complications of regional anesthesia. Recently published clinical practice guidelines recommend ultrasound imaging as a useful tool in performing a central neuraxial block.     

Successful use of sugammadex in a muscular dystrophy patient

Muscular dystrophy requires cautious administration of muscle relaxants due to variable sensitivity and prolonged effects. A 43-year-old man with muscular dystrophy was scheduled for open reduction and internal fixation under general anesthesia. Following patient's TOF ratio with the muscle relaxation monitor, 80 minutes after rocuronium bromide (Rb) administration, we found that TOF ratio was over 0.9. We used sugammadex 4 mg x kg(-1) to reverse Rb-induced neuromuscular block, and then extubated. There was no clinical adverse effect on his muscular function and no respiratory distress after the use of sugammadex in the postoperative phase. Reversal of Rb-induced neuromuscular block by sugammadex in a patient with muscular dystrophy is efficient and safe.     

Reação atípica à anestesia em distrofia muscular de Duchenne/Becker

Background and objectives

Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia.

Severe neurological complications after central neuraxial blockades in Sweden 1990-1999

BACKGROUND: Central neuraxial blockades find widespread applications. Severe complications are believed to be extremely rare, but the incidence is probably underestimated. METHODS: A retrospective study of severe neurologic complications after central neuraxial blockades in Sweden 1990-1999 was performed. Information was obtained from a postal survey and administrative files in the health care system. During the study period approximately 1,260,000 spinal blockades and 450,000 epidural blockades were administered, including 200,000 epidural blockades for pain relief in labor. RESULTS:: The 127 complications found included spinal hematoma (33), cauda equina syndrome (32), meningitis (29), epidural abscess (13), and miscellaneous (20). Permanent neurologic damage was observed in 85 patients. Incidence of complications after spinal blockade was within 1:20-30,000 in all patient groups. Incidence after obstetric epidural blockade was 1:25,000; in the remaining patients it was 1:3600 (P < 0.0001). Spinal hematoma after obstetric epidural blockade carried the incidence 1:200,000, significantly lower than the incidence 1:3,600 females subject to knee arthroplasty (P < 0.0001). CONCLUSIONS:: More complications than expected were found, probably as a result of the comprehensive study design. Half of the complications were retrieved exclusively from administrative files. Complications occur significantly more often after epidural blockade than after spinal blockade, and the complications are different. Obstetric patients carry significantly lower incidence of complications. Osteoporosis is proposed as a previously neglected risk factor. Close surveillance after central neuraxial blockade is mandatory for safe practice.     

Evaluation of acute knee injuries with sciatic/femoral nerve blocks

A preliminary study of the applicability of sciatic and femoral regional nerve blocks in the evaluation of acute knee injuries was conducted. During the period from January 1980 to March 1981, 12 patients with acute knee injuries in whom clinical examination under local anesthesia was considered totally unreliable secondary to patient uncooperation or severe pain were examined at Grady Memorial Hospital. Each of these patients received regional anesthesia by sciatic/femoral nerve block. All patients obtained satisfactory relaxation and analgesia for complete evaluation, and 92% obtained total analgesia for the knee. A full range of motion was present in each patient after the block. No complications were encountered. A satisfactory block was obtained in one attempt in 96% of the patients. (One patient had a failed femoral nerve block, but a repeated block was successful).     

Severe muscular dystrophy and pregnancy: interdisciplinary challenge

The muscular dystrophies are degenerative muscle diseases characterized by progressive muscle weakness. The vast majority of women suffering from muscle diseases develop a deterioration of symptoms during pregnancy. Cardiac and respiratory complications are observed in pregnant women with muscular dystrophy especially in the second and third trimester. The successful perioperative therapy of a 32-year-old pregnant tetraplegic woman with a severe limb-girdle type muscular dystrophy who underwent elective Caesarean section is reported. According to the literature epidural and spinal anesthesia are both possible for perioperative anesthetic management in women with limb-girdle dystrophies. Due to the excellent controllability of intrathecal hyperbaric bupivacaine it was decided to use spinal anesthesia and non-invasive positive pressure ventilation was planned in case of impairment of respiratory function. In summary limb-girdle muscular dysthrophies should be managed on an individual basis and spinal anesthesia can be safely used to avoid intubation.     

Severe Neurological Complications after Central Neuraxial Blockades in Sweden 1990-1999

Background: Central neuraxial blockades find widespread applications. Severe complications are believed to be extremely rare, but the incidence is probably underestimated.

Methods: A retrospective study of severe neurologic complications after central neuraxial blockades in Sweden 1990-1999 was performed. Information was obtained from a postal survey and administrative files in the health care system. During the study period approximately 1,260,000 spinal blockades and 450,000 epidural blockades were administered, including 200,000 epidural blockades for pain relief in labor.

Results: The 127 complications found included spinal hematoma (33), cauda equina syndrome (32), meningitis (29), epidural abscess (13), and miscellaneous (20). Permanent neurologic damage was observed in 85 patients. Incidence of complications after spinal blockade was within 1:20-30,000 in all patient groups. Incidence after obstetric epidural blockade was 1:25,000; in the remaining patients it was 1:3600 (P < 0.0001). Spinal hematoma after obstetric epidural blockade carried the incidence 1:200,000, significantly lower than the incidence 1:3,600 females subject to knee arthroplasty (P < 0.0001).