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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 4 (1988), S. 213-222 
    ISSN: 1433-0350
    Keywords: Prenatal hydrocephalus ; CSF shunt ; Outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical records of 108 infants presenting with hydrocephalus at birth and operated on from 1971 to 1981 were reviewed in order to evaluate the functional results. Premature newborns and spina bifida patients were excluded. Communicated hydrocephalus (39 cases) and aqueductal stenosis (32 cases, excluding 6 X-linked hydrocephalus and 4 toxoplasmoses) were the two main types of hydrocephalus in this series. Eighty-four percent of the infants were operated on before the age of 3 months. The mean follow-up time was 7 years (range 1 to 14 years). The survival rate, calculated by the life table method, was 62% at 10 years. The functional results were evaluated according to intellectual performance, academic level, and psychological status. Of the 75 surviving children, 28% have an I.Q. over 80 and 50% an I.Q. under 60. The mean I.Q. is 54 (range 0 to 130). Of the 52 children who have now reached school age, only 29% have reached a normal academic level. The psychological status is normal or borderline in 46% of the patients. The importance of head enlargement at birth, ventricular size, and the age at the time of surgery are not related to late functional results. The results were best when there were no associated malformations, no shunt infection, when hydrocephalus was due to aqueductal stenosis (excluding X-linked hydrocephalus and toxoplasmosis), or when the first developmental quotient measured at 6 months was over 80.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Key words Optic pathway glioma ; Brain neoplasm ; Neurofibromatosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Progression patterns of optic pathway tumours (OPT) need to be precisely defined for treatment planning. In patients with neurofibromatosis type 1 (NF1), this disease is usually indolent and the available literature rarely reports progression after the age of 6 years. In patients without NF1, the disease course seems to be less favourable. We reviewed the clinical and radiological files of 106 children referred to our institution for the treatment of a symptomatic OPT since 1980. NF1 was present in 51 of them. Progression patterns in children with NF1 differed markedly from those in the other patients. A total of 83 children had tumour extension beyond the chiasm (Dodge type III). Children with NF1 had progressive tumours later during follow-up (47% after the age of 6 years), had more often proptosis and infiltrating tumours but less frequently nystagmus or increased intracranial pressure. 32 children were not treated at diagnosis because they had only mild symptoms related to the OPT. In these patients, progression occurred more often in children without than with NF1 (12/12 versus 12/20 respectively, P=0.04). A high number of patients needed treatment for progression or severe symptoms after 6 years of age. Of the patients, 33% needed treatment for progression or severe symptoms after 6 years of age. Conclusion Progression patterns of optic pathway tumours in children with neurofibromatosis type 1 differ markedly from those in other patients. This study emphasises the need for prolonged follow-up of children with optic pathway tumours, especially in neurofibromatosis type 1.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Key words Arachnoid cyst ; Growth ; Growth hormone deficiency ; Growth hormone treatment ; Insulin ; Leptin ; Puberty ; Precocious puberty
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A suprasellar arachnoid cyst may cause disorders of growth, puberty and hypothalamic-pituitary function, due to the proximity of the cyst to the hypothalamic-pituitary area. A total of 30 patients (17 boys) with cyst diagnosed at 4.3 ± 1 years were routinely evaluated at 5.4 ± 1 years; 24 of them had one or multiple cyst derivations. Some 23 cases had an abnormal height, weight or puberty: short (〈−2SD, 5 cases) or tall (〉2SD, 10 cases) stature, overweight (body mass index, BMI, 〉2SD, 6 cases), central precocious puberty (10 cases) and/or no progression of pubertal development (3 cases). The growth hormone (GH) peaks after pharmacological stimulation test were low (〈10 μg/l) in 16 patients, confirmed by a second evaluation in 8/11 of them. The plasma free thyroxine was low in five patients, prolactin was high in two and the cortisol and concomitant plasma and urinary osmolalities were normal. BMI was correlated negatively with the GH peaks (r=−0.37, P 〈 0.01) and positively with the plasma leptin concentrations (r=0.55, P 〈 0.01). The plasma fasting insulin concentrations were also correlated negatively with the GH peaks (r=−0.55, P 〈 0.02) and positively with the plasma insulin-like growth factor I concentrations (r=0.64, P 〈 0.002). The adult height (12 cases) was at 4SD in 1 and 〈−2SD in 4 patients, two of whom had precocious puberty untreated with gonadotropin releasing hormone (GnRH) analogue, and two had untreated GH deficiency. The adult height of those treated was normal. One girl had primary amenorrhoea and two boys had low plasma testosterone, despite a normal gonadotropin response to a GnRH test. Conclusion Suprasellar arachnoid cysts may cause deficiencies of growth hormone and thyrotropin, stimulation of the hypothalamic-pituitary-gonadal axis, tall stature and/or overweight. These last two disorders may be due to hyperinsulinism, itself due to suprasellar arachnoid cyst.
    Type of Medium: Electronic Resource
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