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Biomedical subjects

F Epstein

Publications and source records attributed to F Epstein.

At least 19 recordsLinked to original sources

Surgical management of symptomatic pineal cysts.

The authors present a series of six patients with large symptomatic benign pineal cysts and review the 27 patients previously reported in the literature. Patients with symptomatic pineal cysts most often present with one of three syndromes: 1) paroxysmal headache with gaze paresis; 2) chronic headache, gaze paresis, papilledema, and hydrocephalus; or 3) pineal apoplexy with acute hydrocephalus. Surgical intervention with radical cyst removal is the treatment of choice for all symptomatic pineal cysts. Complete cyst removal is desirable; however, radical subtotal resection is appropriate if the cyst cannot be easily separated from the quadrigeminal plate. Ventricular shunting should be reserved for patients with persistent hydrocephalus after cyst resection.

Adolescent

Pregnancy in patients with cerebrospinal fluid shunts: report of a series and review of the literature.

Hydrocephalic women with cerebrospinal fluid shunts are now surviving to reproductive age. Twenty-one pregnancies in 18 patients with shunts, including 11 from the present series and 10 from previous series, were analyzed for neurological, obstetrical, and perinatal outcome. Fourteen women had preexisting shunts, and 4 had the onset of symptomatic hydrocephalus and the placement of shunts during pregnancy. Neurological complications occurred in 13 of 17 (76%) pregnancies in patients with preexisting shunts, including symptoms of increased intracranial pressure (ICP) in 10 of 17 (59%) pregnancies, exacerbation of seizure disorder in 2 of 17 (12%) pregnancies, and severe headaches without increased ICD in 1 patient. In 7 of 11 (66%) of the symptomatic patients, symptoms spontaneously resolved postpartum. Four of 17 (23%) of these pregnancies were associated with shunt obstruction requiring antepartum or postpartum surgery. Four patients had a primary shunt placement, and one had a shunt revision during pregnancy without complications. There were no unusual obstetrical or perinatal complications in the series. The clinical management of pregnant patients with hydrocephalus should include preconception counseling and magnetic resonance imaging, as well as the use of serial antenatal magnetic resonance images, ICP monitoring, or the judicious use of radioisotope studies of shunt patency if signs of increased ICP appear. A cesarean section is recommended for the delivery of the neurologically unstable patient. For asymptomatic mothers, a vaginal delivery with a shortened second stage and prophylactic antibiotics are advised.

Adolescent

Glioneurofibroma: renaming the pediatric "gliofibroma": a neoplasm composed of Schwann cells and astrocytes.

Three children had central nervous system tumors with histologic and ultrastructural features corresponding to those of tumors previously described as "gliofibromas." These features, which include a composite appearance with glial and mesenchymal elements, with glial fibrillary acidic protein (GFAP)-containing and GFAP-immunonegative cells, diffuse S-100 immunoreactivity, and basal lamina wrapping processes of both cell types, suggest that the "mesenchymal" cells are Schwann cells, not fibroblasts. We therefore propose to rename this entity "glioneurofibroma." The clinical behavior of these lesions is uncertain but is more often indolent or benign.

Astrocytes

The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients.

171 patients with intramedullary spinal cord tumors were operated on, of which 25 patients (15%), mostly children, developed symptomatic hydrocephalus. Twenty patients (12%) had malignant tumors, with 13 of the 20 cases (63%) complicated by increased intracranial pressure and ventriculomegaly. Of the remaining 151 patients with benign tumors (89%), only 12 (8%) developed symptomatic hydrocephalus. In an effort to understand the relationship between hydrocephalus and intramedullary spinal cord tumor, the authors analyze the level and histology of the neoplasm, as well as its association with spinal cysts. A review of the neurosurgical literature reveals that 34 similar cases of hydrocephalus associated with intramedullary spinal cord tumors have been reported to date. The authors note that the presence of hydrocephalus in patients with malignant intramedullary astrocytomas is associated with a shorter rate of survival than in those patients with high-grade lesions but without hydrocephalus, apparently due to rapid tumor progression. The ventriculomegaly seen with benign spinal cord gliomas has no statistically significant effect upon long-term prognosis.

Adolescent

Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood.

Sixteen children underwent 18 operations for radical resection of chiasmatic-hypothalamic tumors. The clinical presentation correlated with age: infants under 1 year of age presented with macrocephaly, failure to thrive, and severe visual failure; children aged 1 to 5 years predominantly had precocious puberty with mild visual deficits; and older children (greater than 5 years old) had slowly progressive loss of vision. All three infants had biologically aggressive tumors in spite of low-grade histology, and died from progressive tumor growth. Eleven of the 13 children aged 1 year or over are alive and well, without clinical or radiographic evidence of disease progression, 4 months to 4 1/2 years following surgery. Six of these patients, with a follow-up period of 10 months to 4 1/2 years (mean 27 months), have had no adjuvant therapy following radical surgical resection. The authors conclude that: 1) radical surgical resection of chiasmatic-hypothalamic tumors can be performed with minimal morbidity; 2) radical resection may delay the time to disease progression in older children and postpone the need for irradiation; 3) resection of postirradiation recurrent tumors may provide neurological improvement and long-lasting clinical remission; and 4) chiasmatic-hypothalamic tumors of infancy are aggressive neoplasms that require multimodality therapy.

Adolescent

Surgical management of brain stem tumors of childhood and adolescence.

Intrinsic brain stem tumors are a heterogeneous group of neoplasms that may be classified according to clinical and neurodiagnostic criteria. We have described simple anatomic categories that include focal, diffuse, cystic, and cervicomedullary tumors. Although there is no surgical option for the common diffuse brain stem neoplasms, the other tumors may be operated on if the clinical and neurodiagnostic assessment suggests the possibility of a benign neoplasm. It is important to emphasize that although all tumors that were covered by intact ependyma or pia were considered intrinsic tumors in this series, most of these tumors created a "bulge" that was obvious on visual inspection of the external surface of the brain stem. In all of the tumors that were benefitted by surgery, the neoplasms tended to "bulge" from the brain stem rather than infiltrate it, which was the case with the diffuse neoplasms. It seems likely that many of the focal, cystic, and cervicomedullary tumors are similar to the dorsal exophytic brain stem tumors, which are characteristically low-grade astrocytomas that disrupt the ependymal lining and grow posteriorly into the fourth ventricle. Although surgery has been well tolerated and beneficial in many patients, it would be premature to comment on the duration of remission or the possibility of permanent cure.

Adolescent

The vulnerability of the thin descending limbs of Henle's loop in the isolated perfused rat kidney.

In the isolated rat kidney perfused without erythrocytes, the medullary thick ascending limb shows extensive injury. Damage to the thin limbs of Henle's loop has been mentioned only briefly. Thin limbs were examined in the isolated perfused kidney under a variety of conditions that alter oxygenation and active transport in the medulla and are known to affect injury to the medullary thick ascending limb. The thin descending limbs of short loops were preserved in all experimental groups, but those of the long loop showed necrosis that was restricted to the proximal portion, where the epithelium is more complex. In oxygenated kidneys, necrosis involved 41% +/- 5% (mean +/- SE) of the medullary thick ascending limbs and 10% +/- 3% of the proximal portion of long loops of thin descending limbs. Under hypoxic conditions, necrosis involved 90% +/- 3% of the medullary thick ascending limbs and 70% +/- 5% of the proximal portion of long loops of thin descending limbs (P less than 0.0001 compared with oxygenated kidneys). Ouabain and absence of filtration completely prevented necrosis of both nephron segments. Thus, the proximal portions of long loops of thin descending limbs, in resemblance to medullary thick ascending limbs, are especially susceptible to transport-dependent hypoxic injury.

Animals

Real-time intraoperative monitoring during neurosurgical and neuroradiological procedures.

A real-time intraoperative evoked potential (EP) monitoring system is described and evaluated. Unique features include (1) online artifact rejection to reduce noise contamination, (2) optimum digital filtering to improve the signal-to-noise ratio of the EP signal, (3) statistically defined confidence intervals to determine significant EP peak latency deviations, and (4) sliding windows of EP subaverages of various sizes to minimize feedback time to the surgeon. The reliability and validity of this system were determined by comparison with conventional intraoperative EP averaging and by examining the correlation of EP parameter changes with concurrent surgical and radiological manipulations. This system was clearly superior to conventional averaging systems. Reliable EPs could be obtained from neurologically compromised patients within the electrically hostile operating room environment, in cases in which conventional averaging failed to extract a stable EP signal. EP update times of 10-20 s were quite common and allowed direct moment-to-moment correlations with surgical and radiological events. Case histories are presented that show the utility of this system for aiding in the prevention of neurological complications. This utility is examined for neurosurgical and neuroradiological procedures involving spinal cord, brainstem, midbrain, and cortical structures, and affecting the somatosensory, motor, auditory, and visual system pathways.

Adult

Management of hydromyelia.

The authors review their experience in the management of 22 patients with hydromyelia over a 26-month period. Ten children had Chiari I malformations and hydromyelia; 4 children had myelomeningoceles (3 with large thoracic spinal cord cavitations and 1 with cervical hydromyelia); 6 children had distal hydromyelia associated with tethered cords and occult dysraphism; and 2 patients had cavitation subsequent to arachnoiditis. All patients were investigated preoperatively with MRI and intraoperatively with ultrasound. These neurodiagnostic examinations dictated the type of surgical intervention. Patients with Chiari I or Chiari II malformations, cervical hydromyelia, or basal arachnoiditis underwent decompression of the hindbrain malformations, myelotomy with drainage of the cyst, and placement of a stent. When the area of hydromyelia extended to the obex, as demonstrated by intraoperative ultrasound, the obex was plugged. Cyst-pleural shunts were placed in the children who had myelomeningoceles and thoracic hydromyelia. Patients with distal hydromyelia underwent modified terminal ventriculostomy. The classical presentation of brachial amyotrophy and dissociated sensory loss was present in only 3 patients. Progressive scoliosis without neurological deficit, pain, and Lhermitte's phenomenon were common presentations. The patients with tethered cords were generally asymptomatic from their cysts. The authors discuss operative technique, utilization of intraoperative ultrasound, and surgical outcome.

Adolescent

Malignant astrocytomas of the spinal cord.

The authors review their experience with the operative management of 19 consecutive cases of malignant astrocytoma of the spinal cord. There was a male to female ratio of 1.1:1, and the median age of the population was 14 years (range 1 to 32 years). The median duration of symptoms prior to definitive diagnosis was 7 weeks. Radical excision was carried out in all cases, with 18 patients (95%) receiving radiotherapy and 10 patients (53%) receiving chemotherapy as well. To date, 15 (79%) of the 19 patients in this series have died, with a median survival period of 6 months following surgery. No patient improved after operation. Hydrocephalus was present in 11 patients (58%), seven of whom underwent ventricular shunting procedures. Dissemination of disease was found in 11 patients (58%). Extraneural metastases did not occur in the absence of a ventricular shunt. The authors conclude that malignant astrocytomas of the spinal cord are heralded by a short history followed by rapid neurological deterioration and usually death. The rationale for operation is discussed, and an aggressive approach utilizing adjuvant therapy directed at the entire neuraxis is suggested.

Adolescent

Intrinsic brainstem tumors in childhood: surgical indications.

Sixty-six children with intrinsic brainstem gliomas diagnosed between 1980 and 1986 underwent radical surgical resection. Retrospective analysis permitted classification of tumors into four categories: diffuse, focal, cystic and cervicomedullary. All 27 patients with diffuse tumors had malignant neoplasms, were not benefitted by surgery and died within 12-18 months. Five of nine cystic tumors, three of five focal tumors and twenty of twenty-four cervicomedullary tumors had low grade histopathology and are alive one to six years postoperatively. The authors propose a clinical-neuroradiological criteria that accurately predict which patients with brainstem tumors are likely to benefit from radical surgical intervention.

Astrocytoma

Brain tumors in infants less than a year of age.

A retrospective examination of brain tumors in infants less than a year of age was undertaken by reviewing their charts and CT scans. In contradistinction to brain tumors found in older children, most tumors were supratentorial in location. The most common histologic types included: astrocytoma, ganglioglioma and primative neuroectodermal tumors. Apart from their larger size at the time of presentation, these tumors were radiologically and pathologically similar to analogous tumors found in adults.

Brain Neoplasms

Planning ventriculoperitoneal shunts in infants and small children.

A neurosurgeon planning a ventriculoperitoneal shunt in an infant or child must consider the question of how long a shunt to use in order to compensate for linear growth. Ideally, the system should be just long enough to accommodate the child's further development without being any longer than necessary. To approach the problem systematically, we used established criteria for prediction of growth to determining the catheter length required in adulthood. Our aim was to devise a simple 'rule of thumb' method for determining the catheter length necessary for maximal survival of a functioning shunt system.

Adolescent

'Slit-ventricle syndrome': etiology and treatment.

Between 1970 and 1986, 20 patients were treated for the 'Slit-ventricle syndrome'. Six patients had intermittent proximal shunt malfunction and 14 children had increased intracranial pressure with normal shunt function. All of the children in the second group had a relatively small calvarium. Treatment consisted of proximal shunt revision in the first group and a calvarial expansion procedure in the second. The authors discuss the differential diagnosis of the slit-ventricle syndrome and offer guidelines to appropriate treatment.

Cerebral Ventricles

Magnetic resonance imaging of intradural spinal lipoma.

Intradural lipomas are rare tumors of the spinal canal. We report the occurrence of this neoplasm in a 19-year-old girl. Magnetic resonance accurately depicted both the exact location within the spinal canal and the precise tumor histology and was more informative than myelography. This case illustrates the value of magnetic resonance imaging in patients presenting with myelopathy. In many instances, this modality may be all that is needed in preoperative planning.

Adult

Intra-axial tumors of the cervicomedullary junction.

The authors present their experience with the operative management of 20 intra-axial tumors of the cervicomedullary junction. There were two distinct modes of clinical presentation: lower cranial nerve dysfunction and spinal cord dysfunction. Both groups of patients had indolent courses: in 75% of the patients the symptoms had been present for 6 months to 2 years. Radical excision was carried out in all patients. There was no surgical mortality. Postoperative neurological recovery was directly related to the preoperative status; one patient had a significant new deficit. The authors conclude that intrinsic gliomas of the cervicomedullary junction are amenable to radical excision and that an aggressive surgical approach offers the potential for both neurological recovery and long-term survival. The neuroradiological evaluation and operative technique are discussed.

Adolescent