Surgery of epilepsy associated with focal lesions in childhood.

OBJECT: Surgery in children with epilepsy is a new, evolving field. The important practical issues have been to define strategies for choosing the most suitable candidates and the type and optimal timing of epilepsy surgery. This study was undertaken to elucidate these points. METHODS: To identify the factors that correlated with outcome, the authors analyzed a series of 200 children (aged 1-15 years (mean 8.7 years) who underwent surgery between 1981 and 1996 at the Hôpital Necker-Enfants Malades. In 171 cases (85.5%) the epilepsy was medically refractory and was associated with focal cortical lesions. Surgery consisted of resection of the lesion without specifically attempting to identify and remove the "epileptogenic area. "In the group of children whose seizures were medically refractory, the mean follow-up period was 5.8 years. According to Engel's classification, 71.3% of these children became seizure free (Class 1a,) whereas 82% were in Class I. A multivariate statistical analysis revealed that among all the factors studied, the success of surgery in a patient in whom there was a good clinical/electroencephalogram/imaging correlation depended on the patient's having undergone a minimally traumatic operation, a complete resection of the lesion, and a short preoperative seizure duration. After the surgical control of epilepsy, behavior disorders were more improved (31% of all patients) than cognitive function (25%). The patient age at onset, duration and frequency of seizures, intractability of the disease to therapy, and seizure characteristics were correlated with cognitive, behavioral, and academic performance pre- and postoperatively. Multivariate statistical analysis revealed that cognitive dysfunction correlated highly with the duration of epilepsy prior to surgery, whereas behavioral disorders correlated more with seizure frequency. CONCLUSIONS: These data must be taken into account when selecting patients for surgical treatment and when deciding the timing of surgery. Early surgical intervention allows for optimum brain development.

The development of pediatric neurosurgery in Paris. How to create something out of nothing: reflexions on the methodology.

Up to 1960, children in need of neurosurgical treatment in France were treated either in neurosurgical departments where anesthetists, nurses and neurosurgeons had no specialized training in pediatrics or in departments of pediatric surgery in which the specific aspect of neurosurgery was ignored. Clearly a mutation was needed before any progress could be made: the development of pediatric neurosurgery services. This essay tells the story of the creation of the first pediatric neurosurgery service in France, of the difficulties encountered and of the strategies applied to overcome them. It also recalls the discussions about what size such a service should be and whether it should be located in a general hospital or in a pediatric hospital. During the second half of the twentieth century, progress in neurosurgery, and more specifically in pediatric neurosurgery, has been tremendous. The contribution of the pediatric neurosurgery service at Les Enfants Malades in Paris is briefly summarized.

Medical abortion: ethics, laws and religious points of view, A study by the 1994-1995 Ethics and Morals Committee of the ISPN.

Central nervous system malformations are now frequently detected prenatally. Unfortunately, the progress in diagnosis having far outstripped that in therapeutics, the only question to answer is usually whether the pregnancy should be continued or terminated. Pediatric neurosurgeons have to deal with such problems more and more often. They are asked to give an opinion on the prognosis. In many cases the law requires their involvement in the decision about the possibility of a "therapeutic", or more correctly a medical, abortion. The final decision is based on the religious attitutes of the family and the neurosurgeon and on the law of the country in which the medical abortion would be performed. This article reviews the points of view of the main religions and also the laws in different countries. This knowledge may be of some help to neurosurgeons, especially when the religious attitudes of those involved are different or when their final decision is not in accordance with the law of the country.

Intellectual outcome in children with malignant tumors of the posterior fossa: influence of the field of irradiation and quality of surgery.

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.

Consensus: long-term outcome in hydrocephalus.

Developments in the treatment of hydrocephalus and its outcome are traced from the time of Hippocrates up to the present day. Various factors that have been suspected of influencing the outcome are discussed and their relative importance for long-term outcome is reviewed.

Surgical management of brain-stem tumors in children: results and statistical analysis of 75 cases.

A study was made of 75 children treated between 1970 and 1990, with partial, subtotal, or total removal of three intrinsic and 72 exophytic or surface brain-stem tumors. In all cases, the goal of surgery was to remove as much tumor as possible. Extent of removal was defined according to data obtained from postoperative computerized tomography or magnetic resonance imaging, and was considered partial when only a small amount of tumor was removed, subtotal when a few cubic millimeters of tumor was left, and total when no residual tumor was seen on postoperative radiological investigations. An ultrasonic aspirator was used for the 43 most recent operations. Among tumor removals without the aspirator, 24 (75%) were partial, eight (25%) subtotal, and none total; with the use of the aspirator, the number of partial removals decreased to 44.5% while that of subtotal and total removals increased to 32% and 23.5%, respectively. There were 69 gliomas (92%) and 47 benign tumors (62.6%). Forty-nine patients were irradiated postoperatively, and 14 of the 23 patients whose benign tumors were removed totally or subtotally did not undergo irradiation. This study showed that: 1) the overall prognosis of patients with malignant tumors was poor and was not improved by surgery; 2) the survival rate of those with benign tumors was significantly (p < 0.01) lower after partial removal than after total or subtotal removal (52% and 94%, respectively, at 5 years); 3) comparison of means and proportions (Student's and chi-squared tests) between benign and malignant tumors showed a significant difference relating to patient age (p < 0.03), peritumoral hypodensity (p < 0.001), and preoperative duration of symptoms (p < 0.001); 4) stepwise logistic regression analysis confirmed that two of these three variables were related to malignancy: namely, patient age at surgery (p < 0.03) and presence of peritumoral hypodensity (p < 0.001); and 5) routine postoperative irradiation was contraindicated after total or subtotal removal of benign tumors.

Malignant hemispheric tumors in childhood.

Sixty-four malignant hemispheric tumors in children less than 15 years old were treated in the pediatric neurosurgical department of the Hôpital 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.

[Malignant tumors of the cerebral hemisphere in children. A series of 64 cases]. / Les tumeurs cérébrales hémisphériques malignes de l'enfant. Une série de 64 cas.

BACKGROUND: Cerebral hemisphere tumors in children represent about 15% of all tumors of central nervous system. 30 to 40% of them are malignant and have a poor prognosis. PATIENTS AND METHODS: The files of 64 children (30 boys and 34 girls, less than 15 years old) treated between 1970 and 1989 for malignant cerebral hemisphere tumor were analysed. There were 27 astrocytomas, 20 ependymomas, 7 oligodendroblastomas, 6 sarcomas, 2 neuroblastomas, 1 medulloepithelioma and 1 teratoma. The tumor was located in the frontal area in 18 patients; it was temporal in 9, parietal in 8 and occipital in 2 patients. The tumor infiltrated 2 or several lobes in 27 children. The tumor was excised in 58 patients (completely in 36). Radiotherapy was given in 42 children; 18 of them also received chemotherapy. RESULTS: The time between the first clinical manifestation and diagnosis was longer than 6 months in 20% of cases. Symptoms of increased intracranial pressure were present at onset of the disease in 56% of patients and at admission in 73%. Convulsions revealed the tumor in only 30% of cases. Nine children died during the first post-operative month. The actuarial survival rate was 39 +/- 13% at 5 years and 29 +/- 13% at 10 years. This rate was better for ependymomas (48 +/- 23% at 5 years and 41 +/- 23% at 10 years) than for astrocytomas (25 +/- 18% and 13 +/- 16%). Three of the 27 astrocytomas developed in children who had been treated 6 to 7 years earlier for acute lymphoblastic leukemia. One infant developed acute lymphoblastic leukemia 2 years after surgery and chemotherapy for astrocytoma and one patient, treated at 5 months of age for retinoblastoma, developed an astrocytoma 13 years later. CONCLUSIONS: Increased intracranial pressure is more frequently the first symptom than are convulsions in malignant cerebral hemisphere tumors. The long interval between the first manifestations and diagnosis suggests degeneration of benign tumors in 20% of cases. The development of a second malignant neoplasm is not exceptional in children. The better prognosis for ependymomas than for astrocytomas indicates that cerebral ependymomas should be excised and irradiated regardless of their grade.

[Excision of mediastinal nodes using video-thoracoscopy]. / Exérèse d'adénopathie médiastinale sous vidéothoracoscopie.

A man of seventy-one years with gross respiratory failure was suspected of having a cancer of the right upper lobe with metastases to the right pretracheal and intertracheobronchial nodes. A diagnostic mediastinoscopy did not achieve a diagnosis, and a right sub-bronchial node biopsy was performed, using videothoracoscopy and this revealed the presence of tuberculosis without any further delay.

[Percutaneous endoscopic gastrostomy: its value in assisted alimentation in malnutrition in elderly patients. Apropos of 101 consecutive cases in patients over 70 years of age]. / La gastrostomie percutanée endoscopique: intérêt dans l'alimentation assistée du sujet âgé dénutri. A propos de 101 cas consécutifs chez des patients âgés de 70 ans et plus.

This is a prospective study concerning 101 percutaneous endoscopic gastrostomies (PEG) performed in patients aged from 70 to 98 (83.6). We obtained a success in 99%. Indications were stroke and its consequences in 36%, dementia in 22%, depressive syndrome in 38% (7% in the postoperative period) laryngeal or oesophageal tumors in 4%. This technique was carried out in outpatients without anesthesia but PEG must be considered to be a surgical method. Only 5% patients presented major immediate complications with three deaths (3%) due to the technique. Follow-up (30 days) demonstrated 11 other deaths. Would earlier PEG have given better results? Oral feedings (alone or both oral and PEG) were possible in 51% patients. PEG is a valuable technique of feeding assistance in the aged, offering an alternative to nasogastric tube and could be used more extensively in old patients.

Surgery of hydrocephalus: past, present and future.

The treatment of hydrocephalus, over the centuries, underwent three stages of evolution. During antiquity, middle ages and Renaissance, hydrocephalus was not understood. Medical treatment was useless; surgery was hopeless. The second stage extends from the XIXth century to the end of the first half of the XXth century. CSF circulation was now understood; surgery however, remained inefficient, but some patients survived with arrested hydrocephalus. The third stage begins in the nineteen fifties with the development of silicone shunts with a valve. Surgery transforms the prognosis of hydrocephalus, but the number of post-operative complications creates new problems. The different attempts that have been made during these past two decades to solve these problems are reviewed. They have resulted in a reduction of the mechanical and infectious complications. CSF overdrainage has been minimized. Percutaneous ventriculo-cisternostomies have in some cases replaced shunts. In the future, to improve outcome in these hydrocephalics, surgery, when indicated, should be performed as early as possible. Knowledge and prevention of the causes of hydrocephalus should be developed.

A new surgical approach to subcortical lesions: balloon inflation and cortical gluing. Technical note.

A cortical incision performed with inflation of a balloon to create a channel has been used in an approach to deep lesions with minimal damage to cerebral tissue. The balloon is slipped over a blunt needle and, once in place, is inflated through the needle. Postoperative sealing of the incision with fibrin glue avoids the subdural collection of cerebrospinal fluid such as is sometimes observed when the ventricle is opened during surgery.

Cerebrospinal fluid flow dynamics in children with external ventricular drains.

Fifty-five children had 64 external ventricular drains (EVDs) placed predominantly (95%) for cerebrospinal fluid (CSF) shunt infections. In 9 children, a computer monitoring system measured the CSF output each second continuously for up to 24 hours. The monitoring was repeated daily for up to 9 days. The state of arousal of the patients was recorded simultaneously. In all children, daily EVD outputs were related to age, sex, weight, method of establishing the EVD, height of the drip chamber, time since insertion, and type of infecting organism. Computer monitoring revealed wide fluctuations in flow rate, with peak rates frequently greater than 20 ml/h and periods of flow arrest. These changes were usually associated with increased arousal, but also occurred with sleep. The mean EVD flow rate for all children was 6.3 ml/h. EVD output increased with age and weight. EVD output decreased with Gram-negative or multiple-organism infections and with elevation of the drip chamber. Resolution of the infection, sex of the patient, and method of establishing the EVD had no effect on output. These results predict that CSF production increases with brain growth in humans: that CSF production is depressed by Gram-negative and multiple-organism infections: that implanted CSF shunts with standard valves flow at equivalent rates to an EVD in the supine position; and that the CSF drainage requirements in this group are approximately equal to their EVD outputs.

Mechanical complications in shunts.

A retrospective study was conducted on 1,719 hydrocephalic patients, treated between 1974 and 1983 at the Hospital for Sick Children (Toronto) and l'Hôpital des Enfants Malades (Paris), in order to better understand shunt failure. The statistical analysis demonstrates the following: (1) A probability of occurrence of shunt malfunction of 81% at 12 years of follow-up. (2) A high prevalence of shunt obstruction (56.1% of all malfunction) and fracture or disconnection of the tubings (13.6%). (3) A higher risk of proximal occlusion with flanged ventricular catheter. (4) Less malfunction with proximal-non-slit valves as compared to distal-slit valves. (5) The importance of pressure-flow characteristics of the shunt; because of an indirect relation between overdrainage and proximal occlusion. (6) A correlation between connectors and migration or fracture of the shunt. (7) Less distal obstructions when an open-ended distal-catheter was used. These complications were of some clinical, psychological and economical consequences. Their rate could be lowed.

[Overdrainage in the treatment of hydrocephalus]. / L'hyperdrainage dans le traitement de l'hydrocéphalie.

In infants or children, hydrocephalus is usually the consequence of an obstacle in the cerebro-spinal fluid (CSF) pathways and is most frequently treated by the insertion of a ventriculo-peritoneal shunt (rarely ventriculo-atrial). The CSF flow through such a shunt is equal to the ratio of the difference (DP) between the CSF input and output pressures over the valve resistance. When the child is in the upright position, a DP increase occurs due to the height of the hydrostatic column between the inlet and the shunt outlet. Thus if the shunt drains correctly in the prone position, it overdrains in the upright position. As the CSF flow through the shunt in standing patients is higher than the CSF secretion, the excess fluid will be taken out of the ventricles, resulting in pericerebral collections, slit ventricles or post-shunt craniostenoses. Many different techniques have been proposed to reduce overdrainage. The Orbis-Sigma shunt was designed in the "Service des Enfants-Malades" precisely for this purpose. In the upright position this shunt becomes a flow regulator and thus reduces overdrainage. Its use has decreased by 2-fold the number of mechanical complications related to overdrainage.

[Epidural chordoma without bone involvement. A propos of a case in a child. Review of the literature]. / Les chordomes épiduraux sans atteinte osseuse. A propos d'une observation pédiatrique. Revue de la littérature.

The authors report the case of a chordoma developed exclusively in the epidural space at the L1-L2 level in a 6 year old child. The tumor was easily removed in toto. No post-operative radiotherapy was given. The diagnosis of chordoma was based on the histological characteristics of the lesion and the results of immunohistological studies which were both typical of this lesion. This observation is exceptional because of the young age of the patient, the lumbar level of the tumor and, even more, the absence of any bone involvement. The 8 previously published cases of chordoma developed along the craniospinal axis but without bone involvement are reviewed and analysed: all were subdural; 7 were intracranial (6 prepontic, 1 suprasellar): only one was intraspinal. These lesions raise the problem of their diagnosis, of their treatment and of their embryogenesis. They could develop from ectopic notochordal remnants: the "ecchondrosis physaliphura".

[Screening of asymptomatic pulmonary embolisms by systematic scintigraphy in apparently uncomplicated phlebitis]. / Dépistage des embolies pulmonaires asymptomatiques par la scintigraphie systématique au cours des phlébites apparemment non compliquées.

To evaluate the presence of asymptomatic pulmonary emboli, a prospective study was conducted in 21 patients with radiologically confirmed phlebitis. Systematic screening by means of radioisotope perfusion lung scanning revealed 8 pulmonary emboli (38%), 6 of which were confirmed by pulmonary angiography (28% using this criterion). In 3 of these patients, the cutoff affected more than 60% of the lung. All the patients remained asymptomatic throughout the study. It is our opinion that the presence of a latent pulmonary embolus should modify the therapeutic approach. In addition to screening for asymptomatic pulmonary emboli, systematic radioisotope perfusion scanning will enable the compilation of a reference document useful in the interpretation of clinical events arising during treatment.