Congenital high airway obstructive syndrome (CHAOS) is a rare congenital anomaly, frequently caused by laryngeal or tracheal atresia, tracheal stenosis, and obstructing laryngeal cysts. This is a congenital malformation, often fatal, with an unknown prevalence. Laryngeal atresia is the most frequent cause. We report a case of an intrauterine diagnosis of CHAOS and ascites in a 17-week fetus delivered at 38 weeks of gestation without other associated malformations. A fetoscopic procedure was performed at 22 weeks of gestation. An attempt was made to perforate the affected area to ensure pulmonary fluid circulation and the ascites’ resolution. After birth, a tracheostomy was performed. The patient was mechanically ventilated until 11 months of age, when she was discharged with no cerebral or other complications of immediate postnatal anoxia or episodes of respiratory arrest. A laryngotracheoplasty was performed at 2 years old, but decannulation was not possible due to certain complications. At 5 years old, a new surgical intervention was performed, which allowed decannulation 6 months later.

Desmoid tumors are a rare entity, especially in the pediatric population. There are no reports of such a tumor in newborns. They are associated with high rates of morbidity and mortality, even though they are benign soft tissue tumors. This is due to them exhibiting locally aggressive growth with the compression or invasion of adjacent structures. Abdominal localization is most commonly reported, although there are reports of mediastinal desmoid tumors. We present the case of a 6-day male patient with a mediastinal desmoid tumor that led to severe aortic coarctation with hemodynamic instability. The tumor also compressed the left pulmonary artery and obstructed the left main bronchus. The initial management consisted of successful emergency surgery with partial resection of the tumor mass and coarctation repair. In the postoperative setting, the patient evolved with severe respiratory dysfunction which was managed with tracheostomy, allowing weaning the child from the mechanical ventilation one month after surgery, along with chemotherapy. We also review the literature, focusing on the management of desmoid tumors.

Central venous catheterization is, now, one of the most routinely used procedures in the NICUs, helping during the care of very sick infants. Pericardial effusion is a very rare but severe complication, with a high mortality. The cases described are part of an ongoing retrospective study where the use of central catheters inserted in our surgical NICU, and its complications is being analyzed. 16 cases over 13 years are presented in this article, varying in severity from mild, self-resolving cases that were discovered during routine cardiac POCUS to cases with important hemodynamic impact associated with cardiac tamponade and cardiac arrest. Due to immediate intervention, only one of the cases led to catheter-related mortality and that was under particular conditions. Our aim is to highlight the severity of this complication, the importance of early intervention, and the impact of a highly technologized unit and widely available cardiac POCUS.

The vein of Galen malformations (VoGMs) is mainly correlated with the retention of an embryonic pattern of vascularity, inducer of vein of Galen dilation, and formation of arteriovenous communications that give rise to the risk of systemic shunting, causing cardiac dysfunction, vascular steal, and venous hypertension. This is a rare cerebral vascular malformation in the newborn, accounting for 1% of all cerebral arteriovenous malformations and occurring in approximately 1 in 25,000–50,000 live births. We review nine cases of newborns diagnosed with vein of Galen malformations (VoGMs) to assess whether this pathology demonstrates a marked improvement over the past 13 years in diagnostic accuracy, treatment approaches, and patient survival rates within our clinic. Medical treatment was focused on providing inotropic support and tightly controlled peripheral and pulmonary vasodilation with the aim of overriding the effects of high output heart failure. Most of the patients underwent liver failure and flow-mediated pulmonary hypertension, while half of the newborns expressed anomalies of the nervous system due to impaired cerebral hemodynamics. Given the unavailability of endovascular treatment in our unit, which predisposes the newborns to a higher vital risk, we recognize the importance of delivering tailored intensive care aimed at maintaining cardiorespiratory and hemodynamic stability until a curative intervention can be performed in a specialized center.

Capillary malformation–arteriovenous malformation syndrome is a rare spectrum of vascular anomalies characterized by capillary malformations and high-flow vascular malformations, caused by loss-of-function mutations in the RASA1 and/or EPHB4 genes. These mutations disrupt vascular differentiation and lead to complex malformations involving the brain, skin, and systemic vasculature. Since the first description in 2003, more than 200 cases have been reported, but intracranial arteriovenous shunts during the neonatal period remain extremely rare, as well as reports of the dual mutation RASA1 + EPHB4 or the immunological impact of the EPHB4 mutation. We report three cases of neonates presenting with early-onset high-flow shunts, each exhibiting a distinct genetic signature: CM-AVM1 (RASA1 mutation), CM-AVM2 (EPHB4 mutation), and dual variant (combined EPHB4 and RASA1 mutations). We analyzed and compared the clinical evolution, Doppler ultrasound trends, EEG, MRI and genetic data to highlight the distinct genotype–phenotype spectrum. Early multimodal hemodynamic evaluation of neonates with CM-AVM allows the identification and optimum management of life-threatening shunts at the earliest possible stage.

Background: Advances in neonatal intensive care have improved survival in congenital diaphragmatic hernia (CDH), shifting the clinical focus toward long-term complications such as feeding difficulties. Initiation of enteral feeding varies across centers, with evidence suggesting that intra-abdominal pressure (IAP) may influence time to feeding and clinical decision-making. We explored associations of early postoperative heart rate (HR), recorded Apgar score, and intra-abdominal pressure (IAP) with time to enteral trophic feeding. Methods: We retrospectively evaluated 28 consecutive neonates undergoing CDH repair at a national referral center between January 2022 and June 2025. Trophic enteral feeding (≥10 mL/kg/day for ≥24 h) was the primary endpoint. Aalen–Johansen estimates and two parsimonious Fine–Gray models (HR plus Apgar; IAP plus Apgar) were primary analyses and cause-specific Cox models were sensitivity analyses. Results: Within 30 postoperative days, trophic enteral feeding was achieved in 21 cases, six patients died before feeding, and one did not achieve feeding before censoring. Each 10 bpm higher HR was associated with lower feeding incidence (subdistribution HR 0.62, 0.46–0.85; p = 0.003); the cause-specific point estimate was also below 1 (HR 0.65, 0.47–0.89; p = 0.007). Mean IAP was not clearly associated with feeding incidence (subdistribution HR 1.01 per mmHg, 0.82–1.25; p = 0.925). Both Cox models failed global proportional-hazards tests. Conclusions: Each 10 bpm increase in HR was associated with a longer time to trophic enteral feeding, and the cause-specific sensitivity analysis pointed in the same direction. Because both estimates derive from the same 28 infants, their agreement is expected rather than corroborative. These findings are exploratory and do not establish causality, individual predictive value, or superiority of HR over IAP. Prospective multicenter studies with adequate sample sizes are needed to confirm these findings.