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.

Peripheral nerve blocks in advanced cancer pain: retrospective case series

ObjectivesPatients with cancer often suffer severe pain that is not relieved with systemic analgesics and requires further treatment options. This study aims to investigate whether peripheral nerve blocks are a feasible treatment option in patients with incurable cancer who suffer from severe pain.MethodsAll patients with advanced cancer who received a peripheral nerve block for the management of pain at the Tampere University Hospital between January 2015 and December 2018 were included in this retrospective study. The characteristics of the patients’ features of the nerve blocks, opioid dosing (daily morphine equivalent) before and after the blocks, and patient-reported pain relief following peripheral block were assessed from the medical records.ResultsSixteen of the 17 patients included in this study received pain relief through a nerve block. Daily opioid dose was decreased with the block in 12 (71%) patients with a median change in daily morphine equivalent of −20 mg (IQR: −180 to 9). One infection of the catheter and two other transient adverse events occurred, but none was serious or fatal.ConclusionsPeripheral nerve blocks seem safe and may provide considerable analgesia and decrease the need for opioids in patients with advanced cancer.