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  • 1
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background. Midline scalp lesions are frequent in children. They include soft-tissue masses and atretic meningocoeles. Their recognition is important as their treatment differs. Intracranial venous anomalies are known to be associated with atretic cephalocoeles.¶Materials and methods. A retrospective study was undertaken to assess the frequency of intracranial venous anomalies associated with atretic meningocoeles (AT). Thirty-one patients with AT were studied by MRI. There were 13 meningocoeles and 14 encephalocoeles; 4 have not yet received surgery.¶Results. Venous anomalies were found when the cephalocoeles lay above the torcular. They include absence of the straight sinus and duplication of the longitudinal sinus.¶Conclusion. Venous anomalies are frequent in atretic cephalocoeles and are part of the dysraphic state.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 9 (1993), S. 131-135 
    ISSN: 1433-0350
    Keywords: Supratentorial tumors ; Astrocytomas ; Ependymomas ; Secondary malignancies ; Paediatric patients
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sixty-four malignant hemispheric tumors in children less than 15 years old were treated in the pediatric neurosurgical department of the H ôpital des Enfants Malades between 1970 and 1989. (1) These tumors evolved rapidly in most cases. However the pre-operative evolution in 20% of the patients had a duration of more than 6 months, which favors the hypothesis that at least one-fifth of these tumors result from malignant transformation of a benign lesion. This observation should prompt neurosurgeons to operate on all benign hemispheric tumors as soon as they are diagnosed. (2) Five of the 64 patients had two successive malignant diseases. In four cases the other malignant disease was an acute lymphoblastic leukemia. (3) Among the malignant hemispheric tumors, the grade III and IV astrocytomas had a dismal prognosis. As it is known from previous studies that grade I and II astrocytomas have a good prognosis in children, it can be concluded that grading these tumors is essential. By contrast, almost one out of two patients with malignant ependymoma was alive 5 years after treatment. This implies that the grading of ependymomas is of modest prognostic values. (4) The harmful effect of radiotherapy was evaluated by comparing the functional outcome of children operated for a benign hemispheric tumor to that of children operated and irradiated for a malignant hemispheric tumor.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neurochirurgica 48 (1979), S. 1-15 
    ISSN: 0942-0940
    Keywords: Medulloblastoma ; survival ; functional results ; side-effects of radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The authors report on a series of 57 medulloblastomas in children operated upon between 1964 and 1976. Among these children, 44 completed the treatment with radiotherapy to the whole central nervous system. The postoperative mortality rate is 10.5%. Postoperative deaths occurred mainly in infants or when a tumour involved the brain stem. The five-year survival rate is 54% in the whole series. It rose to 71% in the patients who completed the treatment with radiotherapy. The study shows that the life of survivals is frequently impaired by mental or behavioural disturbances. IQ varies from 70 to 90 in 58% of the children; it is below 70 in 31%. Behavioural disturbances are found in 93% of cases. 82% have defective spatial orientation, dysphasia, or dysgraphia. In order to evaluate the responsibility of X-ray therapy for the development of these sequelae, the results have been compared to those of a series of cerebellar astrocytomas surgically removed, but not irradiated. The comparison shows that radiotherapy is at least partially responsible for the mental and behavioural disturbances. No relation was found between these disturbances and a persistent ventricular dilatation or an endocrine dysfunction. However the endocrine assessment showed growth hormone deficiency in 65% of the children, short stature in 55%, and compensated hypothyroïdism in 58%.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 0942-0940
    Keywords: Third ventricle ; surgical approach ; striothalamic vein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In the removal of tumours that develop within the third ventricle, most approaches are not entirely satisfactory. Therefore, a new approach has been devised: transfrontal exposure of the anterior portion of the frontal horn; coagulation and section of the striothalamic vein in order to open up the roof of the third ventricle; use of a blunt spatula introduced in the foramen of Monro and pushed backwards under the choroïd plexus. This approach has been used in ten cases. Postoperative mortality has been nil; the surgical approach has not apparently been responsible for any sequelae. It is simple and gives a good view of the third ventricle.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1920
    Keywords: Key words Neuroepithelial tumor of childhood ; supratentorial ; Desmoplastic astrocytoma ; Desmoplastic ganglioglioma ; Magnetic resonance imaging ; Computed tomography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Desmoplastic neuroepithelial tumours are rare supratentorial neoplasms of infancy with a favourable prognosis. We characterised their imaging findings by reviewing the clinical and radiological reports of five affected children. The tumours were massive, predominantly cystic and with preferential frontal and parietal lobe involvement. The cystic component was in the white matter, with no communication with the ventricle. The solid part was superficially, abutting a meningeal surface, and showed intense contrast enhancement. A heterogeneous predominantly solid mass was observed in one patient. Thinning and deformation of the skull adjacent to the tumour was shown in four cases. Peritumoral oedema was absent or moderate. No calcification or haemorrhage were present. Angiography showed a tumour blush from the internal or external carotid arteries in one case each. Follow-up (3 months–9 years, mean 4.5 years) showed no recurrence.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1920
    Keywords: Neuroepithelial tumor of childhood, supratentorial ; Desmoplastic astrocytoma ; Desmoplastic ganglioglioma ; Magnetic resonance imaging ; Computed tomography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Desmoplastic neuroepithelial tumours are rare supratentorial neoplasms of infancy with a favourable prognosis. We characterised their imaging findings by reviewing the clinical and radiological reports of five affected children. The tumours were massive, predominantly cystic and with preferential frontal and parietal lobe involvement. The cystic component was in the white matter, with no communication with the ventricle. The solid part was superficially, abutting a meningeal surface, and showed intense contrast enhancement. A heterogeneous predominantly solid mass was observed in one patient. Thinning and deformation of the skull adjacent to the tumour was shown in four cases. Peritumoral oedema was absent or moderate. No calcification or haemorrhage were present. Angiography showed a tumour blush from the internal or external carotid arteries in one case each. Follow-up (3 months-9 years, mean 4.5 years) showed no recurrence.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1433-0350
    Keywords: Children ; Medulloblastoma ; Psychological sequelae ; Radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A series of 120 medulloblastomas in children operated on between 1967 and 1987 at the Hôpital des Enfants-Malades has been reviewed in order to check whether the conclusions of our study published 10 years ago have remained valid and, in particular, to verify whether the quality of life of these patients, which had been found to be poor at the time, had improved or worsened over the years. The postoperative mortality for the whole series was 6.5% there have been no deaths in the 35 patients operated on after 1980. The overall survival rate for the 120 children was 60% at 5 years and 53% at 10 years; for the patients who completed radiotherapy, the survival rates was 73% at 5 years and 64% at 10 years. Survival rates were surprisingly better in patients treated when under 6 years of age than in older children. They were also better in girls than in boys, and in desmoplastic compared with other medulloblastomas; however, the differences were not significant. When comparing the groups after total or subtotal resection of tumors, survival rates were not significantly different, but were lower in the small group of partial resections. Cell differentiation did not influence the prognosis. Psychological sequelae were significant and worsened over the years. Five years after treatment 58% of the children showed an IQ above 80; 5 years later this group included only 15% of the patients. These psychological sequelae were related to age at the time of radiotherapy: the younger the child, the lower the final IQ. Five years after treatment, 40% of the children had a normal academic level; 5 years later this group was reduced to 11%. Ten years after treatment, 36% of the patients were unempolyed and 64% worked in a protected environment. No patient had normal employment. These disastrous results concerning the quality of life of these medulloblastoma patients justify new trials with reduced doses of irradiation over the hemispheres, at least in low-risk groups.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1433-0350
    Keywords: Key words Hydrocephalus ; Infancy ; Intellectual outcome ; Scholastic integration ; Prognostic factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The purpose of this report is to analyze the long-term outcome in hydrocephalic children treated by shunt placement and in particular their psycho-intellectual development. In the case of shunt placement postoperative mortality is virtually nil, and the overall mortality rate after 10 years of follow-up has been reduced to less than 5%. Morbidity, in contrast, is far from satisfactory. This study was conducted in 129 consecutive children with nontumorous hydrocephalus who underwent a first shunt insertion before the age of 2 years between 1979 and 1982 and who were followed up for at least 10 years. The final neurological examination revealed a motor deficit in 60%, visual or auditory deficits in 25%, and epilepsy in 30%. The final IQs were above 90 in 32% of the children, between 70 and 90 in 28%, between 50 and 70 in 19%, and lower than 50 in 21%. Integration into the normal school system was possible for 60% of the children, but half of them were 1–2 years behind their age group or having difficulties; 31% were attending special classes or were in institutions; and 9% were considered ineducable. The presence of behavioral disorders was a determinant factor for scholastic and social integration. Such disorders were frequent, and were characterized as severe in 30%. A relationship between final outcome and etiology, initial ventricular size, and epilepsy was observed. These results are used as the basis of a discussion on how morbidity might be improved.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1433-0350
    Keywords: Key words Desmoplastic ; Brain tumour ; Infantile ; Surgery ; Astrocytoma ; Ganglioglioma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The authors report on the clinicopathological aspects of and management strategies for the group of rare, large hemispheric childhood tumours recently classified as desmoplastic infantile ganglioglioma (DIGG), desmoplastic astrocytoma of infancy (DACI) and pleomorphic xanthoastrocytoma (PXA). Between 1985 and 1997, ten children (4 with DACIs, 4 with DIGGs and 2 with PXAs) with a median age of 9.5 months were operated on. All these patients had complete surgical resections, with two having a preoperative biopsy. This led to an erroneous diagnosis in both cases of malignant grade 4 astrocytoma. As a result, one patient had preoperative chemotherapy with no effect. There was one perioperative death. Histology revealed heterogeneous tumours with malignant looking areas in 8 of the specimens. None of the patients has had any postoperative adjuvant treatment. All surviving patients are alive at follow-up (median 4.2 years). Despite their often malignant appearance, these tumours have an excellent prognosis, but they can present formidable surgical challenges when they occur in very young age patients. We believe that surgical excision can offer a cure and that adjuvant treatment is not necessary. Finally, biopsy is of little value and may even lead to an erroneous diagnosis and subsequent mismanagement.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 16 (2000), S. 645-658 
    ISSN: 1433-0350
    Keywords: Keywords Craniosynostosis ; Craniofacial surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Although it is currently thought that surgery is indicated mainly for cosmetic reasons in isolated craniosynostoses, the functional aspects of the treatment must not be underestimated. Prospective studies on intracranial pressure and mental evolution of these children have shown that there were functional consequences in a significant proportion of cases even of single suture fusion. The frequency of increased intracranial hypertension and the risk of mental impairment depend on the age of the child and the type of craniosynostosis. In nonsyndromic cases, the higher risks are observed in multisutural craniosynostoses (brachycephaly, oxycephaly). In syndromic cases, the risk of intracranial hypertension is higher in Crouzon syndrome, and Apert syndrome carries the higher risk of mental retardation. The study of a personal series of 2,137 craniosynostoses shows that the functional and the cosmetic results are better after early surgery, and that the operative risks are not higher in infants than in older children.
    Type of Medium: Electronic Resource
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