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Background: Inadequately controlled postprocedural pain following sclerotherapy in patients with vascular malformations is a well recognised problem. Reliable epidemiological data and risk factors associated with postprocedural pain in children are lacking., Objectives: To evaluate and quantify postprocedural pain in children and identify possible risk factors based on characteristics of the patient, treatment, and medication., Design: A retrospective single centre cohort study., Setting: A tertiary single centre study in the Netherlands., Patients: Two hundred and nine chiuldren with 'simple' subtypes of congenital vascular malformation who had undergone sclerotherapy., Primary Outcome Measure: Quantifying inadequately controlled postprocedural pain., Secondary Outcome Measures: Identifying potential patient and treatment characteristics associated with inadequately controlled postprocedural pain., Results: A total of 209 patients who underwent 679 procedures were included in this study. The mean age at first intervention was 11.8 ± 4.5 years. Inadequately controlled postprocedural pain was found in 34.8% of the 679 procedures. Venous malformations (VM) were the most prevalent subtype of vascular malformation (80%), followed by arteriovenous malformations (AVM) (14.6%) and lymphatic malformations (LM) (5.4%). The odds ratio (OR) (95% confidence intervals), and P values obtained from multivariable mixed effect logistic regression analysis for patient and treatment characteristics found to be associated with inadequately controlled postprocedural pain were: chronic use of analgesics (OR 2.74 (1.40 to 5.34), P  = 0.003), treatment with ethanol (OR 2.39 (1.01 to 5.65, P  = 0.05) or esketamine (OR 7.43 (1.32 to 41.81), P  = 0.02). Patients treated with lauromacrogol (OR 0.42 (0.22 to 0.82, P  = 0.01) and patients receiving intra-operative NSAIDs (OR 0.32, (0.12 to 0.85), P  = 0.02) were less likely to experience inadequately controlled postprocedural pain., Conclusions: Despite aiming to achieve best practice, the 34.8% incidence of unsatisfactory postoperative pain management in the children studied confirms that postprocedural pain after sclerotherapy is a common problem that requires further attention., Competing Interests: Conflicts of interest: none., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society of Anaesthesiology and Intensive Care.)

Background: The noninvasive TensorTip™ MTX measures blood pressure by interpreting blood diffusion color of the finger skin. In addition to blood pressure, the device is able to measure various vital signs: heart rate, oxygen saturation, stroke volume, and cardiac output. Studies about accuracy and precision thus far available have only been conducted by the manufacturer. The aim of our study was to investigate the accuracy and precision of the TensorTip MTX in comparison to invasive radial artery blood pressure values., Methods: Forty-one patients scheduled for elective surgery were enrolled in this study. Placement of the arterial catheter had to be part of the standard of care. Once hemodynamic stable conditions were achieved, blood pressure was measured. Three measurements with the TensorTip MTX were averaged and compared with one invasive blood pressure measurement using Bland-Altman plot and error grid analysis., Results: Systolic, diastolic, and mean blood pressure had a bias of respectively 6.2, -6.9 and 4.4 mm Hg. Corresponding standard deviation were respectively 30.1, 17.0 and 22.2. Calculated percentage errors were 47.6%, 52.9% and 52.3%. Proportional bias was present in all Bland-Altman analyses. Error grid analysis showed 61.0% of systolic blood pressure measurements, and 46.3% of mean blood pressure measurements were in the clinical acceptable zone., Conclusions: The TensorTip MTX was not able to reliably measure blood pressure compared to blood pressure obtained with an arterial catheter and therefore, the measurement performance is not clinically acceptable. Moreover, a high malfunction rate makes the device unsuitable for use in perioperative period., Competing Interests: Parts of this study were presented at the ESAIC conference 2022., (Copyright 2022, Servaas et al.)

Malignant hyperthermia and exertional rhabdomyolysis have conventionally been considered episodic phenotypes that occur in otherwise healthy individuals in response to an external trigger. However, recent studies have demonstrated a clinical and histopathological continuum between patients with a history of malignant hyperthermia susceptibility and/or exertional rhabdomyolysis and RYR1 -related congenital myopathies. We hypothesize that patients with a history of RYR1 -related exertional rhabdomyolysis or malignant hyperthermia susceptibility do have permanent neuromuscular symptoms between malignant hyperthermia or exertional rhabdomyolysis episodes. We performed a prospective cross-sectional observational clinical study of neuromuscular features in patients with a history of RYR1 -related exertional rhabdomyolysis and/or malignant hyperthermia susceptibility ( n = 40) compared with healthy controls ( n = 80). Patients with an RYR1 -related congenital myopathy, manifesting as muscle weakness preceding other symptoms as well as other (neuromuscular) diseases resulting in muscle weakness were excluded. Study procedures included a standardized history of neuromuscular symptoms, a review of all relevant ancillary diagnostic tests performed up to the point of inclusion and a comprehensive, standardized neuromuscular assessment. Results of the standardized neuromuscular history were compared with healthy controls. Results of the neuromuscular assessment were compared with validated reference values. The proportion of patients suffering from cramps ( P < 0.001), myalgia ( P < 0.001) and exertional myalgia ( P < 0.001) was higher compared with healthy controls. Healthcare professionals were consulted because of apparent neuromuscular symptoms by 17/40 (42.5%) patients and 7/80 (8.8%) healthy controls ( P < 0.001). Apart from elevated creatine kinase levels in 19/40 (47.5%) patients and mild abnormalities on muscle biopsies identified in 13/16 (81.3%), ancillary investigations were normal in most patients. The Medical Research Council sum score, spirometry and results of functional measurements were also mostly normal. Three of 40 patients (7.5%) suffered from late-onset muscle weakness, most prominent in the proximal lower extremity muscles. Patients with RYR1 variants resulting in malignant hyperthermia susceptibility and/or exertional rhabdomyolysis frequently report additional neuromuscular symptoms such as myalgia and muscle cramps compared with healthy controls. These symptoms result in frequent consultation of healthcare professionals and sometimes in unnecessary invasive diagnostic procedures. Most patients do have normal strength at a younger age but may develop muscle weakness later in life., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Introduction: Malignant hyperthermia (MH) and exertional rhabdomyolysis (ERM) have long been considered episodic phenotypes occurring in response to external triggers in otherwise healthy individuals with variants in RYR1. However, recent studies have demonstrated a clinical and histopathological continuum between patients with RYR1-related congenital myopathies and those with ERM or MH susceptibility. Furthermore, animal studies have shown non-neuromuscular features such as a mild bleeding disorder and an immunological gain-of-function associated with MH/ERM related RYR1 variants raising important questions for further research. Awareness of the neuromuscular disease spectrum and potential multisystem involvement in RYR1-related MH and ERM is essential to optimize the diagnostic work-up, improve counselling and and future treatment strategies for patients affected by these conditions. This study will examine in detail the nature and severity of continuous disease manifestations and their effect on daily life in patients with RYR1-related MH and ERM., Methods: The study protocol consists of four parts; an online questionnaire study, a clinical observational study, muscle imaging, and specific immunological studies. Patients with RYR1-related MH susceptibility and ERM will be included. The imaging, immunological and clinical studies will have a cross-sectional design, while the questionnaire study will be performed three times during a year to assess disease impact, daily living activities, fatigue and pain. The imaging study consists of muscle ultrasound and whole-body magnetic resonance imaging studies. For the immunological studies, peripheral mononuclear blood cells will be isolated for in vitro stimulation with toll-like receptor ligands, to examine the role of the immune system in the pathophysiology of RYR1-related MH and ERM., Discussion: This study will increase knowledge of the full spectrum of neuromuscular and multisystem features of RYR1-related MH and ERM and will establish a well-characterized baseline cohort for future studies on RYR1-related disorders. The results of this study are expected to improve recognition of RYR1-related symptoms, counselling and a more personalized approach to patients affected by these conditions. Furthermore, results will create new insights in the role of the immune system in the pathophysiology of MH and ERM., Trial Registration: This study was pre-registered at ClinicalTrials.gov (ID: NCT04610619)., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are high-risk extensive abdominal surgery. During high-risk surgery, less invasive methods for cardiac index (CI) measurement have been widely used in operating theater. We investigated the accuracy of CI derived from different methods (FroTrac, ProAQT, ClearSight, and arterial pressure waveform analysis [APWA], from PICCO) and compared them to transpulmonary thermodilution (TPTD) during CRS and HIPEC in the operative room and intensive care unit (ICU)., Methods: Twenty-five patients scheduled for CRS-HIPEC were enrolled. During nine predefined time-points, simultaneous hemodynamic measurements were performed in the operating room and ICU. Absolute and relative changes of CI were analyzed using a Bland-Altman plot, four-quadrant plot, and interchangeability., Results: The mean bias was -0.1 L/min/m2 for ClearSight, ProAQT, and APWA and was -0.2 L/min/m2 for FloTrac compared with TPTD. All devices had large limits of agreement (LoA). The percentage of errors and interchangeabilities for ClearSight, FloTrac, ProAQT, and APWA were 50%, 50%, 54%, 36% and 36%, 47%, 40%, 72%, respectively. Trending capabilities expressed as concordance using clinically significant CI changes were -7º ± 39º, -19º ± 38º, -13º ± 41º, and -15º ± 39º. Interchangeability in trending showed low percentages of interchangeable and gray zone data pairs for all devices., Conclusions: During CRS-HIPEC, ClearSight, FloTrac and ProAQT systems were not able to reliably measure CI compared to TPTD. Reproducibility of changes over time using concordance, angular bias, radial LoA, and interchangeability in trending of all devices was unsatisfactory.

Background: The Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury (AKI) guidelines assign the same stage of AKI to patients whether they fulfil urine output criteria, serum creatinine criteria or both criteria for that stage. This study explores the validity of the KDIGO guidelines as a tool to stratify the risk of adverse outcomes in cardiac surgery patients., Methods: Prospective data from consecutive adult patients admitted to the cardiac intensive care unit (CICU) following cardiac surgery between January 2013 and May 2015 were analysed. Patients were assigned to groups based on the criteria they met for each stage of AKI according to the KDIGO guidelines. Short and mid-term outcomes were compared between these groups., Results: A total of 2267 patients were included with 772 meeting criteria for AKI-1 and 222 meeting criteria for AKI-2. After multivariable adjustment, patients meeting both urine output and creatinine criteria for AKI-1 were more likely to experience prolonged CICU stay (OR 4.9, 95%CI 3.3-7.4, p < 0.01) and more likely to require renal replacement therapy (OR 10.5, 95%CI 5.5-21.9, p < 0.01) than those meeting only the AKI-1 urine output criterion. Patients meeting both urine output and creatinine criteria for AKI-1 were at an increased risk of mid-term mortality compared to those diagnosed with AKI-1 by urine output alone (HR 2.8, 95%CI 1.6-4.8, p < 0.01). Patients meeting both urine output and creatinine criteria for AKI-2 were more likely to experience prolonged CICU stay (OR 16.0, 95%CI 3.2-292.0, p < 0.01) or require RRT (OR 11.0, 95%CI 4.2-30.9, p < 0.01) than those meeting only the urine output criterion. Patients meeting both urine output and creatinine criteria for AKI-2 were at a significantly increased risk of mid-term mortality compared to those diagnosed with AKI-2 by urine output alone (HR 3.6, 95%CI 1.4-9.3, p < 0.01)., Conclusions: Patients diagnosed with the same stage of AKI by different KDIGO criteria following cardiac surgery have significantly different short and mid-term outcomes. The KDIGO criteria need to be revisited before they can be used to stratify reliably the severity of AKI in cardiac surgery patients. The utility of the criteria also needs to be explored in other settings.

Objectives: There is little information on the impact of a high-risk multidisciplinary team (HRMDT) for thoracic surgery. In our unit, patients considered high risk for thoracic surgery have been discussed at this meeting since its inception in June 2013. The aim of this study was to audit our selection of patients discussed at the HRMDT and its effect on patient outcomes., Methods: Data were prospectively collected on all patients (n = 820) who underwent lung resection for lung cancer between July 2013 and September 2014. Patients were analysed as two groups HRMDT versus non-HRMDT. Referral to the HRMDT was at the operating surgeons' discretion. Referred patients usually had a higher-than-expected mortality or morbidity risk for the indicated procedure. The median time from HRMDT to surgery was 27 days (IQR 27.75). The median follow-up for all patients was 415 days (IQR 240)., Results: There were 102 patients in the HRMDT group and 718 in the non-HRMDT group (males 54 vs 46%; P = 0.12). The median duration from HRMDT to surgery was 27 days (IQR 27.75). Mean age (P = 0.0001), cardiac risk score (P = 0.001) and Thoracoscore (P = 0.0001) were significantly higher in the HRMDT group. There was also a significantly higher proportion of pneumonectomies in the HRMDT group (12 vs 4%; P = 0.001). There were no significant differences between the groups in cardiac, cerebrovascular, GI, pulmonary, renal or composite complications. There was no significant difference in 30-day (3 vs 1%; P = 0.24) or 90-day (5 vs 3%; P = 0.48) mortality between the groups. Operated HRMDT patients had better survival at 200 days (P = 0.002), but there was no difference in long-term survival compared with patients turned down for surgery., Conclusions: Despite a higher predicted mortality rate by Thoracoscore, HRMDT patients had the same outcome as lower risk non-HRMDT patients. Within the HRMDT cohort, survival in the operated patients was significantly better than that in non-operated patients in the short term. The HRMDT has managed to offer patients a radical treatment option who might have been refused surgery prior to this due to their higher risk profile. We would recommend this forum as a means to further assess and discuss high-risk patients., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Severe acute pancreatitis is associated with sever multiorgan failure from 15 to 50%, depending on the series. In some of these patients, conventional methods of ventilation and respiratory support will fail, demanding the use of extracorporeal membrane oxygenation (ECMO). Abdominal compartment syndrome is potentially harmful in this cohort of patients. We describe the successful treatment of three patients with severe acute pancreatitis who underwent respiratory ECMO and where intra abdominal pressure was monitored regularly., Methods: Retrospective review of case notes., Results: Three patients with severe acute pancreatitis requiring ECMO suffered from increased intra abdominal pressure during their ICU stay. No surgical interventions were taken to relieve abdominal compartment syndrome. Survival to hospital discharge was 100%., Conclusions: Monitoring intraabdominal pressure is a valuable adjunct to decision making while caring for these high-risk critically ill patients.

A previously fit and well man developed acute respiratory failure due to environmental mould exposure from living in damp rental accommodation. Despite aggressive intensive care management he rapidly deteriorated and required respiratory and cardiac Extracorporeal Membrane Oxygenation. We hypothesize that poor domiciliary conditions may make an underestimated contribution to community respiratory disease. These conditions may present as acute and severe illness with non-typical pathogens identified.

Introduction: Anti-neutrophil cytoplasmic antibody positive pulmonary capillaritis complicated by diffuse alveolar hemorrhage is a potentially fatal condition for which extracorporeal membrane oxygenation can facilitate improved outcomes and potential cure. Diffuse alveolar hemorrhage can be the initial presentation of an autoimmune disorder. The management is centered on the use of immunosuppressive therapy, which requires time, with fatality often occurring for these patients. We showed two very young patients with no previous history of vasculitis presenting with life threatening pulmonary hemorrhage due to anti-neutrophil cytoplasmic antibody positive vasculitis, whose management was facilitated with extracorporeal membrane oxygenation., Methods: We reviewed the clinical presentation and course of the first two patients with diffuse alveolar hemorrhage for anti-neutrophil cytoplasmic antibody positive vasculitis managed with veno-venous extracorporeal membrane oxygenation. We highlighted and analysed the unique challenges encountered in managing these patients., Results: The two patients were referred for extracorporeal membrane oxygenation since conventional ventilation was inadequate to provide physiologic support for respiratory failure. Clinical improvement was achieved without exacerbation of the pulmonary hemorrhage despite the use of anticoagulants. This provided time for the immunosuppressants to take effect. Both patients were discharged and were cured of the underlying condition., Conclusions: Extracorporeal membrane oxygenation has a role in the management of patients with severe respiratory failure due to anti-neutrophil cytoplasmic antibody positive capillaritis. Early recognition and referral for extracorporeal membrane oxygenation are vital to achieve a favourable outcome.

Background & Aims: More than half of patients with gastroesophageal reflux disease (GERD) report heartburn that awakens them from sleep. We aimed to determine the frequency of conscious awakenings associated with acid reflux events during sleep and their relationship with symptoms in patients with GERD compared with normal subjects., Methods: The study included 39 patients with heartburn and/or regurgitation at least 3 times each week and 9 healthy individuals as controls. Subjects underwent pH testing concomitantly with actigraphy. Novel software simultaneously integrated raw actigraphy and pH data matched by time to determine patients' conscious awakenings during sleep and their temporal relationship with acid reflux events and GERD-related symptoms., Results: A total of 104 and 11 conscious awakenings were recorded in 89.7% of patients and 77.8% of normal controls, respectively. The mean number of conscious awakenings was significantly higher in the group with GERD compared with controls (3.0 ± 0.3 vs 1.8 ± 0.4, P < .05). Of the conscious awakenings, 51.9% (51/104) were associated with an acid reflux event in GERD patients and 0 in controls (P < .01). Only 16.3% of total conscious awakenings were symptomatic. In most of the conscious awakenings that were associated with an acid reflux event (85.6%), the awakening preceded the reflux event., Conclusions: Acid reflux events occur primarily after an awakening episode. Conscious awakenings from sleep are common among patients with GERD and are frequently associated with acid reflux events. However, conscious awakenings associated with reflux events are seldom symptomatic., (Copyright © 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Objectives: Little attention has been paid to the effect of the systemic inflammatory response syndrome on intestinal dysfunction in the postoperative period. Several proinflammatory cytokines have been reported to increase the permeability of intestinal mucosa in vitro. We investigated the effect of dexamethasone on gut permeability in pediatric patients undergoing cardiac surgery by using the dual sugar permeability test and absorption of 2 other saccharides., Methods: Thirty-four patients scheduled for cardiac surgery with cardiopulmonary bypass were prospectively randomized to either act as control subjects or to receive dexamethasone (1 mg . kg -1) during induction of anesthesia. Intestinal permeability was measured with 3-O-methyl-D-glucose, D-xylose, L-rhamnose, and lactulose administered orally after induction of anesthesia and 12 and 24 hours later., Results: Lactulose/rhamnose ratios were increased from the outset in both groups (mean 0.57 [95% confidence interval, 0.24-0.91] for the control group and 0.76 [95% confidence interval, 0.35-1.17] for patients receiving dexamethasone). Although the ratios decreased 12 hours (0.29 [95% confidence interval, 0.17-0.42]) and 24 hours later (0.17 [95% confidence interval, 0.08-0.15]) in the dexamethasone group, in the control group there was a rise at 12 hours (0.77 [95% confidence interval, 0-1.64]), with a slight reduction 24 hours later (0.46 [95% confidence interval, 0.06-0.85])., Conclusions: Infants and children undergoing cardiac surgery with cardiopulmonary bypass show a significant reduction in gut permeability when dexamethasone is used during induction of anesthesia. Dexamethasone does not affect the intestinal barrier at the functional level, as assessed on the basis of 3-O-methyl-D-glucose and D-xylose absorption.