Congenital High Airway Obstructive Syndrome (CHAOS) Survival of a Newborn with Laryngeal Atresia.

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.

Consistent Correlation between MTHFR and Vascular Thrombosis in Neonates-Case Series and Clinical Considerations.

BACKGROUND: MTHFR polymorphism has been inconsistently linked to thrombotic events-some studies have shown its contribution to venous thrombosis, arterial thrombosis, and ischemic stroke, whereas others have found no statistically significant correlation between them. METHODS: A descriptive case series study was performed in the Neonatal Intensive Care Unit of "Marie Sklodowska Curie" Emergency Clinical Hospital for Children in Bucharest, Romania. RESULTS: All patients had positive results for MTHFR variants; 14 patients were positive for compound heterozygosity, 13 patients for MTHFR C677T (seven of which were homozygous), and 13 patients for MTHFR A1298C (three of which were homozygous). Eighteen patients received anticoagulants (heparin, enoxaparin, or bivalirudin), and thrombolytics (alteplase) were administered in six cases. In one case, a thrombectomy was performed; in another, vascular plasty was undertaken. Only in six cases was complete revascularization possible. Incomplete revascularization occurred for one patient with a negative outcome. CONCLUSION: The particularity of this case series is that every patient in our unit who developed thrombi had a positive genetic result for MTHFR mutations. MTHFR mutations should be regarded as a thrombotic risk factor for critically ill patients, and screening for MTHFR mutations should be performed in every admitted patient to intensive care units, thus achieving the prevention of thrombi.

Neonatal Brain Abscess with Serratia marcescens after Intrauterine Infection: A Case Report.

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.

Antiproliferative therapy with sirolimus and propranolol for congenital vascular anomalies in newborns (Case reports).

We present a series of four newborns diagnosed with complicated congenital vascular anomalies, with different localization: Congenital lymphatic malformation (CLM) on the left hemithorax extending on the left upper limb; congenital hepatic hemangioma (CHH) with important complications in the first 7 weeks of life; Kaposiform hemangioendothelioma (KHE) of the left lower limb complicated with Kasabach Merritt phenomenon (KMM) and most probable diffuse capillary malformation with overgrowth (DCMO). All patients were treated with combined antiproliferative therapy with sirolimus and propranolol. The initial dose of sirolimus was 0.45-0.5 mg/m2 with doses adjusted according to plasmatic levels. Therapeutic intervals of sirolimus were considered at plasmatic levels of 7-12 ng/ml. Our aim was to use the lowest therapeutic dose in order to avoid possible side effects. Propranolol was initiated in doses of 0.5-1.0 mg/kg/day and was increased up to 3.0 mg/kg/day depending on tolerability. Following two months, every patient showed a marked reduction in the size of the mass, improvement in overall appearance or even calcification in the liver vascular tumor. No patient showed life threatening side effects to the treatment. Hypertriglyceridemia was the only side effect noted in all patients. This is in accordance with several international studies, which try to demonstrate the importance of sirolimus in neonatal vascular malformations in monotherapy or combined with different drugs.

Evolutionary Particularities in a Case of Severe Pneumonia in Children - Case Report.

Necrotizing pneumonia remains an uncommon complication of pneumonia in children, but its incidence is increasing. Pneumococcal infection is the predominant cause of severe necrotizing pneumonia in children, but methicillin resistant Staphylococcus aureus (MRSA) and Panton-Valentine leukocidin (PVL) staphylococcal infections are also important. We present the case of a four-year-old girl,with an unremarkable medical history, who was admitted in our hospital with a history of high fever, productive cough and tachypnea lasting for 10 days, progressive worsening despite empirical oral antibiotic. Following physical examination, laboratory investigations and thoracic radiography, we established the diagnosis of left lower lobe pneumonia with parapneumonic effusion, acute respiratory failure and sepsis. Medical treatment with systemic antibiotics was initiated, but the evolution was unfavorable. Seriated chest X-rays and also high resolution computed tomography with contrast of the lung were performed, revealing the progression to extensive necrotizing pneumonia with multiple cystic lesions causing right mediastinal deflection. The parenteral broad spectrum antibiotic regimen was adjusted, still with unfavorable evolution, requiring surgical treatment (left inferior lobectomy and pleural draining). Postoperatively, recovery was uneventful. The patient was discharged with clinical and laboratory improvement of his condition, a repeated chest X-ray showing good expansion of upper left parenchyma.

Allergic bronchopulmonary aspergillosis in patient with cystic fibrosis - a case report.

Asthma with allergic bronchopulmonary aspergillosis (ABPA), a hypersensitivity disease of the lungs due to an immune response to Aspergillus fumigattus (Af) antigens, is rarely seen in children, other than complicating cystic fibrosis. We present the case of a 14 - year- old female teenager with cystic fibrosis (CF), admitted in our hospital with respiratory failure and persistent cyanosis. Chest X-ray showed perihilar and upper lobes pulmonary infiltrates. Her airway cultures were positive for methicillin resistant staphilococcus aureus (MRSA) and non-mucoid Pseudomonas aeruginosa. She was prescribed intravenous antibiotherapy with ceftazidime and vancomycine (to which MRSA and Pseudomonas aeruginosa were susceptible). Pulmonary function testing (PFT) revealed severe obstructive lung disease. After ten days of intravenous antibiotics and first five days of corticosteroid, the patient's FEV1 was 68% of predicted. Total serum IgE and IgE antibodies to Aspergillus fumigatus were elevated. These results raised the possibility of allergic bronchopulmonary aspergillosis (ABPA). The possibility of ABPA should be considered in all pulmonary exacerbation and in order to determine if ABPA is developing or if an exacerbation is occurring, a serial monitoring of IgE levels should be performed.

Prostaglandin e1 on infradiaphragmatic type of total anomalous pulmonary venous connection - a case report.

We present the case of a newborn with severe pulmonary hypertension, diagnosed with infradiaphragmatic type of total anomalous pulmonary venous connection (TAPVC). The onset was in the first 10 days of life. Diagnosis was made by echocardiography and AngioCT. The pulmonary venous collector was surgically implanted into the left atrium in Germany, but the next month after surgery he developed cardiopulmonary insufficiency and died several days later. We would like to emphasize the importance of prostaglandin E1 administration in this particular case of infradiaphragmatic type of TAPVC and its usefulness in patient's stabilization until surgery. The prognosis in TAPVC, infradiaphragmatic type, is poor and is related mainly to the existence of pulmonary venous obstruction.