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Biomedical subjects

F Epstein

Publications and source records attributed to F Epstein.

At least 55 records · Page 3Linked to original sources

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↗

Early surgery for craniofacial synostosis: an 8-year experience.

A prospective review is presented of 50 patients with one of the craniofacial synostosis syndromes who underwent early interventive craniofacial surgical correction (average age 7.6 months at time of surgery). The study has demonstrated the efficacy and safety of the techniques when employed in the infant. Satisfactory cranio-orbital form was achieved in the majority of the patients, although 10 patients required secondary surgery because of sutural refusion or the development of turricephaly or calvarial contour irregularities. Despite earlier hopes, this surgery did not result in the development of satisfactory occlusal relationships and midfacial form in the craniofacial dysostosis group (Crouzon's, Apert's, etc.). Based on this clinical experience, a surgical treatment plan is presented for the newborn with craniofacial synostosis.

Acrocephalosyndactylia↗

Cerebellocele and associated central nervous system anomalies in the Meckel syndrome.

Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the level of posterior fossa in this condition are classified as those belonging to the Chiari type III group of anomalies. This unusual set of anomalies which forms pathogenetic link between the Dandy-Walker and Chiari-Arnold group of anomalies in the posterior fossa seems to be very frequent in the Meckel syndrome. The therapeutic emphasis is on genetic counseling in view of the recessive inheritance of the syndrome.

Abnormalities, Multiple↗

Intracranial lipomas, hydrocephalus and other CNS anomalies in oculoauriculo-vertebral dysplasia (Goldenhar-Gorlin syndrome).

13 cases of Goldenhar-Gorlin syndrome are presented in which numerous central nervous system anomalies have been found. These include occipital encephalocele, hydrocephalus, aqueductal stenosis, agenesis of corpus callosum, multiple congenital lipomas and many others. Pertinent literature has been reviewed. It is concluded that any part of the central nervous system can be involved in this condition and that careful evaluation is indicated in order to rule out a treatable intracranial anomaly.

Adolescent↗

Extensive advanced maturation of medulloblastoma to astrocytoma and ependymoma.

Four cases of medulloblastoma in children are reported in which a marked degree of differentiation towards neoplastic adult glioma cells was observed. The original immature and highly malignant tissue had largely disappeared in three and completely disappeared in the fourth. The mature tissues which replaced them included astrocytoma tissues in all four, but ependymoma tissues predominated in two. These cases illustrate a general principle applicable to embryonic tumors, defined as those originating in cells that are still immature. Such maturation and differentiation are presumably associated with a decrease in growth rate and an improved prognosis. A continuation of the process of maturation may account for the unexpectedly favorable course sometimes noted with gliomas in children. The processes of differentiation, dedifferentiation, and anaplasia are discussed, particularly the semantic implications of the terms, and it is suggested that the term "dedifferention" is unfortunate since it may have two different meanings. It may be used as a synonym of anaplasia, or it may imply a progression of change backward on the embryologic pathways along which primitive cells normally mature. The latter may not occur, but the assumption that it does leads to views concerning the glioma group as a whole and the phenomena described in this paper, which we hold to be untentable.

Astrocytoma↗

Encephalocele (cerebellocele) in the Goldenhar-Gorlin syndrome.

A patient with unilateral preauricular appendages and occipital mass is described who died on the fifth day of life. An autopsy demonstrated congenital megabladder and megaureters and dysplastic kidneys. The neuropathological examination revealed cranium bifidum in the occipital region, defective posterior arch of the C1 vertebral body, agenesis of vermis and a large cerebellocele. It is suggested that some patients with the Goldenhar-Gorlin syndrome may have prominent central nervous system involvement in spite of only relatively slight facial involvement.

Cerebellar Diseases↗

Meningomyelocele: "pitfalls" in early and late management.

Meningomyelocele is a dynamic ever evolving disease. Children surviving the neonatal period remain at risk for a variety of related neurosurgical problems which are only being recognized as that affected population grows larger and older. The least afflicted children are the most vulnerable, as they have more function which may be further impaired. It is essential that the spina bifida clinic be aware of these potential problems and remain responsive to the changing medical and surgical needs of this "aging" patient population.

Adolescent↗

The Cavitron ultrasonic aspirator in tumor surgery.

The CUSA Ultrasonic Surgical System fragments and aspirates a wide spectrum of firm tumors of the central nervous system with little transmitted movement to adjacent normal neural structures. The CUSA system is clearly of limited value in removing extremely calcified or dense fibrous tumors. It lacks primary hemostatic properties, and conventional hemostatic techniques are necessary.

Brain Neoplasms↗

Accidental hypothermia.

The diagnosis of hypothermia rests solely upon a measured core temperature of 35 degrees C or less; a special thermometer calibrated to record low temperatures should be used whenever hypothermia is suspected. Hospital management of the hypothermic patient consists of definitive rewarming therapy.

Accidents↗

Iniencephaly: a neuropathologic study.

Five cases of iniencephaly are reviewed. Numerous central nervous system malformations were found at all levels, including microencephaly, polymicrogyria, heterotopic glial tissue in the leptomeninges, atresia of the ventricular system, marked disorganization of the brain stem, vermian agenesis, large cerebellar cyst, and disorganization of the spinal cord tissue. The cerebellum was normal in one case. Numerous skeletal anomalies were found as well as marked retroflexion of the craniocervical junction. We concluded that cerebral anomalies, although severe, are not specific for iniencephaly. Cerebellar anomalies, on the other hand, were considered to share some morphologic features between Dandy-Walker and Arnold-Chiari, i.e., Chiari type II and Chiari type III, malformations.

Abnormalities, Multiple↗

Intractable facial pain associated with a ganglioglioma of the cervicomedullary junction: report of a case.

A 6-year-old child with a brain stem tumor presented with the unusual complaint of intractable facial pain resembling trigeminal neuralgia in the absence of other symptoms or signs referable to the 5th cranial nerve. The radiological evaluation included a computed tomographic scan with intravenous contrast administration, which demonstrated an enhancing intramedullary lesion extending from the obex to C-4. After radiation and chemotherapy had failed to achieve symptomatic relief, the tumor, later proven to be a ganglioglioma, was radically removed with the ultrasonic aspirator. Postoperatively the patient experienced full pain relief.

Brain Neoplasms↗