[Epilepsy and focal lesions in children. Surgical management]. / Epilepsie et lésions focales chez l'enfant. Traitement chirurgical.

Epilepsy surgery has gained a large role in the treatment of intractable seizures in the last few decades because of the development of operative techniques and better identification of the cerebral anomalies using electrophysiological recordings and neuroimaging. A series of 419 children, aged from five months to 15 years, with epilepsy (medically refractory in 85.5% of them) associated with focal cortical lesions, who underwent surgery between 1986 and 2006 was analyzed to identify the factors that correlated with outcome. Mean follow-up was 5.2 years. According to Engel's classification; 75.8% of the children were seizure-free. When the lesion was well defined, correlations between clinical data, radiological features and electrophysiological features, suggesting a zone of seizure onset around (or even in) the lesion, was the best guarantee of achieving good seizure control by lesionectomy. Nevertheless, seizure outcome was also determined by other factors: the duration of the epilepsy and the surgery. Persistence of seizures was found to be significantly associated with the preoperative duration of epilepsy, the completeness of the lesional resection and de novo brain damage induced by the surgical procedure itself. Early surgery must be considered in children because of the benefits of seizure control on the developing brain and the risk of secondary epileptogenesis.

[Focus on Dandy-Walker malformation]. / La malformation de Dandy-Walker.

The aim of this review is to summarize our knowledge about Dandy-Walker malformation (DWM) and introduce recent notions about its prognosis. DWM is a malformation associating hypoplasia of the vermis, pseudocystic fourth ventricle, upward displacement of the tentorium, torcular and lateral sinuses and anterio-posterior enlargement of the posterior fossa. It is frequently associated with genetic anomalies, brain malformations (anomalies of gyration, grey matter heterotopias, meningoceles, corpus callosum agenesis...) or systemic malformations (heart, orthopedic, intestinal, urogenital and facial anomalies). It is also part of many syndromes. Its rarity, the difficulty of its diagnosis, the fact that the malformation is mainly known through hospitalized patients, mainly from neurosurgical departments, have made its definition variable and prevents us from having an accurate knowledge of its natural history and prognosis. Hydrocephalus, so frequent in neurosurgical series that some have included it in the definition, is actually rare as has been seen in prenatal series. Treatment, when necessary, is still controversial, the main options remaining cyst fenestration, ventriculo- and/or cystoperitoneal shunts and more recently endoscopic third ventriculostomy. The prognosis, commonly said to be unpredictable, is actually foreseeable after scrutinous observation of the brain anatomy, and mainly of the vermis. A cerebellar vermis with three groups of lobes and two main fissures, as identified on MRI T2 sagittal views, not only has the greatest chance to not be associated with other malformation but also to have a favorable neurocognitive outcome. On the contrary, a deeply dysgenetic vermis with only two or one recognizable lobes is not only constantly associated with other brain malformation but also with poor prognosis. This is a recent advance that may be important for those involved in prenatal counselling.

Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospective series.

BACKGROUND: A prospective study on childhood craniopharyngiomas (CPs) was conducted from 1994 to 1998 to appreciate the pre- and postoperative clinical, endocrine, mental, and intellectual status of the patients and to determine the incidence and severity of the postoperative hypothalamic syndrome. METHODS: The series included 14 consecutive CPs. Twelve were retrochiasmatic and intraventricular, and two were partly prechiasmatic and extraventricular. All were treated with the aim of "total" removal. The removal was "total" in nine cases but incomplete in the other five. Immediate postoperative follow-up was uncomplicated in all cases. CONCLUSION: At 2-year follow-up, the two children with an extraventricular CP and a "total" tumor removal were intellectually normal, had no hypothalamic syndrome, and attended normal school with good results. The 12 others, although still intellectually normal, were more or less severely affected by a hypothalamic syndrome which altered their social insertion and caused academic failure. The authors conclude from this study that radical surgery should be reserved to extraventricular CPs only.

Association of a fetal quadrigeminal cyst with a possibly congenital thalamic astrocytoma.

CASE REPORT: This article reports on the exceptional association of a fetal quadrigeminal malformative cyst with a possibly congenital pilocytic astrocytoma of the pulvinar. Both the cyst and the tumor required treatment due to their postnatal growth. DISCUSSION: Treatment modalities and difficulties are emphasized, as well as the difficulties of prenatal counseling.

Dandy-Walker malformation: prenatal diagnosis and prognosis.

INTRODUCTION: The difficulty in prognosticating the clinical and intellectual outcome of fetuses presenting with a Dandy-Walker malformation (DWM) comes from the great variety of cystic, median, and retrocerebellar malformations that probably have nothing in common and the variability of the definitions given to these lesions. In addition, many of these lesions can mimic each other. A correct diagnosis cannot be made without a good quality MRI including sagittal views of the vermis and T2-weighted images. We limited the diagnosis of DWM to those malformations with all of the following features: 1) a large median posterior fossa cyst widely communicating with the fourth ventricle, 2) a small, rotated, raised cerebellar vermis, 3) an upwardly displaced tentorium, 4) an enlarged posterior fossa, 5) antero-laterally displaced but apparently normal cerebellar hemispheres, 6) a normal brain stem. If any one of the previous criteria were not met, the malformation was considered distinct from DWM. MATERIALS AND METHODS: The charts of 26 patients with DWMs (18 females and 8 males; median age 10.5 years) were reviewed retrospectively. The diagnosis of the malformation was made prenatally in 7 children and postnatally in the 19 others. All the patients had both one MRI including axial and sagittal views of the posterior fossa as well as T1- and T2-weighted sequences, and one neuro-psychological investigation. Syndromic DWMs and Dandy Walker variants were excluded from the study. MRIs were reviewed in a blinded manner looking for brain malformation or damage and studying with particular attention the anatomy of the vermis. Systemic malformations were also recorded. Developmental quotient (DQ) and intellectual quotient (IQ) were said to be normal when equal or greater than 85, and low when below this value. Statistical analysis was performed using a Fisher test to analyze the relationship between intellectual performances, vermis anatomy, ventricular size, brain anatomy, and associated malformations. RESULTS. On scrutiny of sagittal T2 sequences, the vermis, although constantly small, rotated, and pushed towards the tentorium presented as two distinct morphologies, leading us to distinguish two groups of patients. In the first group (n=21), the vermis presented with two fissures, three lobes, and a fastigium as in the normal situation. In this particular group, none of the patients had associated brain malformation and all but 2 were functioning normally. One of the 2 retarded children had a fragile X syndrome. The other had a severe periventricular leukomalacia due to prematurity, which, per se, was sufficient to account for mental delay. In the second group (n=5), the vermis was highly malformed, obviously dysplastic, presenting with only one fissure or no fissure at all. It was constantly associated with major brain anomalies, most often a complete corpus callosum agenesis. All the patients in this group were more or less severely retarded. Vermis anatomy in DWMs was statistically correlated to neurological and intellectual outcome. Is the vermis dysplasia responsible, in itself, for this poor outcome? No answer can be given from this series, because retardation was observed in children who always had both a severely dysplastic vermis and other brain malformations. No other patient-related factor was statistically correlated to the outcome, in particular, hydrocephalus and extracerebral malformations. CONCLUSION: We described two types of DWM. The most frequent is characterized by an isolated and partially agenetic vermis. This malformation is compatible with a normal life. The second type consists of a severely abnormally lobulated vermis and associated brain malformation. This malformation is always accompanied by mental retardation.

Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation.

Half of patients with the Dandy-Walker malformation (DWM) have normal intellectual development. We aimed to identify feature on MRI associated with good intellectual prognosis. We reviewed 20 patients with DWM diagnosed on MRI, mean age 14.6+/-9.9 years. We assessed their intellectual development and related it to the MRI features. We found two groups with a statistically different intellectual outcome. All 14 patients with normal intellectual development had a normal lobulation of the vermis, without supratentorial anomalies. Of the six patients with mental retardation, three had an abnormal vermis, together with dysgenesis of the corpus callosum. In the other three, there were normal vermian anatomy with associated anomalies. Normal lobulation of the vermis, in the absence of any supratentorial anomaly, appears to be a good prognostic factor in DWM. Preservation of cerebrocerebellar pathways and neonatal plasticity could explain the normal intellectual development. These findings might be useful in prenatal diagnosis.

[Treatment of intracranial cysts in children: peritoneal derivation or endoscopic fenestration?]. / Traitement des kystes intracrâniens de l'enfant: dérivation péritonéale ou fenestration endoscopique?

OBJECTIVE: The goal of this study is to evaluate the indications, benefit and complications of shunts and endoscopic fenestrations in the treatment of malformative intracranial cysts. MATERIAL AND METHOD: The records of 172 consecutive children (mean age of 4 years) were reviewed. All had a malformative cyst. Dandy Walker malformation, mega cisterna magna, and cysts from tumoral or porencephalic origin were excluded from the study. The cysts were diagnosed either in utero (n=64) or postnatally (n=108). Most of them were unique (94.8%) and localized in the posterior fossa (26.2%) or at the convexity (23.2%). Indication for surgery was based on clinical symptoms (n=101; 86.3%) or size of the lesion (n=16; 13.7%). Endoscopy was the treatment of choice when cysts were in closed relationship with enlarged ventricles. Shunting procedures were indicated when endoscopy was not feasible and craniotomies when shunt insertion was unsafe or diagnosis uncertain. Fifty children underwent an endoscopic fenestration, 55 a shunting procedure, 7 the puncture or the external drainage of a pericerebral collection and 5 a direct surgical approach. The mean follow-up was 5.5 years. Psycho-motor, intellectual and school performances were evaluated in 93 children (54%). Success was defined by both the disappearance of symptoms of increased intra cranial pressure and regression of the cyst. RESULTS: Compared to shunts, endoscopic fenestrations were more frequently successful (70% vs 61.8%), led to less complications (6% vs 61.8%) and to a lesser number of reoperations (in average 1.6 operation per child vs 2.2). Median developmental and intellectual quotients for the whole series were respectively 98 and 97 and did not depend upon the type of treatment. CONCLUSION: The study of this series shows that treatment modalities necessarily vary according to the site of the cysts but that endoscopic fenestrations are preferable to shunts whenever feasible.

Management of hydrocephalus in pediatric patients with posterior fossa tumors: the role of endoscopic third ventriculostomy.

OBJECT: The authors undertook a study to evaluate the effectiveness of endoscopic third ventriculostomy in the management of hydrocephalus before and after surgical intervention for posterior fossa tumors in children. METHODS: Between October 1, 1993, and December 31, 1997, a total of 206 consecutive children with posterior fossa tumors underwent surgery at Hjpital Necker-Enfants Malades in Paris. Excluded were 10 patients in whom shunts had been placed at the referring hospital. The medical records and neuroimaging studies of the remaining 196 patients were reviewed and categorized into three groups: Group A, 67 patients with hydrocephalus present on admission in whom endoscopic third ventriculostomy was performed prior to tumor removal; Group B, 82 patients with hydrocephalus who did not undergo preliminary third ventriculostomy but instead received conventional treatment; and Group C, 47 patients in whom no ventricular dilation was present on admission. There were no significant differences between patients in Group A or B with respect to the following variables: age at presentation, evidence of metastatic disease, extent of tumor resection, or follow-up duration. In patients in Group A, however, more severe hydrocephalus was demonstrated (p < 0.01): the patients in Group C were in this respect different from those in the other two groups. Ultimately, there were only four patients (6%) in Group A compared with 22 patients (26.8%) in Group B (p = 0.001) in whom progressive hydrocephalus required treatment following removal of the posterior fossa tumor. Sixteen patients (20%) in Group B underwent insertion of a ventriculoperitoneal shunt, which is similar to the incidence reported in the literature and significantly different from that demonstrated in Group A (p < 0.016). The other six patients (7.3%) were treated by endoscopic third ventriculostomy after tumor resection. In Group C, two patients (4.3%) with postoperative hydrocephalus underwent endoscopic third ventriculostomy. In three patients who required placement of CSF shunts several episodes of shunt malfunction occurred that were ultimately managed by endoscopic third ventriculostomy and definitive removal of the shunt. There were no deaths; however, there were four cases of transient morbidity associated with third ventriculostomy. CONCLUSIONS: Third ventriculostomy is feasible even in the presence of posterior fossa tumors (including brainstem tumors). When performed prior to posterior fossa surgery, it significantly reduces the incidence of postoperative hydrocephalus. The procedure provides a valid alternative to placement of a permanent shunt in cases in which hydrocephalus develops following posterior fossa surgery, and it may negate the need for the shunt in cases in which the shunt malfunctions. Furthermore, in patients in whom CSF has caused spread of the tumor at presentation, third ventriculostomy allows chemotherapy to be undertaken prior to tumor excision by controlling hydrocephalus. Although the authors acknowledge that the routine application of third ventriculostomy in selected patients results in a proportion of patients undergoing an "unnecessary" procedure, they believe that because patients' postoperative courses are less complicated and because the incidence of morbidity is low and the success rate is high in those patients with severe hydrocephalus that further investigation of this protocol is warranted.

Repeat endoscopic third ventriculostomy: is it worth trying?

OBJECT: The goal of this study was to evaluate the safety, efficacy, and indications for repeat endoscopic third ventriculostomies (ETV). METHODS AND RESULTS: We reviewed the records of 20 patients who had undergone repeat ETV from 1987 to 1999. Their ages ranged from 8 months to 53 years (mean 17 years). The primary etiologies of hydrocephalus were: primary aqueductal stenosis (9 cases), tumor (5), Chiari malformation (2), prior infection (2), prior intraventricular hemorrhage (1), and blocked foramen of Monro (1 patient). The interval between the first and second ETVs ranged from 8 days to almost 6 years (mean 12.8 months). The intraoperative findings at repeat surgery were: occlusion of the primary orifice by scar (10 cases), virginal floor of the third ventricle (5 cases), pinhole ventriculostomy (3 cases), incompletely penetrated membrane (1 case), and blood clot occluding the orifice (1 case). The follow-up period ranged from 3 to 47 months (median 20 months). Repeat ETV was successful in 13 patients (65%). These patients did not require further shunting or other procedures during follow-up. Seven patients (35%) required placement of a shunt after repeat ETV. Several complications were observed in 1 patient (5%), including seizures, elevated ICP, bilateral pulmonary edema, and cardiac arrhythmia. This patient ultimately recovered fully; the ETV was successful, and the patient did not require a shunt. CONCLUSIONS: Based on the experience of this group of patients, repeat ETV is as effective and as safe as a primary ETV procedure, and should be attempted in selected patients.

Visual agnosia after treatment of a posterior fossa ependymoma in a 16-month-old girl.

We present the clinical observation of a 16-month-old girl treated for a posterior fossa ependymoma who experienced severe and delayed visual dysfunction. She was initially treated by surgery and conventional chemotherapy. When she relapsed at age 3 years, the salvage treatment combined high-dose chemotherapy, second surgery, and local irradiation. At age 4 years, disturbed gait and dysarthric speech appeared rapidly, and she became unable to recognize objects and people. Computed tomography revealed bilateral calcifications in the cerebellum and temporal and occipital lobes but no relapse. The neuropsychologic evaluations revealed signs of visual agnosia and marked intellectual impairment. The role of the different treatment modalities in the pathogenesis of this unusual syndrome is discussed.

Treatment of post-haemorrhage ventricular dilatation with an Ommaya's reservoir: management and outcome of 64 preterm infants.

Over a 5-year period, an Ommaya's reservoir has been inserted in a single neurosurgical centre in each of 64 preterm infants with post-haemorrhage ventricular dilatation (PHVD). Their mean gestational age at birth was 29.2 weeks. The average age at reservoir insertion was 24 days. Seventeen infants received a fibrinolytic agent through the reservoir. Infections occurred in 14 patients. Two patients died after handling of their reservoirs. Thirty-one of the 45 survivors required a shunt placement. After a follow-up ranging from 6 months to more than 4 years, 17 of 43 patients have severe sequelae or are handicapped. Compared with the results of other studies, our experience does not suggest that treatment of PHVD with an Ommaya's reservoir is beneficial in term of mortality, shunt placement, and/or neurological outcome. The place of Ommaya's reservoir among the various means of managing PHVD should be carefully evaluated, as should the best way of using this appliance safely.

Intracranial Ewing sarcoma/'peripheral' primitive neuroectodermal tumor of dural origin with molecular genetic confirmation.

Ewing sarcoma/'peripheral' primitive neuroectodermal tumor (ES/pPNET) is the designation given to a family of small cell neoplasms that typically arise in bone or soft tissue and are unified by their common expression of the MIC2 antigen and specific translocations involving a gene on chromosome 22q12 [the most common being t(11;22)(q24;q12)]. ES/pPNET of intracranial origin is extraordinary. We report the case of a 6-year-old boy with a large left frontal region mass that adhered to dura and was extracerebral at surgery. Histologic study revealed a high-grade, undifferentiated-appearing neoplasm of small cell type that was negative on immunostudy for glial fibrillary acidic protein, synaptophysin, desmin, leukocyte common antigen, smooth muscle actin and epithelial membrane antigen, but positive for vimentin and neuron-specific enolase and diffusely labeled by antibody O13 (which recognizes the MIC2 gene product). RNA-based polymerase chain reaction assay confirmed the diagnosis of ES/pPNET by demonstrating fusion transcripts indicative of t(11;22) translocation. Bone scan, computerized tomography of the chest and bone marrow examination revealed no systemic tumor. The limited observations published to date suggest that primary intracranial ES/pPNET is most likely to present in childhood as a circumscribed, contrast-enhancing and dural-based extracerebral mass. It must be distinguished from a variety of small cell neoplasms, particularly PNETs of central neuroepithelial origin.

Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology.

PURPOSE: To evaluate a strategy that avoids radiotherapy in first-line treatment in children under 5 years of age with brain or posterior fossa ependymoma, by exclusively administering 16 months of adjuvant multiagent chemotherapy after surgery. PATIENTS AND METHODS: Between June 1990 and October 1998, 73 children with ependymoma (82% with high-grade tumors) were enrolled onto this multicenter trial. Children received adjuvant conventional chemotherapy after surgery consisting of seven cycles of three courses alternating two drugs at each course (procarbazine and carboplatin, etoposide and cisplatin, vincristine and cyclophosphamide) over a year and a half. Systematic irradiation was not envisaged at the end of chemotherapy. In the event of relapse or progression, salvage treatment consisted of a second surgical procedure followed by local irradiation with or without second-line chemotherapy. RESULTS: Conventional chemotherapy was well tolerated and could be administered in outpatient clinics. No radiologically documented response to chemotherapy more than 50% was observed. With a median follow-up of 4.7 years (range, 5 months to 8 years), the 4-year progression-free survival rate in this series was 22% (95% confidence interval [CI], 13% to 43%) and the overall survival rate was 59% (95% CI, 47% to 71%). Overall, 40% (95% CI, 29% to 51%) of the patients were alive having never received radiotherapy 2 years after the initiation of chemotherapy and 23% (95% CI, 14% to 35%) were still alive at 4 years without recourse to this modality. In the multivariate analysis, the two factors associated with a favorable outcome were a supratentorial tumor location (P =.0004) and complete surgery (P =.0009). Overall survival at 4 years was 74% (95% CI, 59% to 86%) for the patients in whom resection was radiologically complete and 35% (95% CI, 18% to 56%) for the patients with incomplete resection. CONCLUSION: A significant proportion of children with ependymoma can avoid radiotherapy with prolonged adjuvant chemotherapy. Deferring irradiation at the time of relapse did not compromise overall survival of the entire patient population.

Antenatal diagnosis of spinal lipomas.

OBJECT: The purpose of this study was to highlight the key prenatal diagnostic features of fetal spinal lipomas. METHODS AND RESULTS: All fetuses in whom a spinal lipoma was suspected prenatally or diagnosed postnatally were included in the study. Lumbosacral lipoma was diagnosed in 16 fetuses (group 1), but at birth 3 of these were found to have other forms of occult dysraphism. In 2 other fetuses (group 2) a meningocele was suspected but at birth these lesions were found to be lumbosacral lipomas. The lipomas were covering large meningoceles in 13 fetuses and filling the cul-de-sac in 2 others. Three sets of parents decided on termination. Of the 13 children born with a spinal lipoma, 9 were operated on and 4 were managed conservatively. At the last follow-up, 7 were neurologically intact. CONCLUSION: The distinction between meningomyelocele and spinal lipoma is possible antenatally. The differential diagnosis between lipoma and the other forms of occult dysraphism is more difficult.

When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre.

UNLABELLED: 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.

[Clinical features of gliomas of the chiasma. A retrospective study of a series of 18 patients]. / Particularités cliniques des gliomes du chiasma. Etude rétrospective sur une série de 18 patients.

Eighteen patients with a glioma of the chiasma were managed between 1980 and 1994. A retrospective analysis of this series showed that the most frequent functional ophthalmological signs were visual loss, nystagmus, or strabismus. Funduscopic examination was abnormal in 65%, mostly with optic atrophy. Other exams (visual field and electrophysiology) were inadequate for diagnosis. Our series demonstrated that early diagnosis of involvement of the chiasma could improve functional prognosis as rapid management can improve prognosis.

Growth, puberty and hypothalamic-pituitary function in children with suprasellar arachnoid cyst.

UNLABELLED: 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 MICROg/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.

The management of desmoplastic neuroepithelial tumours in childhood.

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.

The contribution of prenatal diagnosis to the understanding of malformative intracranial cysts: state of the art.

This review evaluates the contribution of prenatal diagnosis to the understanding of intracranial cysts. We describe the outcome of 54 fetuses in which prenatal investigations indicated the presence of such lesions. The cysts were diagnosed between 20 and 30 weeks of gestation. Most (63%) were supratentorial and interhemispheric. There was only a single sylvian cyst. In the infratentorial compartment, median retrocerebellar cysts were predominant. Incisural cysts accounted for 14.8% of the series. Nine pregnancies were interrupted because of the presence of associated brain disorders. Forty-five children are alive. Thirty-four had neuropsychological tests. Cysts rarely progressed, most frequently stabilized and often regressed postnatally. Hydrocephalus was rare. In two cases delivery was precipitated at 36 weeks to allow urgent treatment of rapidly evolving cysts. Thirteen children (28.2%) were treated postnatally, in general for developing cysts. The median follow-up for the whole series exceeds 4 years. Behavior, neurological development, and intelligence are normal in 88% of the cases, and 91% have a normal neurological status. Prognosis at the time of the prenatal consultation was correct in 89% of the cases. We emphasize the value of prenatal magnetic resonance imaging and karyotype studies to limit risks of incorrect prognosis.

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.