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Biomedical subjects

F Epstein

Publications and source records attributed to F Epstein.

At least 37 records · Page 2Linked to original sources

Intrinsic brain-stem tumors of childhood: surgical indications.

This report summarizes the first author's experience with radical excision of intrinsic non-exophytic brain-stem gliomas in 34 pediatric patients. On retrospective analysis, these tumors may be classified into three subgroups: focal, diffuse, and cervicomedullary. A focal neoplasm is a circumscribed mass less than 2 cm in diameter and without associated edema. Tumors of a larger size or in which the "focal" neoplasm is associated with a large area of apparent edema are classified as diffuse. Cervicomedullary neoplasms occur at the junction of the medulla and spinal cord and involve both of the structures but do not extend rostrally into the pons. A radical tumor excision was carried out in all patients, and the only mortality and morbidity occurred in children harboring diffuse gliomas. All of the diffuse gliomas were malignant (grade III or IV astrocytomas), whereas three of the four focal astrocytomas and all of the cervicomedullary tumors were grade II astrocytomas. No patient with a diffuse astrocytoma was benefitted by surgery, while two of the focal astrocytomas and all of the cervicomedullary neoplasms either became stable or improved postoperatively. It is concluded that, although surgery may be accomplished within the substance of the brain stem with low morbidity and mortality rates, it is not indicated for malignant astrocytomas as it has no impact on the biology of the neoplasm. Therefore, while primary radical excision is recommended for cervicomedullary neoplasms, which are often benign, the more traditional radiation therapy and/or chemotherapy remain appropriate for tumors above the medulla.

Astrocytoma

Spinal cord astrocytomas of childhood.

The author has carried out gross total excision of an intramedullary spinal cord astrocytoma in 120 consecutive patients. This experience has led to the following conclusions: Holocord widening occurs in 60% of cases, and is diagnostic of a cystic astrocytoma. Despite the absence of a surgical plane of dissection, these neoplasms may be removed from "inside out" until a glia-tumor interface is recognized. Radical tumor excision is compatible with partial or total recovery of neurological function. The success of surgery is directly related to the preoperative neurological status of the patient. Paralysis or near paralysis was never improved, while mild to moderate preoperative neurological dysfunction often recovered. While this experience has established the efficacy of radical surgery, there is no information to suggest the duration of remission, or the likelihood of permanent cure. This will only become known at the time of retrospective analysis many years from now.

Astrocytoma

Optic pathway tumors. A review with proposals for clinical staging.

Neoplasms of the optic nerves and pathways usually present during the first decade of life with loss of visual acuity. Neurofibromatosis is frequently associated with optic nerve tumors. Computed tomography has improved the capability of diagnosis, exact anatomic delineation of the mass, and clinical follow-up of afflicted patients. The tumors are typically slow-growing, low-grade astrocytomas. Posterior lesions involving the chiasm and hypothalamus may produce considerable morbidity and eventual mortality. Timing and appropriate methods of therapeutic intervention continue to be controversial. Symptomatic patients with blindness and involvement of a single optic nerve may be managed with surgical intervention alone. Posterior lesions with symptomatic progression should be treated with radiation therapy and with cerebrospinal fluid diversion when hydrocephalus occurs. Because these lesions evolve slowly, patients experience prolonged survival, and the benefits of radiation therapy are inconclusive. Clinical staging systems should consider both the functional status of patients and the anatomic extent of the disease.

Adolescent

A staging system for brain stem gliomas.

Intrinsic brain stem neoplasms are almost invariably malignant, and irrespective of radiation treatment and chemotherapy, are associated with a very abbreviated survival. It is possible that occasional long-term survival is the result of a radiation-sensitive low-grade astrocytoma. It is suggested that a primary staging system may separate poor-risk and good-risk patient populations and that this classification may be important in assessing future therapeutic options.

Brain Neoplasms

Radical resection of intramedullary spinal cord tumors in adults. Recent experience in 29 patients.

The management of patients with intramedullary spinal cord tumors is controversial. In the past, these tumors have often been treated with biopsy or subtotal removal followed by irradiation--a therapy that is usually associated with early tumor recurrence and progressive neurological impairment. In an attempt to improve on the outcome of patients with intramedullary tumors, the authors performed radical resection in most of the 29 adult patients who had surgery for these tumors within the past 30 months. The mean duration of symptoms was 9 1/2 years, and all patients presented because of progressive neurological deficit. Patients were evaluated with metrizamide myelography-computerized tomography scanning and intraoperative ultrasound imaging to define the site of the tumor and cystic components. There were 14 ependymomas, 11 astrocytomas, two lipomas, and one case each of intramedullary fibrosis and astrogliosis. Solid tumor spanned a mean of five spinal cord segments and 16 tumors were associated with cysts. Twenty tumors were in the cervical and/or cervicothoracic regions. Total removal was achieved in 14 patients and "99% removal" in seven others. In 21 of 29 patients (72%), the neurological condition was stabilized or improved as a result of the operation. Postoperative deterioration occurred for the most part in patients who could not walk or who had minimal motor function at the time of operation, and these patients are no longer considered as operative candidates. Radical resection of intramedullary tumors can be achieved, with stabilization or improvement of neurological deficit in the majority of patients.

Adult

Atypical visual prognosis with an optic nerve glioma.

A 13-year-old girl presented with a 1-year history of gradual decline of vision in her right eye. She was diagnosed by clinical examination and computed tomographic scan as having a right optic nerve glioma. While awaiting surgery, she experienced spontaneous improvement of her symptoms. Four years after the onset of symptoms, the Snellen acuity, color testing, and visual fields have returned to normal, while the computed tomographic appearance of the tumor is essentially unchanged. Possible mechanisms for such a fortuitous outcome are discussed.

Adolescent

How to keep shunts functioning, or "the impossible dream".

Hydrocephalus is a benign condition, and as our expertise in surgical treatment has increased, many of the "inevitable complications" are disappearing, and a normal life associated with satisfactory shunt function is no longer "the impossible dream." While this chapter has identified a few of the problems that may complicate long-term management, it is important to reemphasize that the most important single factor which determines success or failure is the attitude and experience of the neurosurgeon. While there is a great deal of surgical pride associated with removing a meningioma or clipping an aneurysm, we do not receive accolades from our colleagues when we have inserted or replaced a shunt. Whereas the surgical technique that is employed to remove an acoustic tumor or a meningioma at the base of the brain is a testimony to the skill of the neurosurgeon, it is not a technical feat to revise a shunt in an acutely ill child. When the surgical outcome is a satisfactory one, it is taken for granted, and when recurrent problems evolve they are assumed to reflect problems intrinsic to caring for hydrocephalus, and little consideration is given to the technical and conceptual skills required to retrieve the situation. Neurosurgeons often lose sight of the fact that hydrocephalus is the most benign intracranial disorder which is treated in large numbers. I believe that the most serious problem in the treatment of hydrocephalus has been the reluctance of experienced neurosurgeons to devote their time and energies to understanding the underlying pathophysiology and the potential complications and to instituting appropriate treatment. A neurosurgeon who is devoted to both the patient and the family and is committed to understanding and treating complications associated with shunts and shunt dependency will be the best insurance of the future of the patient, and ultimately as normal adults these patients will be a permanent testimony to the skills of the primary neurosurgeon.

Adolescent

Computed tomography of arachnoid cysts.

The typical CT appearance of intracranial arachnoid cysts may obviate additional neuroradiological procedures. Ventriculography using water-soluble contrast media may differentiate a suprasellar arachnoid cyst from a dilated third ventricle. Twelve cases are presented.

Adolescent

Prophylactic craniofacial surgery.

One-stage radical reconstructive surgery for the common craniofacial deformities has become standard procedure in a few medical centers. With increasing experience and expertise, young children and adolescents, as well as a few adults have been greatly benefited. It has long been recognized that both the cranial and facial deformities of Couzon's disease and Apert's syndrome are progressive, having their inception either before birth or in the neonatal period, and evolving to the full blown syndrome during the first years of life. The authors believe that the progressive nature of these anomalies is secondary to a combination of permaturely fused sutures of the calvarium and cranial base (coronal, sphenozygomatic, frontoethmoidal and frontosphenoidal). In an effort to arrest and correct both the cranial and facial deformities as well as obviate the need for future radical surgery the authors have treated several children with Crouzon's disease and Apert's syndrome with suture craniectomy along the base of the skull. Following this procedure, deformity has regressed and ultimate cosmetic improvement has been dramatic. It is unlikely that radical craniofacial surgery will be necessary in this group of patients at any future time. On the basis of this experience, it is believed that the pendulum is now swinging and that in the future, stripping of the appropriate sutures along the base of the skull will limit deformity and make additional surgery unnecessary.

Child, Preschool

Alphafetoprotein and human chorionic gonadotropin determination in cerebrospinal fluid. An aid to the diagnosis and management of intracranial germ-cell tumors.

The cerebrospinal fluid (CSF) and serum of six patients with histologically verified intracranial germ-cell tumors were assayed serially for the presence of alphafetoprotein (AFP) and the beta subunit of human chorionic gonadotropin (HCG). Two patients had embryonal carcinomas, two had choriocarcinomas, and two had dysgerminomas. The marker profile for a given tumor in either CSF or serum correlated with the histological diagnosis; that is, embryonal carcinoma produced AFP and HCG, choriocarcinoma produced HCG, and dysgerminoma produced no markers. The marker levels in serum and CSF declined with therapy and rose usually prior to the development of overt clinical symptoms if the patient's tumor recurred. A CSF-to-serum gradient of the marker levels was present in three of four patients, and the serum levels were often normal when the CSF values were elevated. Ventricular marker levels were lower than the lumbar levels in two of two patients. The assay of these biological markers is a sensitive indicator of the success of therapy, and the presence of a CSF-to-serum gradient suggests that the major portion of the neoplasm rests within the central nervous system. A histological diagnosis can be inferred without the necessity of surgery in appropriate clinical contexts.

Adolescent

Computerized axial tomography in the preoperative evaluation of posterior fossa tumors in children.

22 cases of pediatric posterior fossa tumors that underwent preoperative evaluation by computerized tomography are presented. 8 of the cases, in the latter part of the series, were successfully operated upon on the basis of computerized tomography alone. In 14 other cases, additional angiographic investigation was carried out. In only 1 case of multiple hemangioblastoma did angiography provide further significant information. On the basis of this experience, it is suggested that computerized tomography is usually sufficient and further contrast studies are unnecessary in the preoperative evaluation of pediatric posterior fossa tumors.

Adolescent

Ouabain inhibition of gill Na-K-ATPase: relationship to active chloride transport.

Ouabain circulating in blood inhibits Na-K-ATPase in the gills of seawater eels at a concentration similar to that necessary for inhibition in vitro. By contrast, a much higher concentration is required when ouabain is applied to the exterior of the gill. Inhibition by external ouabain occurs only when the drug gains access to the circulation of the fish, as evidenced by simultaneous inhibition of Na-K-ATPase in the kidney. These results suggest that the Na-K-ATPase of gill chloride cells faces inward, lining intracytoplasmic tubular channels continuous with the extracellular fluid. Inhibition of gill Na-K-ATPase by ouabain in intact salt water eels results in almost complete inhibition of the efflux of both Na+ and Cl-. The efflux is tritiated water was much less reduced, to 60% of normal. Since chloride is actively transported outward across the gill of seawater teleosts, it is suggested that active chloride transport is coupled to Na-K-ATPase. A neutral sodium chloride carrier is postulated that is energized by the movement of sodium from extracellular fluid down its electrochemical gradient into the chloride cell.

Adenosine Triphosphatases

Myelomeningocele: a progressive intra-uterine disease.

A series of cats were made hydrocephalic by intracisternal injection of kaolin, and four weeks later ventriculography confirmed the presence of hydromyelia. Ten days later, laminectomy in the lower thoracic region was performed. Several days later ventriculography revealed a markedly dilated central canal beneath the entire laminectomy, and pathological examination confirmed the presence of a focal bulge confined to the same region. On sectioning, it was evident that the central canal was massively enlarged to fill the entire spinal cord beneath the bulging region. The conclusion from these findings is that myelomeningocele is the result of progressive intra-uterine disease which begins with pathological hydromyelia and culminates in rupture of the unsupported spinal cord as a result of maldevelopment of associated dermal tissue.

Animals

Evaluation of pediatric hydrocephalus by computed tomography.

Computed tomography (CT) is a safe and reliable technique for the study of children with increased head circumference. Hydrocephalic children requiring drainage of cerebrospinal fluid may be shunted on the basis of the CT scan alone and their postsurgical course followed by serial CT scans thereafter. Any additional pneumographic studies required may be performed via the existing shunt tube, eliminating transcerebral catheterization and its attendant complications.

Brain Edema

Role of computerized axial tomography in diagnosis and treatment of common neurosurgical problems of infancy and childhood.

Computerized axial tomography (CAT) is a new, highly accurate technique for studying the skull and intracranial contents. It is rapid enough to be utilized in acute emergencies and safe enough to be utilized for routine serial followups and evaluation of outpatients with equivocal findings. Because CAT can delineate the ventricular system without need for contrast agent, can distinguish clearly between hematoma and edema and can differentiate cystic from solid tumors, it finds wide application in the evaluation of neurological disease.

Brain Diseases