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Biomedical subjects

J F Hirsch

Publications and source records attributed to J F Hirsch.

At least 55 records · Page 3Linked to original sources
PubMed1988

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↗
PubMed1988

Brain abscesses in neonates. A study of 30 cases.

Since the introduction of ultrasonography and computerized tomography (CT) scanning, brain abscesses are found more frequently in cases of neonatal meningitis and septicemia, particularly when the offending pathogen is Proteus. Thirty cases of brain abscess in neonates are reported, 27 of which were caused by Proteus species infections. Twenty infants had meningitis and 13 had septicemia. Most of the abscesses were enormous, and multiple abscesses were observed in 17 cases. The frontal region was involved in 22 cases (12 unilaterally and 10 bilaterally). The ventricles were enlarged on the first CT scan in 13 cases. The abscesses were treated by aspiration and antibiotics in 25 cases, and by antibiotics alone in five. A shunt for hydrocephalus was necessary in 14 infants. Four infants died, three from the initial illness and one from a shunt complication. Sixteen children have seizures. Subsequent intelligence quotient (IQ) testing was performed in 22 children: eight (36%) have an IQ at or above 80 and eight have an IQ of less than 60. In the 17 children followed for more than 2 years, the proportion with an IQ at or above 80 fell to 24% (four cases). The absence of initial seizures, sterile cerebrospinal fluid, normal ventricles on CT scans, and early aspiration of the abscess seem to be factors portending a better prognosis in terms of epilepsy and mental sequelae.

Anti-Bacterial Agents↗
PubMed1988

[Failure of chemonucleolysis and its surgical treatment. Apropos of 268 cases].

The authors have reviewed 268 cases of herniated lumbar discs treated by chemonucleolysis between 1980 and 1984. 52 patients had a laminectomy (19,40 p. 100) and 18 of theses failures were due to a large disc herniation (34,61 p. 100). The result of the surgical treatment after chemonucleolysis failure was good in 67,30 p. 100 of cases. A chymopapain injection is not indicated in patients with a large disc herniation.

Chymopapain↗
PubMed1988

[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↗
PubMed1987

Pericerebral collections after shunting.

Nineteen pericerebral collections (18 subdural and 1 epidural) occurred in a series of 682 consecutive initial shunt insertions for hydrocephalus in children, performed between 1976 and 1984. No collections were observed in 358 reoperations performed in the same patients during the same period. The incidence rate of this complication is nearly four times higher after 2 years of age than in younger children (6.5% versus 1.7%). The rate is also higher, regardless of age, in noncommunicating than in communicating hydrocephalus. Pericerebral collections are observed with high- as well as low-closing pressure valves. Pericerebral collections after shunting were diagnosed in 80% of the cases less than 2 months after surgery. They were asymptomatic in nearly 60% of the cases but could become symptomatic later when they were not treated. At the onset at least, these pericerebral collections are compensated by the outflow of an equal quantity of intraventricular CSF so that ICP is only moderately elevated. Postshunt pericerebral collections may be the consequence of CSF loss at the time of surgery. In most cases, however, they are due to a CSF overdrainage by the valve. This overdrainage, together with the reversal of CSF flow in the highly resistant CSF pathways of hydrocephalus, establishes a pressure lower in the ventricles than in the subarachnoid space and tends to open up the subdural space. The treatment of postshunt subdural collections is the insertion of a simple tubing without valve between the subdural space and the peritoneum, the ventriculoperitoneal shunt being left in place. With this treatment, more than 80% of the collections disappeared or were improved.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1987

A new approach in the treatment of hydrocephalus.

To date, most patients suffering from hydrocephalus have been treated by insertion of differential-pressure valves that have fairly constant resistance. Since intracranial pressure (ICP) is a variable parameter (depending on such factors as patient's position and rapid eye movement sleep) and since cerebrospinal fluid (CSF) secretion is almost constant, it may be assumed that some shunt complications are related to too much or too little CSF drainage. The authors suggest a new approach to treating hydrocephalus, the aim of which is to provide CSF drainage at or below the CSF secretion rate within a physiological ICP range. This concept has led the authors to develop a three-stage valve system. The first stage consists of a medium-pressure low-resistance valve that operates as a conventional differential-pressure valve until the flow through the shunt reaches a mean value of 20 ml/hr. A second stage consists of a variable-resistance flow regulator that maintains flow between 20 and 30 ml/hr at differential pressures of 80 to 350 mm H2O. The third stage is a safety device that operates at differential pressures above 350 mm H2O (inducing a rapid increase in CSF flow rate) and therefore prevents hyper-elevated ICP. An in vitro study is described that demonstrates the capability of this system to maintain flow rates close to CSF production under a range of pressures similar to those observed under various human physiological and postural conditions. Promising clinical results in 19 patients shunted with this valve are summarized.

Adolescent↗
PubMed1987

[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↗
PubMed1987

[Syringomyelia and scoliosis in children and adolescents. Apropos of 14 cases].

The authors report their experience of 14 cases of scoliosis in children or young adults in association with syringomyelia. The neurological lesion was discovered at a variable time during the development of the scoliosis which may appear to be idiopathic for several years. There were 11 cases of syringomyelia or hydromyelia and three intramedullary tumours with cyst formation. The neurological lesion must be treated first and sufficiently early to avoid the development of muscle weakness and scoliosis. The scoliosis can be treated by normal without risk of additional complications.

Adolescent↗
PubMed1986

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↗
PubMed1986

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↗
PubMed1986

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↗
PubMed1986

[Peroperative echography in pediatric neurosurgery. Experience in a series of 40 procedures].

Forty neurosurgical operations (supra-tentorial: 27; infra-tentorial: 10; intra-spinal: 3) have been performed under real time ultrasonography control. The supra-tentorial anatomical Landmarks visualized on the screen were the base of the skull, the cranial vault, the falx cerebri and the ventricles. The infra-tentorial landmarks were the petrous bone, the tentorium and the brainstem. The dura-mater, the subarachnoid space and the medulla were easily detected in the spinal canal after laminectomy. Intra operative real time ultra sonography performed on the dura-mater or on the cortex allows the detection of pathological structures. Tumors, whatever their location, are hyperechogenic; cysts, on the contrary, are hypoechogenic. Tumors are therefore precisely delimited by intra operative ultrasonography; thus the amount of normal nervous tissue which must be removed to reach a subcortical or intra medullary tumor can be reduced to a minimum. Moreover, the use of intra operative ultra sonography gives control ever the completeness of tumor removal as it shows any residual tumor intra operatively. Echography also facilitates other operations such as placement of a ventricular catheter, tapping of an abscess or biopsy of a tumor. Large arterial vessels or arterio-venous malformations are easily located because their pulsations are visible on the screen. For all these reasons, real time ultrasonography should always be available in the operating theater. In this series, it has provided essential information in 15 cases out of 40.

Brain Abscess↗
PubMed1985

[Statural growth following irradiation of the central nervous system for medulloblastoma of the posterior fossa. Retrospective analysis of 45 cases].

Treatment of medulloblastoma in children with head and spinal irradiation causes growth hormone deficiency and growth retardation. The present study deals with 45 patients presenting a follow-up time superior to 4 years; some of them having reached their final height. The mean final height is 3 standard deviations below normal mean. Growth retardation which occurred in 42 of 45 children, appears to be due to two major factors: 1) GH deficiency in 42 cases as assessed by the arginine insulin tolerance test. 2) Spinal lesions due to irradiation, causing early growth retardation and a reduced trunk length in most of these children. The response to hGH treatment (10 mg/kg/yr) was not sufficient in this group of patients.

Body Height↗
PubMed1984

Germinoma in a boy with precocious puberty: evidence of hCG secretion by the tumoral cells.

The present report concerns a 10-year-old boy in whom diabetes insipidus and short stature were the first manifestations of a suprasellar germinoma. Neuroradiological investigations performed when these symptoms appeared were negative. 2 years later, a rapid and early pubertal development was observed and related to secretion by the germinoma of human chorionic gonadotropin (hCG) identified immunohistochemically. This clinical evolution is unusual and indicates that plasma hCG measurements in patients with so-called idiopathic diabetes insipidus can be of clinical value in predicting the presence of an hCG-secreting tumor.

Brain Neoplasms↗
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↗