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Biomedical subjects

F J Epstein

Publications and source records attributed to F J Epstein.

At least 37 records · Page 2Linked to original sources
PubMed1997

Arachnoid cysts of the cerebellopontine angle: diagnosis and surgery.

OBJECTIVE: The optimal surgical management of arachnoid cysts remains controversial. The cerebellopontine angle (CPA) is a rare location for arachnoid cysts, and only 28 cases of arachnoid cysts occurring in the CPA have been reported in the literature. We discuss the diagnosis, radiographic imaging, and surgical management of CPA arachnoid cysts. METHODS: Five patients (three male and two female patients) with a mean age of 5.6 years have been operated on at our institution since 1980. Magnetic resonance imaging allows for the accurate diagnosis of these arachnoid cysts, which can present with only discrete symptoms, such as headache or ataxia. All five arachnoid cysts compressed the cerebellum or brain stem. One patient had associated hydrocephalus. Three patients presented with refractory headaches associated with nausea and vomiting. The remaining two patients presented with cerebellar signs. No patient had an initial cranial neuropathy. RESULTS: All patients underwent a retrosigmoid suboccipital craniotomy and microsurgical resection and fenestration of the cyst walls. One patient underwent two procedures. A cystoperitoneal shunt was inserted at the first operation. After the shunting procedure, the patient's condition deteriorated; however, after the microsurgical resection and fenestration, his symptoms improved. With a mean 5.2-year follow-up, there has been no evidence of clinical or radiographic recurrence. CONCLUSION: Although CPA arachnoid cysts represent a small number of total arachnoid cysts, the CPA is the second most common location for arachnoid cysts to occur. CPA cysts are congenital lesions found in children who present with subtle signs or symptoms. The definitive treatment for these arachnoid cysts is a retrosigmoid suboccipital craniotomy and microsurgical resection and fenestration of the cyst walls.

Adolescent↗
PubMed1997

The role of motor evoked potentials during surgery for intramedullary spinal cord tumors.

OBJECTIVE: This is a prospective study of the methodology and clinical applications of motor evoked potentials (MEPs) during surgery for intramedullary spinal cord tumors. METHODS: Transcranial electrical stimulation was used to activate corticospinal motoneurons, and the traveling waves of the spinal cord were recorded through catheter-electrodes placed epi- or subdurally. Intraoperative MEP monitoring was performed in 32 consecutive patients (age range, 1-50 yr) undergoing resection of intramedullary spinal cord tumors. In 19 patients, MEPs were present before myelotomy (monitorable group), and in 10 patients, MEPs were absent before myelotomy (unmonitorable group). Placement of an epidural electrode was not possible in two patients, and technical problems prevented recording in one. RESULTS: MEP amplitudes decreased intraoperatively by more than 50% of baseline in three patients, all of whom had postoperative paraplegia. Two of these patients recovered within 1 week after surgery, and one remained paraplegic. None of the patients with preserved MEP amplitude (> 50%) sustained immediate significant postoperative deterioration. Motor function was significantly deteriorated 1 week after surgery in one patient in the monitorable group and in five patients in the unmonitorable group. MEP monitorability was significantly associated with good surgical outcome for adult patients (P < 0.05), although not for pediatric patients (P > 0.6). Preoperative motor status and surgical outcome were not significantly associated for the adult (P = 0.13) or pediatric groups (P > 0.4). CONCLUSION: MEP monitorability was a better predictor of functional outcome than the patient's preoperative motor status for the adult group. Significant predictors of MEP monitorability in the adult group were preoperative motor function (P < 0.01), history of no previous treatment (surgery or irradiation) (P < 0.01), and small tumor size (P < 0.05). Weak associations with monitorable MEPs existed for low-grade tumors (P = 0.09), the presence of baseline somatosensory evoked potentials (P = 0.10), and tumor pathological abnormalities (ependymoma) (P = 0.13). No associations were determined for sex (P > 0.4), associated syrinx (P > 0.3), or tumor location (P > 0.5). In the pediatric group, none of the examined factors were associated with MEP monitorability (P > 0.3). A decline of more than 50% in MEP amplitude during tumor removal should serve as a serious warning sign to the surgeon.

Child↗
PubMed1997

Intraoperative spinal cord monitoring for intramedullary surgery: an essential adjunct.

Intraoperative monitoring of the functional integrity of the spinal cord during removal of intramedullary spinal cord lesions is an aid in intraoperative decision making and a primary tool for the prediction of neurological outcome. Motor evoked potential monitoring has become the neurophysiological monitoring technique of choice for that purpose. In the senior author's experience with over 130 pediatric patients suffering from intramedullary spinal cord tumors, the neurophysiological data of both motor and sensory evoked potentials was utilized in an integrative fashion. Motor evoked potentials, elicited with single transcranial electrical stimuli and recorded directly from the spinal cord with an electrode in the spinal epidural space, reflect the functional integrity of the corticospinal tract. Motor evoked potentials, elicited with a short train of transcranial electrical stimuli and recorded from limb muscles, reflect the functional integrity of the motor system from the cerebral cortex/white matter to beyond the neuromuscular junction. Both epidural and muscle motor evoked potentials correlated closely with postoperative neurological function. Both techniques provide fast, practical and reliable information on the functional integrity of the motor tracts of the spinal cord. No complications attributable to stimulation or recording occurred. Over time both the technique's reliable power of predicting clinical outcome and its practical versatility have altered the surgical approach in that gross total resections are more readily attempted as long as motor evoked potential data indicate the intact functional integrity of the corticospinal tract. This monitoring technique unquestionably had a favorable impact on neurological outcome.

Adolescent↗
PubMed1997

Intra-axial tumors of the cervicomedullary junction: surgical results and long-term outcome.

Until recently, intra-axial brainstem tumors were traditionally regarded as surgically inaccessible lesions with a uniformly poor prognosis. However, increasing data indicate that distinct subgroups of brainstem tumors exist that are amenable to surgical intervention. To address this question, we reviewed our experience in the operative management of 39 consecutive patients, in the magnetic resonance imaging (MRI) era, with intra-axial cervicomedullary tumors, in order to determine those factors associated with long-term outcome. Thirty-nine patients (26 male, 13 female) underwent surgery by a single surgeon (F.J.E.) between 1985 and 1994. Mean age of diagnosis was 14 years (range 3 months - 60 years); mean duration of preoperative symptoms was 24 weeks (range 1-168). Twenty patients presented with lower cranial nerve dysfunction and 19 presented with motor and/or sensory dysfunction. All patients were graded according to the McCormick Scale, pre- and postoperatively, and at the time of follow-up. All patients were evaluated with MRI scanning. Twenty-three patients had either previous biopsy or subtotal resection, 13 previous radiation therapy, and 6 previous chemotherapy. The mean time to follow-up was 48 months (range 7-138). Twelve patients underwent gross total resection, 7 near total resection (>90%), 15 subtotal resection (50-90%), and 5 partial resection (< 50%). Histologically, there were 15 low-grade fibrillary astrocytomas, 9 ependymomas, 7 gangliogliomas, 3 anaplastic astrocytomas, 3 juvenile pilocytic astrocytomas, and 2 mixed gliomas. Although the vast majority of tumors were low grade histologically, a higher proportion of the patients with high-grade lesions experienced tumor progression when compared to low-grade tumors (75 vs. 30%). Overall, the 5-year progression-free and total survivals were 60 and 89%, respectively. There was 1 death within the first postoperative month. Preoperative duration of symptoms greater than 15 weeks was associated with a longer progression-free survival. There was a trend for preoperative neurologic grade to predict functional neurologic outcome at follow-up. In summary, intra-axial tumors of the cervicomedullary junction are a distinct subset of brainstem tumors, predominantly of low-grade histology, with favorable long-term progression-free and total survivals following surgical resection. A long duration of preoperative symptoms may indicate an indolent clinical course and a more favorable prognosis. Our data also indicate that early surgical intervention is warranted prior to neurologic deterioration.

Adolescent↗
PubMed1997

Pediatric intramedullary spinal cord tumors: is surgery alone enough?

Pediatric intramedullary spinal cord tumors occur infrequently with limited data on treatment strategies and outcome. Over an 11-year period, 44 patients with such tumors who were primarily managed by radical surgery were retrospectively reviewed. The majority of tumors (42/44, 95%) were in the cervical or thoracic spine and were histologically low grade. Gross total resection was achieved in 73% (32/44). At a median follow-up of 45 months, 11% (5/44) had severe weakness (modified McCormick scale IV-V), 66% (29/44) had mild-moderate deficits (I-III), and 16% (7/44) had sphincter dysfunction. Tumor recurrence rate was 18% (8/44) after a mean period of 54 months. Age, number of involved spinal cord segments and extent of surgical resection did not significantly impact on tumor recurrence rates (p = 0.28, 0.44 and 0.13, respectively). The only significant prognostic factor was histological type; mixed or higher grade tumors having a higher recurrence rate (p = 0.04). Radical surgery for intraspinal tumors can achieve long tumor-free survival without requiring adjuvant therapy.

Adolescent↗
PubMed1997

Coagulation of herniated cerebellar tonsils for cerebrospinal fluid pathway restoration.

In the past, many different surgical techniques have been proposed for treatment of the Chiari type I malformation. Despite the different technical considerations, in general, the treatment objectives have shared certain features: to prevent/ameliorate tonsillar crowding and to restore normal circulation of cerebrospinal fluid at the foramen magnum. We report a simple technique of coagulation of the herniated cerebellar tonsils in situ to decompress adjacent neural structures and to achieve the above-mentioned goals. When compared to the method of tonsillar resection, the procedure described herein affords the following two advantages: it is relatively less time-consuming and is more hemostatic. These two features minimize inflammation-mediated morbidity which may occur during the postoperative period.

Adolescent↗
PubMed1996

Deferring adjuvant therapy for totally resected intracranial ependymoma.

Radical surgery is the most important treatment modality for ependymoma. The benefit of adjuvant radiotherapy and/or chemotherapy following a gross total resection of a low grade intracranial ependymoma is uncertain. Since 1990 we elected to defer adjuvant therapy in 7 pediatric patients with a median age of 7 years (range 3-16 years) who had a radical resection of an intracranial ependymoma and no evidence of central nervous system metastases. The primary tumor site was the cerebral hemisphere (6) and the cerebellum (1). A gross total resection was radiologically confirmed in 5 of the 7 patients. Two of the patients had a blood clot in the resection site on the postoperative magnetic resonance imaging scan. All patients are alive after a median follow-up of 44 months and the median progression-free survival is 38+ months. Five of the patients remain in continuous remission. The 2 patients with postoperative blood clots developed subclinical local recurrences, 10 and 11 months, respectively, after diagnosis. They remain in remission for 13+ and 27+ months after subsequent radical surgical procedures. Involved field radiotherapy was administered to 1 patient. After a limited period of follow-up, radical surgery alone appears to be sufficient for the majority of children with low grade ependymomas diagnosed at > 3 years of age when postoperative imaging confirms a gross total resection. This is more likely to occur in supratentorial ependymomas arising in older children.

Adolescent↗
PubMed1996

Intramedullary spinal cord tumors in neurofibromatosis.

Neurofibromatosis (NF) describes the most commonly inherited disorders affecting the nervous system. These autosomal dominant, neurocutaneous syndromes are associated with multiple tumors of the nervous system, including neurofibromas, schwannomas, meningiomas, and intracranial gliomas. Spinal cord involvement in NF is typically from extramedullary growth of spinal nerve root tumors. Intramedullary spinal cord tumors in NF have been reported as scattered, single cases in literature. However, this association has not been clearly defined as have other nervous system neoplasms that are typically linked with NF. We present a series of nine patients with NF with intramedullary spinal cord tumors who were managed at our institution from 1984 to 1994. The patients' ages ranged from 4 to 31 years. There were seven male patients and two female patients. Three patients had NF-1 (von Recklinghausen's), five patients had NF-2, and one patient had NF, type uncertain. There were three cervicomedullary tumors, two cervical tumors, three cervicothoracic tumors, and one thoracic tumor. A histological examination revealed five ependymomas, three astrocytomas, and one intramedullary schwannoma. Two patients with malignant intramedullary spinal ocrd tumors have died with progressive disease. One other patient has required a subsequent operation for recurrent tumor. The other six patients are doing well, and their prognosis is expected to be related to their systemic disease. We conclude that there is a clinical entity of intramedullary spinal cord tumors associated with NF.

Adolescent↗
PubMed1996

Functional anatomic relationship between brain-stem tumors and cranial motor nuclei.

OBJECTIVE: To identify patterns of cranial motor nuclei (CMN) displacement in cases of intramedullary brain stem tumor, using neurophysiological mapping of motor nuclei on the floor of the fourth ventricle. METHODS: Relationships between tumors and CMN were reviewed in 18 patients with brain stem tumors (seven pontine, nine medullary, and two pontomedullary tumors) and 2 with cervicomedullary junction spinal cord tumors. CMN VII, IX/X, and XII were mapped by applying electrical stimuli over the surgically exposed fourth ventricular floor through a handheld probe and recording electrical activity in the appropriate cranial muscles. RESULTS: Tumors distorted the anatomic location of CMN in repetitive patterns according to tumor site. Three patterns were identified as follows: Type 1, CMN located around the tumor on the floor of fourth ventricle; Type 2, one or more CMN located ventrally to the tumor; Type 3, CMN in original anatomic position. Six of seven patients with pontine tumors showed the Type 1 pattern. Seven of nine patients with medullary tumors showed Type 2, and the other two showed Type 1. Both patients with pontomedullary tumors showed Type 2. One patient with a cervicomedullary junction spinal cord tumor showed Type 1 and the other Type 3, depending on the tumor extension into the fourth ventricle. CONCLUSION: Pontine tumors push the CMN to around the tumor edge, suggesting that precise localization of CMN before tumor resection is necessary to avoid their damage. Medullary tumors grow more exophytically and compress the CMN ventrally. Understanding patterns of CMN displacement can help surgeons establish the surgical plan, minimize risks, and enable safer surgery of brain stem tumors.

Adolescent↗
PubMed1996

Intrinsic tumors of the medulla: predicting outcome after surgery.

Twenty-four children and young adults who underwent surgery for intrinsic tumors of the medulla were studied, correlating preoperative symptoms to postoperative brain stem dysfunction. 75% harbored benign tumors with a mean survival of 3.6 years, while the remaining 25% with anaplastic tumors had a mean survival of 3.2 years. Six patients required postoperative ventilatory support for an extended period of time, and 9 required feeding gastrostomies. Those patients who experienced frequent symptoms of upper respiratory tract infection, preoperative pneumonia, or an alteration in their voice were at risk of postoperative ventilatory dependency. Those without such symptoms did not experience such a fate. Difficulty in swallowing food or liquids seems to predict the need for postoperative feeding gastrostomies, and a lack of such a history was correlated with no such need 100% of the time. There seem to be preoperative signs and symptoms which predict postoperative brain stem dysfunction for an extended period of time in those undergoing brain stem surgery for tumors intrinsic to the medulla.

Adolescent↗
PubMed1996

Disseminated ependymomas of the central nervous system.

Ependymomas are rare central nervous system (CNS) neoplasms that occasionally disseminate along the neuraxis or to extraneural sites. Definitive criteria predictive of dissemination have yet to be determined. One hundred forty patients with CNS ependymoma (88 primary spinal and 52 primary intracranial tumors) were surgically treated by the senior author (F.J.E.) between 1986 and 1994. Sixteen patients (11.4%) demonstrated tumor dissemination. The disseminated group consisted of 11 (12.5%) of 88 primary spinal and five (9.6%) of 52 primary intracranial ependymomas. The authors retrospectively reviewed the patients with CNS ependymoma and have identified several characteristics associated with dissemination from the primary tumor site. The mean time from diagnosis to dissemination was 6.8 years. The patients with disseminated disease were younger (16.8 vs. 28.3 years old, p = 0.02), had fewer gross-total resections (29% vs. 68%, p = 0.015), and had tumors with higher proliferative indices (MIB-1 staining, 13.14% vs. 2.06%, p = 0.02). High-grade tumors had a mean proliferation index of 21%, versus 2.4% and 1.6% for myxopapillary and low-grade tumors, respectively (p = 0.0003). In contrast to previous studies, tumor histology was the most significant variable for time to dissemination as determined by multivariate analysis (p = 0.008). Myxopapillary and high-grade tumors were 3.6 and 5.6 times more likely to have a shorter time to dissemination than low-grade tumors. In addition, dissemination is associated with a worse prognosis. At follow-up review, 31% of patients with disseminated disease had died compared to 7% of patients without dissemination (p = 0.04). It is concluded that younger patients with subtotal resections, myxopapillary or high-grade histology, and tumors with high proliferative indices are at substantial risk for the development of disseminated disease during their clinical course.

Adolescent↗
PubMed1996

Intramedullary spinal cord tumors in children under the age of 3 years.

Over a 13-year period extending from 1980 to 1993, 27 children less than 3 years of age underwent operation for removal of an intramedullary spinal cord tumor (IMSCT). The majority (18 of 27) of children had undergone surgery before being referred to New York University (NYU) Medical Center. The most common reasons for radiological investigation were pain (42%), motor regression (36%), gait abnormalities (27%), torticollis (27%), and progressive kyphoscoliosis (24%). Forty procedures were performed in 27 children. Nine children underwent two operations and two children underwent three procedures. A gross-total resection was achieved in 72% of the procedures. There was no surgical mortality. A comparison of the preoperative and 3-month postoperative functional grades for the first NYU procedure (NYU-1) yielded the following findings: 20 patients' conditions remained the same, five patients improved, and two patients deteriorated. The functional outcomes of a second operation (NYU-2) were similar. The majority of the children (24 of 27, 89%) had histologically determined low-grade lesions. There were 12 patients with low-grade astrocytomas (Grades I-III), eight with gangliogliomas, two with ganglioglioneurocytomas, one with a glioneurofibroma, and one child with a mixed astro/oligodendroglioma. Two children had anaplastic astrocytomas (Grades II-III) and one child had a glioblastoma multiforme. In a median follow-up review of 76 months, two patients died and two patients were lost to follow up. The 3- and 5-year progression-free survival (PFS) rates were 81.7% (standard error of the mean (SEM) 0.083) and 76.2% (SEM 0.094), respectively. Eight of 24 patients suffered a recurrence within a mean time of 45.4 +/- 28.9 months. All were treated with surgery (NYU-2). Lesions recurred in three of 12 children with low-grade astrocytomas, two of eight children with gangliogliomas, one child with an anaplastic astrocytoma, one child with a ganglioglioneurocytoma, and one child with a glioblastoma multiforme. At follow-up review, most of these children were doing well. Sixteen are in functional Grades I or II and 18 children attend a normal school system. The authors conclude that surgery for the removal of IMSCTs in children less than 3 years of age can be performed radically and safely. The postoperative functional performance is determined by the degree of the preoperative deficit. It is, therefore, of utmost importance to diagnose and treat these children as early as possible. Spinal cord tumors should be recognized as potentially excisable lesions on their initial presentation and when they recur. The optimum treatment for malignant lesions is still to be determined.

Child, Preschool↗
PubMed1995

Residual spasticity after selective posterior rhizotomy.

The technique used in performing selective posterior rhizotomies to treat spastic cerebral palsy remains controversial. One hundred nine children who had undergone selective posterior rhizotomies were studied 6 months after their surgery. Their residual spasticity was correlated to the number of roots and whether or not abnormally responding roots were left, in order to validate the surgical technique used to treat spastic cerebral palsy at most neurosurgical centers in North America. The children were divided into three groups (group A: children who had their L2-S1 roots tested and selectively lesioned, n = 15; group B: children who had their L2-S2 roots tested and selectively lesioned, n = 62; group C: children who had their L2-S2 roots tested and whose lesioning was directed both by the response to the stimulation and mapping of the S1-S3 dorsal roots for afferent pudendal nerve activity, n = 32). Clinically significant residual spasticity was present in the gastrocnemius in 33% of the group A children, 11% of the group B children, and 6% of the group C children. We found that there was no significant increase in residual spasticity in the group C children when abnormally responding roots were not cut in order to preserve pudendal nerve activity. This study shows that the inclusion of the S2 roots decreases the amount of residual spasticity (P < 0.01). It also shows that leaving abnormally responding S2 roots to preserve pudendal nerve activity does not affect the incidence of postoperative spasticity (P > 0.1).

Adolescent↗
PubMed1995

Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis--results and unsolved problems.

Early surgery for isolated craniosynostosis is designed to improve morphology, to prevent functional disturbances, and equally important, to enhance the psychosocial development of the child. As the first of a two-part series, 104 patients with isolated craniofacial synostosis were retrospectively analyzed. Diagnoses included bilateral coronal (10), unilateral coronal (57), metopic (29), and sagittal synostosis (8). All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 8.1 months). Thirteen percent of patients (14) required a secondary cranial vault operation (mean age 22.6 months) to address residual deficits in craniofacial form. Perioperative complications were minimal (5.0 percent), and there was no mortality. Average length of postoperative follow-up was 46.0 months. By the classification of Whitaker et al., which assesses surgical results, 87.5 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. Overall rates of hydrocephalus, shunt placement, and seizures (3.8, 1.0, and 2.9 percent, respectively) were low. Among the isolated craniosynostoses, unilateral coronal synostosis/plagiocephaly poses the most complex problems, including vertical orbital dystopia, nasal tip deviation, and residual craniofacial asymmetry; there is also a wide spectrum of findings and growth patterns in this subgroup.

Craniofacial Dysostosis↗
PubMed1995

Spinal cord ependymomas: MR imaging features.

PURPOSE: To assess the magnetic resonance (MR) imaging characteristics of spinal intramedullary ependymomas. MATERIALS AND METHODS: MR images obtained in 25 patients (aged 12-73 years) with proved intramedullary ependymomas were retrospectively reviewed. T1- and T2-weighted images were obtained in all patients. Gadopentetate dimeglumine was intravenously administered in 23 patients; enhanced sagittal and axial T1-weighted spin-echo images were reviewed. RESULTS: All tumors had hyperintense signal on T2-weighted images. In the 23 patients who received contrast material, all tumors became enhanced; enhancement was heterogeneous in 15 patients and homogeneous in eight patients. Twenty tumors had sharply defined, enhanced borders. Nineteen tumors were centrally located in the spinal cord. A hypointense rim on T2-weighted images was noted in five patients. CONCLUSION: Intramedullary ependymomas become enhanced after administration of gadopentetate dimeglumine; the enhanced borders are usually sharply marginated. They are characteristically located centrally in an expanded spinal cord. Hemosiderin is often present at the periphery of cervical ependymomas.

Adolescent↗
PubMed1995

Prevention of recurrent tethered spinal cord.

One of the most problematic technical considerations in surgery for the release of tethered spinal cord is how to prevent recurrent tethering. Recurrent tethering is common because the spinal canal in the baby is shallow and, therefore, postoperatively, the neural contents are in direct contact with the posterior dura. The only way to prevent a recurrent tethered cord is to be certain that the neural elements remain free within circumferentially patent cerebrospinal fluid. We hereby describe a method where a curved 1.5 mm oval piece of Medpor is used to create a posterior space for the neural elements. The spinal canal is expanded posteriorly, therefore, creating an abnormally wide canal to accommodate the neural elements within subarachnoid space. This methodology was used in 18 neonate patients, and in late tethering cases after myelomeningocele. Technical and theoretical considerations are discussed.

Child, Preschool↗
PubMed1995

The editors' labours: separating the wheat from the chaff.

From the author's standpoint, the editor is viewed as 'one who separates the wheat from the chaff and then prints the chaff'! Since its inception, Pediatric Neurosurgery has tried to provide the reader with the guarantee that only the 'wheat' is published. This paper will examine the policies of the journal's Editorial Board and in particular outline the review process for submitted manuscripts. In addition to certain operational items, the paper will consider how the 'peer' in 'peer review' is identified, the grading instruments for paper acceptance, the likelihood that the readers and peer reviewers agree on manuscript quality, and how authors should view failure. The Society is firmly committed to our journal. Our individual responsibilities begin with paper preparation and then its presentation at the annual meeting. Thereafter, it is important for the membership to appreciate that their participation, as either author and/or reviewer, is critical for the continued success of our journal.

Humans↗