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A Pierre-Kahn

Publications and source records attributed to A Pierre-Kahn.

At least 19 recordsLinked to original sources
PubMed1995

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.

Achievement↗
PubMed1995

Hydrocephalus associated with intramedullary low-grade glioma. Illustrative cases and review of the literature.

Over the past 15 years, eight children affected by intramedullary low-grade gliomas associated with hydrocephalus were treated at l'Hôpital des Enfants Malades. In all cases the diagnosis of hydrocephalus was made prior to that of the spinal tumor. Neuroradiological examination of all patients revealed contrast enhancement of the intracranial subarachnoid spaces. In six cases this was progressive, suggesting subarachnoid spread of the tumor, which was confirmed in two cases by histological examination. The authors analyzed 38 cases of intramedullary low-grade glioma associated with hydrocephalus that were reported in the literature. Fifteen of the cases had intracranial leptomeningeal seeding. Several hypotheses have been proposed to explain this unusual association, such as 1) increase in cerebrospinal fluid (CSF) viscosity because of elevated fluid protein content; 2) obliteration of the cisterna magna due to a rostral extension of the tumor; and 3) blockage of the spinal subarachnoid pathways of CSF resorption. Two other theories seem of particular interest. Bamford and Labadie suggested that the abnormal presence of fibrinogen in the CSF and its transformation into fibrin at the level of the basal cisterns and Pacchioni's granulation may alter CSF hydrodynamics. This mechanism alone is sufficient to induce hydrocephalus of the communicating type. In addition, as suggested by Maurice-Williams and Lucey, the resulting leptomeningeal fibrosis might predispose secondary implantation of neoplastic elements in the subarachnoid spaces of the intracranial compartment.

Adolescent↗
PubMed1995

Chronic tonsillar herniation in Crouzon's and Apert's syndromes: the role of premature synostosis of the lambdoid suture.

The incidence of chronic tonsillar herniation (CTH) was evaluated with magnetic resonance imaging in 44 patients with Crouzon's syndrome and 51 with Apert's syndrome; the incidence was 72.7% in Crouzon's syndrome and 1.9% in Apert's syndrome. All the patients with Crouzon's syndrome and progressive hydrocephalus had CTH, but of 32 individuals with Crouzon's syndrome and CTH, only 15 had progressive hydrocephalus. Five patients with Apert's syndrome were treated for progressive hydrocephalus; none had CTH. The patterns of suture closure in these two groups of patients were studied, and significant differences in coronal, sagittal, and lambdoid sutures were found between patients with Crouzon's and Apert's syndromes. In Crouzon's syndrome, significant differences in the pattern of lambdoid suture closure were found between the groups with and without CTH; in the group with CTH, the lambdoid closure appeared earlier. The authors propose that the high incidence of individuals with CTH who have Crouzon's syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.

Acrocephalosyndactylia↗
PubMed1994

Surgical approach to children with craniopharyngiomas and severely impaired vision: special considerations.

A series of 30 children with craniopharyngiomas is presented, analyzing visual sequelae following surgery. All were operated on with the goal of total removal. Surgical routes used were subfrontal prechiasmatic or translamina terminalis, pterional interopticocarotid, transcallosal, or a combination of these various avenues. The choice of the approach depended upon the type, pre- or retrochiasmatic, of the tumor expansions. Children with large cysts and preoperatively low vision, or vision at risk of rapid deterioration, were submitted to a protocol aiming to preoperatively decompress the visual pathway by repeated aspirations of the cyst with the hope this would improve vision before surgery and limit risks of postoperative visual deterioration. In these cases, the removal of the tumor was postponed until vision had reached a stable new level. The authors emphasize the interest of this procedure in reducing the rate of postoperative visual sequelae. However, they also point out that the interopticocarotid route carries a nonnegligible risk of postoperative visual worsening.

Adolescent↗
PubMed1993

[An uncommon site of malignant germ cell tumor secreting alpha fetoprotein: the posterior mediastinum].

BACKGROUND: While germ cell tumors generally occur in the gonads, they may also appear at other sites, from the sacrococcygeum area to the central nervous system. This report describes a case of such a tumor in the posterior mediastinum that developed intraspinally in a dumb-bell fashion. CASE REPORT: A 2 1/2 year-old girl was admitted for abdominal tenderness, gait disturbance and fever. Clinical examination showed spastic paraparesis and bladder dysfunction. Thoracic X-rays showed a left postero-superior mediastinal mass with rib erosion. MRI showed that this mass had developed intraspinally between the intervertebral foramina and caused spinal cord compression at T4, T5, T6. The tumor was not calcified. Surgical resection via laminectomy was performed in emergency, but the T5 root had to be excised. Pathologic examination showed histologic features of yolk sac carcinoma; the serum alpha-foetoprotein was elevated (12, 400 IU/ml). The patient was given chemotherapy for 6 months and is well 2 years later. CONCLUSION: Germ cell tumors may appear in unusual sites. They can be identified by measuring biological markers, and this avoid primary surgery.

Child, Preschool↗
PubMed1992

Melanotic neuroectodermal tumor of the skull and meninges in infancy.

Three cases of melanotic neuroectodermal tumors of infancy are presented. Two were localized on the midline, involving the skull and extending subdurally. One was located on the inner aspect of the dura and developed intracranially. Two had a benign course following gross total removal. One had a malignant course, recurring locally and spreading within the brain. The difficulties of removing these tumors when they are implanted on the midline are stressed. Histological features of prognostic value are pointed out. Further support for neural crest origin of these tumors is given.

Child, Preschool↗
PubMed1992

[Eye manifestations and treatment of brain chordoma. Apropos of a case].

We report a case of intracranial chordoma revealed by an isolated abducent nerve palsy in a 4 years old boy. We discuss the possible symptoms of intracranial chordomas and their clinical, radiological and histological diagnosis as well as differential diagnosis. We summarize the available therapeutic modalities and we emphasize the future advantages of proton beam therapy in these lesions.

Abducens Nerve↗
PubMed1992

[Primary germinal tumors of the nervous system in children and adolescents. A retrospective study of 35 cases from 1975 to 1987].

Thirty-five cases of primary intracranial germ cell tumor treated at the Institut Gustave-Roussy between 1975 and 1987 were reviewed. There were 23 males and 12 females; the median age was 12 years (range: 3 months to 17 years). Tumors originated in the pineal region in 15 cases, in the suprasellar region in 15 cases, and in both region in 5 cases. The diagnosis was confirmed histologically in 20 cases (14 germinomas and 6 non germinomatous germ cell tumors), and cytologically in 5 cases. There were 11 secreting tumors (alpha-foetoprotein or chorionic gonadotrophin) without histological diagnosis in 6 cases. Four patients were treated with a presumptive diagnosis. Surgical resection was performed in 6 cases. Thirty-two patients were irradiated on the tumor site; 17 received a craniospinal irradiation and 7 a cranial irradiation. Six patients received chemotherapy. Twenty-five patients are alive with a median follow-up of 4 years. Prognosis is better in germinomas than in non germinomatous germ cell tumors (survival rates = 88 and 50% respectively). Chemotherapy is presently being evaluated in the treatment of germ cell tumors with the aim of using lower doses of radiotherapy and decreasing its late effects in germinomas and of improving the prognosis of other histological types.

Adolescent↗
PubMed1991

[Spinal lipomas].

Explore the source record for details and available documents.

Adolescent↗
PubMed1990

Neurosurgery with craniotomy and CT stereotactic guidance in the treatment of intracerebral space-occupying lesions.

When intracerebral space-occupying lesions are small or located deeply within the brain parenchyma, it is sometimes difficult to localize them at surgery or to design the most direct and least hazardous surgical approach. Therefore, we have developed a method that combines conventional neurosurgical techniques with craniotomy using stereotactic guidance. We have used the Brown-Roberts-Wells (BRW) stereotactic system, which allows for computed tomography (CT) or magnetic resonance imaging (MRI) guidance and does not interfere with the absolute sterility mandatory each time a flap is created. Eleven patients were operated on using this method. The deep tumors were approached through a linear incision of the cerebral cortex. Then a needle, fixed in the right position on the arc system of the BRW, was inserted toward the surface of the lesion; the exposure was finally widened by inflation of a rubber balloon set on the stereotactic needle. This technique allows the two lips of the cortical incision to be glued at the end of the operation. Gluing with a fibrin glue avoids the postoperative subdural collections that often develop when the ventricle has been opened. Except for one case, the postoperative course was uncomplicated in these patients. No permanent postoperative neurological worsening was observed even after the removal of an intrathalamic tumor.

Brain Diseases↗
PubMed1990

Medulloblastoma in childhood: progressive intellectual deterioration.

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)

Adolescent↗
PubMed1990

Growth and endocrine disorders in optic glioma.

Hypothalamo-pituitary function in children with optic glioma may be impaired by the tumour itself and by the high cranial radiation doses used in treatment. This study evaluates the effect of optic glioma and its treatment on patient growth and pubertal development. Twenty-one patients (13 boys, 8 girls), treated for optic glioma by cranial irradiation (45-55 Grays) at a mean age of 5.4 years, were evaluated before (n = 10) and/or after (n = 21) irradiation. Growth hormone (GH) deficiency was present in only 1 patient tested before irradiation and in all patients after irradiation. Precocious puberty occurred in 7/21 cases, before irradiation in 5 patients and after irradiation in 2 patients. The cumulative height loss during the 2 years after irradiation was 0.2 +/- 0.2 SD (m +/- SEM) in 7 patients with precocious puberty and 1.1 +/- 0.2 SD in 14 prepubertal patients (P less than 0.01). The corresponding bone age advance over chronological age, evaluated 1-3 years after irradiation, was 1.1 +/- 0.5 and -0.7 +/- 0.3 year in the two groups (P less than 0.01). The mean height loss between time of irradiation and the final height was 2.3 +/- 0.6 SD (n = 6). Primary amenorrhoea, associated with low oestradiol levels, occurred in two of the three girls of pubertal age. These data indicate that the high dose of cranial radiation used to treat optic glioma invariably results in GH deficiency within 2 years and that hGH therapy is required when GH deficiency is documented.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Determination by Skeleton↗
PubMed1990

Presentation and management of suprasellar arachnoid cysts. Review of 20 cases.

The clinical presentation and treatment of suprasellar arachnoid cysts remain controversial. The authors review 20 cases treated at their institution and 86 cases from the literature reported in sufficient detail for analysis. The high frequency of endocrinological disorders, which not only persist following treatment but may also develop years later despite the satisfactory decrease in volume of the cyst, are emphasized and documented. The difficulties of management are discussed, including; subfrontal approaches to these cysts; removal and/or marsupialization of the cysts, procedures that are frequently dangerous and ineffective; and ventricular shunting which often leads to a paradoxical increase in the size of the cysts. The authors emphasize the advantages of percutaneous ventriculocystostomy, which is a simple, benign, and efficacious procedure.

Adolescent↗
PubMed1990

[Treatment of suprasellar arachnoid cysts by percutaneous transfrontal ventriculocystostomy. Apropos of a series of 17 cases].

The treatment of suprasellar cysts is controversial and most often disappointing: on the one hand, subfrontal approaches to the cysts, in view of removal or fenestration, are frequently dangerous and ineffective; on the other hand, ventricular shunting frequently leads to the paradoxical increase in the size of the cyst. Because of these difficulties, the authors have treated 17 consecutive cases by the means of a percutaneous transfrontal ventriculocystostomy. In 11 cases, the cyst was perforated by a leukotome which was guided radiologically in 10 cases and by ultrasounds in 1 case. In the remaining 6 patients, the cystostomy was achieved by a monopolar coagulation under ventriculoscopic control. The results were as follows: in 3 cases, it was not possible to achieve the fenestration of the cyst with the leukotome due to the resistance of the membrane; in 1 case, the ventriculocystostomy was complicated by a poor control of the trocar progression under ultrasounds; the other 14 procedures were successful: signs of increased I.C.P. disappeared; the neurological symptoms regressed; the cysts diminished in size. However, the preoperative endocrine troubles did not decrease. The persistence of a post operative ventriculomegaly apparently did not affect the level of the intellectual development of the patients. The authors emphasize the interest of the percutaneous ventriculocystostomy, this procedure being simple, benign and efficacious. The fenestration of the cyst by coagulation appeared more suitable than by the leukotome.

Adolescent↗
PubMed1989

[Prognosis of antenatal hydrocephalus].

One hundred and eight children born with hydrocephalus were reviewed in order to evaluate their neuropsychological development. Premature children and children with spina bifida were excluded. The most frequent types of hydrocephalus were the communicating type (39 cases) and that where the condition is due to stenosis of the aqueduct of Sylvius (32 cases, not counting 6 cases of Bickers-Adams syndrome and 4 cases of toxoplasmosis); 84 per cent of the children were operated upon before the age of 3 months. The mean follow-up period was 7 years (range: 1 to 14 years). The actuarial survival rate at 10 years was 62 per cent. Among the 75 survivors, 28 per cent had an IQ of at least 80 and 50 per cent and IQ lower than 60; 29 per cent of the 52 children who reached school age had normal education. Forty-six per cent of the children showed little or no disorder of behaviour. The head circumference at birth, the size of the cerebral ventricles and the child's age at the time of surgery had no influence on the neuropsychological development. However, the prognosis seemed to be better in cases without associated malformation or infected valve, in those where the hydrocephalus was due to stenosis of the aqueduct of Sylvius (except when related to sex or toxoplasmosis) and when the first IQ evaluated at the age of about 6 months was higher than 80.

Abnormalities, Multiple↗
PubMed1989

MRI study of lumbosacral lipoma in children.

The authors report 16 cases of lumbosacral lipoma in children studied by MRI. The exact position of the cord and its relationship to the lipoma were well demonstrated in all cases but one. There was as high incidence (25%) of syringomyelia in the terminal conus. Arnold Chiari malformation was never associated, which differentiates lumbosacral lipomas from myelomeningoceles. However, the nerve roots and their relationship to the lipoma were rarely visualized. Despite these drawbacks, MRI is the examination of choice if lumbosacral lipoma is suspected in children.

Adolescent↗
PubMed1989

Benign astrocytic and oligodendrocytic tumors of the cerebral hemispheres in children.

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.

Adolescent↗