Macrophagic activity in intracerebral germinoma: ultrastructural study of a case.

This report presents the ultrastructural study of a germinoma of the third ventricle occurring in a 13 year old boy. The electron microscopic data showed similarities linking this tumor to gonadal and mediastinal germinomas and emphasized the exceptional glycogenic storage in tumor and stroma cells. Another morphological analogy was the intense macrophagic activity that led to tumor cell phagocytosis. The authors discuss the meaning of such an immune response, which is usually not observed in cerebral tumors.

Detection of i(17q) chromosome by fluorescent in situ hybridization (FISH) with interphase nuclei in medulloblastoma.

Medulloblastomas are the most frequent primitive neurectodermal tumors in children. An isochromosome for the long arm of 17, i(17q), is found in 30% of medulloblastomas. For some authors, this abnormality is observed in cases with a shorter survival time. In our cytogenetic studies of 30 medulloblastomas, we observed i(17q) in only three cases, a monosomy 17 in two cases, a monosomy 22 in four cases, nonspecific numerical or structural abnormalities in five cases, and normal karyotypes in 12 cases. We compared the results of karyotypic analysis after culture and FISH with a chromosome 17 alpha satellite DNA probe on interphase nuclei in five cases of medulloblastoma. In one case, i(17q) was only observed in four cells in karyotypic analysis, in three cases a normal karyotype was found, and in one case karyotypic analysis was impossible. In all of these cases, i(17q) was observed in a great number of nuclei by FISH on interphase nuclei. Our study shows that the FISH on interphase nuclei permitted us to observe i(17q) in the cases where it was not or could not be completely detected by karyotypic analysis. The association of these two techniques is required to detect i(17q), an abnormality whose prognosis value in medulloblastomas is now recognized.

Are juvenile pilocytic astrocytomas benign tumors? A cytogenetic study in 24 cases.

We performed a cytogenetic study on 24 pilocytic astrocytomas: 23 in children and 1 in a young adult. We observed 12 normal karyotypes. In 12 karyotypes with structural and/or numerical abnormalities, chromosomes 7, 8, and 11 were most frequently involved. One case recurred and presented chromosomal abnormalities (hyperdiploidy) in the first tumor and additional structural abnormalities in the second tumor. We believe that chromosomal abnormalities in pilocytic astrocytomas are frequent and indicate tumoral progression.

Cytogenetic study of 33 ependymomas.

Ependymomas are glial tumors. They constitute approximately 5-10% of intracranial tumors. Ependymomas are tumors which can recur. Predictive factors of outcome in ependymomas are not well-established. Karyotypic studies on ependymomas are relatively scarce, and no specific chromosomal change has been described in these neoplasms. We performed a cytogenetic study of 33 ependymomas, of which eight were recurrent tumors, to determine the type and incidence of cytogenetic changes.

Shunt implantation: reducing the incidence of shunt infection.

Shunt infection remains the foremost problem of shunt implantation after mechanical malfunctions. Diversionary cerebrospinal fluid shunt implantation has a high complication rate, with 5% to 15% of such shunts becoming infected. Of these infections, 70% are diagnosed within 1 month after surgery and more than 90% within 6 months. Shunt infection in the vast majority of cases is therefore a complication of shunt surgery. The authors review their experience with shunt implantation during two time periods. From January, 1978, to December, 1982, 302 children with hydrocephalus underwent 606 operations. Among these children, 47 (15.56%) developed a proven shunt infection, with an incidence of infection per procedure of 7.75%. As a result of this study, a new protocol for shunt procedures involving modifications in the immediate pre-, intra-, and postoperative management of children undergoing shunt implantation was initiated. With this new protocol, 600 children underwent a total of 1197 procedures between January, 1983, and December, 1990. The incidence of shunt infection decreased dramatically, with two infections (0.33%) in 600 patients and a per-procedure rate of 0.17%. The overall annual risk of a shunt infection in the pediatric neurosurgical unit is currently 1.04%.

Prognostic factors in intracranial ependymomas in children.

OBJECT: The occurrence of intracranial ependymomas in children is relatively infrequent, and their prognostic factors are still controversial, especially regarding histological composition. METHODS: A retrospective study was conducted of 37 children treated during the last 20 years for intracranial ependymomas at the Hôpital de la Timone. Both univariate and multivariate statistical analyses were performed to assess the prognostic relevance of patient age and sex, extent of tumor removal, location of the tumor (supratentorial compared with infratentorial, median compared with lateral), tumor histological composition, and adjuvant therapies in affecting the 5-year progression-free survival (PFS) rate and overall survival (OS) rate. The following histopathological features, either alone or in combination, were analyzed: endothelial proliferation, necrosis, loss of differentiating structures (present compared with absent), the number of mitotic figures per 10 hpf, and cellularity (number of nuclei/5 hpf). In addition, immunohistochemical detection of Ki-67 antigen was performed and the Ki-67 labeling index (LI) evaluated in all cases. The 5-year OS and PFS rates were 45% and 25%, respectively (median follow up 34 months). Four patients died of disease without remission (median 163 days) and disease in 21 patients relapsed: 18 in situ and three both in situ and distantly. On univariate analysis total surgical resection and median infratentorial location were associated with a better outcome (p < 0.002) for both OS and PFS. Loss of differentiating structures was associated with poor prognosis (p < 0.008) and the combination of necrosis, endothelial proliferation, and mitotic index greater than 5 was also a negative predictive factor for both OS (p < 0.002) and PFS (p = 0.02). The PFS time was shorter in patients younger than 4 years of age and in patients in whom a Ki-67 LI greater than 1 was found (p = 0.03 and 0.006, respectively). Adjuvant radiotherapy and chemotherapy were not relevant to prognosis. Moreover, among the 15 patients in whom total excision was achieved, OS was better in those who did not receive adjuvant therapies. In contrast, adjuvant therapies significantly enhanced PFS time in patients in whom tumor excision was incomplete. CONCLUSIONS: This study and analysis of the literature further highlight that total tumor removal is the treatment of choice for ependymomas in children. Postoperative measurement of residual tumor is required, especially because a subgroup of patients might be treated by surgery alone. Median infratentorial ependymomas have to be distinguished from the lateral type. Appropriate and reproducible histological parameters and Ki-67 LI are of interest as predictors of outcome.

Intracranial meningiomas in children: review of 29 cases.

BACKGROUND: Meningiomas are predominantly tumors of the fifth and sixth decades of life. Although rare in infancy and childhood, they represent an important field in pediatric neurosurgery. METHODS: Twenty-nine children under 15 years of age with intracranial meningiomas were treated during the period 1968-1994 in the Department of Pediatric Neurosurgery in Marseille (23 cases) and in the Department of Neurosurgery of Cerrahpasa Medical School in Istanbul (6 cases). These tumors represented 2.4% of all central nervous system (CNS) tumors in this age group. RESULTS: There were 18 boys and 11 girls. Eighteen cases (62%) occurred between 10 and 15 years of age and two cases were infants. Twelve children (41%) had associated neurofibromatosis. Presenting symptoms were related to the tumor location. Thirty-one intracranial meningiomas were observed in these 29 children. Cerebral convexity was the most common location and 13% of the tumors had no dural attachment. Thirty-one tumors were operated on and total removal was achieved in 25 patients (86.2%). Five patients died, one during surgery. There was no mortality among the 17 children without neurofibromatosis. After a mean 6.5 year follow-up period, 13 patients (45%) are neurologically intact, 8 patients (27.5%) have a moderate disability, without evidence of tumor recurrence. CONCLUSION: Childhood meningiomas occur predominantly in males. Absence of dural attachment is more common in children than in adults. Childhood meningiomas have a low recurrence rate. They are frequently associated with neurofibromatosis; this is the most important factor influencing outcome.

Extradural hematomas in infants.

This study reports 19 cases of extradural hematomas in infants for their relative frequency in head injury of children, their clinical findings, their particular evolution and the therapeutic problems specific to this age.

Multiple lateral meningoceles, distinctive facies and skeletal anomalies: a new case of Lehman syndrome.

We describe a 19-year-old boy who presented with facial dysmorphism, multiple lateral meningoceles, skeletal abnormalities and normal intelligence. Neurofibromatosis and Marfan syndrome were excluded. Electron microscopy of the skin showed non-specific abnormalities suggesting a connective tissue disorder. The features of this boy closely resemble those in a mother and daughter with Lehman syndrome.

[Disk calcifications in children. Apropos of 17 cases]. / Calcifications discales de l'enfant. A propos de 17 cas.

Seventeen cases of calcification of the intervertebral discs in childhood have been seen. Only cervical lesions gave symptoms. In several cases the lesions were multifocal (cervical and thoracic) and there was a familial history in one case. Two cases were observed for 10 and 8 years respectively. It was noted that cervical calcifications tend to disappear while thoracic ones persist but are asymptomatic. The authors postulate that this is due to differences in the physiology of the nucleus and in the biomechanics at different levels of the spine. A high frequency of associated spinal deformities was noted. For this reason, the late prognosis must be guarded.

[Brain stem cavernomas in children. Nine case reports and literature review]. / Cavernomes du tronc cérébral chez l'enfant.

We report our experience with 9 cases of brain stem cavernoma in children aged 15 years during the period 1987-2000. All patients were admitted with lesion-related symptoms, except in one where the brain stem cavernoma was discovered on a routine MR scan. A female predominance was noted in the present study. In 2 children, brain stem cavernoma was associated with multiples lesions. All patients except two with multiple cavernomas underwent surgery. One child died 2 months after surgery from recurrent hemorrhage. The eight others are alive. Among the 8 survivors, only one has worsened. Children with brain stem cavernoma who develop persistent or progressive deficit are candidates for surgery. When surgery is indicated, total removal is mandatory to avoid the risk of recurrence and rebleeding.

[14 cases of communicating syringomyelia associated with Chiari I malformation in children]. / 14 cas de syringomyélie communicante associée à une malformation de Chiari type I chez l'enfant.

The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symptoms were motor weakness (57%), sensory signs (50%), scoliosis (50%) and bladder dysfunction (21.5%), while signs of admission were pyramidal signs (78.5%) with motor deficit (43%), spinal deformities (64.2%), syringomyelic syndrome (36%) and sensory deficit (21.5%). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71.4%) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57% of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversies concerning the treatment and concluded with their attitude concerning this pathology in children.

[Arachnoid cysts of the middle cranial fossa in children. A review of 75 cases, 47 of which have been operated in a comparative study between membranectomy with opening of cisterns and cystoperitoneal shunt]. / Les kystes arachnoïdiens temporo-sylviens chez l'enfant. Revue de 75 cas dont 47 ont été opérés et étude comparative entre la membranectomie avec ouverture des citernes et la dérivation kystopéritonéale.

The authors report their experience concerning 75 cases of middle fossa arachnoid cysts observed in children during the period 1975-1993, 47 of which (62.6%) were operated upon. The aim of this study was to study the clinical presentation of these cysts, to discuss the surgical indications and to compare the results of the various techniques used to treat these malformations. Head injury was revealing in 17 cases (22.6%) and among these, 12 patients presented intracranial complications (subdural effusions; 6 cases, subdural hematomas: 4 cases and intracystic hematomas: 2 cases). The most usual signs and symptoms were: intracranial hypertension (25.3%), epilepsy (16%) and temporal bulging (24%). Twenty-one patients (44.7%) underwent a cystoperitoneal shunt; 20 patients (42.5%) were treated by membranectomy with opening of the basal cisterns and removal of intracystic (2 cases) or subdural hematoma (4 cases); 2 patients (4.3%) were treated using membranectomy, opening of the cisterns and cystoperitoneal shunt and 4 patients (8.4%) underwent a subduroperitoneal shunt. The long-term results were good regardless of the surgical procedure; nevertheless, only one patient among 20 cases treated by membranectomy and opening of the cisterns developped complications (5%), while multiple shunt revisions were necessary in 11 children (40.7%) out of 27 where a shunt was inserted. The authors conclude that membranectomy and opening of the basal cisterns is the procedure of choice to treat middle fossa arachnoid cysts in children.

[Surgical posterior approach in spondylolisthesis (author's transl)]. / Opérations des spondylolisthesis par voie postérieure.

The authors report their experience about 60 patients who suffered of a spondylolisthesis and who were treated by a surgical procedure. A posterior approach has been used for all the patients. After a brief review of the clinical and radiological features of the disease, the authors emphazise the advantages of the posterior approach; only this technique allows a correct access both to the bone and the nerve roots lesions. Two types of operations are proposed:--the ablation of the posterior arch of the concerned vertebra (Gill's operation).--a posterior arthrodesis by mean of an homograft, using one of the following technique: an Albee grafting a Bosworth grafting an ilio-transversary grafting. The results are considered from a clinical, and an anatomo-radiological point of view.

[Intraosseous meningioma in a child]. / Méningiome intra-osseux chez un enfant

The authors present a case of intra-osseous meningioma in a 10 years old boy, located on the level of the vertex in the fronto-interparietal region, with the shape of a hand mass, painless, free from all neurological, ophtalmological and angiographic signs. The x-rays examination permit a diagnosis of an osteoma of the cranial vault. The surgery enables the exeresis of a large tumor of the vault 3 cm thick, without any attachment to the dura-matter. The histological examination reveals that it is, in fact, an intra-osseous meningioma. From this observation the authors carried out a review of all litterature in this field, only 12 observations are found again, and none of them deal with children. These intra-osseous meningioma develop from Pacchioni granulations included in the diploic region. They bring about handly any clinical signs and the x-rays findings do not enable us to put forward or suggest the diagnostic of intra-osseous meningioma.

[Aneurysms of the vein of Galen. Angiographic study and morphogenetic considerations]. / Les anévrysmes de la veine de Galien. Etude angiographique et considérations morphogénétiques.

The angiographic and/or anatomic study of a series of 30 cases of Vein of Galen aneurysm (which is defined as an arterio-venous fistula within the wall of the vein of Galen itself, therefore as a purely extra cerebral lesion) permits the analysis of the afferent arteries, of the efferent veins, and of the aneurysmal sac itself. The examination of the afferent arteries confirms the data from the literature, indicating that most often it consists of the abnormal hypertrophy of otherwise normally organized arteries, including the dural arteries. In particular, the involvement of distal subcallosal branches of the anterior cerebral arteries as well as the arterio-arterial maze extending above the collicular plate, fit well the normal anatomy. The venous side of the malformation nevertheless presents with more unusual features; the most striking is the non-opacification of the straight sinus and/or part of the transverse sinuses in half of the cases; also, an aberrant falcine sinus is frequently observed, which actually represents the persistence of a normal channel in the fetal anatomy. A more complex pattern was observed in two cases in which the aneurysmal sac drained into the straight sinus via a loop made of a falcine sinus, the superior sagittal sinus and then backward through a second falcine sinus. Finally, from the systematization of the afferents arteries, the aneurysmal sac appears to belong both to the (prosencephalic) velum interpositum and to the ambient cistern. Such a vessel cannot be either the true vein of Galen or an internal cerebral vein; therefore it has to be a persistent fetal vein, the median prosencephalic vein, which drains the choroid plexuses of the lateral and third ventricles between the 7th and 12th weeks, and disappears normally to be replaced by the internal cerebral veins, when the intrinsic vascularization of the neural tube develops. The adult pattern of the brain artery being attained during the 8th week, the event that led to the arterio venous fistula should have occurred some time during the 3rd month. Unfortunately, no clue was found to orient to a specific cause for the anomaly. The frequently observed venous occlusions are actually not seen in neonates but rather in older children. This, and the fact that spontaneous thromboses within the malformative veins have been observed in vivo, make likely the conclusion that venous occlusions are a secondary phenomenon only.

[Intracranial arterial aneurysm in children. A cooperative study. Apropos of 43 cases]. / L'anévrysme artériel intra-cranien chez l'enfant. Etude coopérative. A propos de 43 observations.

This joint study describes 43 cases of intracranial arterial aneurysms in children diagnosed on the basis of clinical symptoms. In the pediatric age group, this malformation is notable because of the marked sex predilection in males (70%) and an unequal topographic incidence in the circle of Willis, where carotid artery (39.3%) and anterior communicating artery lesions (30%) predominate. The most frequent clinical sign was subarachnoid hemorrhage (81%), although symptoms caused by compression revealed the abnormality in 2.3% of patients. In this series, 11% of the patients suffered a head injury at the time of the hemorrhagic accident; this finding has been reported previously in the literature. Today, treatment is always surgical, consisting in removal of the aneurysmal sac. Surgical results are encouraging; all grade lesions considered together, 63.4% of the children were cured without any sequelae, 19.5% lost one school year but were able to lead a normal life, and 4.8% remained severely handicapped; overall postoperative mortality was 12.3%. Cerebral plasticity and tolerance of spasm in children are fundamental features of this aneurysmal pathology which partially explain the favorable results obtained with surgery.

[Craniopharyngioma in children: MRI study of 43 cases]. / Les crAniopharyngiomes de l'enfant.

BACKGROUND AND PURPOSE: Craniopharyngiomas are intra-cranial tumors, relatively frequent in children, expanding in the pituitary stalk axis, from the third ventricle to the sphenoid body. Plain films and CT scan generally show a calcified lesion, deforming the sella turcica. MRI improves tumor description and topographic and structural analysis of the lesion. The aim of this study is to analyze the MRI aspect of craniopharyngiomas in a pediatric population and to correlate findings with surgical data. METHODS: MR and CT studies of 43 pediatric cases of histology-proven craniopharyngiomas were reviewed retrospectively. Tumor emergence, extensions and signal on different sequences were recorded. We searched for radio-surgical correlations. RESULTS: Craniopharyngiomas can be classified into two groups: intra-sellar tumors and third ventricle floor tumors (infundibulum and tuber cinereum). Preferential routes of extension are observed in each group correlated with consistency (cystic and/or solid). Surgical data confirmed these results. CONCLUSIONS: MRI is crucial for the pre-therapeutic evaluation of craniopharyngiomas allowing not only a detailed description of the tumor but also guiding therapeutic decisions. This series demonstrated that craniopharyngiomas exhibit two different types of localization and behavior. Embryonic development of the tumor explains the topographical differences.