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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The brain of a child with Down syndrome (DS) and vascular abnormalities is described. Neuropathological examination showed a large cerebral infarction. In the circle of Willis there was hypoplasia of the left middle and posterior communicating cerebral arteries, and microscopically there was thickening of intima and focal disruption of internal elastica in some areas of the circle of Willis. Several reports suggest that the incidence of moyamoya disease is higher in children with DS than in other children. The high incidence of congenital heart disease in DS suggests an abnormality of vascular development that may manifest intracranially as a structural vascular defect, creating a vulnerability to unknown factors important in the pathogenesis of the moyamoya abnormality.
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PMID:Vascular dysplasia in Down syndrome: a possible relationship to moyamoya disease. 825 Jan 60

Ischemic cerebrovascular disease in children is relatively rare. To clarify the clinical features of ischemic stroke occurring in infants and children, we evaluated 54 cases of cerebral infarction, excluding cases of moyamoya disease, in patients less than 16 years old at 24 clinics in the Tohoku (northeast) district of Japan. We observed two incidence peaks, one in little children and the other in junior high school students. Infection and minor head trauma were more frequently seen prior to ischemic strokes than was heart disease. The middle cerebral artery region, including the basal ganglia, was most commonly affected (49 patients, 91%) on computed tomograms. Angiography was performed in 48 patients (89%) and showed various types of occlusive lesions, mostly affecting the middle cerebral artery. Hemiparesis was the most common form of disability following ischemic strokes (48 patients, 89%). Surgical treatment was carried out in seven patients (13%). The clinical course of these cases showed that the recovery of children after a stroke tends to be better than that of adults, but that permanent disabilities, such as hemiparesis or mental retardation, occur commonly. Further investigation of juvenile cerebrovascular disease is important to prevent ischemic strokes in children.
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PMID:Clinical survey of ischemic cerebrovascular disease in children in a district of Japan. 202 86

Twenty-seven new cases of stroke in Down syndrome are reported, two probands, 13 patients from review of institutional records, and 12 patients from a survey of child neurologists. Forty-one patients from previous reports are summarized. Most stroke episodes were related to congenital heart disease and infections. Seven cases were associated with angiographic abnormalities; there were three cases of unknown etiology and four cases of moyamoya. The understanding of moyamoya and other causes of stroke may be advanced by further study of stroke in Down syndrome patients.
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PMID:Moyamoya and other causes of stroke in patients with Down syndrome. 297 27

The authors studied 34 patients with juvenile ischemic cerebrovascular disease over a 15-year period. Of the 34 patients, 23 had intracranial occlusions attributed to cerebral thrombosis or embolism and 11 had occlusions resulting from moyamoya disease. Clinicopathological features were evaluated in the 23 cases with ischemic stroke, but not those with moyamoya disease. The cause of the arterial occlusion remained undetermined in 11 patients and was found to be an embolism based on congenital heart disease in 8, on trauma in 3, and on infection in 1. Cerebral angiography was performed in 21 patients. Of these, 17 had stenoses or occlusions corresponding to their symptoms. CT scans were performed in 10 patients; the lesion in question showed no stenosis or occlusion with cerebral angiography. With regard to prognosis, patients with unknown etiology had good outcomes compared with those with congenital heart disease. With respect to "acute infantile hemiplegia", 10 patients had convulsive seizures and 4 had a history of an earlier infection. Angiography and CT scans in patients with congenital heart disease demonstrated arterial occlusive sites in the middle cerebral artery region. Three patients had abscesses after their ischemic lesions.
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PMID:Ischemic stroke in infancy, childhood, and adolescence. 324 46

Authors review 53 children, aged 0 to 14 years, affected with cerebrovascular ischemic strokes. Largest aetiological groups were: a) congenital heart disease, 16 patients; b) arteritis of unknown cause, 11; c) idiopathic arterial occlusion without arteritis images on angiography, 7; d) moyamoya disease, 6; and d) local or systemic infections, 5. The mode of onset was as completed stroke in 72% and stroke in evolution in 24%. After acute stage 17.6% of patients presented other definitive strokes, 11.7% suffered only transient ischemic strokes (TIA), and 4% reversible ischemic neurologic deficits (RIND). Mean follow-up was 4.36 years, 9.8% of patients died, 11.8% recovered completely and 52.9% improved after initial stroke. Poor global evolution was associated with heart disease (p less than 0.05) and with onset of strokes before age 2 (p less than 0.05). Most important sequelae, besides motor impairment, were epilepsy (49%) and mental retardation (50% got less than IQ 80). Late epilepsy was associated with seizures at onset (p less than 0.05). Clinical factors of adverse mental development were: a) seizures at onset, b) late epilepsy and c) stroke before age 2. 66% of cases had two or more arterial lesions in the same or in different arterial trees. Therefore, embolic and arteritic factors probably play an important role in infancy and childhood stroke.
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PMID:[Ischemic cerebrovascular accidents in childhood]. 340 Sep 36

In the present study the pathology and treatment of occlusion of cerebral arteries in children were investigated in an attempt to find out an approach to the surgical treatment of cerebral arterial occlusive disease of childhood. We had a total of 55 children with cerebrovascular disorders seen at our Institute during the past 11 years. In this series there were 19 cases of cerebral arterial occlusive disease. The causes in 15 cases of cerebral arterial occlusive disease except for moyamoya disease were heart disease in 7 cases, trauma in 2 cases and unknown in 6 cases. The cerebral arterial occlusive disease had its onset at the age of less than 6 years in 12 of 15 cases. The disease began with hemiplegia of sudden onset. The disease onset was also attended frequently by a convulsive seizure, which distinguishes the condition from that in adults. In 10 of all 15 lesions the site of occlusion was in the distribution of the middle cerebral artery. One case in which there was occlusion of the basilar artery occurring in association with trauma was described in detail. In one instance the treatment consisted of STA-MCA anastomosis. Paroxysmal black out attacks, which had been of frequent occurrence in addition to hemiplegia were relieved postoperatively. In our opinion, STA-MCA anastomosis for cerebral arterial occlusive disease in children should be considered to be indicated only when 1) cerebral angiographic evidence of occlusion or stenosis of a trunk of cerebral artery is still present after the acute stage or 2) no extensive low density area is demonstrable on CT scan. Surgery is generally less indicated in those instances in which the occlusion is due to embolism. However since abscess may arise from such an arterial lesion, surgery should be considered, or at least its feasibility be evaluated, whenever 1) the underlying cardiac pathology well permits surgical intervention and 2) half a year has passed since an initial attack.
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PMID:[Cerebral arterial occlusive disease in children -- clinical aspects and surgical treatment (author's transl)]. 727 22

Down syndrome (trisomy 21) has many manifestations that affect multiple organ systems, and we describe the wide array of imaging findings. Common cardiovascular and gastrointestinal entities are congenital heart disease (atrioventricular canal), bowel atresias (duodenal and anal), and Hirschsprungs disease. Children with Down syndrome have an 18-20 fold increased incidence of leukemia. Pulmonary hypoplasia, lung cysts, and pig bronchus (origin of the right upper lobe bronchus from the trachea) have been described. Neurologic findings include mineralizing vasculopathy of the basal ganglia, Moyamoya disease, and cerebellar/vermian hypoplasia. Musculoskeletal manifestations are numerous and include eleven ribs, hypersegmented sternum, abnormal pelvis, joint laxity/dislocations, and DDH (developmental dysplasia of the hip). Of special importance is the "triple jeopardy" of the upper cervical spine (atlanoaxial subluxation, hypoplastic posterior arch of C1, and atlantooccipital instability) and the resulting controversial cervical spine radiographic screening of children with Down syndrome. Knowledge of the many anomalies associated with Down syndrome can aid the clinician, not only in diagnosing abnormalities in these patients, but also in counseling families for potential problems that can occur in these children.
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PMID:Down syndrome: imaging of multiorgan involvement. 1045 38

Childhood stoke is increasingly recognized, but studies remain largely descriptive. Important differences from adult stroke include the following: (1) frequently delayed or missed diagnosis, (2) heterogenous and overlapping risk factors, and (3) developmental differences in the cerebrovascular, neurologic, and coagulation systems. These aspects limit the extrapolation of the results of adult stroke research and present challenges in caring for children with stroke. The incidence of childhood ischemic stroke exceeds 3.3 in 100,000 children per year, more than double the estimates from past decades. The increased incidence reflects, in part, increased survival in previously fatal conditions predisposing to stroke, including congenital heart disease, sickle cell anemia, and leukemia. Risk factors for stroke are recognized in more than 75% of children. Common risk factors include congenital heart disease and sickle cell disease. Progressive arteriopathies, including vasculitis and moyamoya syndrome, are rare in children with stroke; however, transient arteriopathies including post-varicella angiopathy are increasingly recognized. Prothrombotic abnormalities are frequently present but of unclear significance. Adverse outcomes after childhood stroke, including death in 10%, recurrence in 20%, and neurologic deficits in two thirds of survivors could be reduced with available stroke treatments. Aggressive prehospital emergency care and transfer could improve access to hyperacute stroke therapies including tPA. Currently, the diagnosis is delayed by more than 24 hours from onset in most children. As in adults, tPA will likely produce unacceptable rates of intracerebral hemmorrhage unless given within 3 hours of stroke symptom onset. The appropriate choices for in hospital treatment and secondary preventative strategies, including aspirin and anticoagulants, are controversial. Empiric recommendations are published; however, age-appropriate clinical trials are urgently needed. The large multinational networks of investigators necessary for designing and conducting these future trials are now being formed.
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PMID:Stroke in children: recognition, treatment, and future directions. 1120 20

Stroke in children is increasingly recognized. The incidence exceeds 8 per 100,000 per year. Important differences in stroke in newborns and children compared with adults, as well as a paucity of clinical trials, create challenges in the diagnosis and management of pediatric arterial ischemic stroke (AIS). The neurological presentation of AIS can be subtle. Radiographic diagnosis of acute AIS is also challenging because CT scan may be normal early on. Risk factors include vascular, intravascular, and embolic disorders; frequently, there are multiple risk factors in a given child, necessitating thorough investigations. More than 50% have a vasculopathy including postvaricella angiopathy, dissection, moyamoya, or vasculitis. Intravascular mechanisms are frequently present, including dehydration. Hematological or prothrombotic conditions are also associated with AIS in children, and include sickle cell disease and prothrombotic disorders. The latter have been identified in from one third to one half of children with AIS, are usually acquired, and frequently act in concert with other risk factors for stroke. The most common embolic source is congenital heart disease, which is present in 25% of children with AIS. Outcomes include death in 6% and neurological deficits in two thirds of children. Given that no clinical trials have been completed in pediatric stroke to date, treatment is empiric. Initial neuroprotective strategies aim to reduce the size of the infarct. For older children antithrombotic agents (antiplatelet drugs and anticoagulants) are given to reduce the 20 to 30% risk of recurrence. There are coordinated research efforts currently being initiated, which over the next decade will result in clinical trials in this understudied condition.
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PMID:Arterial ischemic strokes in infants and children: an overview of current approaches. 1471 73

We report on 2 children with moyamoya angiopathy and bilateral dolichoectatic internal carotid arteries in combination with iris hypoplasia with bilateral fixed dilated pupils and a history of patent ductus arteriosus. Both were symptomatic with moyamoya angiopathy and underwent bilateral extracranial-intracranial (EC-IC) bypass operations for cerebral revascularization. This is the first report on moyamoya angiopathy and bilateral dolichoectatic internal carotid arteries with simultaneous occurrence of ocular and cardiovascular malformations. There have been descriptions of cerebral vascular abnormalities in combination with either congenital heart disease or ocular abnormalities but not with both presenting together. The combination of these separate congenital developmental defects may not be purely coincidental: we propose that the 2 probands are affected with a not yet recognized clinical syndrome of probably genetic etiology.
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PMID:Moyamoya angiopathy with dolichoectatic internal carotid arteries, patent ductus arteriosus and pupillary dysfunction: a new genetic syndrome? 1473 Feb 27


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