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Sacit Bulent Omay, João Paulo Almeida, Yu-Ning Chen, Sathwik R. Shetty, Buqing Liang, Shilei Ni, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Craniopharyngiomas arise from the pituitary stalk, and in adults they are generally located posterior to the chiasm extending up into the third ventricle. The extended endonasal approach (EEA) can provide an ideal corridor between the bottom of the optic chiasm and the top of the pituitary gland (chiasm-pituitary corridor [CPC]) for their removal. A narrow CPC in patients with a prefixed chiasm and a large tumor extending up and behind the chiasm has been considered a contraindication to EEA, with a high risk of visual deterioration and subtotal resection.

METHODS

A database of all patients treated in the authors’ center (Weill Cornell Medical College, NewYork-Presbyterian Hospital) between July 2004 and August 2016 was reviewed. Patients with craniopharyngiomas who underwent EEA with the goal of gross-total resection (GTR) were included in the study. Patients with postfixed chiasm or limited available preoperative imaging were excluded. Using preoperative contrast-enhanced T1-weighted sagittal midline MR images, the authors calculated the CPC as well as the distance from the chiasm to the top of the tumor (CTOT). From these numbers, they calculated a ratio of the CPC to the CTOT as a measure of difficulty in removing the tumors through the EEA and called this ratio the corridor index (CI). The relationship between the CI and the ability to achieve GTR and visual outcome were measured.

RESULTS

Thirty-four patients were included in the study. The mean CPC was 10.1 mm (range 5.2–19.1 mm). The mean CTOT was 12.8 mm (range 0–28.3 mm). The median CI was 0.8; the CI ranged from 0.4 to infinity (for tumors with a CTOT of 0). Thirty-two patients had GTR (94.1%) and 2 had subtotal resection. The CPC value had no relationship with our ability to achieve GTR and no effect on visual or endocrine outcome.

CONCLUSIONS

EEA for craniopharyngioma is generally considered the first-line surgical approach. Although a narrow corridor between the top of the pituitary gland and the bottom of the chiasm may seem to be a relative contraindication to surgery for larger tumors, the authors’ data do not bear this out. EEA appears to be a successful technique for the majority of midline craniopharyngiomas.

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João Paulo Almeida, Armando S. Ruiz-Treviño, Buqing Liang, Sacit B. Omay, Sathwik R. Shetty, Yu-Ning Chen, Vijay K. Anand, Kartikey Grover, Paul Christos, and Theodore H. Schwartz

OBJECTIVE

Surgery is generally the first-line therapy for acromegaly. For patients with residual or recurrent tumors, several treatment options exist, including repeat surgery, medical therapy, and radiation. Reoperation for recurrent acromegaly has been associated with poor results, with hormonal control usually achieved in fewer than 50% of cases. Extended endonasal endoscopic approaches (EEAs) may potentially improve the results of reoperation for acromegaly by providing increased visibility and maneuverability in parasellar areas.

METHODS

A database of all patients treated in the authors’ center between July 2004 and February 2016 was reviewed. Cases involving patients with acromegaly secondary to growth hormone (GH)–secreting adenomas who underwent EEA were selected for chart review and divided into 2 groups: first-time surgery and reoperation. Disease control was defined by 2010 guidelines. Clinical and radiological characteristics and outcome data were extracted. A systematic review was done through a MEDLINE database search (2000–2016) to identify studies on the surgical treatment of acromegaly. Using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, the included studies were reviewed for surgical approach, tumor size, cavernous sinus invasion, disease control, and complications. Cases were divided into reoperation or first-time surgery for comparative analysis.

RESULTS

A total of 44 patients from the authors’ institution were included in this study. Of these patients, 2 underwent both first-time surgery and reoperation during the study period and were therefore included in both groups. Thus data from 46 surgical cases were analyzed (35 first-time operations and 11 reoperations). The mean length of follow-up was 70 months (range 6–150 months). The mean size of the reoperated tumors was 14.8 ± 10.0 mm (5 micro- and 6 macroadenomas). The patients’ mean age at the time of surgery was younger in the reoperation group than in the first-time surgery group (34.3 ± 12.8 years vs 49.1 ± 15.7 years, p = 0.007) and the mean preoperative GH level was also lower (7.7 ± 13.1 μg/L vs 25.6 ± 36.8 μg/L, p = 0.04). There was no statistically significant difference in disease control rates between the reoperation (7 [63.6%] of 11) and first-time surgery (25 [71.4%] of 33) groups (p = 0.71). Univariate analysis showed that older age, smaller tumor size, lower preoperative GH level, lower preoperative IGF-I level, and absence of cavernous sinus invasion were associated with higher chances of disease control in the first-time surgery group, whereas only absence of cavernous sinus invasion was associated with disease control in the reoperation group (p = 0.01). There was 1 case (9%) of transient diabetes insipidus and hypogonadism and 1 (9%) postoperative nasal infection after reoperation. The systematic review retrieved 29 papers with 161 reoperation and 2189 first-time surgery cases. Overall disease control for reoperation was 46.8% (95% CI 20%–74%) versus 56.4% (95% CI 49%–63%) for first-time operation. Reoperation and first-time surgery had similar control rates for microadenomas (73.6% [95% CI 32%–98%] vs 77.6% [95% CI 68%–85%]); however, reoperation was associated with substantially lower control rates for macroadenomas (27.5% [95% CI 5%–57%] vs 54.3% [95% CI 45%–62%]) and tumors invading the cavernous sinus (14.7% [95% CI 4%–29%] vs 38.5% [95% CI 27%–50%]).

CONCLUSIONS

Reoperative EEA for acromegaly had results similar to those for first-time surgery and rates of control for macroadenomas that were better than historical rates. Cavernous sinus invasion continues to be a negative prognostic indicator for disease control; however, results with EEA show improvement compared with results reported in the prior literature.

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João Paulo Almeida, Sacit B. Omay, Sathwik R. Shetty, Yu-Ning Chen, Armando S. Ruiz-Treviño, Buqing Liang, Vijay K. Anand, Benjamin Levine, and Theodore H. Schwartz

Sphenoorbital meningiomas (SOMs) are slow-growing tumors that originate from the sphenoidal wing and are associated with visual deterioration, extrinsic ocular movement disorders, and proptosis caused by hyperostosis of the lateral wall of the orbit. In some cases, the intracranial component is quite small or “en plaque,” and the majority of the symptoms arise from adjacent hyperostosis. Craniotomy has traditionally been the standard of care, but new minimally invasive multiportal endoscopic approaches offer an alternative. In the current study, the authors to present their experience with the transorbital endoscopic eyelid approach for the treatment of 2 patients with SOMs and sphenoid wing hyperostosis.

Clinical and radiological data for patients with SOMs who underwent a transorbital endoscopic eyelid approach were retrospectively reviewed. Surgical technique and clinical and radiographic outcomes were analyzed.

The authors report the cases of 2 patients with SOMs and proptosis due to sphenoid wing hyperostosis. One patient underwent prior craniotomy to debulk the intracranial portion of the tumor, and the other had a minimal intracranial component. Both patients were discharged 2 days after surgery. MR images and CT scans demonstrated a large debulking of the hyperostotic bone. Postoperative measurement of the proptosis with the aid of an exophthalmometer demonstrated significant reduction of the proptosis in one of the cases. Persistence of intraconal tumor in the orbital apex limited the efficacy of the procedure in the other case. A review of the literature revealed 1 publication with 3 reports of the transorbital eyelid approach for SOMs. No measure of relief of proptosis after this surgery had been previously reported.

The transorbital endoscopic approach, combined with endonasal decompression of the medial orbit, may be a useful minimally invasive alternative to craniotomy in a subset of SOMs with a predominantly hyperostotic orbital wall and minimal intracranial bulky or merely en plaque disease. In these cases, relief of proptosis and optic nerve compression are the primary goals of surgery, rather than gross-total resection, which may have high morbidity or be unachievable. In cases with significant residual intraconal tumor, orbital bone removal alone may not be sufficient to reduce proptosis.

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Hazem M. Negm, Rafid Al-Mahfoudh, Manish Pai, Harminder Singh, Salomon Cohen, Sivashanmugam Dhandapani, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Regrowth of the lesion after surgical removal of pituitary adenomas is uncommon unless subtotal resection was originally achieved in the first surgery. Treatment for recurrent tumor can involve surgery or radiotherapy. Locations of residual tumor may vary based on the original approach. The authors evaluated the specific sites of residual or recurrent tumor after different transsphenoidal approaches and describe the surgical outcome of endoscopic endonasal transsphenoidal reoperation.

METHODS

The authors analyzed a prospectively collected database of a consecutive series of patients who had undergone endoscopic endonasal surgeries for residual or recurrent pituitary adenomas after an original transsphenoidal microscopic or endoscopic surgery. The site of the recurrent tumor and outcome after reoperation were noted and correlated with the primary surgical approach. The chi-square or Fisher exact test was used to compare categorical variables, and the Mann-Whitney U-test was used to compare continuous variables between surgical groups.

RESULTS

Forty-one patients underwent surgery for residual/recurrent pituitary adenoma from 2004 to 2015 at Weill Cornell Medical College. The previous treatment was a transsphenoidal microscopic (n = 22) and endoscopic endonasal (n = 19) surgery. In 83.3% patients (n = 30/36) there was postoperative residual tumor after the initial surgery. A residual tumor following endonasal endoscopic surgery was less common in the sphenoid sinus (10.5%; 2/19) than it was after microscopic transsphenoidal surgery (72.7%; n =16/22; p = 0.004). Gross-total resection (GTR) was achieved in 58.5%, and either GTR or near-total resection was achieved in 92.7%. Across all cases, the average extent of resection was 93.7%. The rate of GTR was lower in patients with Knosp-Steiner Grade 3–4 invasion (p < 0.0005). Postoperative CSF leak was seen in only one case (2.4%), which stopped with lumbar drainage. Visual fields improved in 52.9% (n = 9/17) of patients and were stable in 47% (n = 8/17). Endocrine remission was achieved in 77.8% (n = 14/18) of cases, 12 by surgery alone and 2 by adjuvant medical (n = 1) and radiation (n = 1) therapy. New diabetes insipidus occurred in 4.9% (n = 2/41) of patients—in one of whom an additional single anterior hormonal axis was compromised—and 9.7% (n = 4/41) of patients had a new anterior pituitary hormonal insufficiency.

CONCLUSIONS

Endonasal endoscopic reoperation is extremely effective at removing recurrent or residual pituitary adenomas that remain after a prior surgery, and it may be preferable to radiation therapy particularly in symptomatic patients. Achievement of GTR is less common when lateral cavernous sinus invasion is present. The locations of residual/recurrent tumor were more likely sphenoidal and parasellar following a prior microscopic transsphenoidal surgery and sellar following a prior endonasal endoscopic surgery.

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Sacit Bulent Omay, Yu-Ning Chen, Joao Paulo Almeida, Armando Saul Ruiz-Treviño, John A. Boockvar, Philip E. Stieg, Jeffrey P. Greenfield, Mark M. Souweidane, Ashutosh Kacker, David J. Pisapia, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Exome sequencing studies have recently demonstrated that papillary craniopharyngiomas (PCPs) and adamantinomatous craniopharyngiomas (ACPs) have distinct genetic origins, each primarily driven by mutually exclusive alterations: either BRAF (V600E), observed in 95% of PCPs, or CTNNB1, observed in 75%–96% of ACPs. How the presence of these molecular signatures, or their absence, correlates with clinical, radiographic, and outcome variables is unknown.

METHODS

The pathology records for patients who underwent surgery for craniopharyngiomas between May 2000 and March 2015 at Weill Cornell Medical College were reviewed. Craniopharyngiomas were identified and classified as PCP or ACP. Patients were placed into 1 of 3 groups based on their genomic mutations: BRAF mutation only, CTNNB1 mutation only, and tumors with neither of these mutations detected (not detected [ND]). Demographic, radiological, and clinical variables were collected, and their correlation with each genomic group was tested.

RESULTS

Histology correlated strongly with mutation group. All BRAF tumors with mutations were PCPs, and all CTNNB1 with mutations and ND tumors were ACPs. Preoperative and postoperative clinical symptoms and radiographic features did not correlate with any mutation group. There was a statistically significant relationship (p = 0.0323) between the age group (pediatric vs adult) and the mutation groups. The ND group tumors were more likely to involve the sella (p = 0.0065).

CONCLUSIONS

The mutation signature in craniopharyngioma is highly predictive of histology. The subgroup of tumors in which these 2 mutations are not detected is more likely to occur in children, be located in the sella, and be of ACP histology.

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Harminder Singh, Sarang Rote, Ajit Jada, Evan D. Bander, Gustavo J. Almodovar-Mercado, Walid I. Essayed, Roger Härtl, Vijay K. Anand, Theodore H. Schwartz, and Jeffrey P. Greenfield

The authors present 4 cases in which they used intraoperative CT (iCT) scanning to provide real-time image guidance during endonasal odontoid resection. While intraoperative CT has previously been used as a confirmatory test after resection, to the authors’ knowledge this is the first time it has been used to provide real-time image guidance during endonasal odontoid resection. The operating room setup, as well as the advantages and pitfalls of this approach, are discussed. A mobile intraoperative CT scanner was used in conjunction with real-time craniospinal neuronavigation in 4 patients who underwent endoscopic endonasal odontoidectomy for basilar invagination. All patients underwent a successful decompression. In 3 of the 4 patients, real-time intraoperative CT image guidance was instrumental in achieving a comprehensive decompression. In 3 (75%) cases in which the right nostril was the predominant working channel, there was a tendency for asymmetrical decompression toward the right side, meaning that residual bone was seen on the left, which was subsequently removed prior to completion of the surgery.

Endoscopic endonasal odontoid resection with real-time intraoperative image-guided CT scanning is feasible and provides accurate intraoperative localization of pathology, thereby increasing the chance of a complete odontoidectomy. For right-handed surgeons operating predominantly through the right nostril, special attention should be paid to the contralateral side of the resection, where there is often a tendency for residual pathology.

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Richard D. Murray, Rachel Friedlander, Samuel Hanz, Harminder Singh, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

The anterior skull base is a common site for the spontaneous development of meningoceles, encephaloceles, and meningoencephaloceles that can lead to cerebrospinal fluid (CSF) fistula formation, particularly in association with idiopathic intracranial hypertension. In some circumstances the lesions are difficult to localize. Whether all sites in the anterior skull base are equally prone to fistula formation or whether they are distributed randomly throughout the anterior skull base is unknown, although the anterior cribriform plate has been proposed as the most frequent location. The purpose of this study was to identify sites of predilection in order to provide assistance for clinicians in finding occult leaks and increase the understanding of the etiology of this pathology.

METHODS

The authors performed a retrospective review of a prospectively acquired surgical database of all endonasal endoscopic surgeries performed at Weill Cornell Medical College by the senior authors. Spontaneous CSF fistulas of the anterior skull base were identified. The anatomical sites of the defects were located on radiographic images and normalized to a theoretical 4 × 2 grid representing the anterior midline skull base. Data from the left and right skull base were combined to increase statistical power. This grid was then used to analyze the distribution of defects. Frequency analysis was performed by means of a chi-square test, with a subsequent Monte Carlo simulation to further strengthen the statistical support of the conclusions.

RESULTS

Nineteen cases of spontaneous CSF fistulas were identified. Frequency analysis using chi-square indicated a nonrandom distribution of sites (p = 0.035). Monte Carlo simulation supported this conclusion (p = 0.034). Seventy-four percent of cases occurred in the cribriform plate (p = 0.086). Moreover, 37% of all defects occurred in the posterior third of the cribriform plate.

CONCLUSIONS

Anterior skull base spontaneous CSF leaks are distributed in a nonrandom fashion. The most likely site of origin of the spontaneous CSF leaks of the anterior midline skull base is the cribriform plate, particularly the posterior third of the plate, likely because of the lack of significant thick bony buttressing. Clinicians searching for occult spontaneous leaks of the anterior skull base should examine the cribriform plate, especially the posterior third with particularly close scrutiny.

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Peter J. Wilson, Sacit B. Omay, Ashutosh Kacker, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Pituitary adenomas are benign, slow-growing tumors that cause symptoms either through mass effect or hormone overproduction. The decision to operate on a healthy young person is relatively straightforward. In the elderly population, however, the risks of complications may increase, rendering the decision more complex. Few studies have documented the risks of surgery using the endonasal endoscopic approach in a large number of elderly patients. The purpose of this study was to audit a single center's data regarding outcomes of purely endoscopic endonasal transsphenoidal resection of pituitary adenomas in elderly patients and to compare them to the current literature.

METHODS

A retrospective review of a prospectively acquired database of all endonasal endoscopic surgeries done by the senior authors was queried for patients aged 60–69 years and for those aged 70 years or older. Demographic and radiographic preoperative data were reviewed. Outcomes with respect to extent of resection and complications were examined and compared with appropriate statistical tests.

RESULTS

A total of 135 patents were identified (81 aged 60–69 years and 54 aged 70 years or older [70+]). The average tumor diameter was slightly larger for the patients in the 70+ age group (mean [SD] 25.7 ± 9.2 mm) than for patients aged 60–69 years (23.1 ± 9.8 mm, p = 0.056). There was no significant difference in intraoperative blood loss (p > 0.99), length of stay (p = 0.22), or duration of follow-up (p = 0.21) between the 2 groups. There was a 7.4% complication rate in patients aged 60–69 years (3 nasal and 3 medical complications) and an 18.5% complication rate in patients older than 70 years (4 cranial, 3 nasal, 1 visual, and 2 medical complications; p = 0.05 overall and 0.013 for cranial complications). Cranial complications in the 70+ age category included 2 postoperative hematomas, 1 pseudoaneurysm formation, and 1 case of symptomatic subdural hygromas.

CONCLUSIONS

Endonasal endoscopic surgery in elderly patients is safe, but there is a graded increase in complication rates with increasing age. The decision to operate on an asymptomatic or mildly symptomatic patient in these age groups should take this increasing complication rate into account. The use of a lumbar drain or lumbar punctures should be weighed against the risk of subdural hematoma in patients with preexisting atrophy.

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A. Gabriella Wernicke, Andrew W. Smith, Shoshana Taube, Menachem Z. Yondorf, Bhupesh Parashar, Samuel Trichter, Lucy Nedialkova, Albert Sabbas, Paul Christos, Rohan Ramakrishna, Susan C. Pannullo, Philip E. Stieg, and Theodore H. Schwartz

OBJECTIVE

Managing patients whose intraparenchymal brain metastases recur after radiotherapy remains a challenge. Intraoperative cesium-131 (Cs-131) brachytherapy performed at the time of neurosurgical resection may represent an excellent salvage treatment option. The authors evaluated the outcomes of this novel treatment with permanent intraoperative Cs-131 brachytherapy.

METHODS

Thirteen patients with 15 metastases to the brain that recurred after stereotactic radiosurgery and/or whole brain radiotherapy were treated between 2010 and 2015. Stranded Cs-131 seeds were placed as a permanent volume implant. Prescription dose was 80 Gy at 5-mm depth from the resection cavity surface. The primary end point was resection cavity freedom from progression (FFP). Resection cavity freedom from progression (FFP), regional FFP, distant FFP, median survival, overall survival (OS), and toxicity were assessed.

RESULTS

The median duration of follow-up after salvage treatment was 5 months (range 0.5–18 months). The patients' median age was 64 years (range 51–74 years). The median resected tumor diameter was 2.9 cm (range 1.0–5.6 cm). The median number of seeds implanted was 19 (range 10–40), with a median activity per seed of 2.25 U (range 1.98–3.01 U) and median total activity of 39.6 U (range 20.0–95.2 U). The 1-year actuarial local FFP was 83.3%. The median OS was 7 months, and 1-year OS was 24.7%. Complications included infection (3), pseudomeningocele (1), seizure (1), and asymptomatic radionecrosis (RN) (1).

CONCLUSIONS

After failure of prior irradiation of brain metastases, re-irradiation with intraoperative Cs-131 brachytherapy implants provides durable local control and limits the risk of RN. The authors' initial experience demonstrates that this treatment approach is well tolerated and safe for patients with previously irradiated tumors after failure of more than 1 radiotherapy regimen and that it results in excellent response rates and minimal toxicity.

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Harminder Singh, Walid I. Essayed, Ajit Jada, Nelson Moussazadeh, Sivashanmugam Dhandapani, Sarang Rote, and Theodore H. Schwartz

OBJECTIVE

The authors describe the supraorbital keyhole approach to the contralateral medial optic nerve and tract, both in a series of cadaveric dissections and in 2 patients. They also discuss the indications and contraindications for this procedure.

METHODS

In 3 cadaver heads, bilateral supraorbital keyhole minicraniotomies were performed to expose the ipsilateral and contralateral optic nerves. The extent of exposure of the medial optic nerve was assessed. In 2 patients, a contralateral supraorbital keyhole approach was used to remove pathology of the contralateral medial optic nerve and tract.

RESULTS

The supraorbital keyhole craniotomy provided better exposure of the contralateral superomedial nerve than it did of the same portion of the ipsilateral nerve. In both patients gross-total resections of the pathology was achieved.

CONCLUSIONS

The authors demonstrate the suitability of the contralateral supraorbital keyhole approach for lesions involving the superomedial optic nerve.