The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants.
Background/Objectives: The gut microbiota (GM) is pivotal for immune regulation, metabolism, and neurodevelopment. Infants undergoing surgery for congenital gastrointestinal anomalies are especially prone to microbial imbalances, with a paucity of beneficial bacteria (e.g., Bifidobacteria and Bacteroides) and diminished short-chain fatty acid production. Dysbiosis has been associated with severe complications, including necrotizing enterocolitis, sepsis, and feeding intolerance. This narrative review aims to critically examine strategies for microbiota modulation in this high-risk cohort. Methods: An extensive literature analysis was performed to compare the evolution of GM in healthy neonates versus those requiring gastrointestinal surgery, synthetizing strategies to maintain eubiosis, such as early nutritional interventions—particularly the use of human milk—along with antibiotic management and supplementary treatments including probiotics, prebiotics, postbiotics, and lactoferrin. Emerging techniques in metagenomic and metabolomic analysis were also evaluated for their potential to elucidate microbial dynamics in these patients. Results: Neonates undergoing gastrointestinal surgery exhibit significant alterations in microbial communities, characterized by reduced levels of eubiotic bacteria and an overrepresentation of opportunistic pathogens. Early initiation of enteral feeding with human milk and careful antibiotic stewardship are linked to improved microbial balance. Adjunctive therapies, such as the administration of probiotics and lactoferrin, show potential in enhancing gut barrier function and immune modulation, although confirmation through larger-scale studies remains necessary. Conclusions: Modulating the GM emerges as a promising strategy to ameliorate outcome in neonates with congenital gastrointestinal surgical conditions. Future research should focus on the development of standardized therapeutic protocols and the execution of rigorous multicenter trials to validate the efficacy and safety of these interventions.
IgG4-related disease (IgG4-RD) is a recently discovered immune-mediated fibroinflammatory condition, uncommon in the pediatric population, that could involve multiple organs and induce cancer-like lesions and organ damage. Its main features are multiple injuries in different sites, a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells, storiform fibrosis, and often high serological concentrations of IgG4. Autoimmune pancreatitis is the most common manifestation, mainly in adults. Two cases of IgG4-RD in children with lymph node localization of disease are reported. Localized or systemic lymph node involvement is common, but lymph node enlargement as the first and only manifestation of IgG4-RD is unusual, and therefore, hard to differentiate from other diseases. IgG4-related lymphadenopathy (IgG4-LAD) is most likely a distinct disease, described as isolated lymphadenopathy, related to the presence of elevated numbers of IgG4-positive plasma cells. Both disorders are likely to be misdiagnosed in children because they are characterized by rare and polymorphic features. IgG4-RD and IgG4-LAD should be considered in the differential diagnosis of disorders characterized by lymphadenopathy of uncertain etiology.
Background: Transumbilical laparoscopic-assisted (TULA) surgery is a minimally invasive technique that combines laparoscopic exploration with extracorporeal surgical management, offering potential advantages in neonatal abdominal surgery. However, comparative data with conventional open surgery in neonates remain limited. This study reports our single-center experience with TULA and compares its outcomes with those of a matched cohort of neonates undergoing open surgery. Methods: We performed a retrospective study on neonatal patients (<28 days of life) treated at our Pediatric Surgery Unit between 2015 and 2023. Twenty-five neonates underwent TULA for various intra-abdominal malformations. Each TULA patient was matched in a 1:2 ratio with neonates treated with open surgery based on gestational age, birth weight, and underlying diagnosis, resulting in a matched cohort of 50 patients. Primary outcomes included operative and anesthesia times, conversion rate, postoperative complications, length of hospital stay, and mortality. Results: The TULA cohort included 11 males and 14 females, with a mean gestational age of 37.5 ± 1.9 weeks and a mean birth weight of 2989 ± 675 g. Indications comprised intestinal malrotation, ileal atresia, duodenal stenosis, meconium ileus, and other abdominal pathologies. Mean operative time was comparable between groups (116 ± 37 min in the TULA group vs. 137 ± 65.9 min in the open surgery group; p = 0.52). Conversion from TULA to open surgery occurred in 16% of cases. No significant differences were observed in major postoperative complications or length of hospital stay between groups (p > 0.05). No mortality was reported. Conclusions: TULA represents a safe and effective surgical option for selected neonatal abdominal pathologies, with outcomes comparable to conventional open surgery. When performed in specialized centers with appropriate patient selection and multidisciplinary expertise, TULA offers favorable safety and cosmetic results.
Background/Objectives: Hypovolaemia and hypotension in the postoperative neonatal period are life-threatening conditions difficult to diagnose with standard clinical parameters. The inferior vena cava collapsibility index (IVCCI) is a validated non-invasive tool in adults; its application in mechanically ventilated surgical neonates remains poorly defined. This study aimed to evaluate the association of the IVCCI with echocardiographic haemodynamic indices and its changes following volume expansion in surgically treated neonates. Methods: This monocentric observational study enrolled 36 surgical neonates (mean gestational age 38.1 weeks; mean weight 2799 g) with postoperative hypovolaemia and oliguria at the UOC NICU-Neonatology, AOU Policlinico G. Rodolico–San Marco, Catania (July 2020–September 2023). All patients were on controlled invasive mechanical ventilation. The IVCCI, cardiac output (CO), stroke volume (SV), and peak Doppler velocities (Vmax) of the pulmonary artery and aorta were measured before and 4–6 h after colloid infusion (10–20 mL/kg). Results: The IVCCI decreased significantly from 44.05 ± 7.8% to 15.14 ± 4.3% (p < 0.001). MAP improved from 36.89 ± 5.12 to 55.99 ± 4.87 mmHg (p < 0.001) and pH from 7.32 ± 0.04 to 7.36 ± 0.03 (p < 0.01). The Vmax, SV, and CO of both ventricles increased significantly (all p < 0.001). Significant inverse correlations were found between the IVCCI and MAP, Vmax, CO, and SV (all p < 0.001). No correlation was found with gestational age, birth weight, or pain scores. Conclusions: The IVCCI is a feasible and clinically useful non-invasive echocardiographic parameter for haemodynamic monitoring in critically ill surgical neonates. Its significant correlation with cardiac function indices supports its potential role in the bedside assessment of suspected hypovolaemia. Larger prospective studies are warranted.