Outcome of Neonates with Vein of Galen Malformation Presenting with Severe Heart Failure: A Case Series.

BACKGROUND: Neonatal presentation of vein of Galen aneurysmal malformations (VGAMs) complicated by cardiac failure and pulmonary hypertension is frequently associated with a poor prognosis. Interventional neuroradiology with embolization can offer a chance for survival, although neurological damage can represent a limitation. OBJECTIVE: This article determines if aggressive intensive care and drug management of cardiac failure before urgent embolization can influence morbidity and mortality. PATIENTS AND METHODS: Twelve infants (7 boys, 5 girls) were diagnosed with symptomatic vein of Galen malformations in the neonatal period during the period 2000 to 2014. Due to high output cardiac failure, endovascular treatment was attempted as soon as stabilization was achieved. RESULTS: Endovascular procedures successfully reverted cardiac failure in 5 patients who survived without significant neurological damage, while in 7 patients the causes of death were refractory cardiac failure, multiorgan failure, and severe brain damage. Bidimensional echocardiography assessment was performed at presentation and after early embolization procedures. CONCLUSION: Aggressive intensive care approach to heart failure and pulmonary hypertension leading to early neurointervention results in good survival rates with low morbidity even in cases of high-risk neonatal VGAM. Combined hemodynamic treatment can improve outcome in neonates with cardiac failure secondary to VGAM, although there is the risk of precipitating systemic hypoperfusion and renal failure. A moderate prematurity may not prevent both interventional approach and good outcome.

Neonatal emergencies associated with cardiac rhabdomyomas: an 8-year experience.

During the foetal-neonatal period, rhabdomyomas represent the majority of cardiac tumours and are closely associated with tuberous sclerosis. Cardiac rhabdomyomas may be completely asymptomatic and are incidentally discovered during an echocardiogram, or may cause cardiac dysfunctions requiring medical and/or surgical intervention. During foetal life and the early neonatal period, life-threatening conditions, mostly due to arrhythmias, cardiac failure or obstruction, do occur on rare occasions. We reviewed the medical records of all cases of cardiac rhabdomyomas diagnosed prenatally or postnatally over an 8-year period. The present study reviews 7 cases of life-threatening conditions. Arrhythmic episodes were described in 5 patients, and blood flow obstruction was reported in 2 cases. Antiarrhythmic agents successfully controlled the clinical and electrophysiological conditions. Obstructive conditions were associated with poor outcomes. In conclusion, when prenatal diagnosis of rhabdomyoma is made, appropriate planning at delivery for the management of potential haemodynamic complications may prevent adverse neonatal outcomes. The clinical outcome is more influenced by obstructive rather than by dysrhythmic complications. Appropriate antiarrhythmic treatment is of primary importance. In all cases discovered through prenatal and/or neonatal life-threatening conditions, an accurate follow-up should always be performed to anticipate the development of tuberous sclerosis.

Neonatal Life-Threatening Nonoliguric Hyperkalemia Under Therapeutic Hypothermia.

To illustrate our experience with two cases of neonatal life-threatening hyperkalemia during therapeutic hypothermia (TH) despite a normal acid-base status, urine output, and preserved renal function. Clinical cases are presented from Pediatric Intensive Care Unit (PICU) admission to the onset of the hyperkalemia, with related complications and after resolution. Similar cases were not retrieved from a critical review of pertinent literature. Severe hyperkalemia pathophysiology and risk factors have been debated. Two full-term adequate for weight female neonates were admitted to PICU because of perinatal asphyxia who underwent TH. Prenatal history was completely uneventful, nor hereditary genetic conditions were reported; moreover, long-term follow-up ruled out any metabolic or renal disease. Despite an accurate evaluation of previous clinical series and literature on TH and perinatal asphyxia, these hyperkalemic episodes remain unexplained. The hypoxic-ischemic insult may affect multiple organs, mainly central nervous system, heart, lung, and kidneys; acute muscle breakdown and consequent rising of myoglobin may also have a precipitating role in acute kidney failure (AKF) and hyperkalemia. Electrolyte imbalance is a possible finding as a consequence of combined cell injury and AKF. In contrast, an isolated severe hyperkalemia is exceedingly rare in nonoliguric neonates.

Fontaine-Farriaux syndrome: a recognizable craniosynostosis syndrome with nail, skeletal, abdominal, and central nervous system anomalies.

Craniosynostosis is an etiologically heterogeneous malformation, which may present as an isolated finding or in association with other anomalies. The concurrence of craniosynostosis together with specific central nervous system, abdominal, genital, and limb malformations defines the Fontaine-Farriaux syndrome, described so far in only two patients. We report on a stillborn who mainly presented severe intrauterine growth retardation, bilateral coronal synostosis, generalized nail hypo/aplasia more evident on the posterior side, tapered digits, mild cutaneous syndactyly, abdominal muscle hypoplasia, micropenis and bilateral cryptorchidism. Skeletal radiographs revealed universal platyspondyly and necropsy findings comprised intestinal malrotation, abnormal cortical gyral formation, periventricular heterotopia, and cerebellar hypoplasia. Comparison between the present and the two previously described patients demonstrates that our case shows a combination of features strikingly resembling the original description. Conversely, the second reported patient shows a very atypical phenotype and is, most probably, affected by a distinct clinical entity. The triad of craniosynostosis, anonychia, and abdominal muscle hypo/aplasia emerges as the most consistent core phenotype, although skeletal and brain anomalies are relevant ancillary findings. An in-depth differential diagnosis with other partially overlapping conditions is carried out.

Massive blood transfusion in choroid plexus tumor surgery: 10-years' experience.

STUDY OBJECTIVE: To describe our 10 years of experience with childhood choroid plexus tumors (CPTs). DESIGN: Retrospective chart analysis. SETTING: Operating room and pediatric intensive care unit (PICU) of a university hospital. PATIENTS: 18 infants and children undergoing CPT surgery from 1995 to 2004, 11 of whom were younger than 12 months. MEASUREMENTS: Perioperative hematologic and coagulation data were measured as well as estimated red cell volume variations (as a reliable index of blood loss) in the perioperative period, together with coagulation parameters. RESULTS: Greater blood loss was recorded in the infant group vs older children (percentage of estimated red cell volume loss, 1.31 +/- 1.79% vs 0.20 +/- 0.17% [P < 0.01] and 1.50 +/- 1.86% vs 0.29 +/- 0.21% [P < 0.01] on PICU admission and after 72 hours, respectively). Platelet count decrease was similarly noted (51.60 +/- 28.06 vs 27.57 +/- 11.98, P < 0.05, as percentage of preoperative count). Patients operated on in the neonatal period showed the highest blood loss and related coagulation impairment. CONCLUSION: Younger CPT surgery patients present an increased risk versus their older counterparts of massive bleeding resulting in hemodynamic instability and coagulative impairment.

Sharing features of uncommon respiratory syncytial virus complications in infants.

We describe 4 nonconsecutive cases of infants admitted to Catholic University pediatric intensive care unit (PICU) because of complicated respiratory syncytial virus (RSV) infection during winter RSV outbreaks from the year 2000 to the year 2003. A hyponatremic epileptic status (as in the first case) has been reported by several authors as a rare RSV complication, potentially leading to death. The second infant developed a serious pulmonary edema after a subglottic obstruction (croup) associated with RSV infection. The remaining 2 infants developed a pneumothorax and subcutaneous emphysema while breathing spontaneously during an RSV bronchiolitis. In all infants, a full recovery and PICU discharge was achieved despite the need for mechanical ventilation in cases 1 and 2. Increased intrapleural negative pressure or its combination with hypoxia/hypercapnia has been suggested as the common factor possibly joining these different clinical pictures.

Lung injury from "liquid ecstasy": a role for coagulation activation?

We describe our second clinical observation of pulmonary injury after a "liquid ecstasy" ingestion/inhalation by a 3-year-old girl. Apart from the deep coma state, a markedly asymmetric pulmonary compromise was recorded. A transient coagulation activation was detected, possibly triggered off by the toxic effect on the lung alveolar-capillary membrane.

Genotypic analysis by 27A DNA fingerprinting of Candida albicans strains isolated during an outbreak in a neonatal intensive care unit.

We describe an outbreak of Candida albicans systemic infection involving five premature infants in a neonatal intensive care unit. Molecular and epidemiologic characterization of all C. albicans isolates was performed by DNA fingerprinting with the 27A probe. This genotypic analysis demonstrated that the isolates were identical, providing evidence for the circulation of a unique C. albicans strain.

Nebulized iloprost and noninvasive respiratory support for impending hypoxaemic respiratory failure in formerly preterm infants: a case series.

OBJECTIVE: To describe a series of ex-preterm infants admitted to pediatric intensive care unit due to impending hypoxaemic respiratory failure complicated by pulmonary hypertension (PH) who were treated electively combining noninvasive ventilation (NIV) and nebulized iloprost (nebILO). DESIGN: Open uncontrolled observational study. SETTING: Pediatric Intensive Care Unit, University Hospital. PATIENTS: Ten formerly preterm infants with impending hypoxaemic respiratory failure and PH, of whom eight had moderate to severe bronchopulmonary dysplasia. MEASUREMENTS AND MAIN RESULTS: Median age and body weight were 6.0 (2.75-9.50) months and 4.85 (3.32-7.07) kg, respectively. We observed a significant early oxygenation improvement in terms of PaO(2) /FiO(2) increase (P = 0.001) and respiratory rate reduction (P = 0.01). Hemodynamic also improved, as shown by heart rate (P = 0.002) and pulmonary arterial pressure systolic/systolic systemic pressure (PAPs/SSP) ratio reduction (P = 0.0137). NebILO was successfully weaned in positive response cases: 4 infants were discharged on oral sildenafil. Three patients failed noninvasive modality and needed invasive mechanical ventilation; hypoxic-hypercarbic patients were most likely to fail noninvasive approach. Only one patient requiring invasive ventilation died and surviving babies had a satisfactory 1-month post-discharge follow-up. CONCLUSIONS.: The noninvasive approach combining NIV and nebILO for ex-preterm babies with impending respiratory failure and PH resulted to be feasible and quickly achieved significant oxygenation and hemodynamic improvements.

Levosimendan in two neonates with ischemic heart failure and pulmonary hypertension.

We report the successful and safe use of levosimendan, a new calcium-sensitizing agent with positive inotropic and vasodilatory action, in 2 critically ill term newborns with acute heart failure and pulmonary hypertension in the absence of any underlying heart malformation and/or previous cardiosurgical procedures. During the neonatal period, levosimendan may represent an ideal drug for immature myocardium characterized by a higher calcium-dependent contractility than in adults.

Cardiac tamponade and bilateral pleural effusion in a very low birth weight infant.

A very low birth weight premature newborn developed a sudden cardiac tamponade due to parenteral fluid extravasation 1 month after central line insertion. Besides tensive pericardial effusion a bilateral pleural effusion also developed. An emergency pericardiocentesis was really life-saving and after pleural fluid removal a complete cardiorespiratory recovery was obtained. Pericardial and bilateral pleural effusions very rarely coexist as a complication of central line extravasation.

Noninvasive ventilation options in pediatric myasthenia gravis.

A 10-month-old female infant with congenital myasthenic syndrome suffering from acute respiratory failure was supported using face mask positive pressure ventilation until definitive diagnosis and specific treatment was achieved. A 12-year-old girl suffering from seronegative myasthenia gravis was treated by helmet-delivered noninvasive ventilation during recurrent myasthenic episodes. Noninvasive support was really beneficial in the myasthenic crisis with respiratory muscle weakness, whereas a shift to tracheal intubation was necessary when pulmonary infection and multiple atelectasis occurred. The new helmet interface for noninvasive positive pressure ventilation can represent a valuable means of respiratory support in the early phase of respiratory failure in older children.

Fatal coronary artery anomaly presenting as bronchiolitis.

UNLABELLED: During winter outbreaks of respiratory syncytial virus bronchiolitis from 2002 to 2004, three infants presented with a presumptive diagnosis of lower respiratory tract infection and wheezing. The clinical condition in two cases was rapidly progressive and precipitated into intractable shock; clinical and instrumental examinations revealed a cardiac origin of their illness. A subacute presentation permitted a cardiological assessment and a proper treatment in the third infant. An abnormal origin of the left coronary artery from the pulmonary trunk was demonstrated in all cases. The concurrent acute airway infection had a catastrophic effect on the underlying cardiovascular anomaly leading to refractory cardiogenic shock and death. CONCLUSION: Admission chest X-ray film and arterial gas analysis can raise the suspicion of cardiac involvement when treating a severe wheezing episode in young infants. Paediatric cardiological evaluation with two-dimensional echocardiography may eventually reveal this rare condition, whereas cardiac catheterisation with aortography remains the standard means of diagnosis.

Massive congenital intracranial teratoma: perioperative coagulation impairment.

Massive congenital intracranial teratoma is a rare neoplasm with a poor prognosis. Surgery may be curative only if complete resection can ben obtained. Several single case reports have been published in the pediatric literature, mostly focusing on prenatal diagnosis. The authors describe six patients with congenital intracranial teratoma treated at their institution in the past decade. Perioperatively, a marked hemostatic derangement was observed in three of them undergoing surgery: the pathophysiology of this complication is discussed. The surgical indication itself represents an ethical dilemma when treating a large intracranial tumor with partial destruction and replacement of brain structures.