Six-month survival of a monochorionic monoamniotic twin with sirenomelia.

BACKGROUND: Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1/100,000) and usually lethal in the perinatal period. CASE: A 2,042 g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34 weeks of gestation by elective caesarean section. Sirenomelia was prenatally diagnosed. Single midline lower limb, bilateral dysplastic kidneys, an omphalomesenteric fistula, colon atresia, imperforate anus, indiscernible genital structures, and myelomeningocele were detected at birth. The amniotic fluid volume was normal throughout the pregnancy course, which led to appropriate lung maturation of the twin with sirenomelia. Although renal replacement therapy was initiated soon after birth, stable peritoneal dialysis was difficult because of the limited intraperitoneal space, and the infant frequently developed peritonitis. He died of sudden cardiorespiratory arrest at 6 months of age. Postmortem examination showed bilateral dysplastic kidneys, agenesis of the ureters and urinary bladder, abnormal branching and agenesis of the distal colon, bilateral inguinal hernias, and small testes. CONCLUSION: Infants with sirenomelia, even those with end-stage kidney disease at birth, may survive if they have a stable cardiorespiratory status at birth and renal replacement therapy is appropriately initiated.

Estimation of change in pleural pressure in assisted and unassisted spontaneous breathing pediatric patients using fluctuation of central venous pressure: A preliminary study.

BACKGROUND: It is important to evaluate the size of respiratory effort to prevent patient self-inflicted lung injury and ventilator-induced diaphragmatic dysfunction. Esophageal pressure (Pes) measurement is the gold standard for estimating respiratory effort, but it is complicated by technical issues. We previously reported that a change in pleural pressure (ΔPpl) could be estimated without measuring Pes using change in CVP (ΔCVP) that has been adjusted with a simple correction among mechanically ventilated, paralyzed pediatric patients. This study aimed to determine whether our method can be used to estimate ΔPpl in assisted and unassisted spontaneous breathing patients during mechanical ventilation. METHODS: The study included hemodynamically stable children (aged <18 years) who were mechanically ventilated, had spontaneous breathing, and had a central venous catheter and esophageal balloon catheter in place. We measured the change in Pes (ΔPes), ΔCVP, and ΔPpl that was calculated using a corrected ΔCVP (cΔCVP-derived ΔPpl) under three pressure support levels (10, 5, and 0 cmH2O). The cΔCVP-derived ΔPpl value was calculated as follows: cΔCVP-derived ΔPpl = k × ΔCVP, where k was the ratio of the change in airway pressure (ΔPaw) to the ΔCVP during airway occlusion test. RESULTS: Of the 14 patients enrolled in the study, 6 were excluded because correct positioning of the esophageal balloon could not be confirmed, leaving eight patients for analysis (mean age, 4.8 months). Three variables that reflected ΔPpl (ΔPes, ΔCVP, and cΔCVP-derived ΔPpl) were measured and yielded the following results: -6.7 ± 4.8, - -2.6 ± 1.4, and - -7.3 ± 4.5 cmH2O, respectively. The repeated measures correlation between cΔCVP-derived ΔPpl and ΔPes showed that cΔCVP-derived ΔPpl had good correlation with ΔPes (r = 0.84, p< 0.0001). CONCLUSIONS: ΔPpl can be estimated reasonably accurately by ΔCVP using our method in assisted and unassisted spontaneous breathing children during mechanical ventilation.

A novel method for transpulmonary pressure estimation using fluctuation of central venous pressure.

The objective of the study is to develop a correction method for estimating the change in pleural pressure (ΔPpl) and plateau transpulmonary pressure (PL) by using the change in central venous pressure (ΔCVP). Seven children (aged < 15 years) with acute respiratory failure (PaO2/FIO2 < 300 mmHg), who were paralyzed and mechanically ventilated with a PEEP of < 10 cmH2O and had central venous catheters and esophageal balloon catheters placed for clinical purposes, were enrolled prospectively. We compared change in esophageal pressure (ΔPes), ΔCVP, and ΔPpl calculated using a corrected ΔCVP (cΔCVP-derived ΔPpl). cΔCVP-derived ΔPpl was calculated as κ × ΔCVP, where κ was the ratio of the change in airway pressure (ΔPaw) to ΔCVP during the occlusion test. cΔCVP-derived ΔPpl correlated better than ΔCVP with ΔPes (R2 = 0.48, p = 0.08 vs. R2 = 0.14, p = 0.4) with lesser bias and precision in Bland-Altman analysis. The plateau PL calculated using the cΔCVP-derived ΔPpl (17.6 ± 2.6 cmH2O) correlated well with the ΔPes-derived plateau PL (18.1 ± 2.3 cmH2O) (R2 = 0.90, p = 0.001). Our correction method can estimate ΔPpl and plateau PL from ΔCVP with a reasonable accuracy in paralyzed and mechanically ventilated pediatric patients with respiratory failure.

Effects of Healthcare-Associated Infections on Length of PICU Stay and Mortality.

OBJECTIVES: To identify the effects of healthcare-associated infections on length of PICU stay and mortality. DESIGN: Retrospective, single-center, observational study. SETTING: PICU of a tertiary children's hospital. PATIENTS: Consecutive patients who stayed greater than 48 hours in the PICU between January 2013 and December 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Data were retrospectively collected from medical records. We identified occurrences of common healthcare-associated infections, including bloodstream infection, pneumonia, and urinary tract infection, defined according to the 2008 definitions of the Centers for Disease Control and Prevention and National Healthcare Safety Network. We assessed the effects of each healthcare-associated infection on length of PICU stay and PICU mortality using multivariable analysis. Among 1,622 admissions with a PICU stay greater than 48 hours, the median age was 299 days and male patients comprised 51% of admissions. The primary diagnostic categories were cardiovascular (58% of admissions), respiratory (21%), gastrointestinal (8%), and neurologic/muscular (6%). The median length of PICU stay was 6 days, and the PICU mortality rate was 2.5%. A total of 167 healthcare-associated infections were identified, including 67 bloodstream infections (40%), 43 pneumonias (26%), and 57 urinary tract infections (34%). There were 152 admissions with at least one healthcare-associated infection (9.4% of admissions with a stay > 48 hr). On multivariable analysis, although each healthcare-associated infection was not significantly associated with mortality, bloodstream infection was associated with an extra length of PICU stay of 10.2 days (95% CI, 7.9-12.6 d), pneumonia 14.2 days (11.3-17.2 d), and urinary tract infection 6.5 days (4.0-9.0 d). Accordingly, 9.7% of patient-days were due to these three healthcare-associated infections among patients with a stay greater than 48 hours. CONCLUSIONS: Although healthcare-associated infections were not associated with PICU mortality, they were associated with extra length of PICU stay. As 9.7% of patient-days were due to healthcare-associated infections, robust prevention efforts are warranted.

Accuracy of Reported Tidal Volume During Neonatal Ventilation With Airway Leak: A Lung Model Study.

OBJECTIVES: To determine the accuracy of tidal volume reported by neonatal ventilators, with and without leak compensation, in invasive and noninvasive ventilation modes in the presence of airway leak; and, to determine what factors have a significant effect on the accuracy of tidal volume reported by ventilators with leak compensation in the presence of airway leak. We hypothesized that ventilators with a leak compensation function that includes estimation of tidal volume could accurately report tidal volume in the presence of airway leak, but that the accuracy of reported tidal volume may be affected by variables such as the identity of the ventilator, lung mechanics, leak size, positive end-expiratory pressure level, and body size. DESIGN: In vitro assessment of ventilator volume delivery was conducted for seven acute care ventilators using a passive lung simulator. SETTING: Laboratory-based measurements. INTERVENTIONS: The error of reported tidal volume was calculated under three ventilation modes (noninvasive-pressure-control, invasive-pressure-control, and invasive-dual-control ventilation), three models of lung mechanics (normal and restrictive and obstructive lung disease), a range of airway leak values, two positive end-expiratory pressure values, and two body weights for each ventilator. Ventilators with and without leak compensation were studied. MEASUREMENTS AND MAIN RESULTS: In the absence of airway leak, all ventilators reported tidal volume accurately. In the presence of airway leak, the error of reported tidal volume increased for all ventilators without a leak compensation algorithm while ventilators with leak compensation that included estimation of tidal volume accurately reported tidal volume. In the presence of airway leak, clinically significant effects on the error of reported tidal volume by ventilators with leak compensation were associated with the choice of ventilator in all modes and with lung mechanics in invasive ventilation modes. CONCLUSIONS: Reported tidal volume is affected by the presence of airway leak, but in many ventilators a leak compensation algorithm that includes estimation of tidal volume can correct for the discrepancy between actual and reported tidal volume. However, even in ventilators with leak compensation, choice of ventilator and lung mechanics in invasive ventilation modes have a significant effect on error of reported tidal volume.

Risk Factors for Healthcare-Associated Infections After Pediatric Cardiac Surgery.

OBJECTIVES: Healthcare-associated infections after pediatric cardiac surgery are significant causes of morbidity and mortality. We aimed to identify the risk factors for the occurrence of healthcare-associated infections after pediatric cardiac surgery. DESIGN: Retrospective, single-center observational study. SETTING: PICU at a tertiary children's hospital. PATIENTS: Consecutive pediatric patients less than or equal to 18 years old admitted to the PICU after cardiac surgery, between January 2013 and December 2015. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: All the data were retrospectively collected from the medical records of patients. We assessed the first surgery during a single PICU stay and identified four common healthcare-associated infections, including bloodstream infection, surgical site infection, pneumonia, and urinary tract infection, according to the definitions of the Centers for Disease Control and Prevention and National Healthcare Safety Network. We assessed the pre-, intra-, and early postoperative potential risk factors for these healthcare-associated infections via multivariable analysis. In total, 526 cardiac surgeries (394 patients) were included. We identified 81 cases of healthcare-associated infections, including, bloodstream infections (n = 30), surgical site infections (n = 30), urinary tract infections (n = 13), and pneumonia (n = 8). In the case of 71 of the surgeries (13.5%), at least one healthcare-associated infection was reported. Multivariable analysis indicated the following risk factors for postoperative healthcare-associated infections: mechanical ventilation greater than or equal to 3 days (odds ratio, 4.81; 95% CI, 1.89-12.8), dopamine use (odds ratio, 3.87; 95% CI, 1.53-10.3), genetic abnormality (odds ratio, 2.53; 95% CI, 1.17-5.45), and delayed sternal closure (odds ratio, 3.78; 95% CI, 1.16-12.8). CONCLUSIONS: Mechanical ventilation greater than or equal to 3 days, dopamine use, genetic abnormality, and delayed sternal closure were associated with healthcare-associated infections after pediatric cardiac surgery. Since the use of dopamine is an easily modifiable risk factor, and may serve as a potential target to reduce healthcare-associated infections, further studies are needed to establish whether dopamine negatively impacts the development of healthcare-associated infections.

Predicting extubation readiness by monitoring the electrical activity of the diaphragm after prolonged mechanical ventilation: a pediatric case report.

BACKGROUND: The intensity of the electrical activity of the diaphragm (Edi) correlates with inspiratory effort. The ratio of tidal volume to the Edi is known as neuroventilatory efficiency (NVE) and is used as an index for ventilation efficiency. Here, we present a case showing that Edi and NVE may be effective parameters to predict successful extubation. CASE PRESENTATION: A 6-month-old female infant required prolonged mechanical ventilation after cardiac surgery. Fifty-two days after surgery, her trachea was extubated but required reintubation. Edi monitoring was initiated to assess diaphragm function. The Edi was > 70 mcV just after the reintubation, and her NVE was 1.0 mL/mcV, but gradually decreased. On day 59, her Edi values during the spontaneous breathing trials were 13 mcV with the improvement of NVE (2.5 mL/mcV) and her trachea was extubated without complications. CONCLUSIONS: The Edi and NVE were valuable for deciding the extubation readiness in a long-term mechanically ventilated patient.