Biomedical subjects

C Sainte-Rose

Publications and source records attributed to C Sainte-Rose.

At least 55 records · Page 3Linked to original sources

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

Prevention of venous air embolism in paediatric neurosurgical procedures performed in the sitting position by combined use of MAST suit and PEEP.

We studied 60 children undergoing neurosurgical procedures in the sitting position. Routine monitoring included ECG, pulse oximetry, invasive arterial pressure, in particular mean arterial pressure (MAP), and right atrial pressure (RAP). Children were allocated to two groups. In group B lower body positive pressure and positive end-expiratory pressure (PEEP) were used for preventing venous air embolism (VAE). In this group, antishock trousers (MAST suit) were adjusted in supine children. After induction of anaesthesia, different positions were studied: supine and sitting before MAST suit inflation, sitting with MAST suit inflated up to a pressure of 40 mmHg in the lower compartments and 30 mmHg in the abdominal compartment, and finally a combination of lower body positive pressure and PEEP of 8-10 cm H2O. In group A no MAST suit or PEEP was used. Continuous monitoring of end-tidal carbon dioxide pressure throughout (PE'CO2) was used to detect VAE. In order to evaluate the transmission of pressures from the right atrium to the veins at the base of the skull, jugular bulb venous pressure (JBVP) was measured in 20 patients by retrograde catheterization. The incidence of VAE was compared in the two groups. On placing children into the sitting position, a significant decrease in RAP and JBVP was noted without significant changes in MAP in the two groups. Inflation of the MAST suit induced a dramatic increase in RAP and JBVP, reinforced by addition of PEEP. There was a strong positive relationship between RAP and JBVP. There were no deleterious side effects or differences between the two groups in peroperative blood product requirements or surgical general conditions.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

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

Localized dumbbell neuroblastoma: a study of 25 cases treated between 1982 and 1987 using the same protocol.

Among the 108 non-metastatic neuroblastomas treated at the Institut Gustave Roussy between 1982 and 1987, 25 dumbbell neuroblastomas were observed. Therapeutics included: 1) an initial laminectomy in forms with neurological deficit; 2) surgical excision of the primary tumor; 3) preoperative chemotherapy in children in which the primary tumor was considered as unresectable at diagnosis and postoperative chemotherapy in cases of incomplete resection; and 4) radiation therapy on macroscopic residual disease. Fifteen out of 25 (60%) presented a neurological deficit. A laminectomy was performed in 14 cases. Neurological recovery was good in 4 cases, partial in 4 cases, and absent in 4 cases. Two patients were worse after the procedure. The event-free survival was 88%. This high survival rate is linked: 1) to the non-metastatic stage; 2) to a high proportion of children under 1 year of age (18/25) (median age = 7 months); and 3) to a high proportion of thoracic location (12/25). The high incidence of macroscopically incomplete excision (13/25) did not jeopardize the prognosis. Out of the 22 survivors, there were 8 cases of major neurological sequellae (36%) and 5 cases of major orthopedic ones (26%). The coexistence of a serious functional prognosis and an excellent vital prognosis for these patients has led us to analyse the therapeutic modalities, to reevaluate the necessity of routine initial neurosurgical removal by laminectomy and to discuss the use of first line chemotherapy.

Abdominal Neoplasms↗

PubMed1993

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.

Adolescent↗

PubMed1993

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.

Adolescent↗

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↗

PubMed1991

Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis.

In 14 children with Sturge-Weber syndrome, cortical calcifications on CT scan was present in 12, localized brain atrophy in 10, enlargement of the choroid plexus in 7, and abnormal veins in 7. Cortical enhancement was present on 12 CTs performed shortly after an episode of severe seizures or hemiplegia but was absent or considerably less marked at a distance from the acute episodes. We suggest that cortical enhancement is related to seizure activity and/or blood-brain disturbances rather than to the extension of pial angioma.

Adolescent↗

PubMed1991

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.

Adolescent↗

PubMed1991

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.

Brain Neoplasms↗

PubMed1991

[Overdrainage in the treatment of hydrocephalus].

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.

Cerebrospinal Fluid Shunts↗

PubMed1991

[Dumbbell neuroblastoma. Experience at the Gustave Roussy Institute in 38 cases treated from 1982 to 1987].

Among the 282 neuroblastomas treated at the Institut Gustave-Roussy between 1982 and 1987, 38 dumbbell forms were observed. Therapeutic approaches included: 1) An initial laminectomy in forms with neurological deficit; 2) Surgical excision of the primary tumor; 3) Preoperative chemotherapy for metastatic forms and non-metastatic forms in which primary tumors considered unresectable at diagnosis; 4) Radiation therapy on macroscopic residual disease. Twenty three of 38 children presented with a neurological deficit. A laminectomy was performed in 21 cases. Neurological recovery was good in 8 cases, partial in 5 cases and absent in 5 cases. Three patients were aggravated after the procedure. The event free survival was 76%. This high survival rate is linked with: 1) The predominantly non metastatic stages (25/38); 2) A high proportion of children under 1 year of age (25/38); 3) A high proportion of thoracic locations. Out of the 29 survivors, there were 10 cases of major neurological sequelae (34%) and 9 cases of major orthopedic sequelae (31%). The coexistence of a serious functional prognosis and an excellent vital prognosis led us to analyse the therapeutic modalities and reevaluate the necessity of routine initial neurosurgical excision by laminectomy, and using a first line chemotherapy in selected indications.

Adolescent↗

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

[Antishock trousers in the prevention of air embolism].

This study aimed to assess the efficiency and safety of military anti-shock trousers (MAST) in preventing venous air embolism in children undergoing neurosurgical procedures in the sitting position. It included 26 children, 7.7 +/- 4-year-old and weighing 25.4 +/- 10.5 kg. All had the same anaesthetic protocol including premedication with oral lorazepam, induction with thiopentone or propofol with fentanyl and vecuronium, and maintenance with isoflurane and a continuous infusion of fentanyl. In 10 patients, a retrograde central venous catheter was inserted for measurement of pressure in the superior bulb of the internal jugular vein. Haemodynamic measurements were carried out with the patient supine; with the patient sitting, the MAST not being inflated; 10 min after inflation of the MAST (40 mmHg in the limbs, 30 mmHg in the abdominal part); after starting 10 cmH2 O positive end expiratory pressure; and after fluid administration with 5 ml.kg-1 macromolecules (Plasmion). The criterion for air embolism was a decrease in PetCO2 of more than 5 mmHg over more than 30 s, not preceded by respiratory or cardiovascular impairment. Inflation of the MAST resulted in a dramatic rise in venous pressure, still reinforced by minor PEEP. Transmission of pressure from the right atrium to the dural sinus was linear, and seemed to be sufficient to prevent occurrence of detectable venous air emboli. No deleterious side-effects were noted. This method would therefore seem to be very efficient, preserving the advantages of the sitting position, and restricting its disadvantages. Unfortunately, the limited availability of different paediatric sizes of MAST reduces the possible uses of this method.

Adolescent↗

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↗