1/138. Fatal haemorrhagic infarct in an infant with homocystinuria.Thrombotic and thromboembolic complications are the main causes of morbidity and mortality in patients with homocystinuria. However, it is unusual for thrombosis and infarction to be the presenting feature leading to investigation for homocystinuria and cerebrovascular lesions in the first year of life. We describe a previously healthy 6-month-old infant who presented with a large middle-cerebral-artery territory infarction and died of massive brain swelling. homocystinuria due to cystathionine beta-synthase (CBS) deficiency was diagnosed by metabolite analysis and confirmed by enzymatic activity measurement in a postmortem liver biopsy. homocystinuria should be considered in the differential diagnosis of venous or arterial thrombosis, regardless of age, even in the absence of other common features of the disease. We recommend systematic metabolic screening for hyperhomocysteinemia in any child presenting with vascular lesions or premature thromboembolism.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/138. Large chronic cephalohematoma without calcification.Cephalohematomas following birth normally resorb within the 1st month of life. In cases of prolonged resorption, over greater than 1 month, cephalohematomas typically begin to calcify. We report the case of a 3-month-old child with a persistent, large cephalohematoma that did not calcify. After observation alone failed to demonstrate a decrease in the size of the hematoma, 30 cm3 of old blood was aspirated, and the patient's head was wrapped. Unlike calcified cephalohematomas, this noncalcified lesion did not require open surgical intervention. A treatment protocol for cephalohematomas is presented.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/138. Massive intracranial bleeding requiring emergency splenectomy in a patient with CMV-associated thrombocytopenia.We describe a previously healthy male patient, with severe immune thrombocytopenic purpura (ITP) following CMV infection which was refractory to steroids and intravenous immunoglobulin, who developed massive intracranial bleeding. Despite an extremely low platelet count (2x10(9)/liter) which was refractory to platelet transfusions, successful emergency splenectomy was performed, with rapid resolution of the thrombocytopenia. Bleeding complications are extremely rare in viral-associated ITP. Emergency splenectomy should be considered in the presence of life-threatening bleeding when other modalities fail to produce a rise in the platelet count. infection with CMV should be ruled out in cases of severe, treatment-resistant ITP.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/138. Cerebral arteriovenous malformation in pregnancy: presentation and neurologic, obstetric, and ethical significance.Cerebral arteriovenous malformations infrequently complicate pregnancy. We sought to determine the neurologic, obstetric, and ethical significance of such malformations. We present the clinical course of 2 pregnant women with arteriovenous malformations who experienced cerebral hemorrhage and a loss of capacity for decision making. We also review the neurologic and obstetric significance of arteriovenous malformations in pregnancy. Various treatment options with concern for pregnancy and the prognosis for arteriovenous malformations are outlined. The ethical issues involved for pregnant patients whose decisional capacity is compromised as a result of cerebral injury are explored. A review of persistent vegetative state and brain death (death by neurologic criteria) occurring in pregnancy allows us to explore many issues that are applicable to decisionally incapacitated but physiologically functioning pregnant women. We outline a document, the purpose of which is to obtain advance directives from pregnant women regarding end-of-life decisions and to appoint a surrogate decision maker. We believe that evaluation and treatment of the arteriovenous malformation may be undertaken without regard for the pregnancy and that the pregnancy should progress without concern for the arteriovenous malformation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/138. cleidocranial dysplasia with neonatal death due to central nervous system injury in utero: case report and literature review.cleidocranial dysplasia (CCD), an uncommon disorder involving membranous bones, is rarely lethal in early life. The calvaria is defective and wormian bones are present. Abnormalities of the clavicles vary in severity from a minor unilateral defect to bilateral absence. This report concerns pre- and postmortem anatomical and radiological findings in a 15-day-old female neonate with CCD. Her postnatal course was characterized by seizures and recognition of hydrocephalus during the first day of life. The calvaria was hypoplastic with numerous wormian bones. A pseudofracture of the right clavicle was present. hydrocephalus was present in the brachycephalic brain which had a severely thinned cerebral cortex. hemosiderin in the ventricular lining and marked subependymal gliosis were interpreted as evidence of old intraventricular hemorrhage that had occurred in utero. A CCD-related condition, Yunis-Varon syndrome (YVS), is noted for early lethality and for developmental and secondary abnormalities of the central nervous system. The present case only partially matches the phenotype of YVS and might represent a part of a spectrum of phenotypic variants ranging from viable CCD to lethal YVS.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
6/138. Unilateral posthaemorrhagic hydrocephalus in the neonatal period or later in infancy.Five infants who developed unilateral hydrocephalus associated with antenatal or perinatal intraventricular haemorrhage (IVH) in the neonatal period or later in infancy are reported. Unilateral hydrocephalus occurred following discharge home in four of our five cases, two of whom had been treated during the neonatal period with either serial lumbar punctures or punctures from a Rickham reservoir. An obstruction at the level of the foramen of Monro following a large subependymal matrix bleed appeared to be the underlying aetiology. These data suggest that infants who suffer a predominantly unilateral IVH, with or without parenchymal involvement, can subsequently develop unilateral hydrocephalus. Cranial ultrasound examinations should be repeated at regular intervals during the first year of life, as unilateral hydrocephalus can still develop after a period of apparent stabilization.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/138. Periventricular haemorrhagic infarct in a preterm neonate.magnetic resonance imaging (MRI) was performed on an infant born, at 28 weeks gestational age who suffered a sudden episode of bradycardia and desaturation on the 3rd day of life. Imaging demonstrated bilateral germinal layer haemorrhage and intraventricular haemorrhage, with parenchymal involvement in a fan-shaped pattern in the periventricular white matter on the left. These appearances are consistent with a combination of intravascular thrombi and perivascular haemorrhage along the course of the medullary veins. We believe that this is the first report of the MRI appearance of an acute periventricular haemorrhagic infarct associated with a germinal layer haemorrhage/intraventricular haemorrhage in a preterm neonate.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/138. hellp syndrome with haemaglobin vasospasm.The syndrome of haemolysis, elevated liver enzymes and low platelets (hellp syndrome) is a life threatening, severe complication of pre-eclampsia with typical laboratory findings. An unusual case of a 36-year-old woman with hellp syndrome and the initial complication of intracerebral haemorrhage is presented. The diagnosis of hellp syndrome was confirmed by elevated liver enzymes, low platelets, increased total bilirubin and increased lactate dehydrogenase. The intracranial haematoma was removed with good neurological recovery ensuing. However, this case was complicated by cerebral vasospasm on the eleventh day, confirmed by cerebral angiography and computer tomographic findings. The patient died from brain swelling. Possible vasospam should be considered during the treatment of patients with hellp syndrome.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/138. association of herpes simplex virus encephalitis and paraneoplastic encephalitis - a clinico-pathological study.A 57 year-old woman developed acute limbic encephalitis and brainstem dysfunction. Anti-HU antibodies were repeatedly detected in serum and CSF. Postmortem examination showed necrotic and hemorrhagic lesions in the temporal lobes characteristic of herpes simplex virus encephalitis, which was confirmed by immunocytochemistry, and Purkinje cell loss with proliferation of Bergman glia and myelin loss in the external aspect of the dentate nuclei characteristic of paraneoplastic encephalitis. PCR-assay performed on temporal tissue extracts was positive for HSV-1. There was no identifiable neoplasm. This unusual association raises the possibility of a link between the two diseases.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/138. Haemorrhagic complication after total extirpation of huge arteriovenous malformations.Two cases with huge arteriovenous malformations (AVMs) who developed haemorrhagic complications after surgery are described. The cause of the postoperative haemorrhage was considered to be the normal perfusion pressure breakthrough phenomenon and/or occlusive hyperaemia. These two haemodynamic insults possibly occur simultaneously and induce life-threatening haemorrhage. It was concluded that a huge high-flow AVM with a large venous ampulla in its deep drainers has a high risk of a postoperative intravenous thrombosis, resulting in haemorrhage together with normal perfusion pressure breakthrough phenomenon.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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