Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery

The objective of this study was an evaluation of the prevalence of myopathies in paediatric patients scheduled for orthopaedic surgery (clubfoot) performed under regional anaesthesia. Seventeen infants scheduled for lower limb orthopaedic surgery were studied to verify coexisting neuromuscular disorders with electromyography and muscle biopsy during surgery. All surgical procedures were performed under caudal block or spinal anaesthesia, associated with light general anaesthesia. No major cardiorespiratory, neurological or malignant hyperthermic complications (muscle rigidity, arrhythmias, hyperpyrexia) were observed. Combined neurological, electromyographic and biopsy studies showed a high rate of myopathic changes (70%). Performance of clubfoot surgery under light general anaesthesia with regional techniques was free from any problems. The high rate of myopathic changes (70%) observed in the muscle biopsies suggests that precautions should be taken with paediatric patients for clubfoot surgery and a regional anaesthesia technique with adequate monitoring may be helpful to prevent possible malignant hyperthermia related problems.     

Malignant hyperthermia and central core disease causative mutations in Swedish patients

BACKGROUND: Malignant hyperthermia (MH) susceptibility is a pharmacogenetic disorder of intracellular calcium homeostasis. In susceptible individuals, halogenated anaesthetics and/or suxamethonium may trigger an MH reaction. The diagnosis of MH susceptibility is made by an in vitro contracture test of biopsied muscle strips. METHODS: In 27 MH susceptible (MHS) probands and four MH negative (MHN) probands, exons 17, 39, 40, 45 and 46 of the RYR1 gene were screened for MH causative mutations. In addition, in three patients with established central core disease (CCD), exons 17, 39, 40, 45 and 46 and exons 95, 100, 101 and 102 were screened for MH and CCD causative mutations. All screenings were performed by direct sequencing of the entire exons. RESULTS: MH causative mutations were found in five of the 27 MHS probands (19%). CCD causative mutations were found in two of three CCD patients in the C-terminal exons. None of the CCD patients showed a mutation in N-terminal exon 17 or in the central exons. CONCLUSIONS: In a Swedish population, screening of N-terminal exon 17 and the central exons for MH causative mutations in the RYR1 gene covers 19% of families. Thus, other mutations must also be responsible for MH susceptibility in Sweden. Although the number of CCD patients in this study was small, screening of the C-terminal exons for CCD causative mutations seems to be a promising tool in the process of making a diagnosis.     

Anesthetic considerations in myofibrillar myopathy

Myofibrillar myopathy (MFM) is a relatively newly recognized genetic disease that leads to progressive muscle deterioration. MFM has a varied phenotypic presentation and impacts cardiac, skeletal, and smooth muscles. Affected individuals are at increased risk of respiratory failure, significant cardiac conduction abnormalities, cardiomyopathy, and sudden cardiac death. In addition, significant skeletal muscle involvement is common, which may lead to contractures, respiratory insufficiency, and airway compromise as the disease progresses. This study is the first report of anesthetic management of a patient with MFM. We report multiple anesthetic encounters of a child with genetically confirmed BAG3‐myopathy, a subtype of MFM with severe childhood disease onset. A review of the anesthetic implications of the disease is provided, with specific exploration of possible susceptibility to malignant hyperthermia, rhabdomyolysis, and sensitivity to other anesthetic agents.     

Progressive scoliosis in central core disease

Central core disease (CCD) is a rare congenital myopathy with autosomal dominant inheritance. Here, we report on two cases of progressive scoliosis in CCD, pointing out the value of a muscle biopsy to establish the correct diagnosis. The first case involves a 13-year-old boy with severe progressive scoliosis and joint contractures. The patient was initially diagnosed with arthropgryposis multiplex congenita. The second case involves a 45-year-old man with severe scoliosis that had slowly progressed over the years. Both patients suffered from unexplained muscle weakness and severe restriction of pulmonary function. The correct diagnoses were established through muscle biopsies taken from the paravertebral musculature during scoliosis surgery. Correction of the spinal deformities was achieved through posterior instrumentation in both patients, with prior anterior release in one patient. Although scoliosis is a common feature in CCD, the correct diagnosis can be missed in scoliosis patients. Therefore, we recommend a muscle biopsy in patients with scoliosis, unexplained muscle weakness and multiple joint problems.     

Anaesthetic management of coronary artery bypass grafting in a patient with central core disease and susceptibility to malignant hyperthermia on statin therapy

Central core disease and malignant hyperthermia (MH) are bothassociated with mutations in the RYR1 gene. We report the anaestheticmanagement of one such patient presenting for coronary arterybypass grafting. Her medication included aspirin 75 mg, atorvastatin20 mg, isosorbide mononitrate 60 mg, atenolol 25 mg and glyceryltrinitrite sublingual spray as required. The use of aprotinin,statins and moderate hypothermia in patients with central coredisease and known susceptibility to MH has not been documented. Br J Anaesth 2003; 91: 744–7     

Anesthesia for sickle cell disease and congenital myopathy in combination

We report on the perioperative management of anesthesia and analgesia in a child with sickle cell disease and a congenital myopathy, presenting for corrective orthopedic surgery. The case illustrates two valuable points of interest: the many benefits of regional anesthesia in complex medical cases and the successful use of tourniquets in children with sickle cell disease.     

Anaesthetic management of a child with a positive family history of malignant hyperthermia for posterior fossa surgery in the sitting position

A 6-year-old boy with a positive family history of malignant hyperthermia presented for posterior fossa craniectomy and excision of medulloblastoma. A nontriggering anaesthetic was therefore planned using infusions of propofol and remifentanil and a vapour free anaesthetic system delivering an oxygen/air mixture. The surgery was carried out with the child in the sitting position.     

全麻复合头部周围神经阻滞对颅脑手术应激反应的影响

目的观察全麻复合头部周围神经阻滞对颅脑手术患者应激反应的影响。方法选择择期幕上颅脑手术患者80例,随机均分为静脉全麻联合头部周围神经阻滞组(S组)和单纯静脉全麻组(C组),每组40例。麻醉诱导插管后S组用0.596%甲璜酸罗哌卡因行眶上神经(2ml)、滑车神经(2ml)、耳颞神经(5ml)、枕大神经(5ml)和枕小神经(5ml)阻滞,对侧头钉所在的位置作相应的区域神经阻滞。C组不行神经阻滞。两组术中TCI丙泊酚血浆浓度3.0～3.5μg/ml和瑞芬太尼血浆浓度2ng/ml维持麻醉。记录诱导前(T0)、诱导后(T1)、上头架(T2)、切皮(T3)、分离骨膜(T4)、去骨瓣(T5)、术中(T6)、缝皮下(T7)、缝皮(T8)的SBP、DBP、HR,以及血浆皮质醇(Cor)、血管紧张素Ⅱ(AⅡ)、血糖(Glu)的变化和麻醉镇痛药的用量。结果与T1时比较,T2～T4、T7、T8时C组SBP、DBP明显升高(P0.05);T2、T8时C组HR明显增快(P0.05);与C组比较,S组SBP、DBP在T2、T7、T8时明显降低,HR明显减慢(P0.05)。T2～T8时C组Glu明显升高(P0.05),T6～T8时S组Glu明显升高(P0.05);T2～T8时两组Cor明显降低(P0.05);T2时C组AⅡ明显升高(P0.05),T2时S组AⅡ明显降低(P0.05);T3～T8时两组AⅡ明显降低(P0.05)。与T6时比较,T7、T8时两组SBP、DBP、Cor、AⅡ、Glu明显升高,HR明显增快(P0.05)。与C组比较,T2、T7、T8时S组SBP、DBP、HR、Cor、AⅡ明显降低(P0.05);T2～T8时S组Glu明显降低(P0.05);瑞芬太尼用量S组明显少于C组(P0.05)。结论头部周围神经阻滞联合全麻对血流动力学和应激反应影响小,并能减少全身麻醉性镇痛药的用量,是颅脑手术比较理想的麻醉方法。     

New trends in regional anesthesia for shoulder surgery: Avoiding devastating complications

Surgeons and patients are often reluctant to support regional anesthesia (RA) for shoulder and other orthopedic surgeries. This is because of the sometimes true but usually incorrectly perceived "slowing down" of operating room turnover time and the perceived potential for added morbidity. Recently, severe devastating and permanent nerve injury complications have surfaced, and this article attempts to clarify the modern place of RA for shoulder surgery and the prevention of these complications. A philosophical approach to anesthesiology and regional anesthesiology is offered, while a fresh appreciation for the well-described and often forgotten microanatomy of the brachial plexus is revisited to explain and avoid some of the devastating complications of RA for shoulder surgery.     

Femoral and lateral femoral cutaneous nerve block for muscle biopsies in children

Retrospective chart review (1978–1993) of 179 children less than age 18 (10.0 ± 3.8 SD yrs) undergoing muscle biopsy for determination of susceptibility to malignant hyperthermia provided data. One hundred and forty-six patients received femoral and lateral femoral cutaneous nerve blocks as their primary anaesthetic. We examined age, weight, duration of surgery, time to discharge from hospital, choice and dosage of local anaesthetics, choice and dosage of sedation, postoperative pain medications, and complications. All children receiving this form of anaesthesia remained outpatients. Between 1978 and 1985 procaine (10 mg·kg^-1) with hyaluronidase or 2-chloroprocaine (12 mg·kg^-1) provided nerve blockade; after 1985, lignocaine (6.8 mg·kg^-1), or a combination of lignocaine or mepivacaine and 2-chloroprocaine, were the preferred agents. More recently the combination of 2-chloroprocaine and bupivacaine has been popular. Three patients required admission to the recovery room postoperatively, due to heavy sedation. Forty-three children (29%) received pain medication during recovery. Femoral and lateral femoral cutaneous block anaesthesia with light to moderate sedation is well tolerated in children undergoing anterior thigh procedures.     

快速康复外科联合腹腔镜技术治疗婴儿先天性巨结肠

目的：评估快速康复外科（FTS）理念联合腹腔镜技术治疗婴儿先天性巨结肠的安全性及临床效果。方法回顾性分析南京医科大学附属南京儿童医院新生儿外科2010年6月至2013年6月接受择期手术治疗的72例年龄为2．5～5．0月的先天性巨结肠患儿的临床资料,根据家长意愿,其中33例予以快速康复外科理念指导下联合腹腔镜手术（FTS组）,39例予以单纯腹腔镜手术（对照组）,比较两组患儿手术以及术后肠功能恢复时间、总住院时间、住院费用和并发症发生率等情况。结果 FTS组与对照组患儿术中出血和手术时间比较,差异无统计学意义（均P＞0．05）。术后肠功能恢复时间FTS组（42±9） d,对照组（46±8） d;虽两组差异无统计学（P＝0．078）,但FTS组要快于对照组。FTS组总住院时间为（10±2） d,住院费用（15316±2273）元,明显低于对照组的（14±4） d和（18641±3082）元（P＜0．01）。随访4周,两组术后并发症发生率和术后恢复情况比较,差异均无统计学意义（P＞0．05）。结论快速康复外科理念联合腹腔镜技术治疗婴儿先天性巨结肠安全、有效。     

Peripheral venous pressure is an alternative to central venous pressure in paediatric surgery patients

BACKGROUND: Peripheral venous pressure (PVP) is easily and safely measured. In adults, PVP correlates closely with central venous pressure (CVP) during major non-cardiac surgery. The objective of this study was to evaluate the agreement between CVP and PVP in children during major surgery and during recovery. METHODS: Fifty patients aged 3-9 years, scheduled for major elective surgery, each underwent simultaneous measurements of CVP and PVP at random points during controlled ventilation intraoperatively (six readings) and during spontaneous ventilation in the post-anaesthesia care unit (three readings). In a subset of four patients, measurements were taken during periods of hypotension and subsequent fluid resuscitation (15 readings from each patient). RESULTS: Peripheral venous pressure was closely correlated to CVP intraoperatively, during controlled ventilation (r=0.93), with a bias of 1.92 (0.47) mmHg (95% confidence interval = 2.16-1.68). In the post-anaesthesia care unit, during spontaneous ventilation, PVP correlated strongly with CVP (r = 0.89), with a bias of 2.45 (0.57) mmHg (95% confidence interval = 2.73-2.17). During periods of intraoperative hypotension and fluid resuscitation, within-patient changes in PVP mirrored changes in CVP (r = 0.92). CONCLUSION: In children undergoing major surgery, PVP showed good agreement with CVP in the perioperative period. As changes in PVP parallel, in direction, changes in CVP, PVP monitoring may offer an alternative to direct CVP measurement for perioperative estimation of volume status and guiding fluid therapy.     

Perioperative truncal peripheral nerve blocks for bariatric surgery: an opioid reduction strategy

BackgroundBariatric surgical patients are vulnerable to cardiopulmonary depressant effects of opioids. The enhanced recovery after surgery (ERAS) protocol to improve postoperative morbidity recommends regional anesthesia for postoperative pain management. However, there is limited evidence that peripheral nerve blocks (PNB) have added benefit.ObjectiveStudy the effect of PNB on postoperative pain and opioid use following bariatric surgery.SettingAcademic medical center, United States.MethodsWe conducted a cohort study of patients who underwent sleeve gastrectomy (SG) or Roux-en-Y gastric bypass (RYGB) surgery. A total of 44 patients received the control ERAS protocol with preoperative oral extended-release morphine sulfate (MS), while 45 patients underwent a PNB with either intrathecal morphine (IM) or oral MS per local ERAS protocol. The PNB group either underwent preoperative bilateral T7 paravertebral (PVT) PNBs (27 patients) with IM or postoperative transversus abdominis plane (TAP) PNBs (18 patients) with oral MS. The primary outcome compared total opioid consumption between the ERAS control group and the PNB group up to 48 hours postoperatively. Secondary outcomes included comparison by block type and postoperative pain scores.ResultsPVT or TAP PNB patients had a reduction in mean postoperative oral morphine equivalent (OME) requirements compared with the ERAS protocol cohort at 24 hours (93.9 versus 42.8 mg), P < .0001; at 48 hours (72.6 versus 40.5 mg); and in pain scores at 24 hours (5.64/10 versus 4.46/10), P = .02. OME and pain scores were higher in the SG cohort.ConclusionAddition of truncal PNB to standard ERAS protocol for bariatric surgical patients reduces postoperative total opioid consumption.     

超声引导下选择性颈神经根阻滞在肩关节镜术后镇痛中的应用

目的比较超声引导下选择性颈神经根阻滞与传统肌间沟臂丛阻滞在肩关节镜术后镇痛的应用效果。方法择期行肩关节镜手术全麻患者70例,男25例,女45例,年龄18~75岁,随机分为两组,选择性颈神经根阻滞组(S组)和传统肌间沟臂丛阻滞组(ISB组),每组35例。S组在超声引导下分别给予C5、C6神经根0.5%罗哌卡因各5ml;ISB组在超声引导下给予0.5%罗哌卡因10ml。记录神经阻滞起效时间;记录术后4、12、24h的VAS评分和前臂(屈肘、屈腕、屈指)MBS运动评分;记录术后24h曲马多用量,以及患者满意度和不良反应发生情况。结果 S组起效时间明显短于ISB组[(8.24±2.96)min vs(13.85±7.45)min,P0.01];S组术后12h的VAS评分明显低于ISB组[(1.7±0.8)分vs(3.6±0.7)分,P0.05],术后4h前臂(屈肘、屈腕、屈指)MBS运动评分明显高于ISB组[(3.5±0.6)分vs(0.8±0.3)分,(3.4±0.5)分vs(0.9±0.4)分,(3.6±0.6)分vs(0.7±0.4)分,P0.01];术后24h曲马多用量明显少于ISB组[(37.5±35.9)mg vs(112.5±43.5)mg,P0.05],患者满意率明显高于ISB组(88%vs 56%,P0.05),术后不良反应两组差异无统计学意义。结论在肩关节镜手术后镇痛中,超声引导下选择性颈神经根阻滞优于臂丛神经阻滞。     

Regional anesthetic techniques are an alternative to general anesthesia for infants with Pompe's disease

Pompe's disease or glycogen storage disease type II is a genetic disorder affecting skeletal and cardiac muscle. The infantile form is associated with gross hypertrophic cardiomegaly and death in the early years. General anesthesia is associated with potential major morbidity in these patients. We present our experience of regional anesthetic blocks used in five patients with the infantile form of glycogen storage disease type II with and without sedation for 11 surgical procedures during a clinical trial of replacement therapy for this condition. Both femoral nerve blockade and caudal epidural blockade were used with good result. The relative merits of the type of block are discussed in addition to the choice of sedation and risks of general anesthesia. The avoidance of general anesthesia in the newly presenting patient with Pompe's disease may reduce potential morbidity until enzyme replacement has been established.     

Factors that predict conversion in patients undergoing laparoscopic surgery for Crohn's disease

BACKGROUND: To identify factors that would predict conversion in patients undergoing laparoscopic surgery for Crohn's disease. METHODS: Data were collected retrospectively. All procedures were performed by an experienced laparoscopic surgeon. RESULTS: Forty-eight patients underwent 57 laparoscopic procedures. Twenty-six of these procedures were for recurrent disease. The conversion rate was 42.3% (11 of 26) for recurrent disease and 13% (4 of 31) for primary disease. On univariate analysis age (P = 0.02), surgery for recurrence (P = 0.02), time from diagnosis (P = 0.03), and the presence of a clinical mass (P = 0.03) were factors that predicted conversion. On multivariate analysis these factors except time from diagnosis still remained significant. Patients who did not undergo a conversion had significantly better post operative outcomes. There was no difference in the incidence of major or minor complications or the number of resections in both groups. CONCLUSIONS: The conversion rate for the whole group was 28%. The rate is higher in patients who are over 40 years of age who are undergoing procedures for recurrence.     

超声引导下神经阻滞技术用于患儿腹部手术后镇痛的进展

区域神经阻滞是多模式镇痛的基础方案。与静脉镇痛比较,区域神经阻滞具有更强的针对性和有效性,神经阻滞麻醉镇痛可有效减少阿片类药物使用带来的不良反应。联合应用超声引导技术,能够提高神经阻滞的安全性和精确性。患儿腹部神经阻滞解剖结构以及局麻药物扩散途径、临床应用以及并发症的报道较少,药物浓度与剂量缺乏统一标准,需要进一步的临床研究。本文分别从患儿腰方肌阻滞（QLB）、腹横肌平面阻滞（TAPB）、腹直肌鞘阻滞（RSB）、竖脊肌平面阻滞（ESPB）和椎旁阻滞（PVB）的局麻药物扩散途径、浓度剂量、临床应用及相关并发症等方面进行综述,以期为患儿术后镇痛提供参考。     

胸椎旁神经阻滞与多点肋缘下腹横肌平面阻滞在开腹肝脏手术术后镇痛的应用比较

目的:探讨胸椎旁神经阻滞(thoracic paravertebral nerve block,TPVB)与多点肋缘下腹横肌平面阻滞(transverse abdominal plane block,TAPB)在开腹肝脏手术术后镇痛中的应用价值。方法:拟行开腹肝脏手术的患者60例(手术切口均为经典反"L"型切口),ASA分级Ⅰ~Ⅲ级,采用随机数字表法分为TPVB组(TP组)和多点肋缘下TAPB组(TA组),每组30例。两组均在术前行超声引导下神经阻滞,TP组行双侧T 7-T 8和T 8-T 9 TPVB,TA组行双侧肋缘下和经典肋缘与髂前上棘之间的TAPB,共4个阻滞点。记录并比较两组阻滞操作时间,记录切皮前(T 0)、切皮后(T 1)、上腔静脉阻断时(T 2)、肝切除时(T 3)、缝皮时(T 4)的MAP和心率,记录手术时间、术中液体入量、瑞芬太尼消耗量、术后PACU停留时间,记录术后即刻(患者拔管苏醒后,可进行正常语言交流)、术后2h、术后6h的静息及运动VAS评分,记录术后6h内给予阿片类药物的例数、术后第1次给予阿片类药物的时间。结果:TA组的阻滞操作时间短于TP组(P<0.05)。两组患者T 0时MAP和心率差异没有统计学意义(P>0.05),但在T 1、T 3、T 4时TP组的MAP和心率均低于TA组(P<0.05)。TP组术中瑞芬太尼消耗量、术后PACU停留时间、术后即刻和术后2h的静息及运动VAS评分也明显少于TA组(P<0.05),但术后第1次给予阿片类药物的时间,TP组长于TA组(P<0.05)。其余观察指标两组差异无统计学意义(P>0.05)。结论:在开腹肝脏手术中,TPVB较多点肋缘下TAPB能提供更加平稳的血流动力学变化,减少阿片类药物的用量,提供更完善的围手术期镇痛。