Intestinal perforation (IP) associated with necrotizing enterocolitis (NEC) is one of the leading causes of mortality in premature neonates; with major nutritional and neurodevelopmental sequelae. Since predicting which neonates will develop perforation is still challenging; clinicians might benefit considerably with an early diagnosis tool and the identification of critical factors. The aim of this study was to forecast IP related to NEC and to investigate the predictive quality of variables; based on a machine learning-based technique. The Back-propagation neural network was used to train and test the models with a dataset constructed from medical records of the NICU; with birth and hospitalization maternal and neonatal clinical; feeding and laboratory parameters; as input variables. The outcome of the models was diagnosis: (1) IP associated with NEC; (2) NEC or (3) control (neither IP nor NEC). Models accurately estimated IP with good performances; the regression coefficients between the experimental and predicted data were R2 > 0.97. Critical variables for IP prediction were identified: neonatal platelets and neutrophils; orotracheal intubation; birth weight; sex; arterial blood gas parameters (pCO2 and HCO3); gestational age; use of fortifier; patent ductus arteriosus; maternal age and maternal morbidity. These models may allow quality improvement in medical practice.
Introduction: Intracardiac rhabdomyomas can cause severe ventricular dysfunction and outflow tract obstruction.Case report: A term newborn infant with antenatal diagnosis of giant left ventricle rhabdomyoma presented with cardiac failure and duct-dependent systemic circulation after birth. She was treated successfully with everolimus, showing decrease in tumour size and improvement in left ventricular ejection fraction.Discussion: Tumour regression rate was 0.32 cm2/day and improved to 0.80 cm2/day with the use of everolimus. Herein we report a newborn with inoperable giant left ventricular cardiac rhabdomyoma and significant regression of the tumour. To our knowledge, this is the largest left ventricular rhabdomyoma reported. A review of the literature was undertaken for comparison.Conclusion: Everolimus has proven to be efficacious in size reduction of cardiac rhabdomyomas in cases when surgical resection is not possible.
Propofol infusion syndrome (PRIS) is a rare syndrome originally described in critically ill children undergoing long-term (> 48 h) propofol infusion at high doses (> 4 mg/kg/h). Severe metabolic acidosis, rhabdomyolysis, renal failure and fatal cardiac failure are the features. Herein, we present a case of a newborn who developed PRIS after a single bolus dose of propofol at 3.2 mg/kg/do, developing rhabdomyolysis and severe metabolic acidosis, with a successful outcome after medical therapy.
Background While research indicates comparable quality of life (QOL) in congenital diaphragmatic hernia (CDH) and healthy populations, the effect of CDH severity on patients’ health perceptions remains unexplored. We aimed to assess QOL perception in CDH, hypothesising a decline correlated with increased disease severity. Methods In this prospective observational study, we analysed patients with CDH aged 5 years and above participating in a longitudinal outpatient programme. We excluded bilateral CDH, genetic/syndromic conditions, prematurity and late diagnosis. Participants self-administered the age-adapted Pediatric Quality of Life Inventory (PedsQL) survey, covering four domains (physical, emotional, social, school). After enrolment, data were collected blind to severity status (larger defects denoting significant/‘severe’ disease). Repeated measurements were managed using a random mixed-effects model. Results Of 34 participants (50% males) who completed the PedsQL, 10 provided measurements at two visits. Eight required a patch (type C), while 26 had primary repairs (type A=8; type B=18). Age at first evaluation was comparable across groups (no patch: median 11 (7–16), patch: 13 (8–15) years, p=0.78). Severe CDH correlated significantly with lower PedsQL scores (adjusted β: −18%, 95% CI −28%; −7%, adjusted for age at visit and sex). Lower scores specifically occurred in walking, exercising, social and academic functioning. Conclusion Severe CDH significantly lowers QOL. This finding is crucial for resource allocation in long-term CDH health surveillance and advocates for regular inclusion of patient experiences in quality improvement efforts.
Background: Preterm infants with hemodynamically significant patent ductus arteriosus (HsPDA) are exposed to low cerebral tissue oxygen saturation (rScO2) values. Additionally, infants requiring surgical ligation are at risk of further changes in cerebral oxygenation and postligation cardiac syndrome (PLCS). Previous studies have assessed the effect of PDA ligation on rScO2 with variable results. Cases description: In this report we analyse near-infrared spectroscopy (NIRS) and echocardiographic findings of two patients who underwent ligation of PDA and presented low cardiac output. Literature on regional tissue oxygenation saturation (rSO2) before and after PDA ligation was briefly reviewed. Discussion: Cerebral oxygenation values before and after PDA ligation may be influenced by gestational age, vasopressor use, ductal shunt volume, time of exposure HsPDA, chronological age and the presence of cerebral autoregulation. PLCS complicates 28–45% of all PDA ligations and is associated with higher mortality. Cerebral and somatic NIRS monitoring in the postoperative period may enhance the identification of PLCS at early stages. Conclusion: Cerebral oxygenation in the perioperative period of PDA ligation may be influenced by numerous clinical factors. Early detection of PLCS using multisite NIRS after ligation could prevent further alterations in cerebral hemodynamics and improve outcomes. A decrease in somatic-cerebral difference and/or a significant drop in somatic NIRS values may precede clinical signs of hypoperfusion. NIRS values should be interpreted as trends along with echocardiographic findings to guide goal directed interventions.
Chronic pulmonary hypertension (cPH) associated with bronchopulmonary dysplasia (BPD) is a major contributor to morbidity and mortality in extremely preterm infants. Despite improvements in neonatal care, the burden of BPD and its pulmonary vascular complications remains significant. Early detection and standardized management of cPH are essential to improve outcomes. Echocardiography plays a central role in screening and guiding treatment, particularly in high-risk infants requiring respiratory support at or beyond 36 weeks postmenstrual age. The Targeted Neonatal Echocardiography—Quebec (TnECHO-Qc) collaborative has developed a province-wide screening and management algorithm for cPH in preterm infants with BPD. This initiative outlines a stepwise approach to echocardiographic evaluation, including specific criteria for identifying elevated pulmonary arterial pressures, grading severity, and scheduling follow-up based on clinical and imaging findings. Additional management elements encompass biomarker use, respiratory and nutritional optimization, and consideration of airway anomalies, reflux, and aspiration. Pharmacologic therapies, including inhaled nitric oxide and pulmonary vasodilators, are considered for moderate to severe cPH with a pre-capillary component (“pulmonary arterial hypertension”) after stabilization of ventilation and oxygenation, and guided by echocardiography follow-up. This collaborative initiative establishes a standardized, multidisciplinary framework to enable timely recognition and individualized management of chronic pulmonary hypertension (cPH) in preterm infants. The primary goal is to reduce adverse outcomes and support long-term health, with the effectiveness of the framework to be evaluated through longitudinal outcome assessments.