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Daryl R. Fourney and Ziya L. Gokaslan

Sacral chordomas are relatively rare, locally invasive, malignant neoplasms. Although metastasis is infrequent at presentation, the prognosis for patients with chordoma of the sacrum is reported to be poor and attributable in most cases to intralesional resection. The value of adjuvant treatment is uncertain, and resection remains the primary mode of treatment. Chordomas are difficult to excise completely, but recent improvements in imaging and surgical techniques have allowed surgeons to perform more frequently en bloc sacral resections with wide surgical margins. The technical challenges of such operations, and the functional costs for the patient (with respect to anorectal and urogenital dysfunction) are significantly increased when the tumor involves high sacral levels. The authors review the clinical presentation and natural history of sacral chordoma and discuss the current treatment techniques and outcomes.

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Daryl R. Fourney and Ziya L. Gokaslan

In addition to tumor resection, a major goal of spine surgery involving tumors is the preservation or achievement of spinal stability. The criteria defining stability, originally developed for use in trauma, are not directly applicable in the setting of neoplasia. The authors discuss the most common patterns of tumor-related instability and deformity at all levels of the spinal column and review the surgical options for treatment.

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Use of “MAPS” for determining the optimal surgical approach to metastatic disease of the thoracolumbar spine: anterior, posterior, or combined

Invited submission from the Joint Section Meeting on Disorders of the Spine and Peripheral Nerves, March 2004

Daryl R. Fourney and Ziya L. Gokaslan

✓ The surgical treatment of thoracolumbar metastases is controversial, and various approaches have been described. No single approach, however, is always applicable, and the optimal surgical strategy for any individual is determined by several interrelated factors. The authors have grouped these factors into four preoperative planning considerations that form the mnemonic “MAPS”: 1) method of resection; 2) anatomy of spinal disease; 3) patient's level of fitness; and 4) stabilization. The choice of approach is also considered in light of the goals of surgery, including the relief of pain, neurological palliation, spinal stabilization, and oncological control.

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Daryl R. Fourney, Gregory N. Fuller and Ziya L. Gokaslan

✓ Extradural ependymomas of the sacrococcygeal region are very rare, with most arising from the soft tissues of the presacral area or from the regions dorsal to the sacrum. In even rarer circumstances, the tumor may arise within the sacral canal, likely as a result of ependymal cells of the extradural filum terminale. Because of bone erosion caused by extension of the tumor into the pelvis or dorsal to the sacrum, a truly intraspinal extradural ependymoma in this region has until now never been clearly demonstrated. The authors present a patient with a myxopapillary ependymoma arising from the filum terminale externa in which there was no involvement of the intradural filum or extension outside the sacral canal. A review of the literature is presented, with emphasis on the pathogenesis and clinical management of these rare tumors.

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Daryl R. Fourney, Dima Abi-Said, Frederick F. Lang, Ian E. McCutcheon and Ziya L. Gokaslan

Object. Few reports are available on the use of pedicle screw fixation for cancer-related spinal instability. The authors present their experience with pedicle screw fixation in the management of malignant spinal column tumors.

Methods. Records for patients with malignant spinal tumors who underwent pedicle screw fixation at the authors' institution between September 1994 and December 1999 were retrospectively reviewed.

Results. Ninety-five patients with malignant spinal tumors underwent 100 surgeries involving pedicle screw fixation: metastatic spinal disease was present in 81 patients, and locally invasive tumors were demonstrated in 14 patients. Indications for surgery were pain (98%) and/or neurological dysfunction (80%). A posterior (48%) or a combined anterior—posterior (52%) approach was performed depending on the extent of tumor and the patient's condition. At the mean follow up of 8.2 months, 43 patients (45%) had died; median survival, as determined by Kaplan—Meier analysis, was 14.8 months. At 1 month postsurgery, self-reported pain had improved in 87% of cases (p < 0.001), which is a finding substantiated by reductions in analgesic use, and 29 (47%) of 62 patients with preoperative neurological impairments were functionally improved (p < 0.001). Postoperative complications were associated only with preoperative radiation therapy (p = 0.002) and with preexisting serious medical conditions (p = 0.04). In two patients asymptomatic violation of the lateral wall of the pedicle was revealed on postoperative radiography. The 30-day mortality rate was 1%.

Conclusions. For selected patients with malignant spinal tumors, pedicle screw fixation after tumor resection may provide considerable pain relief and restore or preserve ambulation with acceptable rates of morbidity and mortality.

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Lissa Peeling, Evan Frangou, Stephen Hentschel, Ziya L. Gokaslan and Daryl R. Fourney

The treatment of complex thoracolumbar disorders occasionally requires combined anterior and posterior approaches. Traditionally, these are either sequentially staged to occur during the same anesthesia procedure or alternatively performed on separate days. A less common option is the simultaneous anterior-posterior approach. The authors discuss the rationale for this approach in selected cases and illustrate a number of modifications to previous descriptions of the procedure. By slightly altering the incision, the risk of wound breakdown and infection has been reduced. The use of newly available positioning devices has allowed easy incorporation of fluoroscopy to guide the placement of spinal instrumentation. The authors have also expanded the use of the approach beyond the original oncological indications to include trauma and infection.

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Total cervical spondylectomy for primary osteogenic sarcoma

Case report and description of operative technique

Zvi R. Cohen, Daryl R. Fourney, Rex A. Marco, Laurence D. Rhines and Ziya L. Gokaslan

✓ The authors describe a technique for total spondylectomy for lesions involving the cervical spine. The method involves separately staged anterior and posterior approaches and befits the unique anatomy of the cervical spine. The procedure is described in detail, with the aid of radiographs, intraoperative photographs, and illustrations. Unlike in the thoracic and lumbar spine—for which methods of total en bloc spondylectomy have previously been described—a strictly en bloc resection is not possible in the cervical spine because of the need to preserve the vertebral arteries and the nerve roots supplying the upper limbs. Although the resection described in this case is by definition intralesional, it is oncologically sound, given the development of effective neoadjuvent chemotherapeutic regimens for osteosarcoma.

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En bloc resection of multilevel cervical chordoma with C-2 involvement

Case report and description of operative technique

Laurence D. Rhines, Daryl R. Fourney, Abdolreza Siadati, Ian Suk and Ziya L. Gokaslan

✓ Chordomas are locally aggressive neoplasms with an extremely high propensity to recur locally following resection, despite adjuvant therapy. This biological behavior has led most authors to conclude that en bloc resection provides the best chance for the patient's prolonged disease-free survival and possible cure.

The authors present a case of an extensive upper cervical chordoma treated by en bloc resection, reconstruction, and long-segment stabilization. Total spondylectomy of C2–4 with sacrifice of the right C2–4 nerve roots and a segment of the right vertebral artery was performed. The inherent anatomical complexities of en bloc resection in the upper cervical spine are discussed. To the authors' knowledge, this represents the first report of an en bloc resection for multilevel cervical chordoma.

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Giant cell ependymoma of the spinal cord

Case report and review of the literature

Daryl R. Fourney, Abdolreza Siadati, Janet M. Bruner, Ziya L. Gokaslan and Laurence D. Rhines

✓ Several rare histological variants of ependymoma have been described. The authors report on a patient in whom cervical spinal cord astrocytoma was originally diagnosed after evaluation of a limited biopsy specimen. More abundant tissue obtained during gross-total resection included areas of well-differentiated ependymoma. The histological features of the tumor were extremely unusual, with a major component of pleomorphic giant cells. Its histological, immunohistochemical, and electron microscopic features, however, were consistent with ependymoma. Only two cases of terminal filum and two of supratentorial giant cell variant of ependymoma have been reported. To the authors' knowledge, this represents the first case of giant cell ependymoma of the spinal cord. The clinical significance is the potential for misdiagnosis with anaplastic (gemistocytic) astrocytoma, especially in cases in whom limited biopsy samples have been obtained.

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Daryl R. Fourney, Julie E. York, Zvi R. Cohen, Dima Suki, Laurence D. Rhines and Ziya L. Gokaslan

Object. The treatment of atlantoaxial spinal metastases is complicated by the region's unique biomechanical and anatomical characteristics. Patients most frequently present with pain secondary to instability; neurological deficits are rare. Recently, some authors have performed anterior approaches (transoral or extraoral) for resection of upper cervical metastases. The authors review their experience with a surgical strategy that emphasizes posterior stabilization of the spine and avoidance of poorly tolerated external orthoses such as the rigid cervical collar or halo vest.

Methods. The authors performed a retrospective review of 19 consecutively treated patients with C-1 or C-2 metastases who underwent surgery at The University of Texas M. D. Anderson Cancer Center between 1994 and 2001.

Visual analog pain scores were reduced at 1 and 3 months (p < 0.005, Wilcoxon signed-rank test); however, evaluation of pain at 6 months and 1 year was limited by the remaining number of surviving patients. Analgesic medication consumption was unchanged. There were no cases of neurological decline or sudden death secondary to residual or recurrent atlantoaxial disease during the follow-up period. One patient underwent revision of hardware at 11 months. The mean follow-up period was 8 months (range 1–32 months). Median survival determined by Kaplan—Meier analysis was 6.1 months (95% confidence interval 2.99–9.21).

Conclusions. Occipitocervical stabilization provided durable pain relief and preservation of ambulatory status over the remaining life span of patients. Because of the palliative goals of surgery, the authors have not found an indication for anterior-approach tumor resection in these patients. Successful stabilization obviates the need for an external orthosis.