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Chandranath Sen and Karin Hague

✓ Despite advances in the surgical treatment of meningiomas located at the skull base, surgery for meningiomas involving the cavernous sinus remains controversial. The controversy centers on whether complete resection of such a meningioma is possible while preserving cranial nerve function. To evaluate this question, the authors examined six patients with benign meningiomas involving the cavernous sinus. The pathological features of these tumors were evaluated and compared with the normal histoarchitecture of the cavernous sinus. The tendency of these tumors to be infiltrative is evident and this seems to occur along connective tissue planes within the cavernous sinus. This invasiveness can be explained by the peculiar structure of this region. The trigeminal nerve and ganglion seem to be particularly prone to invasion; this does not correlate with the degree of preoperative impairment of nerve function. Internal carotid artery invasion occurs frequently and can be seen even when there is no narrowing of the artery on arteriography. The pituitary gland can also be invaded by the tumor, which penetrates the thin dural barrier.

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Chandranath Sen and Aymara Triana

Object

The authors analyze their experience with the treatment of 29 patients who underwent radical excision of skull base chordomas.

Methods

Modern skull base surgical techniques were used in all patients who were treated between August 1991 and July 2000. The degree of tumor resection was gauged according to intraoperative inspection and postoperative high-resolution imaging findings. There were 21 patients with primary disease and eight with recurrent disease. Total resection was accomplished in 18 patients. Five patients had undergone radiotherapy prior to the present surgery, and an additional eight patients underwent postoperative radiotherapy. There were no surgery-related deaths. In five patients who died of the disease, surgery and radiotherapy had failed to effect a cure. Two of the remaining patients are alive with recurrent disease, and there is questionable evidence of recurrence in another patient. All 24 patients are functioning independently. Cranial nerve impairment was the most common postoperative deficit, followed by cerebrospinal fluid (CSF) leakage and infection.

Conclusions

The use of skull base techiniques in radical surgery provides an opportunity to excise the tumor and the involved bone. In most cases the procedure-related cranial nerve deficits improve over time. The complications of CSF leakage and infection can be minimized and are preventable. Proton beam irradiation is an excellent adjuvant treatment but is reserved for patients with definite tumor recurrence or residual tumor that can be identified on the imaging studies.

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Michael H. Brisman, Chandranath Sen and Peter Catalano

✓ To evaluate the results of surgery in patients with head and neck cancers that involved the internal carotid artery at the skull base the authors retrospectively reviewed a consecutive series of 17 patients who underwent surgery at Mount Sinai Hospital over a 4-year period. In general, patients who underwent tumor resection with carotid preservation had less advanced disease (two of seven tumors were recurrences) than patients who underwent tumor resection with carotid sacrifice (seven of 10 tumors were recurrences).

Of seven patients who underwent resection with carotid preservation, six had good outcomes (five patients alive in good condition, one dead at 2.2 years) and none had strokes. Of seven patients who underwent resection with carotid sacrifice and bypass, five had good outcomes (four alive in good condition, one dead at 2.5 years with no local recurrence) and two suffered graft occlusions that led to strokes, one of which was major and permanently disabling. Of three patients who underwent resection with carotid sacrifice and ligation without revascularization, there were no good outcomes: all three patients died within 6 months of surgery, two having suffered major permanently disabling strokes. The overall results (11 [65%] of 17 with good outcomes at an average follow-up period of 2.1 years) compared very favorably with historical nonsurgical controls.

The authors conclude that tumor resection with carotid perservation carries the lowest risk of stroke and should usually be the treatment of choice. For patients with more advanced and recurrent disease, in whom it is believed that carotid preservation would prevent a safe and oncologically meaningful resection, carotid sacrifice with carotid bypass may be a useful treatment option. Carotid sacrifice without revascularization seems to be the treatment option with the least favorable results.

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Adesh Tandon, Sid Chandela, David Langer and Chandranath Sen

Cervical radiculopathy secondary to compression from congenital anomalous vertebral arteries (VAs) is a known entity. Patients present with a variety of symptoms ranging from upper-extremity numbness to true occipital neuralgia. Treatment options for extracranial tortuous VAs include conservative management or some form of surgical microvascular decompression (MVD). The authors report on a patient with a congenital anomalous VA loop causing cervical nerve root compression. Successful MVD was conducted with relief of the patient's symptoms. A novel sling technique was used for mobilization of the VA. To the authors' knowledge, this is the first MVD described utilizing this technique.

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Hyunchul Shin, Ignacio J. Barrenechea, Jonathan Lesser, Chandranath Sen and Noel I. Perin

Object

Surgical access to tumors at the craniovertebral junction (CVJ) requires extensive bone removal. Guidelines for the use of occipitocervical fusion (OCF) after resection of CVJ tumors have been based on anecdotal evidence. The authors performed a retrospective study of factors associated with the use of OCF in 46 patients with CVJ tumors. The findings were used to develop recommendations for use of OCF in such patients.

Methods

The authors retrospectively reviewed the cases of 51 patients with CVJ tumors treated by their group between March 1991 and February 2004. Forty-six patients were available for follow up. Charts were reviewed to obtain data on demographic characteristics, presenting symptoms, and perioperative complications. Preoperative computerized tomography scans and magnetic resonance imaging studies were obtained in all patients. Occipitocervical fusion was performed in patients who had undergone a unilateral condyle resection in which 70% or more of the condyle was removed, a bilateral condyle resection with 50% removal, or C1–2 vertebral body destruction. Of the 46 patients, 16 had foramen magnum meningiomas, 17 had chordomas, one had a chondrosarcoma, two had Schwann cell tumors, two had glomus tumors, and eight had other types of tumors. Twenty-three (50%) of the 46 patients underwent OCF, including 15 of the 17 patients with chordomas (88%). None of the patients with meningiomas required fusion. Seventeen (71%) of the 24 patients presenting with neck pain preoperatively underwent OCF.

Conclusions

Patients presenting with neck pain had a 71% chance of undergoing OCF. Patients with chordomas and metastatic tumors were most likely to require OCF. One patient with a 50% unilateral condylar resection returned with OC instability for which OCF was required. Based on their clinical experience and published biomechanical studies, the authors recommend that OCF be performed when 50% or more of one condyle is resected.

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Raj K. Shrivastava, Chandranath Sen, Peter D. Costantino and Robert Della Rocca

Object

Sphenoorbital meningiomas (SOMs) are complex tumors involving the sphenoid wing, orbit, and cavernous sinus, which makes their complete resection difficult or impossible. Sphenoidal hyperostosis that results in incomplete resection makes these tumors prone to high rates of recurrence with postoperative morbidity resulting in a nonfunctional globe. High-dose radiation therapy has often been described as the only treatment capable of achieving tumor control, although often at the expense of the patient's progressive visual deterioration.

Methods

This series consisted of 25 patients who were retrospectively analyzed over a 12-year period. Visual function was evaluated pre- and postoperatively in all patients. A standardized surgical approach to a frontotemporal craniotomy and orbitozygomatic osteotomy with intra- and extradural drilling of the optic canal and all the hyperostotic bone was performed. Orbital and cranial reconstruction was performed in all patients. The follow-up period was 6 months to 12 years (average 5 years).

The patients presented with the classic triad of SOM: proptosis (86%), visual impairment (78%), and ocular paresis (20%). A gross-total resection was achieved in 70% of patients with surgery limited by the superior orbital fissure and the cavernous sinus. Proptosis improved in 96% of patients with 87% improvement in visual function. Ocular paresis improved in 68%, although 20% of patients experienced a temporary ocular paresis postoperatively. There were no perioperative deaths or morbidity related to the surgical approach or reconstruction. Ninety-five percent of patients reported an improved functional orbit. There was tumor recurrence in 8% of patients; in one case recurrence was delayed for longer than 11 years.

Conclusions

Sphenoorbital meningiomas are a distinct category of tumors complicated by potentially extensive hyperostosis of the skull base. Successful resection requires extensive intra- and extradural surgery, necessitating drilling of the optic canal and an orbital osteotomy within anatomical limitations. The bone resection requires reconstruction with autograft, allografts, or alloplast for improved orbital function. All aspects of the clinical triad improved. A radical resection can be achieved with low morbidity, providing a significantly improved clinical outcome in the long-term period.

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Chandranath Sen, Aymara I. Triana, Niklas Berglind, James Godbold and Raj K. Shrivastava

Object

Chordomas are rare malignant neoplasms arising predominantly at the sacrum and skull base. They are uniformly lethal unless treated with aggressive resection and proton beam irradiation. The authors present results of the surgical management of a large number of patients with clivus chordomas. Factors that influence the surgeon's ability to achieve radical tumor resection are also evaluated.

Methods

Between 1991 and 2005, 71 patients with clivus chordomas underwent surgery. The average follow-up was 66 months (median 60 months, range 3–189 months). Sixty-five patients had complete records that were analyzed in the present report. Thirty-five percent of them had undergone surgery before being treated by the authors. They were evaluated with MR imaging and CT scanning and underwent surgery utilizing a variety of skull base techniques aimed at achieving radical excision. Many also underwent postoperative radiation, usually in the form of proton beam therapy. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation.

Results

Radical tumor resection was achieved in 58% of the group. The overall 5-year survival rate was 75%. Radical resection had a positive impact on survival. The ability to achieve radical resection was dependent on the preoperative tumor volume and the number of anatomical areas involved by the tumor. Cranial nerve impairment and CSF leakage were the most frequent postoperative complications.

Conclusions

Radical excision is the ideal surgical goal in the treatment of clival chordomas and can be achieved with reasonable risks. Several different surgical approaches may be necessary to accomplish this.

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Sid Chandela, Juan Alzate, Chandranath Sen, Joon Song, Yasunari Nimi, Alejandro Berenstein and David Langer

✓Endovascular and cerebral bypass therapies are rarely used in children. The authors describe the treatment of a partially coiled giant distal vertebral artery (VA)–posterior inferior cerebellar artery (PICA) aneurysm in a child. They performed a side-to-side PICA–PICA anastomosis followed by endovascular VA aneurysm deconstruction with PICA preservation.

A healthy 11-year-old boy developed progressive holocephalic headaches over the course of 2 months. Magnetic resonance imaging and magnetic resonance angiography revealed a large right PICA aneurysm causing brainstem compression. In November 2005, 2 Neuroform stents and Guglielmi detachable coils and Matrix were placed in the aneurysm at an outside institution. In 2006, angiography demonstrated aneurysm enlargement from which the PICA originated, coil compaction, and increased mass effect. The patient underwent a PICA–PICA bypass with intraoperative flow measurements followed by endovascular embolization of the aneurysm and parent VA. An angiogram obtained after the procedure demonstrated filling of the right PICA medullary branch through the bypass and obliteration of the aneurysm. The patient remained neurologically intact.

Giant aneurysms of the posterior circulation are rare but do occur in children. With the aid of combined surgical and endovascular strategies the authors were able to safely eliminate the aneurysm from circulation with good outcome. Cerebral bypass and endovascular deconstructive therapies can be used safely in children but should be reserved for cases in which direct treatment carries significant risk. Careful surgical and endovascular planning with intraoperative flow assessment is essential for good outcome.

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Ignacio J. Barrenechea, Noel I. Perin, Aymara Triana, Jonathan Lesser, Peter Costantino and Chandranath Sen

Object

Chordomas of the cervical spine are rare tumors. Although en bloc resection has proven to be the ideal procedure in other areas, there is controversy regarding this approach in the cervical spine. The goal in this study was to determine whether piecemeal tumor resection was efficient in the management of chordomas that arise in this location.

Methods

The authors retrospectively reviewed all 74 cases of chordoma treated by their group. Seven patients with isolated cervical chordomas who were treated between October 1992 and January 2006 were identified. There were four male and three female patients, whose ages ranged from 6 to 61 years (mean 34.4 years). Follow-up duration ranged from 7 to 169 months (median 23 months). All cases were managed using a retrocarotid approach with mobilization of the vertebral artery. When the tumor could not be completely resected via the initial anterior approach, a subsequent posterior resection was performed. Tumor resection was intralesional in all cases, and gross-total tumor resection was achieved in six cases. One patient required a second resection 4 months later. In all cases, a posterior stabilization procedure was performed. Five patients underwent anterior fusion (three with fibular allograft and two with iliac crest), whereas two underwent occipitocervical fusion. In two patients with dedifferentiated chordoma metastasis developed, and one of them died 7 months later. The other patient with metastasis died suddenly at home 26 months postsurgery, presumably from aspiration. At the time of this submission, there were no signs of recurrence in five patients.

Conclusions

The authors believe that, in most cases, en bloc resection of cervical chordoma is not feasible. This is due to the tendency of chordomas to involve multiple compartments at the time of diagnosis. In the authors' experience, intralesional radical resection remains an effective surgical approach to this disease entity.

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Raj K. Shrivastava, Salomao Segal, Martin B. Camins, Chandranath Sen and Kalmon D. Post

✓ The search for the origin of the commonly held principle in current neurosurgery regarding the resectability of the anterior one third of the superior sagittal sinus unravels the many fascinating developments that occurred in neurosurgery during the early 20th century. All these occurrences can be traced back to, and are uniquely contextualized in, Harvey Cushing's seminal text, Meningiomas, Their Classification, Regional Behaviour, Life History, and Surgical End Results. Written with Louise Eisenhardt and published in 1938, Meningiomas is a monograph of incredible description and detail. The meticulous categorization of meningiomas, their presentation, clinical outcome, and surgical therapies are even further supplemented by Cushing's personal commentary, questions, and recollections. Cushing's genius was evident in his ability not only to make insightful clinical observations, but also to synthesize these ideas within the neurosurgical context of his era. As he says in Meningiomas, “Thus the pathological curiosity of one day becomes in its proper time a commonplace … most of which are one and the same disorder—had, for their interpretation, to await the advent of the Neurosurgeon.”