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  • 1
    Electronic Resource
    Electronic Resource
    Medulloblastoma in childhood: progressive intellectual deterioration (1990)
    Springer
    Child's nervous system 6 (1990), S. 60-65 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00307922
    Library Location Call Number Volume/Issue/Year Availability
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  • 2
    Electronic Resource
    Electronic Resource
    Antley-Bixler syndrome Description of two new cases and a review of the literature (1997)
    Springer
    Child's nervous system 13 (1997), S. 275-281 
    Details
    ISSN: 1433-0350
    Keywords: Key words Antley-Bixler syndrome ; Plagiocephaly ; Imperforate anus ; Choanal stenosis or atresia ; Craniosynostosis ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Antley-Bixler syndrome was first described in 1975, and to date 20 cases have been reported. In addition to brachycephaly, the syndrome is associated with midface hypoplasia, often with choanal stenosis or atresia, bilateral radiohumeral synostosis, multiple joint contractures, femoral bowing and long bone fractures, ``pear-shaped'' nose, dysplastic ears and, occasionally, urogenital or cardiac defects. Survival is closely linked to upper airway obstruction. This, in addition to craniosynostosis, also affects mental prognosis. The cluster of malformations and their severity are variable, and while numerous children have died early from respiratory distress, one third of them are alive and have had quite satisfactory development. With early and effective prevention of respiratory complications and early treatment of craniosynostosis, the overall prognosis can be favorable. The mode of inheritance is probably autosomal recessive, and midtrimester prenatal diagnosis is feasible. Genetic counseling depends on accurate prognostic and therapeutic data. We describe two new cases, a 4-year-old boy with unilateral coronal synostosis and radio-humeral synostosis on the same side and an 18-month-old girl with brachycephaly and imperforate anus.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003810050082
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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