1/22. Severe perinatal liver disease associated with fetal thrombotic vasculopathy.Three neonates with fetal thrombotic vasculopathy in the placenta and severe neonatal liver disease are described. Symptoms included a bleeding disorder on the first day of life, followed by direct hyperbilirubinemia and elevated liver transaminases. All patients also had evidence of thrombosis outside the placenta, including cerebral infarct in two infants and thrombosis of the inferior vena cava in one infant. liver disease was demonstrated to be thrombotic in one infant who died with budd-chiari syndrome. Two infants survived and had liver biopsy with cholestasis, bile duct proliferation, and portal fibrosis demonstrated at 4 weeks and 11 weeks of age, respectively. The etiology of thrombosis is unknown, though in one patient an excessively long and coiled umbilical cord may be implicated. The prenatal onset of thrombosis suggests an inherited or acquired thrombophilic state. In cases of enigmatic neonatal liver disease, an association with thrombosis should be considered and thrombi sought in placenta, umbilical cord, major blood vessels, and other organs. Evaluation for a hypercoagulable state is also suggested.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/22. Abnormal umbilical vessels and systemic circulatory reversal in thoracopagus twins.A human thoracopagus of slightly unequal twins having a common umbilical cord with exomphalos is described with special reference to the cardiovascular system. The larger twin had single umbilical artery. The smaller twin had a large left umbilical artery which was in direct continuity with the aorta, a small right umbilical artery with sole connection to the right external and internal iliac arteries (right common iliac artery was absent), missing umbilical vein, a rudimentary nonfunctional heart with atresia of the truncus arteriosus, and a right-sided aortic arch. Exomphalos of both twins is accounted by the possible defective growth of umbilical mesoderm caused by abnormal umbilical vessels. Reversal of sustemic circulation in the upper part of the body of small twin is discussed. The importance of a careful study of umbilical veins is emphasized.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
3/22. Hereditary infantile hemiparesis, retinal arteriolar tortuosity, and leukoencephalopathy.BACKGROUND: The main hereditary vascular conditions involving both retinal and cerebral vessels include cerebroretinal vasculopathy, HERNS (hereditary endotheliopathy with retinopathy, nephropathy, and stroke), and hereditary vascular retinopathy; all are linked to the same locus on chromosome 3p21. Hereditary retinal arteriolar tortuosity is a distinct, autosomal dominant condition characterized by retinal arteriolar tortuosity and recurrent retinal hemorrhages. This condition is known to affect only retinal vessels. methods: Clinical and brain MRI investigations of eight members of a three-generation family and extensive biological and systemic vascular investigations within one affected family member were conducted. RESULTS: Six of eight family members were clinically symptomatic; disorders included infantile hemiparesis (2), migraine with aura (3), and retinal hemorrhage (1). Five individuals had retinal arteriolar tortuosities. A diffuse leukoencephalopathy in association with dilated perivascular spaces was observed in six individuals. Two family members had silent, deep cerebral infarcts as demonstrated on MRI. genetic linkage analysis strongly suggests that this disorder is not linked to the 3p21 hereditary vascular retinopathy/cerebroretinal vasculopathy/HERNS locus. CONCLUSIONS: The authors describe a novel hereditary autosomal dominant condition affecting both retinal and cerebral vessels and characterized by infantile hemiparesis, migraine with aura, retinal hemorrhage, retinal arterial tortuosity, and leukoencephalopathy with dilatation of perivascular spaces and microbleeds on brain MRI. Investigation of additional families should help to map the gene and to better categorize the spectrum of hereditary cerebroretinal small vessel diseases.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
4/22. Arteriovenous malformation of the vein of Galen: treatment in a neonate.heart failure in a 2-day-old infant was not readily explained by clinical examination. cardiac catheterization suggested an intracranial arteriovenous (AV) fistula, and cerebral arteriography showed a malformation of the vein of Galen. The major feeding arteries were surgically obliterated. At age 27 months, the boy has normal mentation but moderate left hemiparesis. review of the literature disclosed 39 other infants with AV malformations of the vein of Galen producing heart failure before age 3 months. Most of them were boys, and had cyanosis, a systolic murmer, cranial bruit, cardiomegaly, and right bentricular hypertrophy. Only three of the 13 who had surgery for their malformation survived. Removal of the malformation is difficult; obliteration of the nutrient vessels, using the operating microscope, is the currently accepted treatment.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/22. Vein of Galen malformation combined with atrial septal defect in a neonate.An arteriovenous fistula (AVF) is an abnormal connection between an artery and a vein, whereby the interconnecting capillary network is missing. Such a malformation frequently occurs in the deep midline regions of the brain, and the subsequent increased flow into the draining vein of Galen substantially dilates in an aneurysmal manner. Congenital forms of the aneurysmal dilatation of the vein of Galen (AVG) often lead to death in the neonatal period, predominantly due to cardiac failure caused by the increased venous inflow as a consequence of the intracerebral arteriovenous shunting. In the presented case a male baby suffered from a rare combination of a cerebral AVF and an atrial septal defect (ASD). He was born at week 38 of pregnancy and subsequently developed tachydyspnoe. Ultrasound (US) and CT scans revealed a large bilateral AVF with dilated basal venous sinuses, hydrocephalus and brain atrophy. In the heart, severe right ventricular hypertrophy, patent ductus arteriosus and an ASD were detectable by US. Neurosurgical consultation rejected the possibility of an operative treatment due to size and localization of the lesion and the existing irreversible brain damage. The child died because of cardiac failure 6 days after birth. autopsy examination in the brain demonstrated a large conglomerate of dilated blood vessels predominantly in the midline and left occipital lobe, edema and hydrocephalus. In the heart, the ASD detected by US proved to be an ostium secundum-type lesion. Histologically, the conglomerate of vessels revealed features of an AVF and matched the characteristics of AVG. Consequences of chronic ischemic brain injury were also present, with ferruginated neurons suggesting intrauterine damage caused by a congenital AVF. Based on data in the literature, we assume that the left-to-right shunt due to increased venous influx into the heart caused not only cardiomegaly, but may have also interfered with the normal development of the atrial septum leading to an ASD, contributing to the rapid progression of the cardiac failure.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
6/22. Studies on subcutaneous fat necrosis of the newborn.biopsy specimens from the skin and subcutaneous fat tissue of four cases with neonatal subcutaneous fat necrosis were made and investigated by light and electron microscopy at 2, 4, and 6 weeks, and 5 months (Case 2) from the onset of the disease. Three stages of ultrastructural change of fat cells were observed. The evolution of crystal formation in the fat cells was seen and phagocytosis of crystals and fat droplets by macrophages and foreign-body giant cells was also noted. In the light microscope accumulation of calcium concretions in the spaces between and inside the fat cells was found. In the electron microscope we detected foci of highly electron-dense granules, which were similar in distribution and structure to calcium salts stained with the von Kossa method. Changes in small and medium size blood vessels were observed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/22. Neuroradiological and pathological studies on neonatal aneurysmal dilation of the vein of Galen.Neuroradiologic and neuropathologic findings on neonatal aneurysmal dilation of the vein of Galen are reported. Doppler echocardiography revealed a diastolic reversed Doppler signal, which suggested the presence of a cerebral arteriovenous fistula. Cerebral ultrasonography with intravenous injection of contrast medium was useful in the diagnosis. Digital subtraction cerebral angiography clearly demonstrated both the feeding and draining vessels and the aneurysmal dilation of the vein of Galen, although it failed to detect the abnormal vascular channels. Pathologic and histopathologic findings demonstrated intimal thickening of the abnormal vascular channels, which could produce higher vascular resistance and result in the detection failure with digital subtraction angiography.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/22. Ventricular opacification after intravascular injections of contrast material.The cerebral ventricles of two neonates were demonstrated on CT scans and plain radiographs after the intra-arterial injection of contrast material. Leakage of contrast material into the ventricles after intravenous injection probably indicates damage of the blood-brain barrier. Breakdown of this barrier results only from severe and prolonged anoxia because cerebral vessels are much more resistant to anoxia than neuronal tissues. The authors have not observed ventricular opacification in adults.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/22. Rhabdomyomatous dysplasia of the lung.This article deals with the presence of nontumoral striated muscle fibers in the lungs of 3 neonates. These cells were diffusely distributed in one lung (case 1) or in both (case 2), or focally localized to the lung parenchyma adjacent to the liver in a case with a large right diaphragmatic hernia (case 3). The striated muscle fibers were located in the walls of small bronchi and bronchioli or in the alveolar interstitium. Other major lung malformations found simultaneously were absence of lobation, hypoplastic lungs, and hypoplastic pulmonary vessels. The origin of striated muscle fibers in the neonatal lung has been attributed to anomalous differentiation of mesoblastic cells (as in cases 1 and 2). The presence of striated muscle cells in the lower margin of a hypoplastic lung associated with a right diaphragmatic hernia (case 3) suggests that intrapulmonary inclusion of diaphragmatic muscle fibers might be a source as well. Striated muscle fibers in the lung are commonly associated with major malformations involving heart and lungs, suggesting a much wider morphogenetic error.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/22. Systemic air embolism in the respiratory distress syndrome of the newborn.Details of 25 cases of neonatal systemic air embolism, including three of our patients, are reviewed. This rare complication of respiratory distress syndrome (RDS) was observed in 21 premature infants and 4 fullterm newborns, of whom 23 required intermittent positive pressure ventilation (IPPV). In 21 of these patients air embolism was preceded by pulmonary interstitial emphysema, pneumomediastinum, pneumothorax and/or pneumopericardium. air embolism occurred between 3 and 288 hours after delivery. The outcome was lethal in 24 cases, only one patient survived. Two main pathogenic mechanisms are discussed; entrance of air from ruptured alveoli into the lung capillaries and introduction of air via catheters in umbilical vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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