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Biomedical subjects

A Pierre-Kahn

Publications and source records attributed to A Pierre-Kahn.

At least 91 records · Page 5Linked to original sources
PubMed1984

Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants?

From a previous study of achondroplasia as well as from the observation of patients with hydrocephalus associated with craniostenosis, the authors have concluded that an increased superior sagittal sinus venous pressure (SSVP) could be the cause of the enlarged ventricles. However, other workers have demonstrated that an increased SSVP could be the consequence of increased intracranial pressure (ICP). Therefore, the authors undertook a study to determine if there was a physiological test that could distinguish between rare instances of increased SSVP caused by structural and irreversible narrowing of the sinus and those caused by increased ICP. In 20 hydrocephalic infants and children, pressure was simultaneously measured in the lateral ventricle, the superior sagittal sinus, and the jugular vein. Stable baseline pressures were recorded, as well as the variations observed after the withdrawal of an amount of cerebrospinal fluid (CSF) sufficient to lower ICP to zero. Similar recordings were taken after reinjection of an equal quantity of CSF. In all of the patients, SSVP was increased, but not as much as the ICP. In the cases of hydrocephalus without any associated cranial malformation, and therefore without any likely anatomical interruption of the sinus, CSF withdrawal induced a simultaneous decrease of ICP and SSVP. However, whereas ICP could be lowered to zero, SSVP never fell below the jugular venous pressure, which remained stable (around 5 mm Hg) throughout the recording session. Results were different when sinography demonstrated an anatomical interruption of the sinus, as in cases of hydrocephalus associated with achondroplasia or craniostenosis. In these cases, although ICP was normally lowered by CSF withdrawal, SSVP remained nearly unchanged, usually greater than the jugular venous pressure. The present study demonstrated that SSVP recording during ICP variations induced by CSF withdrawal permits differentiation between a reversible collapse of the sigmoid sinus due to increased ICP and a fixed obstructive lesion of the sinuses. Based upon this test and the results of sinography, the authors inserted a venous bypass between the lateral sinus and a jugular vein in three patients.

Achondroplasia↗
PubMed1984

The Dandy-Walker malformation. A review of 40 cases.

Forty cases of Dandy-Walker malformation referred to the Hôpital Necker Enfants-Malades between 1969 and 1982 have been reviewed. The incidence of the malformation in hydrocephalus was 2.4%. There was a slight, statistically insignificant, female prevalence. Hydrocephalus should not be included in the definition of the syndrome. In 80% of the cases, it was actually a post-natal complication of the malformation and most often developed within 3 months after birth. In 80% of the cases, a communication, although insufficient, was found between the dilated 4th ventricle and the subarachnoid space. Since this communication is probably established through the foramina of Luschka, the definition of the Dandy-Walker malformation should only include atresia of the foramen of Magendie. Associated brain and systemic malformations were numerous. Among facial anomalies, facial angiomas were found in 10% of our cases. The association of facial and cardiovascular anomalies favors the hypothesis that the onset of the malformation occurs between the formation and the migration of the cells of the neural crest (that is, between the 3rd and the 4th post-ovulatory week, earlier than previously thought). Except in selected patients, membrane excision has a high rate of failure and should be abandoned. Cyst-peritoneal shunting avoids the risk of an entrapped fourth ventricle and is presently the best surgical procedure. The overall mortality in this series was 12.5%. Intelligence quotients were over 80 in 60% of the patients. Other studies will be necessary to understand why the communication between the fourth ventricle and the subarachnoid spaces, sufficient in utero, usually becomes insufficient for a normal cerebrospinal fluid (CSF) circulation in the first months following birth. Two hypotheses are discussed: a change in CSF circulation, or bleeding in the dilated fourth ventricle during delivery.

Brain↗
PubMed1984

Factors causing acute shunt infection. Computer analysis of 1174 operations.

A series of 1174 operations performed on 802 hydrocephalic children was analyzed in an effort to find the factors causing acute postoperative infection. Culture of the cerebrospinal fluid (CSF) samples during the operation was positive in 33 cases. These cases were excluded from the series. Ninety infections were observed in the remaining 1141 operations, an overall rate of 7.9%. Most of these infections were meningitis (56 cases). Staphylococcus epidermidis was the bacterium most frequent identified (44%). Statistically significant relationships were found between shunt infection and the following factors: 1) age: infection was 2.6 times as frequent before 6 months than after 1 year of age (p = 0.03); 2) poor condition of the skin; 3) presence of intercurrent seats of infection at the time of surgery; 4) type of operation: the rate of infection was 8.4% in primary shunt insertions, 5% in shunt revisions, and 17.5% in reinsertions following shunt removal for infection (p = 0.0001); 5) end of the shunt requiring revision: an infection rate of 7.7% followed revision of the ventricular catheter alone, and 2.6% followed revision of the other end alone (p = 0.012); and 6) postoperative wound dehiscence or scalp necrosis. The surgical team involved was poorly correlated with the rate of infection (p = 0.12). No statistically significant relationships have been found between infection and 1) etiology of hydrocephalus: 2) sex; 3) recent neurosurgical operation before the shunt procedure; 4) preoperative presence of an external drainage tube or CSF fistula; 5) lumbar or ventricular taps, or ventriculography; 6) number of previous operations performed on the shunt; 7) time (month and year) of operation; or 8) sugar level and cell count in the CSF.

Cerebrospinal Fluid Shunts↗
PubMed1984

[Benign osteoblastoma in children. Apropos of 8 cases, 4 with spinal localization].

In a 20 year period the authors have seen 8 cases of osteoblastoma in patients less than 15 years old. Four were in the spine, three in the hand and one in the tibia. Three of the four limb osteoblastomas were lost to follow up after curettage, one of them after a recurrence. The fourth one was cured after 9 years. None of the spine lesions recurred after a follow up of from two to 14 years but two of them had a rigid kyphosis. It is concluded that osteoblastoma is normally benign in children. The pathological diagnosis may be difficult. In one the initial diagnosis had been of osteosarcoma. The treatment is based on complete excision. In the spine, it should be accompanied by arthrodesis.

Adolescent↗
PubMed1984

[Abdominal complications of ventriculo-peritoneal shunts in children. 65 cases].

From 1971 to 1982, among 1 200 children with hydrocephalus cured by ventriculo-peritoneal shunt, 65 have presented an abdominal complication. Some of them are rare and easily explainable: 2 intra-operative viscus perforations, 1 intestinal obstruction, 3 extrusions of the catheter into an inguinal hernia. Also rare, C.S.F. ascites (5 cases) et C.S.F. pseudocysts (3 cases) presented with typical symptomatology easily diagnosed, but their etiology is not clear. The main complications are peritonitis without perforation (32 cases) and migrations of the catheter in the colon (19 cases): their etiology is an intra-operative bacterial contamination, they happen during the following weeks or months after the shunt procedure. They are associated with 8 among 9 deaths of our data by gram negative germ meningitis. Removal of the peritoneal catheter and temporary external shunt are their main treatment.

Ascites↗
PubMed1983

Intracranial ependymomas in childhood. Survival and functional results of 47 cases.

A pediatric series of 47 intracranial ependymomas (15 supra- and 32 infratentorial) is presented. All children were operated upon between 1969 and 1979. Among these children, 35 completed the treatment with radiotherapy. The irradiation was usually limited to the intracranial content but in 7 patients, it was extended to the spinal axis as well. Malignant ependymomas represented 69% of the whole series (86% of the supratentorial and 53% of the infratentorial ependymomas). The operative mortality rate was 17%. The 5-year survival rate was 39% in the whole series and 51% when excluding postoperative mortality. Recurrences developed in 41% of cases and metastases in 20%. The study of this series and of the literature points out that: (1) metastases from supratentorial ependymomas are not frequent, almost always supratentorial and secondary to a malignant ependymoma, and (2) metastases from infratentorial ependymomas are almost always intraspinal and occur in one third of these tumors. The rate of seeding is especially high in the case of malignant infratentorial ependymomas (50%) but is also 15% in the case of benign tumors. These data lead the authors to propose a craniospinal irradiation in the case of infratentorial ependymomas whether benign or malignant, an irradiation of only the brain in the case of malignant supratentorial ependymoma, an irradiation of only the tumoral bed when ependymomas are benign and supratentorial.

Brain Neoplasms↗
PubMed1983

[Lumbosacral lipomas with spina bifida. Anatomo-clinical correlations. Therapeutic results].

From 1971 to 1982, the authors saw 41 patients who presented lumbo sacral lipomas with spina bifida; 9 remained outpatients and had regular clinical follow up; the other 32 were operated on; 1 was lost sight of, shortly after the operation. 40 patients were then regularly followed; of these, 15 (37%) developed neurological deficits (14 before and 1 long after surgery). Intraspinal lipomas were classified into 2 groups: group I (18 cases), when the lipomas displaced but did not include the lumbo sacral roots; group II (14 cases) when it included most or all of these roots. Despite an inevitably less satisfactory liberation of the spinal cord in the group II, post operative results were not significantly different in the 2 groups. Comparing the post operative results of this series to others in the literature, the authors come to the following 3 conclusions: 1) these operations are benign; 2) they are all the more effective when the neurological deficits are either recent or evolutive, and 3) they protect most of the patients from eventual neurological deterioration. Therefore the authors agree that patients with lumbo sacral lipomas should be operated on at the onset of neurological deficits but also recommend early intervention, even in the absence of any neurological disturbance.

Adolescent↗
PubMed1983

[Intracranial ependymoma in children. Prognosis and therapeutic perspectives].

The authors report 47 cases of intracranial ependymomas occurring in children less than 15 years of age, who were operated on from 1969 to 1979. The aim of the study was to study the incidence of intraspinal metastases and to suggest a logical protocol for postoperative radiotherapy. The operative mortality rate was 17%. The 5 year survival rate, after exclusion of postoperative mortality, was 51%. Recurrences developed in 41% of cases and metastases in 20%. Among these, intraspinal metastases occurred in 14.5% and were found only in infratentorial ependymomas, whether benign or malignant. The results and those in the literature suggest that a protocol of irradiation adapted to the site and the histological grading of these tumors should be discussed with the teams of radiotherapy; in infratentorial ependymomas, the irradiation should include the whole craniospinal axis.

Adolescent↗
PubMed1983

[Hemolymphangioma of the orbit in children].

Orbital hemolymphangiomas are extremely rare. Clinical and computed tomographic brain scan aspects mimic "inflammatory orbital pseudotumors". They frequently fluctuate in size with upper respiratory tract infections and may bleed, causing acute exophthalmos. Therapeutic approach remains difficult as complete surgical excision is always impossible and postoperative complications frequent with acute inflammatory relapses and/or hemorrhages. We describe 2 children with orbital lymphangiomas presenting as orbit tumors.

Child↗
PubMed1983

Brain abscess in childhood. A study of 34 cases treated by puncture and antibiotics.

During the last 15 years, 34 children were treated for a brain abscess. 13 (38%) had cyanotic heart disease; 12 (35%) had an ENT infection. The standardized treatment protocol included puncture of the abscess and administration of antibiotic and antiedematous drugs. Postoperative permanent epidural monitoring of intracranial pressure and, in the last 5 years, repeated CT scans indicated if retapping the abscess or reinforcing the antiedematous treatment was necessary. In 7 out of these 34 cases the abscess had to be excised. Postoperative mortality rate was 6%. Overall mortality rate was 12%. Neurological sequelae were slight. Epilepsy frequency was 10%. Mental retardation (18%) was found only in cyanotic congenital heart disease to which it is related. Repeated CT scans demonstrated the progressive disappearance of the capsule within a few months. This study shows an improvement in the results when brain abscesses in children are punctured rather than excised.

Adolescent↗
PubMed1982

Endocrine aspects and tumoral markers in intracranial germinoma: an attempt to delineate the diagnostic procedure in 14 patients.

This work outlines the endocrine abnormalities associated with intracranial germinomas (14 patients before treatment). Diabetes insipidus of various intensity was present in all cases. Adipsia is often present as well. In six patients, the first neuroradiologic examination after the onset of DI was normal. Growth hormone secretion was deficient in 11 of 13 patients. Abnormalities of TSH response to TRH were present in nine of ten (four insufficient and five exaggerated responses to TRH). Anterior pituitary dysfunction could not be predicted by the tumor site as determined by radiologic criteria. Tumoral markers have also been studied. Elevated plasma tumoral markers were found in four patients of 11 studied. Tumoral germinoma cells were present in CSF in five patients of ten, in one of them before radiologic confirmation. Pertinent endocrine evaluation and search for tumoral markers was of great value in systematic follow-up of patients with central diabetes insipidus, and could lead to early diagnosis and treatment of the tumor.

Adolescent↗
PubMed1982

[Intra-cranial abscesses and congenital heart disease in children (author's transl)].

The authors report on 13 cerebral abscesses developed in children with cyanogenic heart disease. The abscesses have been treated by one or several punctures and by a general antibiotherapy adjusted to the germ when it was isolated. C.T. Scan and epidural Intra-cranial pressure monitoring have defined the Therapeutic tactics. The mortality rate is 7.7%. 60% of the survivors lead a normal life; 40% are mentally retarded; however this mental retardation is not directly related to the abscess; it appears to be a consequence of the cyanogenic cardiopathy. The neurological sequelae are one epilepsy and two visual defects compatible with normal life. Successive C.T. Scans in the months following treatment have shown the progressive disappearance of the abscess membranes. The conclusions of this study are under antibiotherapy, it is better to tap abscesses than to remove them and that their prophylaxy is the suppression of the shunt responsible of the cyanosis.

Adolescent↗
PubMed1982

[Cerebral abscesses in children treated by puncture. A 16 year-experience].

In the last 16 years, 33 children have been treated for brain abscesses. Fifteen (45.5%) presented with cyanogenic congenital heart disease; eleven (33.5%) with E.N.T. infection. The standardized treatment protocol included puncture of the abscess, antibiotherapy and anti-edematous drugs. Postoperative permanent epidural ICP monitoring and, in the last 6 years, repeated CT scans indicated when it was necessary to tap the abscess again or to reinforce the anti-edematous treatment. However, in 3 of these 33 cases, the abscess had to be removed. Postoperative mortality rate was 3%. Overall mortality rate was 6%. Neurological sequelae were slight. Epilepsy occurred in 10%. Mental retardation (16%) was only found in children with cyanogenic congenital heart diseases. Repeated CT scans showed the progressive disappearance, within a few months, of the abscess membranes. This study shows an improvement in the results when brain abscesses in children are punctured rather than excised.

Brain Abscess↗
PubMed1982

[Congenital intraspinal neuroblastomas. A propos of 7 cases treated surgically].

Neuroblastomas appear to be one of the most frequent congenital solid tumors. Nevertheless, intraspinal congenital neuroblastomas are uncommon. The authors report seven cases of congenital intraspinal neuroblastomas operated on in the "Service de Neurochirurgie des Enfants-Malades" from 1970 to 1980. Their study and that of 16 cases published in the literature allows to stress several points (7, 12). The dumb-bell intraspinal neuroblastomas present a large extension. They usually determine a severe and definitive paraplegia. They probably have a long prenatal evolution. The tumoral prognosis of these congenital neuroblastomas is good. Success should be expected in nearly 80% of the cases. Surgery, in two steps, intra and extra spinal, remains compulsory even though the neurological prognosis is poor. It is useless to operate upon the neuroblastomas as emergencies, when neurological involvement is total at birth. The complementary treatment, X ray-therapy or chemotherapy is not without any danger on newborns. It necessary is not proved.

Female↗
PubMed1980

Vasomotor origin of intracranial pressure waves in hydrocephalic infants.

By measuring cerebral blood volume (CBV) and intracranial pressure (ICP) variations at the same running time during sleep, it has been demonstrated that the ICP wave which appears during the REM sleep in hydrocephalic infants is produced by intracerebral vaso-dilatation. Nine infants with stabilized hydrocephalus were investigated by non-invasive means: REM phases were distinguished with the usual polysomnographic electrodes. Intracranial pressure was measured with a fontanel palpation transducer and CBV variations were obtained by recording 99mTc activity at the head level after in vivo labelling of red cells with 99mTc--pertechnetate. The time-activity curves, obtained from regions of interest and selected on the sequential radioisotope images, show that an increased ICP wave, occurring during the REM period, is related to a simultaneous increase in the blood volume, limited to the cerebral sector and not to the area of the external carotid artery.

Blood Volume↗
PubMed1980

Hydrocephalus and achondroplasia. A study of 25 observations.

Two series of achondroplastic patients were studied. The first series included 5 patients referred to our neurosurgical department for macrocranium and/or mental retardation. The second series was composed of 20 patients arbitrarily chosen from a medical group. Macrocranium was observed in 60% of the patients and was related to hydrocephalus in all but 2 cases. Radiological studies demonstrated that the posterior fossa was deformed and narrowed in its 3 planes, while its volume was conserved because of an abnormal ascension of the tentorium. The conflict between normal brain development and the craniostenosis at the base of the skull is responsible for an upward tilt of the petrous pyramids, a lowering of the mastoid process, and the illusion of a basilar invagination. Ventriculographies, pneumoencephalographies and isotopic transits demonstrated normal CSF circulation. The study suggests that hydrocephalus is related to the stenosis of the sigmoid sinus at the level of narrowed jugular foramina with a resulting raise in intracranial venous pressure. The majority of patients with macrocranium stabilizes spontaneously. Thus, a surgical indication should be extremely limited.

Achondroplasia↗
PubMed1980

[Neurological complications of hemophilia in children (author's transl)].

Although the treatment of hemophilia has greatly improved, the disease is still sometimes responsible for neurological or neurosurgical disorders. Such disorders were observed in the department of Neurosurgery at the "Hopital des ENFANTS MALADES' in twelve hemophiliacs, five of them having antifactor antibodies. Four main points results from this study: 1) A traumatic etiology was found only in one third of the cases. 2) CT Scan is the main investigation since it allows to find out what cases should be operated upon. The ratio of neurosurgical cases varies from one statistic to another from 40% to 70%. 3) The frequency of neurological disorders is correlated with the importance of the deficit in antihemophilic factors, which is usually less than 1%. 4) The death rate in this series was nil. Hemophiliacs without antifactor antibodies should receive a substitutive treatment as soon as possible. However, in hemophiliacs with antifactor antibodies, this treatment should only be given when surgery is required.

Brain Diseases↗