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• W2555119428 abstract "A 36-week appropriate-for-gestational age male was born with hydrops fetalis that was not diagnosed prenatally. The birth hospital had a Level II nursery.Pertinent points in the history include:Born to a 24-year-old G2P0010 (previous first-trimester spontaneous abortionwoman by emergency cesarean section due to failure to progress and decreased fetal heart rate variability.Preterm labor occurred 1 week prior to delivery, but was stopped successfully with tocolysis, and the mother was sent home after 6 hours of observation. Rupture of membranes occurred 6 hours prior to delivery with clear fluid.At delivery, the infant appeared grossly edematous, dusky, and floppy, with no spontaneous respiratory effort. Endotracheal intubation was difficult because of airway edema. Resuscitation consisted of positive pressure ventilation, chest compressions, and epinephrine administration via an umbilical venous catheter.A chest radiograph showed bilateral pleural effusions. A kidney-ureter-bladder (KUB) radiograph showed ascites. Pleurocentesis released 45 mL of straw-colored fluid from the left side of the chest, but none from the right side of the chest. A total of 15 mL of straw-colored fluid was removed from the abdomen via paracentesis.On physical examination, the infant exhibited gross body edema but no dysmorphic features. A II/VI systolic murmur was heard at the left sternal border. The liver was palpable 3 cm below the costal margin. There was mild glandular hypospadias with a minimal chordee, but testes were descended bilaterally.The infant developed seizures and received a loading dose of 20 mg/kg of phenobarbital before being transferred to a Level III nursery.The infant was placed on a high-frequency oscillating ventilator, and bilateral chest tubes were placed, which drained copious amounts of serous fluid.Initial C-reactive protein concentration was 3.8mg/dL, and the infant was placed on antibiotics for 1 week. All cultures (blood, pleural fluid, ascites fluid) showed no growth of bacteria.Initial chemistry values and results of liver function tests were within normal limits.Urine was negative for reducing sugars and ketones. Urine cytomegalovirus culture was negative.Toxoplasmosis IgG was negative, but IgM was positive.Chromosomal studies documented a normal 46,XY karyotype.Head ultrasonography showed a large, high-flow vascular structure within the midline of the brain extending to the transverse sinus posteriorly and possibly a small right choroid plexus hemorrhage.Abdominal ultrasonography showed moderate intra-abdominal ascites with normal liver, spleen, pancreas, and kidneys.Echocardiography revealed severe right ventricular hypertension with right-to-left ductal shunting. Right-to-left shunting also was documented at the atrial level, suggesting right ventricular diastolic dysfunction. Retrograde flow was seen in the distal aortic arch during diastole.The infant suffered from severe hypotension requiring a continuous dopamine infusion and multiple doses of fresh frozen plasma.Magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) of the head was ordered, but could not be performed immediately due to the infant’s clinical instability. It was performed on postnatal day 3.Cardiovascular systemChromosomal abnormalityAnemiaInfection Vascular obstructionLymphatic obstructionMetabolic diseaseGenitourinary malformationIdiopathicVein of Galen malformation causing severe cardiac failure and hydrops fetalis.High-flow arteriovenous shunts of the brains of young children are among the most challenging conditions treated in modern medicine. In the neonatal and infantile age groups, the most common type of arteriovenous malformation in the brain is the vein of Galen malformation (VGM). Approximately 40% to 60% of pediatric cases of VGM present in the neonatal period and occur with a male-to-female ratio of 3:1.The essential feature of the VGM is aneurysmal dilation of the venous structure generally characterized as the vein of Galen (although careful anatomic studies have suggested that the median prosencephalic vein of Markowski, a transitory venous structure that normally disappears by the 11th week of gestation, is the vein involved). The most common feeding arterial vessels into this dilated venous structure are, in order of frequency, the posterior choroidal artery, anterior cerebral artery, middle cerebral artery, anterior choroidal artery, and posterior cerebral artery. The cause of VGMs is unknown, but an early insult (between the 6th and 11th week of fetal development) that results in a somatic mutation in neural crest or adjacent cephalic mesoderm in the early embryo could cause such vascular abnormalities.The associated neuropathologic findings observed with VGM consist of a variety of ischemic, hemorrhagic, and mass effects of the malformation. The ischemic phenomena are related primarily to a combination of intracranial “steal” phenomena, caused by the absence or even the reversal of diastolic cerebral blood flow, and congestive heart failure. The heart failure is caused by the remarkably high cardiac output due to both the marked decrease in cerebrovascular resistance and the increased venous return to the heart and by marked cardiac ischemia. Cardiac ischemia results from decreased coronary blood flow, which normally occurs during diastole, because of the low diastolic pressure caused by the VGM.The consequences of a VGM in the developing brain differ from those seen in the adult, principally because of the immature cerebral venous system. The arachnoid granulations by which cerebrospinal fluid returns to the cerebral venous sinuses are not fully mature until 16 to 18 months of age. In infancy, cerebrospinal fluid is reabsorbed across the ventricular ependyma and brain parenchyma into the medullary veins. The presence of a large VGM raises venous sinus pressure, which subsequently is transmitted to the cortical and finally the medullary veins. This results in water congestion of the brain parenchyma and impaired oxygenation that leads to subependymal atrophy and, in severe cases, a progressive “melting brain syndrome.”VGMs occasionally are detected on antenatal ultrasonography (from about 25 weeks’ gestation) as cystlike structures in the region of the posterior fossa. Color Doppler facilitates diagnosis by showing high flow in the structure. Antenatal MRI can confirm the diagnosis (differentiating VGM from other arteriovenous malformations) and allows for assessment of any pre-existing damage to the brain. Once diagnosed, the mother should be referred for fetal echocardiography to analyze the degree of heart failure, and delivery should be planned at a center that has appropriate facilities and expertise in neonatology, pediatric cardiology, and interventional neuroradiology.More commonly, VGMs are diagnosed after birth. The clinical manifestations reflect the large size of the lesion and consist predominately of congestive heart failure. The features accompanying the congestive heart failure aid in diagnosis and include a continuous cranial bruit localized over the posterior cranium and bounding carotid pulses. Prerenal azotemia, a consequence of the congestive heart failure, also may be seen. A small number (5% to 10%) of cases present with hydrocephalus, subarachnoid hemorrhage, or intraventricular hemorrhage. Seizures and other neurologic signs are very rare.A VGM should be considered in any newborn who has unexplained congestive heart failure, especially high-output heart failure, or unexplained intracranial hemorrhage or hydrocephalus. The initial diagnostic evaluation should be cranial ultrasonography. The aneurysmally dilated vein presents as a large echolucent region in the region of the vein of Galen. Color Doppler studies of flow velocity in the arterial feeders and in the vein further define the hemodynamics and the anatomy of the lesion. Computed tomography is useful for assessment of parenchymal ischemic injury or intracranial hemorrhage. A dilated third ventricle filled with blood in the absence of a large amount of blood in the lateral ventricles is a clue that a VGM is the source of an intraventricular hemorrhage.MRI and MRA are used to define the anatomic details of the VGM. Delineation of the arterial feeders and the size and location of the aneurysmally dilated vein is crucial for determination of intervention.Ideally, the initial treatment of a VGM is conservative. Embolization is a high-risk procedure in a neonate and, where possible, we treat the child medically (for cardiac failure) until age 5 to 6 months. Elective embolization can be scheduled for this time, with the aim of closing the feeder arteries with a liquid adhesive agent (eg, bucrylate). The venous approach (although technically easier) is associated with a higher risk of hemorrhagic complications and a lower success rate. If the infant deteriorates (as indicated by seizures, failure to thrive, or worsening cardiac failure), embolization is performed earlier. Surgery has little role in the modern treatment of VGM. Surgical attempts at closure of the shunt are associated with very high mortality or severe morbidity, and shunting of the ventricles before embolization may accelerate progressive atrophy. It is difficult to navigate microcatheers to the site of the shunt because of the often extreme tortuosity of the arterial system. Restraints on the amount of parenteral fluid that can be administered to a neonate or infant impose limits on the duration of the procedure. Often, a large shunt that has many feeding vessels requires several embolization sessions.Untreated VGMs have a poor prognosis and almost always are fatal, but it is important to recognize that treatment may be inappropriate in some cases. Where there is evidence of pre-existing brain damage (eg, progressive atrophy, parenchymal calcification) or severe multiorgan failure, a poor outcome (death or survival with severe brain damage) is inevitable.When treatment is performed before significant brain damage has occurred, a good outcome can be anticipated. In Lasjaunias’ series (the largest discussing the management of VGMs), among 21 babies diagnosed antenatally, 14 were considered fit for embolization, and 12 of these (86%) had normal neurologic development. Among the 50 babies who were diagnosed in the neonatal period, 29 were considered fit for embolization (the rest had pre-existing irreversible damage), and 50% of these had permanent neurologic deficits. The worst prognosis was seen in babies who had the largest shunts, presenting as neonates who had severe cardiac failure.Allison Murphy, MD and JoDee M. Anderson, MD.• Patrick Barnes, MD (1).• Departments of Pediatrics (Division of Neonatal and Developmental Medicine) and Radiology (1), Stanford University Medical Center, Stanford, CA." @default.
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• W2555119428 title "A Newborn Who Has Previously Undiagnosed Hydrops Fetalis" @default.
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