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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK; Malden, USA : Munksgaard International Publishers
    Journal of cutaneous pathology 32 (2005), S. 0 
    ISSN: 1600-0560
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A 1-year-6-month-old girl presented with a subcutaneous tumor of the forehead, which had developed since birth. The preoperative examinations showed nasofrontal bone defect with meningocele and subcutaneous tumor with cyst. The patient underwent excision of the tumor and reconstruction of the bone defect. Histologically, the skin tumor exhibited disordered arrangement of striated muscle fibers among normal dermal components, and the cyst was lined by cornified epithelium with a few hair adnexa and contained lamellated keratin. These findings were consistent with rhabdomyomatous mesenchymal hamartoma (RMH) and dermoid cyst. This is an interesting case of RMH co-existing with nasofrontal meningocele and dermoid cyst in the same area. We suggest embryologic errors as a possible etiology, which is incomplete dysjunction of the neural ectoderm from the cutaneous ectoderm. Failure of insertion of mesoderm between the ectoderms caused the bone defect and the disordered proliferation and differentiation of mesoderm-derived tissue, leading to formation of hamartoma.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1433-0350
    Keywords: Key words Moyamoya disease ; Brain ischemia ; Anesthesia ; Revascularization ; Perioperative management
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In purpose of clarifying the risk factors of perioperative ischemic complications in cases with moyamoya disease, a retrospective study was conducted on the perioperative care of 36 patients who underwent vascular reconstruction for moyamoya disease. Following surgery, 8 patients developed ischemic complications (5 with minor completed strokes and 3 with transient ischemic attacks). Three factors including the presence of a pre-operative low density area (LDA), intra-operative urinary output, and a reduction in the hematocrit significantly differed with complications and those without. In an evaluation of individual cases, intra-operative hypercapnia, hypotension, and a reduction in the circulating blood volume also were identified as potential factors associated with complications. On the basis of these findings, it was concluded that it is necessary to infuse an adequate amount of fluids, maintain normocapnic and normothermic states, and actively correct anemia in patients undergoing vascular reconstruction for moyamoya disease. It is also necessary to pay particular attention to perioperative care in patients in whom a LDA is detected by pre-operative CT.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 15 (1999), S. 209-211 
    ISSN: 1433-0350
    Keywords: Key words Anterior sacral meningocele ; Epidermoid tumor ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 2-year-old girl presented with an anterior sacral meningocele completely occupied by an epidermoid tumor. Preoperative magnetic resonance imaging had shown the meningocele with contents of the same intensity as cerebrospinal fluid. Surgery via a posterior sacral approach disclosed the tumor beneath an unexpected membrane inside the meningocele. Additionally, the presence of pus inside epidermoid tumor suggested that possible episodes of asymptomatic meningitis or other infection might have occurred before treatment, these being the major complication in anterior sacral meningocele. Therefore, we recommend that surgical treatment should be performed at the earliest possible stage in childhood, once the diagnosis is established, and dural plasty carried out to prevent infectious complications.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1433-0350
    Keywords: Extracerebral fluid collection ; 123IMP-SPECT ; Regional cerebral blood flow ; Infant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral blood flow in seven infants with extracerebral fluid collections was investigated using single photon emission computed tomography (SPECT) with 123I-N-isopropyl-p-iodoamphetamine. Early and delayed SPECT imaging was carried out. Areas of hypoperfusion were observed in five cases. The watershed zone of the major cerebral arteries or the territory of the anterior cerebral arteries were common areas of low perfusion. The hypoperfusion area was redistributed in two cases with intracranial hypertension. Subduroperitoneal shunts produced improvement of clinical symptoms in these cases. Hypoperfusion without redistribution was observed in three patients. In these areas, permanent tissue damage caused by a primary disease existed. Normal circulation patterns were observed in two patients. They showed normal development and follow-up CT revealed a decrease in the size of the extracerebral fluid collection. Measurement of regional cerebral blood flow may be helpful in considering surgical indications and in following up extracerebral fluid collection in infants.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1433-0350
    Keywords: Cerebral angiography ; Childhood onset ; Intracranial hemorrhage ; Long-term follow-up ; Moyamoya disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To clarify the differences between childhood-onset moyamoya disease and that with onset in adulthood, we studied the clinical course and angiographic findings of adult patients (over 20 years of age) with moyamoya disease of childhood onset (up to 15 years of age). The clinical course in 25 patients could be assessed. The follow-up period was 5–27 years. Neurological deficits were noted in 11/23 and mental disorders in 9/21. In all except one, the illness had started before the age of 7 years. Neither neurological nor mental condition changed during or after adolescence (15–20 years of age). Two patients died of intracranial hemorrhage. The disease progressed in angiographic stage until adolescence, but had stabilized or almost stabilized by the age of 20 years. This study indicates that moyamoya disease with onset in childhood carries high morbidity and mortality. The disease advances in angiographic stage between childhood and adolescence, but stabilizes or almost stabilizes between adolescence and adulthood.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1433-0350
    Keywords: Infant ; hydrocephalus ; Cerebral blood flow ; Oxygen metabolism ; PET
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In this study, regional cerebral blood flow (rCBF) and the cerebral metabolic rate of oxygen (rCMRO2) were measured using positron emission tomography (PET) with oxygen-15 radiopharmaceuticals to clarify the pathophysiology of ventriculomegaly in the developing brain. Four hydrocephalic infants without severe neurological deficit were studied. Hypoperfusion was observed in the frontal, parietal, and visual association cortices which surrounded dilated anterior or posterior horns of the lateral ventricle. Lower rCMRO2 values than adult rates were observed in all cases. In the infants with markedly enlarged anterior or posterior horns, the surrounding cortices showed relatively lower rCMRO2 values with the fall of rCBF. Postoperative studies were performed in two infants. rCMRO2 increased in every region after ventriculoperitoneal shunting, but little change was observed in rCBF. These results indicate that metabolic deterioration occurs in the developing brain with hydrocephalus.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1433-0350
    Keywords: Moyamoya disease ; Cerebral blood flow ; Cerebral metabolism ; Re-build-up ; EEG ; Positron CT
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The cerebral blood flow and cerebral metabolic rate of oxygen (CBF and CMRO2) of three cases of childhood moyamoya disease were examined by positron-emission-computed tomography for the purpose of investigating the mechanism of the “re-build-up” phenomenon on EEG. Decrease in both CBF and CMRO2 were observed following hyperventilation. However, dissociation between the decrease in CBF and CMRO2 was also observed. Arterial blood-gas analysis disclosed hypocapnea during hyperventilation and hypoxia following hyperventilation. These results clearly indicate that the re-build-up seen on EEG is the manifestation not only of ischemic hypoxia but also of hypoxic hypoxia characteristically seen in moyamoya disease.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 8 (1992), S. 432-436 
    ISSN: 1433-0350
    Keywords: Cerebral infarction ; Angiography ; Children ; Vasculitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A cooperative study was undertaken in the Tohoku district of Japan to investigate the relatively rare phenomenon of cerebral infarction in children. The purpose of the present paper is to describe the cerebral angiographic findings in 48 children whose ischemic lesions were confirmed by CT scan. The majority of lesions were considered to be idiopathic. The areas of cerebral infarction appearing in the CT scans were located in the territory of the middle cerebral artery including the basal ganglia. Angiographical abnormalities were observed in 40 patients (83%). The majority occurred in the supraclinoid portion of the internal carotid artery and in the cisternal portion of the middle and anterior cerebral arteries. Multiple lesions, such as in the C1, A1, and M1 or the C1, M1, and M2 segments were observed in 22 cases. These lesions generally appeared in continuation; no bilateral intracranial lesions were observed. Repeated angiography was performed in 22 cases, and in 55% of these some recovery of the lesions was seen.
    Type of Medium: Electronic Resource
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