Forty cases of Dandy-Walker malformation referred to the Hôpital Necker Enfants-Malades between 1969 and 1982 have been reviewed. The incidence of the malformation in hydrocephalus was 2.4%. There was a slight, statistically insignificant, female prevalence. Hydrocephalus should not be included in the definition of the syndrome. In 80% of the cases, it was actually a post-natal complication of the malformation and most often developed within 3 months after birth. In 80% of the cases, a communication, although insufficient, was found between the dilated 4th ventricle and the subarachnoid space. Since this communication is probably established through the foramina of Luschka, the definition of the Dandy-Walker malformation should only include atresia of the foramen of Magendie. Associated brain and systemic malformations were numerous. Among facial anomalies, facial angiomas were found in 10% of our cases. The association of facial and cardiovascular anomalies favors the hypothesis that the onset of the malformation occurs between the formation and the migration of the cells of the neural crest (that is, between the 3rd and the 4th post-ovulatory week, earlier than previously thought). Except in selected patients, membrane excision has a high rate of failure and should be abandoned. Cyst-peritoneal shunting avoids the risk of an entrapped fourth ventricle and is presently the best surgical procedure. The overall mortality in this series was 12.5%. Intelligence quotients were over 80 in 60% of the patients. Other studies will be necessary to understand why the communication between the fourth ventricle and the subarachnoid spaces, sufficient in utero, usually becomes insufficient for a normal cerebrospinal fluid (CSF) circulation in the first months following birth. Two hypotheses are discussed: a change in CSF circulation, or bleeding in the dilated fourth ventricle during delivery.
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.
The authors review 42 consecutive cases of benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children undergoing surgery in the pediatric service of the Hôpital des Enfants Malades between 1975 and 1987. Epilepsy was the presenting sign in 76% of the children and remained the only clinical sign at diagnosis in 62%. Partial or complex partial seizures were observed in half of the cases, but other seizure types were also frequent. Diagnosis of the tumor as the etiological agent rested upon the results of computerized tomography or magnetic resonance imaging. Postoperative mortality (5%) and morbidity (16%) rates were low. The postoperative intelligence quotient was above 80 in 71% of the patients, and 77% of the children had no major problem in school. Although only two of the 42 patients were given postoperative radiotherapy, there were no recurrences in 82% of the survivors. The actuarial probability of nonrecurrence of the tumor was 95% at 5 years. Three patients with recurrent tumor underwent further surgery and are, at the present time, recurrence-free. The incidence of epilepsy fell from 76% before surgery to 19% after removal of the tumor alone; therefore, intraoperative electrocorticography and resection of the electrically abnormal cortex at the time of the first surgical procedure do not appear necessary. It is possible that tumor removal restores a mechanism that limits the spread of seizures and, thus, the clinical manifestations of epilepsy. Benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children should not be treated with adjuvant radiotherapy, at least initially.
The purpose of this study was to determine the respective parts played by cerebral hemisphere irradiation, posterior fossa irradiation, and surgery in the poor late functional results often observed in children treated for medulloblastoma. To do this we compared the intellectual outcome in a series of 59 children operated on for medulloblastoma, who had received whole-brain irradiation, to that observed in a series of 37 children operated on for ependymoma of the posterior fossa, who had received radiotherapy only on the posterior fossa. Only patients who had survived for more than 2 years without recurrence were included. At the assessment 1 year after treatment, intellectual outcome was somewhat better in the ependymoma group, but the difference was not statistically significant. At the long-term follow-ups at 5 and 10 years the results remained stable in the children treated for ependymoma, around 60% having an IQ above 90, whereas the intellectual level of the children treated for medulloblastoma was seen to have deteriorated progressively: 20% had an IQ above 90 5 years after treatment and only 10% at the 10-year follow-up. This progressive degradation is most likely due to the irradiation of the cerebral hemispheres, as this prophylactic irradiation constituted the only difference between the two groups. Moreover, irradiation to the posterior fossa did not seem to affect intellectual functions, since in the group of children with ependymomas the proportion of IQs above 90 was high and remained stable over the years. Surgery was certainly responsible for some poor results. The percentage of IQs above 90 observed 1-2 years after treatment was between 70 and 80 when no postoperative complications occurred, and only between 20-40% in the presence of postoperative complications. Postoperative aggravation was in most cases related to a brain-stem lesion. These results encourage the reduction, when possible, of irradiation to the cerebral hemispheres and underline the importance of the quality of surgery.
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 rate 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.(ABSTRACT TRUNCATED AT 250 WORDS)
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