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Biomedical subjects

A Pierre-Kahn

Publications and source records attributed to A Pierre-Kahn.

At least 73 records · Page 4Linked to original sources

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↗

[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↗

[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↗

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↗

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↗

[X-ray computed tomography coupled with cisternography in chronic pericerebral effusions in young children].

The authors study 26 children with macrocranium and pericerebral effusion as diagnosed by C.T. Three groups are described: 10 with the classical C.T. features of subdural effusion, 12 with features of external hydrocephalus and 4 with massive pericerebral effusion. C.T. performed after lumbar subarachnoid injection of contrast medium, shows that all the effusions are subdural. In only one case, opacification of the subdural space occurred. The authors conclude that plain C.T. alone is unable to demonstrate the precise localization of pericerebral effusion in children and that all these effusions are, in their experience, subdural in localization.

Chronic Disease↗

[Treatment of chronic subdural collection in infants by subdural peritoneostomy].

Between 1980 and 1987, 50 chronics bilateral subdural collections in infants were treated by means of a subdural peritoneal shunt (unilateral in 48 cases, bilateral in 2 cases). In 2 patients with huge macrocranium the drains were left in situ. In the 48 others, the drains were removed after an average of 10 months. In these 48 cases, the drainages either bilateral (2 cases) or unilateral (46 cases) led to the disappearance of the collections; however, in 1 of these 48, a drain had to be reinserted due to the reproduction of the collection following the removal of the initial drainage. High level of C.S.F. protein content did not alter the peritoneal resorption. Obstruction of the catheter by a clot occurred in 3 cases with grossly bloody collections. Unilateral subdural peritoneal drainage for bilateral pericerebral collection is an efficient and safe treatment. The authors should advocate temporary external drainage only in the cases where the collection is grossly bloody.

Chronic Disease↗

[Stereotaxic technics with an open skull in the treatment of space-occupying brain lesions].

When intra-cerebral space-occupying lesions are small or when they are deeply located in 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 which combines conventional neurosurgical techniques with craniotomy using stereotaxic guidance. We have used the Brown-Roberts-Wells (B.R.W.) stereotaxic system which allows for CT scan or MRI guidance and does not interfere with the absolute sterility mandatory every 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 B.R.W. was inserted toward the surface of the lesion; the exposure was finally widened by the inflation of a rubber balloon set on the stereotaxic needle. The post-operative course was very uncomplicated in all these patients. No permanent post-operative neurological worsening was observed, even after the removal of an intra-thalamic tumor.

Brain Neoplasms↗

Lumbosacral lipomas with spina bifida.

The pathological and metabolic characteristics, as well as the anatomical and functional evolution of lumbosacral lipomas, were studied in a series of 73 patients operated on between 1970 and 1983. The pathological study showed that they are mainly composed of adipocytes, but that they also possess fibrous tissue, vessels, and nerve fibers. Innervated muscle fibers, contracting under proper nerve stimulation, were found in several cases. Lipogenesis and lipolysis are the same in lumbosacral lipomas and in normal fat tissue. However, these lipomas can grow with the rest of the fatty pool. Moreover, spontaneous progressive worsening of the clinical status has been observed in 36% of the cases. Surgery is efficient and not harmful to the patient. Postoperative mortality was nil. Early postoperative worsening occurred in 2.7% of the cases. The 6% rate of late postsurgical deteriorations should be compared to the 36% to 56% rate found when patients are not operated on. Three different mechanisms are responsible for clinical worsening: compression or stretching of the cord, and cord injury on the posterior upper limit of the spinal defect. In each case, one mechanism is prevalent. Three different types of lumbosacral lipomas can thus be individualized. The surgical implications of these data are discussed. The necessity for early and systematic surgical treatment is pointed out.

Adult↗

Prenatal hydrocephalus: outcome and prognosis.

The clinical records of 108 infants presenting with hydrocephalus at birth and operated on from 1971 to 1981 were reviewed in order to evaluate the functional results. Premature newborns and spina bifida patients were excluded. Communicated hydrocephalus (39 cases) and aqueductal stenosis (32 cases, excluding 6 X-linked hydrocephalus and 4 toxoplasmoses) were the two main types of hydrocephalus in this series. Eighty-four percent of the infants were operated on before the age of 3 months. The mean follow-up time was 7 years (range 1 to 14 years). The survival rate, calculated by the life table method, was 62% at 10 years. The functional results were evaluated according to intellectual performance, academic level, and psychological status. Of the 75 surviving children, 28% have an I.Q. over 80 and 50% an I.Q. under 60. The mean I.Q. is 54 (range 0 to 130). Of the 52 children who have now reached school age, only 29% have reached a normal academic level. The psychological status is normal or borderline in 46% of the patients. The importance of head enlargement at birth, ventricular size, and the age at the time of surgery are not related to late functional results. The results were best when there were no associated malformations, no shunt infection, when hydrocephalus was due to aqueductal stenosis (excluding X-linked hydrocephalus and toxoplasmosis), or when the first developmental quotient measured at 6 months was over 80.

Age Factors↗

[Treatment of craniopharyngiomas in children. Retrospective analysis of 50 cases].

In order to evaluate the results of surgery and radiotherapy upon craniopharyngiomas in children, the authors review their own series of 50 cases treated from 1968 to 1985 and the literature. After subtotal removal, the recurrence-free survival rate, 10 years postoperatively, was 37%. This rate was significantly higher (72%) when the subtotal removal was followed by irradiation with a dose of 50 to 55 grays: however, deafness and severe neuro-psychological and intellectual sequelae were frequent in these patients. After radical excision the rate of recurrence was the lowest, with a 10 year-recurrence-free survival rate of 88%. The postoperative mortality was low in case of pre-chiasmatic craniopharyngiomas, but high in case of retro-chiasmatic ones. Nevertheless, it appears from the recent literature data that impressive surgical improvements are to be expected from new surgical routes, associated with the use of the most recent technologies. The conclusions of this study are: 1. Radical excision is the treatment of choice; 2. If radical excision is not possible, surgery should be followed by irradiation to lower the risk of recurrence; 3. However, in view of the dangers of radiotherapy to the growing brain, it should be delayed as long as possible, particularly in the case of young children, and used only when tumor recurrence has been demonstrated.

Adolescent↗

[Craniopharyngioma in children. Endocrine evaluation and treatment. Apropos of 37 cases].

Endocrine function was studied in 37 children treated for craniopharyngioma by total (22 cases) or partial (12 cases) excision and complementary or isolated irradiation (9 cases). Height deficiency was the only revealing sign in only 20% of cases. Skull X-rays showed patent abnormalities at the first examination in 36 of 37 cases. Analysis of 24 children before and after surgery helps defining the part played by surgery in endocrine deficiencies: the frequency of thyroid and GH deficiencies is poorly changed after surgery as they are most often already present before surgery. On the other hand, the frequency of corticotropin and antidiuretic deficiencies is highly increased after surgery. Gonadotropic deficiency is almost constant after surgery. The final height is greater than 2 SD in 9 of the 14 patients whose growth is completed.

Adolescent↗

Are the metabolic characteristics of congenital intraspinal lipoma cells identical to, or different from normal adipocytes?

Congenital intraspinal lipomas are frequently responsible for progressive neurological deficits caused by distortion or compression of the nervous system. Since fat metabolism in these lesions has not been previously studied, the aim of this study was to determine whether intraspinal lipoma cells behave like lipomas or like normal adipocytes. In 11 patients, intraspinal lipoma cells were compared with normal adipocytes isolated from adjacent subcutaneous adipose tissue for the following parameters: lipoprotein lipase (LPL), lipogenesis from U14C glucose, beta-receptor number, adenylate cyclase activity, cyclic AMP production, and lipolysis in response to beta- and alpha 2-adrenergic agonists. No significant difference between these two cell populations was found, suggesting that intraspinal lipomas are not lipomatous tumors, but hamartomatous lesions capable of growth and regression along with the changes in the rest of the fatty pool. This emphasizes the danger of an abnormal weight gain, as well as the possible usefulness of an hypocaloric diet in patients who worsen in spite of previous surgery.

Adipose Tissue↗

Acute intracranial hematomas in term neonates.

Acute intracranial hematomas in 17 term neonates are reported: 3 were subdural in the posterior fossa; 14 supratentorial, either subdural, intracerebral (1 frontal, 4 occipital) or intraventricular. The hematomas were aspirated in 3 cases and surgically evacuated in 10. In the remaining 4, the effusion was left in situ. Three patients died: 2 due to dramatic preoperative deterioration and 1 to hemostatic difficulties during surgery. In the 14 survivors, neurological sequellae are major in 3, nil or minor in 11. Comparison of these cases to the 110 others already published allows the following conclusions: the cause of such hematomas is usually multifactorial, combining obstetrical trauma, anoxia and/or coagulation disorders; early diagnosis of the hematoma requires early echography and/or tomodensitometry; when the hematoma causes a midline shift, the authors advocate its early evacuation by aspiration, or craniotomy if it is clotted; the prognosis is poor when there is associated severe perinatal anoxia; if there is no anoxia, the prognosis seems good, but as the follow-up is usually short, definite conclusions are difficult; in our series hydrocephalus requiring treatment occurred in 2 of the 14 survivors.

Cerebral Hemorrhage↗

Intraspinal lipomas with spina bifida. Prognosis and treatment in 73 cases.

The authors present a series of 73 cases of intraspinal lipomas in the lumbosacral region. Sixty-four patients were operated on, 43 of these under intraoperative monitoring of neural function. The results of this series and of major series published in the past 10 years demonstrate both the potential severity of these lesions (which are responsible for progressive neurological deficits in 56% of affected cases) and the benignity of their surgical treatment. The authors emphasize the usefulness of systematic early surgical treatment of these lesions.

Adolescent↗

Anorectal motility after surgery for spina bifida.

Anorectal motility was studied in 93 children (aged 15 days to 16 1/2 years) who had undergone surgery for myelocele or meningomyelocele, and 80 controls, matched for age and sex. Mean resting pressure in the anal canal of patients was markedly decreased, particularly in the presence of incontinence, and unstable. The recording of a rectoanal inhibitory reflex both in the upper anal canal and at the anal margin was associated with the presence of fecal incontinence and motor disorders of the lower extremities. Patients could be divided into four groups according to manometric pattern: 1) those with normal recordings (incontinence in 63 per cent), 2) those with spontaneous intermittent relaxations of the upper anal canal and simultaneous rectal contractions (incontinence in 91 per cent), 3) those with normal manometry except for unstable upper anal canal pressure (incontinence in 92 per cent), and 4) those with absence of the rectoanal inhibitory reflex and hypotonic anal canal (patients too young to appraise continence, all under 2 years).

Anal Canal↗