Anesthesia for children with anterior mediastinal masses.

Children with an anterior mediastinal mass may have cardiopulmonary compromise that can be exacerbated under general anesthesia. Signs and symptoms such as cough, shortness of breath, stridor, orthopnea, accessory muscle use, a history of respiratory arrest, and the presence of a pleural effusion and upper body edema are predictive of perioperative complications. A larger mediastinal mass on imaging is predictive of perioperative complications. Risk stratification of patients, together with an individualized plan, will best guide operative management for patients with an anterior mediastinal mass. General anesthesia (GA) should be avoided if possible, but a spontaneous breathing technique is recommended if GA is required.

Pulsatile Perfusion during Cardiopulmonary Bypass: A Literature Review.

The use of cardiopulmonary bypass (CPB) in cardiac surgery has often been associated with postoperative organ dysfunction. Roller and centrifugal pumps produce non-pulsatile flow (NPF) by default, and this still is the most widely used mode of perfusion. The development of pulsatile pumps has allowed comparisons to be made with NPF. Pulsatile flow (PF) mimics the arterial pulse generated by the heart and is thought to be more physiological by some. This review aims to examine the proposed mechanisms behind the potential physiological benefits of PF during CPB and to summarize the current clinical evidence. MEDLINE and EMBASE were used to identify articles published over a 25 year period from 1995 to 2020. A literature review was conducted to determine the effects of PF on organ functions. A total of 44 articles were considered. Most of the articles published on PF were randomized controlled trials (RCTs). However, there was a wide variation in study methodology, method of pulse generation and how pulsatility was measured. Most of the evidence in favor of PF showed a marginal improvement on renal and pulmonary outcomes. In these studies, pulsatility was generated by an intra-aortic balloon pump. In conclusion, there is a lack of good quality RCTs that can inform on the short- and long-term clinical outcomes of PF. Further research is required in order to draw a conclusion with regards to the benefits of PF on organ function.

Positive role of continuous positive airway pressure for intensive care unit patients with severe hypoxaemic respiratory failure due to COVID-19 pneumonia: A single centre experience.

OBJECTIVES: Continuous positive airway pressure (CPAP) may be a useful treatment strategy for patients with severe COVID-19 pneumonia but its effectiveness in preventing mechanical ventilation is unknown. We aimed to evaluate the outcomes of COVID-19 patients treated with CPAP and determine predictors of CPAP response. DESIGN: This was a retrospective observational cohort study. SETTING: The study took place in the intensive care unit (ICU) at Royal Papworth Hospital (RPH) in Cambridge, UK. PATIENTS: We included all consecutive patients with confirmed COVID-19 pneumonia who were transferred from neighbouring hospitals between 14th March and 6th May, 2020 for consideration of ventilatory support. INTERVENTION: We instituted the use of CPAP for all patients who arrived in RPH not intubated and were not making satisfactory progress on supplemental oxygen alone. MEASUREMENTS AND MAIN RESULTS: Of 33 self-ventilating patients included in this study, 22 (66.7%) were male and the mean age was 54 ± 13.23 patients received CPAP. They were more hypoxaemic than those treated with oxygen alone (PaO2/FiO2 ratio; 84.3 ± 19.0 vs 170.0 ± 46.0 mmHg, p = 0.001). There was a significant improvement in PaO2/FiO2 ratio 1-2 hours after CPAP initiation (167.4 ± 49.0 from 84.3 ± 19.0 mmHg, p = 0.001). 14 (61%) patients responded to CPAP and 9 required intubation. There was no difference between these two groups in terms of the severity of baseline hypoxaemia (PaO2/FiO2 ratio; 84.5 ± 16.0 vs 83.9 ± 23.0 mmHg, p = 0.94) but CPAP responders had significantly lower C-reactive protein (CRP) (176 ± 83 vs 274 ± 63 mg/L, p = 0.007), interleukin-6 (IL-6) (30 ± 47 vs 139 ± 148 pg/mL, p = 0.037), and D-dimer (321 ± 267 vs 941 + 1990 ng/mL, p = 0.003). CT pulmonary angiogram was performed in 6 out of 9 intubated patients and demonstrated pulmonary emboli in 5 of them. All patients were discharged from ICU and there were no fatalities. CONCLUSIONS: In this cohort, CPAP was an effective treatment modality to improve hypoxaemia and prevent invasive ventilation in a substantial proportion of patients with severe respiratory failure. Accepting the small sample size, we also found raised biomarkers of inflammation (CRP and IL-6) and coagulopathy (D-Dimer) to be more useful predictors of CPAP responsiveness than the severity of hypoxaemia, and could help to guide intubation decisions in this clinical setting.

Lung ultrasound and the role of lung aeration score in patients with acute respiratory distress syndrome on extracorporeal membrane oxygenation.

OBJECTIVE: This was a pilot study to determine the utility of daily lung ultrasound (LUS) in patients requiring veno-venous extracorporeal membrane oxygenation (VV-ECMO) for acute respiratory distress syndrome (ARDS). DESIGN: This was a prospective, observational study. SETTING: The study took place in the intensive care unit at Royal Papworth Hospital in Cambridge, UK. PARTICIPANTS: We recruited adult patients receiving VV-ECMO for ARDS. INTERVENTIONS: All patients received a lung computed tomography (CT) scan and LUS on admission. Bedside chest radiography (CXR) and LUS were done on a daily basis until patients were decannulated. MEASUREMENTS AND MAIN RESULTS: Daily LUS aeration scores were calculated according to the appearance of four defined patterns. An independent radiologist calculated corresponding scores for CT and CXR, retrospectively. These were checked for correlation with LUS aeration scores. There were statistically significant correlations between LUS versus CT (r = 0.868, p = 0.002) and LUS versus CXR (r = 0.498, p = 0.018) with good agreement and no evidence of proportional bias. LUS was able to detect 13.5% of pleural effusions and 54.2% of pneumothorax that were not picked up on CXR.In most of the patients who were weaned off VV-ECMO, a progressive reduction of LUS aeration scores corresponding to lung re-aeration was observed. CONCLUSIONS: LUS correlated with findings on CT and CXR for quantifying lung aeration and the clinical presentation of patients. LUS also picked up more pleural effusions and pneumothorax than CXR. Together with traditional imaging techniques, the routine use of LUS should be considered for this patient group.

Anaesthetic management of a patient with Leigh's syndrome with central hypoventilation and obstructive sleep apnoea.

Leigh's syndrome, which is characterised by progressive neurodegeneration involving the brainstem and basal ganglia, belongs to a family of disorders classified as mitochondrial myopathies. It is most commonly transmitted by an autosomal recessive mode of inheritance, but can sometimes occur in a mitochondrial pattern. It typically presents during infancy with developmental delay and deterioration of brainstem function. Respiratory failure is the common cause of death and postoperative morbidity in patients with Leigh's disease. Herein, we report the case of a 17-year-old female patient with Leigh's syndrome who underwent general anaesthesia for a tracheostomy, which was performed in view of the patient's requirement for long-term ventilation and frequent toileting for secretions. Her respiratory complications included central hypoventilation secondary to brainstem involvement, and obstructive sleep apnoea due to obesity and muscle dystonia. She was hospitalised for acute respiratory decompensation secondary to hospital-acquired pneumonia. We review the anaesthetic implications of this disease and discuss its impact on preoperative, intraoperative and postoperative management.

Gastroschisis with intestinal atresia--predictive value of antenatal diagnosis and outcome of postnatal treatment.

PURPOSE: The purpose of this study is to evaluate (1) the predictive value of fetal bowel dilatation (FBD) for intestinal atresia in gastroschisis and (2) the postnatal management and outcome of this condition. METHODS: A retrospective review of all gastroschisis cases diagnosed in our fetal medicine unit between 1992 and 2010 and treated postnatally in our center was performed. RESULTS: One hundred thirty cases had full postnatal data available. Intestinal atresia was found at surgery in 14 neonates (jejunum, n = 6; ileum, n = 3; ascending colon, n = 3; multiple, n = 2). Polyhydramnios and FBD were more likely in the atresia group compared with infants with no atresia (P = .0003 and P = .005, respectively). Fetal bowel dilatation had 99% negative predictive value (95% confidence interval, 0.9-0.99) and 17% positive predictive value (95% confidence interval, 0.1-0.3) for atresia. Treatment of intestinal atresia included primary anastomosis (n = 5), delayed anastomosis (n = 2), and stoma formation followed by anastomosis (n = 7). Infants with atresia had longer duration of parenteral nutrition, higher incidence of sepsis, and cholestasis compared with infants with no atresia (P = .0003). However, the presence of atresia did not increase mortality. CONCLUSIONS: Polyhydramnios and FBD are associated with atresia. Absence of FBD in gastroschisis excludes intestinal atresia. In our experience, atresia is associated with a longer duration of parenteral nutrition but does not influence mortality. These findings may be relevant for antenatal counseling.

Gastroschisis: sonographic diagnosis, associations, management and outcome.

Gastroschisis is a defect in the abdominal wall, typically on the right side of a normally inserted umbilical cord through which bowel and other abdominal contents herniate. Classically, no membrane covers the herniated abdominal contents, which distinguishes the defect from exomphalos, an important differential diagnosis. Gastroschisis is usually diagnosed prenatally using ultrasound examination. The prevalence is increasing worldwide from approximately 0.1 per 10,000 total births in the 1970s to over 5 in the early 2000s. The reasons for this are unknown, but factors such as maternal smoking, recreational drugs and young maternal age are strongly associated with the defect. The increasing prevalence is causing concern because the cost of treating gastroschisis is high. Neonatal morbidity depends on significant complicating factors such as bowel atresia or necrosis and prolonged post-operative ileus. Foetuses with gastroschisis are more likely to be born premature and with intra-uterine growth restriction, both of which contribute to the morbidity. Gastroschisis requires early surgery after birth, often followed by prolonged neonatal care. However, advances in surgical and post-operative care in the last decade have meant that currently 90% of affected neonates survive, with few long-term problems.

Sonographic diagnosis of SEDC and double heterozygote of SEDC and achondroplasia--a report of six pregnancies.

OBJECTIVE: To define the sonographic features of spondyloepiphyseal dysplasia congenita (SEDC) and the double heterozygote for SEDC and achondroplasia. METHODS: A retrospective review of 6 pregnancies in one family where one parent has achondroplasia and the other SEDC. RESULTS: There were 4 double heterozygote pregnancies and 2 where the fetus had SEDC. Shortening of long bones was evident in both conditions from around 16 weeks gestation. Other findings such as an increased nuchal translucency were more variable. CONCLUSIONS: Molecular analysis of the FGFR3 and COL2AI gene once mutations are known in a family such as reported here can inform prenatal diagnosis and help to distinguish between the double heterozygote and a fetus which has inherited a single mutation. The data presented here on the growth of the long bones and other sonographic features associated with SEDC may aid prenatal diagnosis in cases where the mutation is not known.