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Biomedical subjects

F Epstein

Publications and source records attributed to F Epstein.

At least 19 recordsLinked to original sources

Pediatric intramedullary spinal cavernous malformations.

STUDY DESIGN: A retrospective analysis of a large single institution pediatric intramedullary tumor database. The database was searched for all pediatric intramedullary cavernous malformations. OBJECTIVES: To review the authors' experience with three pediatric intramedullary cavernous malformations, review the previous literature, and discuss the surgical management for these rare lesions. SUMMARY OF BACKGROUND DATA: Cavernous malformations can occur throughout the central nervous system; however, the intramedullary spinal cord is a rare location. More recent estimates indicate that cavernous malformations constitute 5% of all vascular malformations. There is no series that reports the management of pediatric intramedullary cavernous malformations. METHODS: There were 181 pediatric intramedullary lesions in the tumor database. A review revealed three children with a mean age of 13.3 years (range, 8-19 years) with intramedullary cavernous malformations. All these children presented with an acute motor deficit that prompted radiographic imaging and diagnosis. All the children underwent laminectomy and gross total removal of the lesion. All the malformations abutted the dorsal pial surface and created a blue discoloration, which was evident after opening the dura. RESULTS: Approximately 10% of all intramedullary cavernous malformations present in the pediatric population. Pediatric lesions, unlike those in adults, have an acute presentation, and the lesions are typically found in the cervical region. The surgical removal of these malformations results in a good functional outcome. The entire neuraxis should be screened because there is a high incidence of multiple lesions. CONCLUSIONS: The surgical removal of these malformations results in a good functional outcome. The entire neuraxis should be screened because there is a high incidence of multiple lesions.

Adolescent↗

Intramedullary low-grade astrocytomas: long-term outcome following radical surgery.

The management of low-grade intramedullary astrocytomas is controversial. Unlike ependymomas, which have a distinct cleavage plane, astrocytomas are diffuse infiltrative tumors. The intramedullary tumor database at NYU Medical Center was searched to identify all patients with intramedullary astrocytoma from 1988 to 1994. Seventeen patients presented with a low-grade diffuse astrocytoma. The medical, surgical and office records were reviewed retrospectively and statistically analyzed. All patients underwent a radical resection of the intramedullary tumor; of these, 12 patients had gross-total removal and 5 had subtotal removal. Nine patients received adjuvant radiotherapy. The median follow-up period was 7.4 years. Fourteen patients are alive and have stable disease. Eleven patients (79%) are functionally independent at last follow-up. The remaining patients are at functional Grade III. The 5-year overall survival was 82% for this group. These results indicate that aggressive surgery is associated with a prolonged survival for patients with intramedullary astrocytomas. Radiation therapy should only be reserved for patients who have radiographic progression or inoperable disease.

Adult↗

Natural history of brainstem cavernous malformations.

OBJECTIVE: To review the natural history and determine the rates of intra- and extralesional hemorrhaging of brainstem cavernous malformations (cavernomas) monitored by one neuro-ophthalmology service. METHODS: A record review of all patients with brainstem cavernomas who were evaluated by a neuroophthalmology service between 1987 and 1999 was performed. We recorded the clinical symptoms and Rankin disability grade at presentation, during the worst clinical episode, and at the last follow-up examination. Magnetic resonance imaging scans were reviewed for evidence of intralesional hemorrhage (a bleeding episode), edema, or venous anomalies, and the cavernoma size was assessed. RESULTS: Thirty-seven patients (age range, 6-73 yr; mean age at presentation, 37.5 yr) underwent a mean of 4.9 years of follow-up monitoring. At presentation, there were 27 bleeding events and 8 nonhemorrhagic events; 2 patients did not exhibit symptoms. Patients who were at least 35 years of age exhibited a lower risk of bleeding episodes (odds ratio, 0.15; 95% confidence interval, 0.1-0.4). Cavernomas of at least 10 mm were associated with a higher risk of bleeding episodes (odds ratio, 3.48; 95% confidence interval, 1.3-9.4). Thirty-nine bleeding episodes occurred in 31 patients, yielding a bleeding rate of 2.46%/yr. There were eight rebleeding episodes, yielding a rebleeding rate of 5.1%/yr. Three patients experienced extralesional bleeding episodes; all of these patients experienced rebleeding. Of the 39 follow-up magnetic resonance imaging scans, the cavernoma size was unchanged in 66.7%, smaller in 18%, and larger in 15%. At the last follow-up examination, the mean Rankin grade was 1.0 for all patients, 0.6 for the 25 nonsurgically treated patients, and 1.4 for the 12 surgically treated patients. CONCLUSION: Rebleeding is not more common among patients who first present with bleeding, and it often has little effect on the neurological status of patients. Significant morbidity attributable to a brainstem cavernoma occurred in 8% of patients during follow-up monitoring of medium duration.

Adolescent↗

Spinal intramedullary cavernoma: clinical presentation and surgical outcome.

OBJECT: Improved neuroimaging techniques have led to an increase in the reported cases of intramedullary cavernomas. The purpose of this study was to define the spectrum of presenting signs and symptoms in patients with spinal intramedullary cavernomas and to analyze the role of surgery as a treatment for these lesions. METHODS: The authors reviewed the charts of 16 patients who underwent surgery for spinal intramedullary cavernomas. All patients underwent preoperative magnetic resonance imaging studies. Cavernomas represented 14 (5.0%) of 280 intramedullary lesions found in adults and two (1.1%) of 181 intramedullary lesions found in pediatric cases. A posterior laminectomy and surgical resection of the malformation were performed in all 16 patients. CONCLUSIONS: Magnetic resonance imaging is virtually diagnostic for spinal cavernoma lesions. Patients with spinal intramedullary cavernomas presented with either an acute onset of neurological compromise or a slowly progressive neurological decline. Acute neurological decline occurs secondary to hemorrhage within the spinal cord. Chronic progressive myelopathy occurs due to microhemorrhages and the resulting gliotic reaction to hemorrhagic products. There is no evidence that cavernomas increase in size. The rate of rebleeding is unknown, but spinal cavernomas appear to be clinically more aggressive than cranial cavernomas, probably because the spinal cord is less tolerant of mass lesions. Complete surgical removal of the cavernoma was possible in 15 of 16 of the authors' cases.

Adolescent↗

Aneurysmal bone cyst of the temporal bone.

OBJECTIVE AND IMPORTANCE: Aneurysmal bone cysts (ABC) are benign bone neoplasms which typically involve the spine and long bones. We present a rare case of an ABC in the temporal bone with significant cerebellar compression. CLINICAL PRESENTATION: The patient was a young boy who presented with several weeks of left posterior auricular pain. Computed tomography, magnetic resonance imaging and angiogram showed an expansile bone neoplasm involving the left petrous temporal bone. INTERVENTION: A retrosigmoid-transmastoid craniectomy was performed, and total removal of the bone lesion was achieved. The pathological examination revealed the diagnosis of ABC. CONCLUSION: The postoperative course was uneventful, and the imaging studies demonstrated total removal of the neoplasm. This case represents the imaging and surgical management of a rare ABC in the temporal bone. This diagnosis should be considered in the differential of bone neoplasms in this region.

Bone Cysts, Aneurysmal↗

Treatment of intracranial ependymoma by surgery alone.

OBJECTIVE: This study aimed to determine the safety of deferring radiotherapy in pediatric intracranial ependymoma following a radiographically confirmed gross total resection in patients with localized disease. METHODS: Children over age 3 were recruited prospectively from 1990 to 1997, following a surgical impression and radiologic confirmation of a gross total resection of an intracranial ependymoma. RESULTS: 10/32 cases of intracranial ependymomas were both eligible and gave consent. 7 remain free of disease without further intervention. 3 recurred, 2 were salvaged with surgery and radiotherapy, none died. CONCLUSIONS: Deferral of radiotherapy following gross total resection alone is a safe option in supratentorial ependymomas. The pattern of recurrence is usually local and patients may be salvaged with additional surgery with or without radiotherapy.

Adolescent↗

Treatment of high-grade spinal cord astrocytoma of childhood with "8-in-1" chemotherapy and radiotherapy: a pilot study of CCG-945. Children's Cancer Group.

OBJECT: The purpose of this study was to devise an improved method of treating high-grade gliomas of the spinal cord in children who have a dismal prognosis following conventional treatment. METHODS: Eighteen children with newly diagnosed high-grade astrocytomas arising in the spinal cord were enrolled in the Children's Cancer Group (CCG) protocol 945. Following surgery, they were all assigned to receive two cycles of "8-drugs-in-1-day" (8-in-1) chemotherapy prior to radiotherapy and eight additional cycles thereafter. A centralized neuropathology review was used to confirm the diagnosis of high-grade astrocytoma in 13 of the 18 children: anaplastic astrocytoma (eight patients), glioblastoma multiforme (four patients), and mixed malignant glioma (one patient). Diagnoses were discordant in five patients. There were eight boys and five girls in the group with confirmed diagnoses, with a median age of 7 years (range 1-15 years). The extent of resection was confirmed by computerized tomography or magnetic resonance (MR) evaluation in five of 13 patients. There were six gross-total or near-total resections (> 90%), four partial or subtotal resections (10-90%), and three biopsies. Six patients showed evidence of leptomeningeal metastases at diagnosis based on staging MR examinations. Eight of the 13 patients completed at least eight of the prescribed 10 cycles of chemotherapy; five received craniospinal radiotherapy and five spinal radiotherapy. CONCLUSIONS: The 5-year progression-free and total survival rates for the 13 children were 46 +/- 14% and 54 +/- 14%, respectively. Seven patients suffered a relapse at the primary site, four of whom also had leptomeningeal metastases. Seven of the 13 patients (54%) remain alive at the time of this report at a median of 76 months (range 51-93 months) from study entry. Six patients died between 8 and 38 months after diagnosis, all with active disease. Intensification of therapy may further improve outcome in this high-risk population.

Adolescent↗

Diffuse infiltrating astrocytoma of the cervicomedullary region: clinicopathologic entity.

Recent imaging and neurosurgical techniques have led to an improvement in the surgical management of children with brainstem tumors (BSTs). Many children with tumors previously considered 'inoperable' can now benefit from surgery. Increased experience has brought about new theories concerning the growth pattern, natural history, classification and optimal management of these tumors. Cervicomedullary (CM) tumors commonly have an indolent presentation reflecting either medullary or cervical spinal cord dysfunction and tend to arise in the upper cervical cord growing into the medulla in a posterior exophytic fashion. Intrinsic BSTs often present acutely with cranial nerve dysfunction and generally arise in the pons with a diffuse infiltrating growth pattern. A 21-month-old patient had developed feeding difficulty and reactive airway disease at approximately 8 months of age. MRI showed a diffuse, nonenhancing tumor in the CM region. Following radical resection, and an unremarkable perioperative course, he aspirated, developed pulmonary insufficiency and expired. Postmortem examination revealed a low-grade diffuse fibrillary astrocytoma extending from C6 to the medulla. The medullary portion arose in a paramedian location and infiltrated dorsally into the fourth ventricle, the obex, the leptomeninges, and the adjacent cerebellum. This case demonstrates the growth pattern of a distinct subset of CM tumors that behave in a manner similar to intrinsic diffuse BST. Future identification of these subsets by a careful analysis of the clinical presentation and MRI images will enable better operative planning and optimal management.

Astrocytoma↗

Medical abortion: ethics, laws and religious points of view, A study by the 1994-1995 Ethics and Morals Committee of the ISPN.

Central nervous system malformations are now frequently detected prenatally. Unfortunately, the progress in diagnosis having far outstripped that in therapeutics, the only question to answer is usually whether the pregnancy should be continued or terminated. Pediatric neurosurgeons have to deal with such problems more and more often. They are asked to give an opinion on the prognosis. In many cases the law requires their involvement in the decision about the possibility of a "therapeutic", or more correctly a medical, abortion. The final decision is based on the religious attitutes of the family and the neurosurgeon and on the law of the country in which the medical abortion would be performed. This article reviews the points of view of the main religions and also the laws in different countries. This knowledge may be of some help to neurosurgeons, especially when the religious attitudes of those involved are different or when their final decision is not in accordance with the law of the country.

Abortion, Legal↗

Intramedullary subependymoma of the spinal cord.

A consecutive series of six patients underwent operative resection of intramedullary spinal cord subependymomas between January 1981 and August 1993. Three men and three women between the ages of 26 and 66 years experienced symptoms for a mean of 50 months preceding diagnosis. The locations of the tumors were predominantly cervical or cervicothoracic, except in one patient. At operation, a complete extirpation was achieved in each patient. No patient received further adjunct therapy. There has been no tumor recurrence in any patient after a mean follow-up period of 39 months. Most of the intramedullary spinal cord tumors are either ependymomas or astrocytomas. Clinical histories, physical examinations, and radiographic investigations are not conclusive for absolute diagnosis of subependymomas; however, intraoperative gross observations have shown these well-demarcated tumors to be located eccentrically within the spinal cord. Pathological examinations demonstrate tumors with sparse cellularity, clustering of cells, and dense fibrillary stroma. Proliferation studies with the mouse monoclonal antibody MIB-1 reconfirm the slow growth potential of these benign tumors.

Adult↗

Practical decisions in the treatment of pediatric brain stem tumors.

The treatment of brain stem gliomas has evolved over the last few decades, reflecting advances in imaging (MR), microsurgical techniques and biological understanding. The aim of this chapter is to provide a preoperative classification for intrinsic brain stem lesions that will predict histopathology and biological behavior from the clinical syndrome and the MR appearance. Such a classification system may help in selecting children with brain stem tumors that can benefit from surgery. Technical considerations, potential surgical complications, and the ways to avoid them are discussed.

Brain Neoplasms↗

Spontaneous involution of an intra-axial brain stem lesion: a case report.

This report describes the spontaneous involution of an intrinsic brain stem lesion whose clinical and MRI characteristics strongly suggest a tumor etiology. Nonoperative experiences with central nervous system tumors have shown that they may sometimes exhibit prolonged periods of dormancy. There are several reports of 'disappearing' CT lesions which have all been inflammatory, infective, or immunological. No histologically proven tumor has been shown to spontaneously involute, nor have 'disappearing' lesions been described for MRI.

Brain Neoplasms↗

The use of motor evoked potentials in the diagnosis of psychogenic quadriparesis. A case study.

We present a case illustrating the usefulness of motor evoked potentials (MEPs) in differentiating psychogenic from organic postoperative paralysis. Discussed is a 12-year-old girl who underwent surgery for the repair of a recurrent syringomyelia. On the 6 day after a proximal revision of her syringoperitoneal shunt she returned to the hospital with deep quadriparesis, bowel and bladder incontinence, and complaining of severe headache. An MRI scan showed the syrinx to be collapsed, and removal of the shunt had no impact on her clinical symptoms. Repeat somatosensory evoked potentials (SEPs) showed no change in comparison to those obtained at the end of her preceding surgery. MEPs were normal for the lower extremities, a finding which is inconsistent with a severe upper motor neuron lesion. She was diagnosed with psychogenic paralysis, and fully recovered within 1 month. We propose that a neurophysiological evaluation including MEPs is useful in the differential diagnosis of psychogenic and organic motor weakness.

Child↗

Late radiation necrosis of the brain: case report.

We report a case of radiation necrosis occurring 47 years after resection and radiotherapy for a juvenile pilocystic cerebellar astrocytoma, the longest yet reported. The patient presented with progressive lower cranial nerve dysfunction, and eventually died from cardiopulmonary arrest secondary to aspiration. The presentation, diagnosis, pathological features, and management of radiation necrosis are discussed.

Astrocytoma↗

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↗