Search

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.

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a rare motility disorder with high mortality rate described by Berdon in 1976. We present the first case of Berdon’s syndrome (heterozygous pathogenic variant in the ACTG2 gene) reported in Romania, a female newborn admitted in NICU “Marie S. Curie” Emergency Children’s Hospital Bucharest for intestinal obstruction after birth. Total parenteral nutrition, ileostomy, gastrostomy, clean intermittent bladder catheterisation, evaluation for multivisceral transplantation were performed. She was discharged from our NICU ward at the age of 4 years and 2 months with home total parenteral nutrition administered by her mother in sterile condition, clean intermittent catheterisation for bladder evacuation performed by her mother, monitored monthly for about three years, with normal cardio-respiratory function, no signs of thrombosis, she maintained relatively low platelet count without positive blood culture, good liver and renal function test. Normal neurological and psychomotor development according to age. Her course was complicated by multiorgan failure with death ensuing at the age of 7 years and 10 months.

Background: Acute kidney injury occurs commonly in the Neonatal Intensive Care Unit and is associated with increased mortality and morbidity. We report a case of a neonate with congenital heart disease who developed acute kidney injury after cardiac surgery, administration of iodinated contrast media for cardiac catheterization, and a combination of nephrotoxic drugs. Case report: A term neonate without a prenatal diagnosis of congenital heart disease and with a good postnatal transition was transferred at 13 days of life to the MS Curie Emergency Hospital for Children, Newborn Intensive Care Unit, from a regional hospital where he was admitted at 10 days of life with severe general status, respiratory distress, cyanosis, and arterial hypotension. The cardiac ultrasound detected critical aortic valve stenosis, hypoplastic descending aorta, acute heart failure, and pulmonary hypertension. The patient was intubated and mechanically ventilated and received antibiotherapy (meropenem, vancomycin, and colistin), inotropic and vasoactive support (epinephrine, norepinephrine, dopamine, and milrinone), and diuretic support (furosemide, aminophylline, and ethacrynic acid). A balloon aortic valvuloplasty was performed several hours after admission, but after two days the patient required reintervention by open heart surgery due to relapsing severe aortic stenosis. He developed oligo-anuria, generalized edema, and altered renal function tests on the second postoperative and fourth day post-contrast media administration. Continuous renal replacement therapy was initiated for 75 h, leading to almost instant improvement in blood pressure, then diuresis and creatinine levels. The patient required long-term treatment for heart, respiratory, and liver failure. He was discharged at almost four months of age with normal renal function tests, blood pressure, and good urine output without diuretic support. The literature review indicates that contrast-associated acute kidney injury (CA-AKI) requiring continuous renal replacement therapy is rare. Conclusions: Our current case proves that iodinated contrast media administration in a neonate with concomitant insults, such as cardiac surgery for a specific pathology, aortic stenosis, coarctation, arch stenosis, arterial hypotension, and administration of nephrotoxic drugs, may lead to severe kidney injury.

Background: Neonates have an increased risk of vitamin D insufficiency due to the inadequate supplementation of mothers and infants after birth. Insufficiency of vitamin D is frequently detected in critically ill patients and is associated with disease severity and mortality. There is yet to be a consensus on the appropriate regimen of vitamin D3 supplementation in high-risk infants. Aim: The main objectives of this study were to determine the prevalence of vitamin D insufficiency in neonates with severe comorbidities and to evaluate whether high-dose vitamin D3 oral administration leads to normal plasmatic concentrations without side effects. Methods: The current study was a randomized, prospective trial of 150 patients admitted to the Neonatal Intensive Care Unit (NICU) at Maria Sklodowska Curie Emergency Children’s Hospital in Bucharest. Patients were divided into three subgroups based on the chronological order of their admission date. Each subgroup received a different pharmaceutical product of vitamin D3. We administered a dosage of 10,000 IU/kg of vitamin D3 orally in three steps, as follows: at admission, one week after admission, and one month from the first administration, targeting a serum 25-hydroxyvitamin D concentration of at least 40 ng/mL. Results: Most neonates (68%) achieved an optimum vitamin D level after one month, even though only 15% of patients had an optimum concentration at admission. After the first high dose of vitamin D3, there was a 27% increase in the mean vitamin D plasmatic level compared to admission levels. However, after one month, the concentrations decreased in all subgroups due to the gap of three weeks between the last two administrations. Conclusions: An intermittent, weekly high-dose vitamin D3 oral administration leads to a steadier increase and normalization of vitamin D concentration in most critically ill neonates. However, high-dose vitamin D3 administered orally after three weeks decreases vitamin D levels in this high-risk population.

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.

The main goal of surgical treatment for gastroschisis and omphalocele is the reduction of viscera in the abdominal cavity and closure of the abdomen, but the challenge is to succeed without the detrimental effects of increased intraabdominal pressure. In this regard, we performed a retrospective study for all patients admitted for gastroschisis and omphalocele to the Neonatal Intensive Care Unit of ‘Marie Sklodowska Curie’ Emergency Clinical Hospital for Children, from January 2011 until June 2021. Our aim was to highlight the presence of postoperative abdominal compartment syndrome. We observed that six out of forty-seven patients developed clinical signs of abdominal compartment syndrome, five associated with primary closure and one with staged closure with a polyvinyl chloride patch. Following the results, we decided to implement the trans-bladder measurement of intraabdominal pressure to avoid closing the abdomen at pressures higher than 10 mmHg in order to prevent the development of abdominal compartment syndrome. We consider that there is still place for the improvement of congenital abdominal wall defects management and that the measurement of intraabdominal pressure might help us reach our goal.

We present the case of a newborn diagnosed with perinatal asphyxia and secondary renal injuries, transposition of the great vessels and low systemic blood flow, treated with Prostaglandin, atrioseptostomy, followed by arterial switch surgery After the cardiac surgery the patient is oliguric and requires hemodiafiltration for 12 days, after which renal function is restored. In evolution, however, AVB (atrioventricular block) grade III occurs, followed by implanta-tion of permanent pacemaker, but another postoperative complication – chylothorax – leads to stopping electrical stimulation followed by severe cardiac dysfunction and, consequently, recurrent renal injury and anuria. Re-estab-lishing hemodiafiltration for another 7 days without recovery of renal function. Perinatal asphyxia, cardiac heart disease with low systemic blood flow, prostaglandin, atrioseptostomy, cardiac rhythms disturbances, chylothorax, sepsis, cardiac arrest are intriguing factors that bring renal injury. Their association greatly decreases the chance of survival even if the patient benefits from supportive treatment and early hemodiafiltration.

Prezentăm cazul unui nou-născut diagnosticat cu suferintă perinatală și injurie renală secundară, transpoziție de vase mari și debit sistemic scăzut, tratat cu prostaglandină, atrioseptostomie, urmate de operația de switch arterial. Postoperator, pacientul este oliguric și necesită hemodiafiltrare pentru 12 zile, după care funcția renală este resta-bilită. În evoluție, apare bloc atrioventricular (BAV) grad III, urmat de implantare de stimulator cardiac permanent, dar o altă complicație postoperatorie – chilotorax – duce la oprirea stimulării electrice, urmată de disfuncție cardia-că severă și, consecutiv, injurie renală recurentă și anurie. Se reinstituie hemodiafiltrarea pentru încă 7 zile, fără recuperarea funcției renale. Asfixia perinatală, malformația cardiacă cu debit sistemic scăzut, prostaglandina, atri-oseptostomia, circulația extracoroporeală, tulburările de ritm, chilotoraxul, sepsisul, stopul cardiac sunt factori intri-caţi care aduc injurie renală importantă. Asocierea lor scade major șansele de supraviețuire chiar dacă pacientul beneficiază de tratament suportiv și hemodiafiltrare precoce.

Introduction. Obtaining vascular access that allows long-term hemodialysis of children with stage 5D chronic kidney disease is a priority in the management of these patients. Complications of central venous catheters and arteriovenous fistula are directly correlated with the morbidity and quality of life of these children. The objective of the study was to assess complications of vascular access in children on long-term hemodialysis by arteriovenous fistula or central venous line. Methods. We followed 40 patients in the hemodialysis program for a period of 18 months; in 22, vascular access was by arteriovenous fistula, and 18 were dialyzed on a central venous line. Results. All 22 patients were accepted for AVF at a vein diameter of at least 3 mm, and this probably contributed to the high rate of primary maturation (86%). In the majority of these patients (64%) was brachiocephalic fistula versus radiocephalic (18%), in most cases the decision being supported by small diameters of radial artery and distal cephalic vein. Three of the arteriovenous fistulas performed had a primary failure, and one a secondary failure; 2 of the patients with arteriovenous fistula failure had to be subsequently dialyzed by central venous line and one by peritoneal dialysis, while the fourth benefited from a new arteriovenous fistula. Six patients developed fistula aneurysms, one of them evolving into a pseudoaneurysm with spontaneous rupture and hemorrhagic shock and requiring emergency ligation. Two patients developed significant vein stenosis in the proximity of the anastomosis. All 6 patients with aneurysmal fistula had hypertension, and 3 of them had thrombotic venous stenoses/occlusion in the main deep veins upstream of the fistula in the context of prolonged use of central venous lines in the jugular veins and subclavian veins. The average age of the children was 13.35 years (range 9-18 years) and the average weight was 38.6 kg (range 23-58 kg). Of the patients on dialysis on central venous lines, 76% (16 patients) had at least one episode of sepsis that required hospitalization, and all patients who were dialyzed on the catheter (from the beginning or after fistula failure) experienced at least one episode of vein thrombosis that required catheter change. In 8 (38%) of the 21 patients using central venous lines, thrombotic occlusion/stenosis of at least one persistent deep vein was observed at 3 months after the removal of the central venous line from the venous axis. Conclusions. Complications of venous access in children with hemodialysis are very common and have a major impact on the morbidity and quality of life of these patients. In this context, a multidisciplinary team (pediatric nephrologist, specialist in vascular imaging, and vascular surgeon) is needed to follow these patients in order to prevent and treat these complications.

Congenital heart disease (CHD) remains a predisposing cardiac condition for infective endocarditis (IE). Case report: We present the case of 8-year-old boy with no known pre-existing cardiac disease diagnosed with infective endocarditis (IE) with Gemella sanguinis. After admission, he underwent transthoracic echocardiography (TTE), which revealed the presence of Shone syndrome with a bicuspid valve, mitral parachute valve and severe aortic coarctation. He developed a paravalvular aortic abscess with severe aortic regurgitation and left ventricle (LV) systolic dysfunction for which he required a complex surgical intervention after six weeks of antibiotic treatment, consisting of Ross operation and coarctectomy, with a complicated postoperative course, cardiac arrest and ECMO support for five days. The evolution was slow and favorable, with no significant residual valvular lesions. However, persistent LV systolic dysfunction and increased muscle enzymes required further investigation to establish a genetic diagnosis of Duchenne disease. As Gemella is not considered a frequent pathogen of IE, no current guidelines refer specifically to it. Additionally, the predisposing cardiac condition of our patient is not currently classified as “high-risk” for IE; this is not considered an indication for IE prophylaxis in the current guidelines. Conclusion: This case illustrates the importance of accurate bacteriological diagnosis in infective endocarditis and poses concerns regarding the necessity of IE prophylaxis in “moderate risk” cardiac conditions such as congenital valvular heart disease, especially aortic valve malformations.

Brain abscesses are a possible complication of bacterial sepsis or central nervous system infection but are uncommon in the neonatal period. Gram-negative organisms often cause them, but Serratia marcescens is an unusual cause of sepsis and meningitis in this age group. This pathogen is opportunistic and frequently responsible for nosocomial infections. Despite the existing antibiotics and modern radiological tools, mortality and morbidity remain significant in this group of patients. We report an unusual unilocular brain abscess in a preterm neonate caused by Serratia marcescens. The infection had an intrauterine onset. The pregnancy was achieved through assisted human reproduction techniques. It was a high-risk pregnancy, with pregnancy-induced hypertension, imminent abortion, and required prolonged hospitalization of the pregnant woman with multiple vaginal examinations. The infant was treated with multiple antibiotic cures and percutaneous drainage of the brain abscess associated with local antibiotic treatment. Despite treatment, evolution was unfavorable, complicated by fungal sepsis (Candida parapsilosis) and multiple organ dysfunction syndrome.

(1) Background: Transposition of the great arteries (TGA) is the most common congenital heart disease, accounting for 5–7% of all cardiac anomalies, with a prevalence of 0.2–0.3 per 1000 live births. (2) Aim: Our main objectives were to evaluate the clinical safety of balloon atrial septostomy in neonates and the possible complications. Furthermore, we tried to establish whether the procedure should be performed in all TGA patients with small atrial septal defects, regardless of oxygen saturation, within a center where corrective surgery cannot be performed on an emergency basis due to the lack of a permanent cardiac surgery team for arterial switch surgery. (2) Methods: We conducted an observational, retrospective, single tertiary-care center study between January 2008 and April 2022, which included 92 neonates with TGA transferred to our institution for specialized treatment. (3) Results: The median age at the time of the Rashkind procedure was four days. The rate of immediate complications after balloon atrial septostomy (BAS) was high (34.3%), but most were transient (metabolic acidosis and arterial hypotension—21.8%). Twenty patients with TGA managed in our hospital underwent definitive and corrective surgical intervention (arterial switch operation) at a median age of 13 days. Most patients (82.6%) were term neonates, but 16 were born preterm. (4) Conclusions: Urgent balloon atrial septostomy is often the only solution to restore adequate systemic perfusion. Bedside balloon atrial septostomy is a safe, effective, and initial palliative intervention in neonates with TGA, which can be performed in the neonatal unit.

One of the main markers of arterial stiffness is pulse wave velocity (PWV). This parameter is well studied as a marker for end-organ damage in the adult population, being considered a strong predictor of major cardiovascular events. This study assessed PWV in children with chronic kidney disease (CKD) as a marker of cardiovascular risk. We conducted a prospective observational single-center cohort study of 42 consecutively pediatric patients (9–18 years old) with terminal CKD and dialysis, at the Hemodialysis Department of the “M. S. Curie” Hospital, Bucharest. We measured PWV by echocardiography in the ascending aorta (AscAo) and the descending aorta (DescAo), and we correlated them with left ventricular hypertrophy (LVH). Fifteen patients (35.7%) presented vascular dysfunction defined as PWV above the 95th percentile of normal values in the AscAo and/or DescAo. Cardiac disease (LVH/LV remodeling) was discovered in 32 patients (76.2%). All patients with vascular damage also had cardiac disease. Cardiac damage was already present in all patients with vascular disease, and the DescAo is more frequently affected than the AscAo (86.6% vs. 46.9%). Elevated PWV could represent an important parameter for identifying children with CKD and high cardiovascular risk.

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.

We present the first successful case of extracorporeal membrane oxygenation (ECMO) at „M.S. Curie” Emergency Clinical Hospital for Children, Newborn Intensive Care Unit: a term neonate with cardiorespiratory failure secondary to left sided congenital diaphragmatic hernia. The patient was placed on veno-venous ECMO at three days of life; however, on the fourth day, due to unstable right ventricular function, conversion to veno-arterial ECMO was mandatory. At one week of life, the patient was operated on-site for diaphragmatic hernia without ECMO support and then reintroduced on ECMO immediately after the surgical procedure, being on ECMO support for a total of 8 days. Antithrombotic treatment was administered for significant occlusion of the right common carotid artery and right internal jugular vein (complication of the cannulation for ECMO) and also long term treatment for Persistent Pulmonary Hypertension was needed. The patient was discharged at the age of four months with moderate neurodevelopmental delay. The literature review indicates that neonatal ECMO procedure in Romania is still in its early stages. Despite this state, our current case proves that ECMO can be successfully performed with increased chances of survival for neonates with severe prognosis after failure of conventional therapy.