Spinal anesthesia for a patient with type I sialidosis undergoing abdominal surgery

Type I and II sialidosis are autosomal recessively inherited glycoprotein storage disorders. Until now, there has been no published reports of patients with these conditions requiring anesthesia. We present the case of a 31-year-old male afflicted with type I sialidosis who underwent a surgical jejunostomy. Regional (spinal) anesthesia was carried out uneventfully. We discuss the anesthetic challenges posed by patients with type I and II sialidosis. Airway assessment and management is particularly crucial.     

Obstetric anesthesia management of the patient with cardiac disease

Cardiovascular disease is the leading cause of maternal mortality in much of the developed world. Risk stratification models can predict which patients are at greatest risk for maternal or fetal morbidity or mortality. Particular cardiac diseases hold significant risk of mortality during pregnancy including pulmonary hypertension, aortic aneurysm, left-ventricular outflow tract obstruction, and severe cardiomyopathy. High-risk patients should deliver at high-resource hospitals under the care of experts in cardiology, obstetrics, perinatology, neonatology and anesthesiology. The obstetric anesthesiologist should formulate delivery plans for cardiac monitoring, labor analgesia, cesarean anesthesia, postpartum monitoring, as well as plans for obstetric or cardiac emergencies. Carefully co-ordinated multidisciplinary care of pregnant women with cardiac disease can result in successful outcomes.     

Bone ultrasonometry, bone density, and turnover markers in type 1 Gaucher disease

In 12 patients (mean age, 33 ± 13 years) with type 1 Gaucher disease (GD), we evaluated bone mass by broadband ultrasound attenuation (BUA) of the calcaneus and dual X-ray absorptiometry (DXA) of the total body, lumbar spine, and hip. In all patients, we measured serum levels of osteocalcin (OC) and bone-specific alkaline phosphatase (BAP) and urinary excretion of pyridinoline (Pyr/Cr) and deoxypyridinoline (D-Pyr/Cr) cross-links. Compared to age- and sex-matched healthy controls, patients with GD showed marked osteopenia at all measuring sites as expected. Values of BUA (67.25 ± 15.83 dB/MHz) were also significantly reduced. OC and BAP concentrations were within the normal range. Pyr/Cr and D-Pyr/Cr were significantly higher than in controls. Calculating T- and Z scores, we found a significant correlation between the Bone Severity Score Index (BSSI) and both BUA and BMD measurements. A significant correlation was also found between pyridinoline urinary excretion and both BSSI and BUA at the calcaneus. Our data suggest that type 1 GD in adulthood is associated with increased bone resorption and that BUA at the calcaneus may be a relevant tool in the assessment of bone status in these patients. Received: April 2, 2001 / Accepted: August 6, 2001     

Prevalence of autoantibodies in the course of Gaucher disease type 1: A multicenter study comparing Gaucher disease patients to healthy subjects

Objectives

Type 1 Gaucher disease may be related to the presence of autoantibodies. Their clinical significance is questioned.Primary endpoint was to compare the prevalence of autoantibodies in type 1 Gaucher disease patients with healthy subjects, seeking correlations with autoimmune characteristics. Secondary endpoints were to determine whether patients with autoantibodies reported autoimmunity-related symptoms and if genotype, splenectomy or treatment influenced autoantibodies presence.

Subarachnoid anesthesia in young patients: a comparative analysis of two needle bevels

BACKGROUND AND OBJECTIVES: This prospective, randomized, double-blind study compares the efficacy of two spinal needles in terms of their performance characteristics and associated perioperative complaints in young patients. METHODS: ASA I and II patients aged from 20 to 40 years undergoing lower limb orthopedic surgery were included during a 12-month period. After application of the protocol, 158 patients were recruited. The patients were randomized to two groups: group I: 26-gauge Atraucan (n = 79) and Group II: 27-gauge Whitacre (n = 79). A study was made of the demographic parameters, technical characteristics, and peri- and postoperative complications. RESULTS: No significant differences were found in the technical handling of the needles, number of attempts made to achieve the puncture, or the time required to perform the technique. No technical failures (spinal anesthesia inadequate for the planned surgery) were reported, and the frequency of complications during the procedure was identical in both groups. Frequency of postdural puncture headache (3.8%) or severity and duration showed no difference between the two groups. The overall assessment of postoperative complications revealed similar scores for both needles. CONCLUSIONS: Technical handling (ease with which block was performed) of the needles analyzed was extremely easy, showing a high success rate which associated with the similar incidence of complications. Type of bevel does not appear to be a determining factor in the quality or morbidity associated with subarachnoid block when fine-gauge needles are used in young patients.     

Anesthesia for obstetric patients with Gaucher disease: survey and review

Pregnancy and delivery in patients with non-neuronopathic Gaucher disease, whether treated with enzyme replacement or untreated, are usually uncomplicated. Various factors may influence mode of delivery, vaginal or cesarean section, as well as type of anesthesia, general or regional, used during delivery. This retrospective review was intended to highlight some of the practical issues relating to obstetric anesthetic management, based on a review of the literature and experiences from a large referral clinic for Gaucher disease. In the past decade, there were 16 deliveries in 11 women in our institution. There were five normal vaginal deliveries, two vacuum extractions, one placental extraction, and eight cesarean sections. Platelet counts were 27-215 x 10(9)/L. Two spontaneous deliveries and one vacuum extraction were performed under epidural anesthesia; two other women having vaginal deliveries and one vacuum extraction were given i.v. analgesia; the fifth was given i.v. patient-controlled analgesia. The placental extraction was performed under general anesthesia. Seven of the women having cesarean deliveries received spinal anesthesia; the breech presentation required general anesthesia. There were no anesthesia-related side effects or complications, although there were some instances of post-partum bleeding irrespective of enzyme therapy. Gaucher disease affects multiple organs and can be a challenge to the anesthesiologist. Based on this survey we suggest that anesthetic management requires particular attention to hematological parameters before delivery. A multidisciplinary approach and extensive communication among obstetrician, hematologist and anesthesiologist is required to anticipate the possibility of post-partum hemorrhage, and preclude skeletal damage.     

Bone density changes with enzyme therapy for Gaucher disease

Gaucher disease is the most common lysosomal storage disease. Enzyme replacement therapy engenders improvement in hematological and visceral parameters; however, improvement in bone density (BMD) with treatment has not been confirmed. This study presents follow-up of BMD in the first ten patients in Israel treated with low-dose recombinant enzyme for up to 108 months. BMD at femoral neck and lumbar spine was determined by dual-energy X-ray absorptiometry (DEXA) at the start of the trial, after 3–6 months, after 18–24 months, and at the most recent follow-up. BMD in all patients was very low at onset and never normalized. There was a decrease in BMD in all patients at 3–6 months. Older patients (four women, two men; >30 years of age) showed some improvement in BMD during treatment. Younger patients (four females; 18–23 years of age) did not show a statistically significant improvement. These findings might reflect the failure of patients with Gaucher disease to achieve expected peak bone density at appropriate chronological milestones despite treatment. Nonetheless, the z-scores of the older patients were better than those of the younger patients, implying some catch-up period. Yet, some patients with Gaucher disease evince rapid onset of osteoporosis in early adulthood. Enzyme treatment per se, as well as attendant improved well-being and increased physical activity, may induce amelioration in BMD at this later stage. One may consider adding anti-osteoporosis therapy in young adults to induce earlier catch up to peak bone mass, and then enzyme replacement in later adulthood to prevent decrements in bone mass related to Gaucher cell infiltration.     

Hemodynamic changes during spinal anesthesia in premature infants with congenital heart disease undergoing inguinal hernia correction

Introduction: There are conflicting reports on the effects of spinal anesthesia (SA) on hemodynamics. Data on the hemodynamic effects of SA in infants with congenital heart disease (CHD) are limited. Methods: We reviewed our experience with 44 unsupplemented SA with 1 mg·kg^?1 of either hyperbaric tetracaine or bupivacaine in premature and former premature infants with noncyanotic CHD. Hemodynamics and oxyhemoglobin saturation (SpO₂) were assessed. Neither preoperative fluid boluses nor atropine was administered to any of the infants. Results: There was no significant change in systolic, diastolic, or mean blood pressures from pre‐SA induction compared with end of surgery. Heart rate showed a small but systematic decline (mean change of 10 beats per minute, P < 0.01) but was within the normative range values for age. There was a small, but clinically insignificant increase in SpO₂ across the time course. Intraoperatively, two infants developed transient apneic spells. No infant developed postoperative apnea, oxygen desaturation, or bradycardia. Conclusions: The data suggest that SA with 1 mg·kg^?1 of either hyperbaric tetracaine or bupivacaine can be used safely as the sole anesthetic for inguinal hernia repair in infants with noncyanotic CHD even when fluid restricted and apparently causes minimal respiratory complications in these infants.     

Subarachnoid anesthesia for cesarean section in a patient with Ehlers-Danlos syndrome type II

A 32 year-old woman with full-term pregnancy and a history of Ehlers-Danlos syndrome type II underwent cesarean delivery owing to breech presentation and the presence of oligohydramnios. Anesthesia was provided with a subarachnoid block with 6.5 mg of bupivacaine and 32.5 microg of fentanyl to T4. Depth of anesthesia was adequate and hemodynamic stability was good. The intervention was uneventful and the outcomes were excellent for both mother and fetus. A subarachnoid block with low doses of local anesthetic and fentanyl is an appropriate, safe alternative to general anesthesia for cesarean section in women with Ehlers-Danlos who have normal coagulation tests and no history of bleeding.     

罗哌卡因复合舒芬太尼连续蛛网膜下腔阻滞用于全产程分娩镇痛的临床效果

目的观察罗哌卡因复合舒芬太尼连续蛛网膜下腔阻滞用于全产程分娩镇痛的效果。方法选择2018年3-10月在北京妇产医院全产程分娩镇痛的足月妊娠单胎头位初产妇97例,年龄23~35岁,体重60~90 kg,ASAⅠ或Ⅱ级,采用随机数字表法分为两组:连续蛛网膜下腔阻滞组(CSA组,n=48)和腰-硬联合阻滞组(CSEA组,n=49)。产妇子宫规律收缩后行分娩镇痛,CSA组经蛛网膜下腔推注罗哌卡因0.3 mg/ml+舒芬太尼1μg/ml共5 ml,20 min后连接镇痛泵;CSEA组经蛛网膜下腔推注罗哌卡因0.3 mg/ml+舒芬太尼1μg/ml共5 ml,20 min后连接硬膜外镇痛泵。所有产妇持续应用镇痛泵至第三产程结束。记录镇痛即刻(T1)、镇痛后10 min(T2)、30 min(T3)、60 min(T4)、宫口开全(T5)、胎头娩出(T6)、胎盘娩出(T7)时的MAP和VAS疼痛评分;记录镇痛前、镇痛后0~30 min、30~60 min、90~120 min和第二产程的宫缩持续时间和宫缩间隔时间;记录缩宫素使用情况和分娩方式;记录恶心呕吐、瘙痒、产后出血和硬脊膜穿刺后头痛(PDPH)的发生情况;记录新生儿体重、出生后1、5、10 min Apgar评分和脐带动脉血血气分析。结果与CSEA组比较,T2、T5、T6时CSA组MAP和VAS疼痛评分明显降低(P<0.05);镇痛后0~30 min CSA组宫缩持续时间明显缩短(P<0.05),宫缩间隔时间明显延长(P<0.05)。CSA组缩宫素使用率、瘙痒发生率明显高于CSEA组(P<0.05)。两组分娩方式以及恶心呕吐、产后出血、PDPH发生率差异无统计学意义。两组新生儿体重、出生后1、5、10 min Apgar评分和脐带动脉血血气分析差异无统计学意义。结论与腰-硬联合阻滞比较,连续蛛网膜下腔阻滞具有用药量小的特点,对产妇、胎儿影响较小,可安全用于全产程分娩镇痛。     

Continuous spinal anesthesia/analgesia vs. single-shot spinal anesthesia with patient-controlled analgesia for elective hip arthroplasty

BACKGROUND: In total hip replacement surgery several anesthesiological techniques can be used. In this study we compared continuous spinal anesthesia (CSA) and postoperative analgesia vs. single-shot spinal anesthesia (SPA) and postoperative patient-controlled intravenous analgesia with morphine (SPA). METHODS: In a prospective randomized study, 68 patients, ASA I-III, between 50 and 85 years of age were allocated to these two groups. Quality of analgesia, hemodynamic stability and technical difficulties, as well as incidence of postoperative nausea and vomiting (PONV) and post dural puncture headache (PDPH), were recorded during a 24-h period. RESULTS: Visual analog scale (VAS) scores were significantly lower in the CSA group from 3 h post operation (P < 0.05). Mean arterial pressure dropped by 21 +/- 11 mmHg in the CSA group and 29 +/- 14 in the SPA group during induction (P < 0.05). Technical difficulties and incidence of PDPH were similar in both groups. Postoperative nausea and vomiting was lower in the CSA group (P < 0.05). CONCLUSIONS: Continuous spinal anesthesia/analgesia is a very practicable method providing better postoperative analgesia and better hemodynamic stability during anesthesia induction than SPA followed by morphine PCA analgesia after total hip replacement surgery.     

Need for additional anesthesia after single injection spinal analgesia for labor: a retrospective cohort study

BackgroundThere is little information about the use and efficacy of single injection spinal blocks for labor analgesia; specifically, how frequently subsequent analgesia or anesthesia is needed. This study determined how frequently an additional anesthetic intervention was needed in women who received single injection spinal analgesia.MethodsThis retrospective study examined electronic medical records to find all single injection spinal analgesic blocks for labor analgesia over a 14-year (2003–2016) period. Patient and block characteristics and patient outcomes were recorded. The primary outcome was need for an additional anesthetic intervention following single injection spinal for labor analgesia.ResultsFour-hundred-and-twenty-eight patients received single injection spinal blocks for labor and 60 (14.0%) needed an additional anesthetic either for labor analgesia (n=49) or an unexpected procedure (n=11). Two of these (0.5%) required general anesthesia. Parity of zero (nulliparous), a low cervical dilation at the time of the spinal injection, and induction of labor status, were associated with an increased risk of needing an additional anesthetic intervention.ConclusionsThis retrospective review provides evidence that single injection spinal anesthesia may be used for multiparous women with spontaneous labor and more advanced cervical dilation.     

Outpatient anesthesia for oral surgery in a juvenile with Leigh disease

We report a case of anesthesia for elective outpatient third molar extraction in a juvenile with Leigh disease, a progressive neurodegenerative disorder related to respiratory chain deficiency. This syndrome usually presents in infancy and is characterized by nervous system dysfunction and respiratory abnormalities. Anesthesia has been reported to aggravate respiratory symptoms and frequently precipitate respiratory failure. Preoperative swallowing difficulty or respiratory symptoms should be carefully diagnosed, because they can be a warning sign of postoperative complications or mortality. Adverse effects of anesthesia may quickly lead into metabolic acidosis. Anesthetics should be carefully chosen that do not interfere with mitochondrial respiration, which can lead to lactic acidosis.     

罗哌卡因经Spinocath导管行连续腰麻及PCA用于下腹部术的临床观察

目的观察罗哌卡因经Spinocath导管行连续腰麻(CSA)及术后自控镇痛(PCA)应用于下腹部手术的安全性和有效性。方法50例ASAⅠ-Ⅱ级拟行下腹部手术的患者,于L2～3间隙穿刺置入24GSpinocath连续腰麻导管,分次注入0.75%罗哌卡因2.5ml。术后PCA随机分为三组,A组(n=16):0.15%罗哌卡因;B组(n=16):0.125%罗哌卡因;C组(n=18):0.125%罗哌卡因 0.0002%芬太尼,容量均为60ml,三组背景输注速度1ml/h,PCA单次剂量0.5ml,时间锁定30min。观察术中HR、MAP、SpO2的变化,最高阻滞平面,运动阻滞程度(改良Bromage分级法),麻醉效果分级,PCA镇痛效果(视觉模拟评分法VAS)及不良反应和并发症。结果所有患者HR、MAP、SpO2均在正常范围内。最高阻滞平面为T7.2±0.9,改良Bromage评级均为Ⅲ级,麻醉效果满意率为96%。VAS评分A、C两组明显低于B组(P<0.01)。A组运动阻滞恢复时间较B、C两组明显延长(P<0.01)。结论下腹部手术中,采用罗哌卡因经Spinocath导管行连续腰麻及PCA,安全有效,具有可行性。     

Moyamoya disease and anesthesia

Moyamoya disease is a condition that results from bilateral stenosis or obstruction of the intracranial arteries at the base of the brain. Patients exhibit ischemic symptoms, and vascular reconstruction is the therapy of choice. Surgical treatment for Moyamoya disease is often complicated by cerebral ischemia, so the goal in perioperative management is to maintain the balance between oxygen supply and demand in the brain. This report presents three cases of Moyamoya disease in patients under 3 years of age, and discusses anesthesia management issues for pediatric patients with this condition.     

Treatment with desmopressin before epidural anesthesia in a patient with type I von Willebrand disease

A 33-year-old primipara with von Willebrand disease type I was admitted in labor at 37 weeks, requesting epidural analgesia. The consultant hematologist advised treating with desmopressin acetate (DDAVP) before inserting an epidural catheter. Desmopressin at a dose of 0.3 microgram/Kg was administered intravenously and the catheter was inserted to L3-L4 to infuse 0.1% bupivacaine with 2 micrograms/mL of fentanyl at a rate of 12 mL/h. Four hours later the patient was brought to the operating room for forceps delivery of a healthy boy. One hour later, she had recovered normal motor tone followed by normal sensitivity in the lower extremities. The catheter was then withdrawn with no signs of bleeding. A woman with von Willebrand's disease can receive an epidural block for analgesia during childbirth. The decision to perform the block should be individualized, based on coagulation tests. DDAVP may play a role in improving hemostasis.