Case Report: Anesthetic Management and Electrical Cardiometry as Intensive Hemodynamic Monitoring During Cheiloplasty in an Infant With Enzyme-Replaced Pompe Disease and Preserved Preoperative Cardiac Function

Introduction: Pompe disease is caused by deficiency of the lysosomal enzyme acid α-glucosidase, which results in cardiac and muscular complications that can jeopardize perioperative outcomes. We report a 4-month-old infant with Pompe disease receiving cheiloplasty under general anesthesia with the aid of peripheral nerve blocks and intensive hemodynamic monitoring.Case Description: This case report describes a 4-month-old full-term Taiwanese female infant who presented with left unilateral cleft lip and palate in the prenatal examination. She was diagnosed with infantile-onset Pompe disease after acidic α-glucosidase (GAA) gene sequencing. She also received enzyme replacement therapy (ERT) 15 days after birth and regular ERT every other week. Cheiloplasty was performed under general anesthesia uneventfully, and peripheral nerve blocks were adopted for analgesia. Intensive hemodynamic monitoring using electrical cardiometry technology (ICON®) and pulse contour analysis (FloTrac system) were applied during the operation. No adverse effects were observed, and the wound healed well. Therefore, the patient was discharged 4 days after surgery.Conclusion: With the availability of ERT, severe organ dysfunction in infantile-onset Pompe disease patients is no longer common. However, moderate cardiac depression can still occur while increasing inspiratory pressure and deepening the anesthesia level despite a normal preoperative echocardiogram report. Therefore, careful, gradual titration is desirable. Furthermore, electrical cardiometry can detect hemodynamic changes more instantaneously and reliably than pulse contour analysis. In addition, we suggest taking advantage of the peripheral nerve block as a part of balanced anesthesia to alleviate the cardiac suppression caused by general anesthesia.

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Interpretation of Noninvasive and Invasive Information

10.2310/7ccsp.8000 ◽

2017 ◽

Author(s):

Borzoo Farhang ◽

Erik P Anderson ◽

Mark P Hamlin

Keyword(s):

Critically Ill ◽

Hemodynamic Monitoring ◽

Critically Ill Patients ◽

Pulse Contour Analysis ◽

Pulse Contour ◽

Response To Therapy ◽

Contour Analysis ◽

Central Venous ◽

Arterial Blood ◽

Pulmonary Arterial

Traditional, static measures of resuscitation, such as vital signs, central venous pressure, and pulmonary arterial pressure, provide momentary glimpses evolving hemodynamic states. In patients with shock, these measures of resuscitation are poor indicators of response to therapy. As a result, dynamic assessments of cardiovascular status are now used in critically ill patients to facilitate resuscitation. Some of these approaches focus on fluid responsiveness. These assessments allow care to be tailored to each patient’s response to interventions. An evolving aspect of hemodynamic monitoring is evaluation of the adequacy of tissue perfusion and oxygen delivery. In this review, we consider the use of arterial, central venous, and pulmonary arterial blood pressure monitoring; echocardiography; transesophageal Doppler technology; pulse contour analysis; bioimpedance and bioreactance; and partial rebreathing monitoring modalities to assess hemodynamic status in critically ill patients. This review contains 22 figures, 5 tables, and 38 references. Key words: echocardiography, esophageal Doppler technology, invasive and noninvasive hemodynamic monitoring, pulse contour analysis, shock

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Hemodynamic monitoring by pulse contour analysis in critically ill children with congenital heart disease

Pediatric Critical Care Medicine ◽

10.1097/pcc.0b013e318220a405 ◽

2011 ◽

Vol 12 (5) ◽

pp. 609-610

Author(s):

Francois Proulx ◽

Joris Lemson ◽

Ghassan Choker ◽

Shane M. Tibby

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Critically Ill ◽

Hemodynamic Monitoring ◽

Congenital Heart ◽

Pulse Contour Analysis ◽

Pulse Contour ◽

Critically Ill Children ◽

Contour Analysis

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Comparative outcomes of peripheral nerve blocks versus general anesthesia for hip fractures in geriatric Chinese patients

Patient Preference and Adherence ◽

10.2147/ppa.s61903 ◽

2014 ◽

pp. 651

Author(s):

Hong Zhang ◽

Gong MaoWei ◽

Wang XiaoLin ◽

Mai HaiXing ◽

Shujun Pei ◽

...

Keyword(s):

Peripheral Nerve ◽

General Anesthesia ◽

Hip Fractures ◽

Chinese Patients ◽

Nerve Blocks ◽

Peripheral Nerve Blocks

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The Relationship Between Current Intensity for Nerve Stimulation and Success of Peripheral Nerve Blocks Performed in Pediatric Patients Under General Anesthesia

Survey of Anesthesiology ◽

10.1097/sa.0b013e31817c5b7e ◽

2008 ◽

Vol 52 (4) ◽

pp. 177

Author(s):

&NA;

Keyword(s):

Peripheral Nerve ◽

General Anesthesia ◽

Nerve Stimulation ◽

Pediatric Patients ◽

Current Intensity ◽

Nerve Blocks ◽

Peripheral Nerve Blocks ◽

The Relationship

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Peripheral nerve blocks in pediatric anesthesia

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh0910497n ◽

2009 ◽

Vol 137 (9-10) ◽

pp. 497-501

Author(s):

Dejan Novakovic ◽

Ivana Budic ◽

Dusica Simic ◽

Nina Djordjevic ◽

Andjelka Slavkovic ◽

...

Keyword(s):

Peripheral Nerve ◽

General Anesthesia ◽

Age Groups ◽

Pediatric Anesthesia ◽

Anesthetic Techniques ◽

Older Children ◽

Nerve Blocks ◽

Patient Age ◽

Peripheral Nerve Blocks ◽

Patient Âgé

Introduction Most children undergoing surgery can benefit from regional anesthetic techniques, either as the sole anesthetic regimen or, as usual in pediatric practice, in combination with general anesthesia. The use of peripheral nerve blocks (PNBs) in pediatric anesthesia is an effective way to decrease the side-effects and complications associated with central blocks. In spite of their many advantages, including easy performance end efficacy, peripheral nerve blocks are still underused. Objective This article discusses a general approach to PNBs in children and provides data concerning the practice of this regional technique in different age groups. Methods Data from 1,650 procedures were prospectively collected during the period from March 1, 2007 to February 29, 2008. The type of PNB, if any, as well as the patient age were noted. Our patients were divided into four groups: 0-3 years, 4-7 years, 8-12 years and 13-18 years. Results During the investigated period, PNBs as a sole technique or in anesthetized children were performed in 7.45% of cases. Ilioingunal/iliohypogastric nerve block and penile block were the most common (70% of all PNBs) distributed mainly among the children between 4-7 years of age (p<0.05). In older children, extremity PNBs predominate in regard to other types of blocks. PNBs are most frequently performed under general anesthesia (85%), so the perineural approach requires a safe technique to avoid nerve damage. Conclusion The observed differences in PNB usage seem to be related to patient age and correlate with common pathology and also with technical availability of PNB performance.

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Hemodynamic monitoring by pulse contour analysis during trans-catheter aortic valve replacement: A fast and easy method to optimize procedure results

Cardiovascular Revascularization Medicine ◽

10.1016/j.carrev.2018.07.015 ◽

2019 ◽

Vol 20 (4) ◽

pp. 332-337 ◽

Cited By ~ 2

Author(s):

Francesca Ristalli ◽

Salvatore Mario Romano ◽

Miroslava Stolcova ◽

Francesco Meucci ◽

Giovanni Squillantini ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Hemodynamic Monitoring ◽

Pulse Contour Analysis ◽

Pulse Contour ◽

Contour Analysis ◽

Easy Method

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Efficiency of ultrasound guided lower limb peripheral nerve blocks in perioperative pain management for knee arthroscopy in children: A randomized study

Medicinski pregled ◽

10.2298/mpns1602005m ◽

2016 ◽

Vol 69 (1-2) ◽

pp. 5-10

Author(s):

Dragan Marinkovic ◽

Jovana Simin ◽

Biljana Draskovic ◽

Ivana Kvrgic ◽

Marina Pandurov

Keyword(s):

Postoperative Pain ◽

Peripheral Nerve ◽

General Anesthesia ◽

Lower Limb ◽

Knee Arthroscopy ◽

Opioid Analgesics ◽

Ultrasound Guided ◽

Nerve Blocks ◽

Peripheral Nerve Blocks ◽

Group B

Introduction. Ultrasound guided lower limb peripheral nerve blocks are efficient for perioperative pain treatment in children. The aim was to see if lower limb peripheral nerve blocks reduced the amount of propofol and opioid analgesics used intraoperatively, as well as the level of pain and consumption of systemic analgesics postoperatively. Material and Methods. A randomized, prospective clinical trial was carried out. It included 60 children between 11 and 18 years of age scheduled for elective knee arthroscopy. The patients were divided into two groups. Group A received general anesthesia, group B received lower limb peripheral nerve blocks with sedation or general anesthesia. Postoperative level of pain was assessed using visual analogue scale. Results. Less propofol and fentanyl was used to induce and maintain anesthesia in group B (p<0.001). The level of postoperative pain was significantly lower in group B (p<0.001), as well as the postoperative consumption of analgesics (p<0.001). As mahbny as 47% of the patients were discharged without receiving any analgesics postoperatively. The average duration of peripheral nerve blocks was 468 minutes. Conclusions. Ultrasound guided lower limb peripheral nerve blocks are an efficient technique of regional anesthesia in children. They reduce the amount of general anesthetics and opioid analgesics needed intraoperatively as well as the level of postoperative pain and consumption of analgesics postoperatively.

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