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Shaan M. Raza and Franco DeMonte

This video describes the surgical management of an epidermoid cyst within the cerebellopontine angle and petroclival region with involvement of cranial nerves V through XI and the vertebrobasilar system. A retrosigmoid craniotomy was performed for gross total resection of the lesion. The key steps of the procedure are discussed, including: positioning, soft tissue dissection, craniotomy, microsurgical dissection/resection, closure. Additionally, surgical nuances with regards to the safe maximal resection of such lesions are detailed.

The video can be found here: http://youtu.be/VEROVO5cYdU.

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Rohan Ramakrishna, Shaan M. Raza, Michael Kupferman, Ehab Hanna and Franco DeMonte

OBJECT

Adenoid cystic carcinoma (ACC) is a locally aggressive tumor of salivary gland origin. Little data exist to guide treatment when this tumor extends to involve the structures of the skull base.

METHODS

Fifty-one patients with a diagnosis of ACC affecting the skull base were identified from a prospective database at MD Anderson Cancer Center (from 1992 to 2010).

RESULTS

Median follow-up for study patients was 6.75 years. The 5- and 10-year overall survival (OS) rates were 78% and 50%, respectively. Sixty-six percent of patients had progression of their disease. The 5- and 10-year progression-free survival (PFS) rates were 46.7% and 21.0%, respectively. Gross-total resection was achieved in 75% of patients, with 49% having microscopically negative margins at the time of first operation. On univariate analysis, resections with microscopically negative margins were associated with a significant OS advantage (20.1 ± 3.3 years) compared with resections that left residual disease, even if microscopic (10.3 ± 1.6 years, p = 0.035). In patients who underwent reoperation, the effect persisted, with improved OS in those with negative margins (21.4 ± 0.0 vs 16.7 ± 4.0 years, p = 0.06). The use of adjuvant radiotherapy was associated with an OS advantage (16.2 ± 2.5 vs 5.5 ± 2.2 years, p = 0.03) at initial diagnosis and improved PFS (7.8 ± 1.0 vs 2.1 ± 0.62 years, p = 0.005), whereas repeat irradiation provided no benefit. The use of adjuvant chemotherapy at diagnosis or at recurrence was not associated with any significant advantage. Multivariate analysis revealed margin-negative resection at initial operation and at recurrence retained OS significance, even after controlling for age, radiation therapy, and T stage.

CONCLUSIONS

ACC of the skull base is best treated with a multidisciplinary approach aimed at maximal, safe resection. Adjuvant radiotherapy should be offered, whereas chemotherapy does not confer benefit.

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G. Rene Alvarez Berastegui, Shaan M. Raza, Vijay K. Anand and Theodore H. Schwartz

Visual deterioration after dopamine-agonist treatment of prolactinomas associated with empty sella syndrome and secondary optic apparatus traction is a rare event. Chiasmapexy has been described as a viable treatment option, although few cases exist in the literature. Here, a novel endonasal endoscopic approach to chiasmapexy is described and its efficacy is demonstrated in a case report.

A 55-year-old female patient with a history of a giant prolactinoma and 14 years of treatment using dopaminergic agonist therapy presented to our institution with a 1-month history of visual changes. Neuroophthalmological examination confirmed severe bitemporal field defects, and MRI revealed a large empty sella with downward optic chiasmal herniation. Endoscopic endonasal chiasmapexy was performed by elevating the chiasm with lumbar drainage and filling the clival and sellar defect with an extradural liquid (HydroSet; a cranioplasty bone cement), and a piece of AlloDerm was used to cover and cushion the chiasm. Postoperative imaging demonstrated successful anatomical elevation of the optic apparatus, and the patient showed functional improvement in the visual field at 3 months postoperatively.

Although rare, massive empty sellar and chiasmal descent from macroadenoma treatment can result in progressive visual loss. Here, a novel technique of endonasal endoscopic extradural cranioplasty aided by lumbar drainage is reported, which appears to be an effective technique for stabilizing and possibly reversing anatomical and visual deterioration.

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Pablo F. Recinos, Shaan M. Raza, George I. Jallo and Violette Renard Recinos

Object

Microsurgical removal is the preferred treatment for most deep-seated, intraaxial tumors in the pediatric population. The feasibility of surgery as an option has improved with advances in surgical technology and technique. Tubular retractors disperse retraction forces over a greater surface area than do conventional retractors, which can lower the risk of ischemic complications. The authors describe their experience utilizing a new tubular retractor system specifically designed for cranial applications in conjunction with frameless neuronavigation.

Methods

The Vycor ViewSite retractor was used in 4 pediatric patients (ages 15 months and 9, 10, and 16 years) with deep-seated intraaxial tumors. The lesions included a papillary tumor of the pineal region, a low-grade astrocytoma in the occipital lobe, a dysembryoplastic neuroepithelial tumor arising from the basal ganglia, and an intraventricular low-grade glioma. The extent of white matter damage along the surgical trajectory (based on T2 or FLAIR and diffusion restriction/apparent diffusion coefficient signals) and the extent of resection were assessed on postoperative imaging.

Results

Satisfactory resection or biopsy was achieved in all patients. A comparison of pre- and postoperative MR imaging studies revealed evidence of white matter damage along the surgical trajectory in 1 patient. None of the patients demonstrated new neurological deficits postoperatively.

Conclusions

Obtaining surgical access to deep-seated, intraaxial tumors is challenging. In this small series of pediatric patients, the combination of the ViewSite tubular retractor and frameless neuronavigation facilitated the surgical approach. The combination of these technologies adds to the armamentarium to safely approach tumors in deep locations.

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Shaan M. Raza, Angela M. Donaldson, Alpesh Mehta, Apostolos J. Tsiouris, Vijay K. Anand and Theodore H. Schwartz

Object

Because multiple anatomical compartments are involved, the surgical management of trigeminal schwannomas requires a spectrum of cranial base approaches. The endoscopic endonasal approach to Meckel's cave provides a minimal access corridor for surgery, but few reports have assessed outcomes of the procedure or provided guidelines for case selection.

Methods

A prospectively acquired database of 680 endoscopic endonasal cases was queried for trigeminal schwannoma cases. Clinical charts, radiographic images, and long-term outcomes were reviewed to determine outcome and success in removing tumor from each compartment traversed by the trigeminal nerve.

Results

Four patients had undergone endoscopic resection of trigeminal schwannomas via the transpterygoid approach (mean follow-up 37 months). All patients had disease within Meckel's cave, and 1 patient had extension into the posterior fossa. Gross-total resection was achieved in 3 patients whose tumors were purely extracranial. One patient with combined Meckel's cave and posterior fossa tumor had complete resection of the extracranial disease and 52% resection of the posterior fossa disease. One patient with posterior fossa disease experienced a sixth cranial nerve palsy in addition to a corneal keratopathy from worsened trigeminal neuropathy. There were no CSF leaks. Over the course of the study, 1 patient with subtotal resection required subsequent stereotactic radiosurgery for disease progression within the posterior fossa.

Conclusions

Endoscopic endonasal approaches appear to be well suited for trigeminal schwannomas restricted to Meckel's cave and/or extracranial segments of the nerve. Lateral transcranial skull base approaches should be considered for patients with posterior fossa disease. Further multiinstitutional studies will be necessary for adequate power to help determine relative indications between endoscopic and transcranial skull base approaches.

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Shaan M. Raza, Rohan Ramakrishna, Randal S. Weber, Michael E. Kupferman, Paul W. Gidley, Ehab Y. Hanna and Franco DeMonte

OBJECT

A relative paucity of information exists regarding outcomes from craniofacial resection for advanced nonmelanoma skin cancers involving the skull base. In light of advances in surgical technique and adjuvant therapy protocols, the authors reviewed their surgical experience to determine disease control rates, overall survival (OS), morbidity, and mortality.

METHODS

A retrospective review of 24 patients with nonmelanoma cutaneous cancers with skull base involvement treated with craniofacial resection at The University of Texas MD Anderson Cancer Center from 1994 to 2012 was performed. Of these patients, 19 (79%) had squamous cell carcinoma (SCC), 4 (17%) had basosquamous carcinoma (BSCC), and 1 patient (4%) had adenocarcinoma. Factors as assessed were prior treatment, TNM staging, tumor involvement, extent of intracranial extension, margin status, postoperative complications, recurrence, disease status at last follow-up, and long-term survival. The majority of tumors were T4 (67%) according to the TNM classification; perineural extension was noted in 58%, cavernous sinus involvement in 25%, and dural involvement in 29%.

RESULTS

Postoperative complications occurred in 4 patients (17%) including 1 death. Kaplan-Meier estimates were calculated for OS and progression-free survival (PFS). Median OS was 43.2 months with an 82% 1-year OS and 37% 5-year OS; the median PFS was 91.2 months. Margin status was positively associated with median OS in SCC (91 months [for negative margins] vs 57 months, p = 0.8) and in BSCC (23.7 vs 3.2 months, p < 0.05). Postoperative radiotherapy was associated with improved median OS (43.2 vs 22 months, p = 0.6). Brain involvement was uniformly fatal after 1 year, while cavernous sinus involvement (31 vs 43 months, p = 0.82), perineural disease (31 vs 54 months, p = 0.30), and T4 stage (22 vs 91.2 months, p = 0.09) were associated with worsened OS. Similar associations were found with median PFS.

CONCLUSIONS

Aggressive multimodality management with surgery and postoperative radiotherapy can positively impact locoregional control and OS. With improvements in technique and adjuvant therapy protocols, treatment can still be considered in situations of perineural disease and cavernous sinus involvement and as a salvage option for patients in whom prior treatment has failed. As patients with advanced NMSCs often have few options, craniofacial resection, as part of a coordinated multimodal management plan, is justified if it can be performed safely.

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Shaan M. Raza, Matei A. Banu, Angela Donaldson, Kunal S. Patel, Vijay K. Anand and Theodore H. Schwartz

OBJECT

The intraoperative detection of CSF leaks during endonasal endoscopic skull base surgery is critical to preventing postoperative CSF leaks. Intrathecal fluorescein (ITF) has been used at varying doses to aid in the detection of intraoperative CSF leaks. However, the sensitivity and specificity of ITF at certain dosages is unknown.

METHODS

A prospective database of all endoscopic endonasal procedures was reviewed. All patients received 25 mg ITF diluted in 10 ml CSF and were pretreated with dexamethasone and Benadryl. Immediately after surgery, the operating surgeon prospectively noted if there was an intraoperative CSF leak and fluorescein was identified. The sensitivity, specificity, and positive and negative predictive power of ITF for detecting intraoperative CSF leak were calculated. Factors correlating with postoperative CSF leak were determined.

RESULTS

Of 419 patients, 35.8% of patients did not show a CSF leak. Fluorescein-tinted CSF (true positive) was noted in 59.7% of patients and 0 false positives were encountered. CSF without fluorescein staining (false negative) was noted in 4.5% of patients. The sensitivity and specificity of ITF were 92.9% and 100%, respectively. The negative and positive predictive values were 88.8% and 100%, respectively. Postoperative CSF leaks only occurred in true positives at a rate of 2.8%.

CONCLUSIONS

ITF is extremely specific and very sensitive for detecting intraoperative CSF leaks. Although false negatives can occur, these patients do not appear to be at risk for postoperative CSF leak. The use of ITF may help surgeons prevent postoperative CSF leaks by intraoperatively detecting and confirming a watertight repair.

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Gautam U. Mehta, Franco DeMonte, Shirley Y. Su, Michael E. Kupferman, Ehab Y. Hanna and Shaan M. Raza

Chondrosarcomas of the skull base are malignant tumors for which surgery is the primary therapeutic option. Gross-total resection has been demonstrated to improve survival in patients with these tumors. Chondrosarcomas arising from the petroclival synchondrosis harbor particularly unique anatomical considerations that have long been a barrier to achieving such a resection. Endoscopic endonasal transpterygoid approaches have been recently used to gain improved access to such lesions; however, these approaches have classically relied on a medial to lateral transclival trajectory, which provides limited exposure for complete resection of lateral disease. In this paper the authors describe an endoscopic endonasal transpterygoid transnasopharyngeal approach that provides comprehensive access to the petroclival region through dissection of the eustachian tube with resection of the cartilaginous torus tubarius. Of note, the authors have previously demonstrated the superior outcomes and validity of this approach relative to other cranial base techniques for petroclival chondrosarcomas. Surgical outcomes in 5 cases of chondrosarcoma without medial extension are detailed. Gross-total resection was achieved in 4 of 5 patients. Postoperative complications included transient palatal numbness in all patients and eustachian tube dysfunction due to the approach. With tympanostomy tube placement, no patient had persistent hearing loss. Overall, this approach appears to be a safe and effective technique for resection of petroclival chondrosarcomas.

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Kunal S. Patel, Shaan M. Raza, Edward D. McCoul, Aikaterini Patrona, Jeffrey P. Greenfield, Mark M. Souweidane, Vijay K. Anand and Theodore H. Schwartz

OBJECT

Craniopharyngiomas are benign parasellar tumors for which surgical removal, although potentially curative, often leads to morbidity with resulting decreases in quality of life (QOL). The endonasal endoscopic approach is a minimal-access technique for removing these tumors and may reduce postoperative morbidity. The QOL following this method for resection of craniopharyngiomas has not been documented.

METHODS

The authors reviewed a database of consecutive endonasal endoscopic surgeries done at Weill Cornell Medical College. Adult patients with histologically proven craniopharyngiomas were included who had completed either only postoperative (> 9 months) or both pre- and postoperative QOL forms, the Anterior Skull Base Quality of Life (ASBQ) questionnaire, and the 22-Item Sinonasal Outcome Test (SNOT-22). Rates of gross-total resection (GTR), complications, and visual and endocrine function were collected. Retrospective independence (Wen score) was also assigned. A contemporaneous group of patients undergoing endonasal endoscopic pituitary macroadenoma resection was used as a control.

RESULTS

This study included 33 procedures performed in 31 patients. The average postoperative ASBQ score was 3.35 and the SNOT-22 score was 19.6. Better QOL was associated with GTR and postoperative radiation. Worse QOL was associated with persistent visual defects, hypopituitarism, tumor recurrence, increase in body mass index, and worsening Wen score. In a subset of 10 patients, both pre- and postoperative (> 9 months) QOL scores were obtained. Both ASBQ and SNOT-22 scores showed stability and a trend toward improvement, from 2.93 ± 0.51 to 2.96 ± 0.47 (ASBQ) and 23.7 ± 10.8 to 18.4 ± 11.6 (SNOT-22). Compared with 62 patients undergoing endoscopic pituitary macroadenoma resection, patients with craniopharyngiomas had worse postoperative QOL on the ASBQ (3.35 vs 3.80; p = 0.023) and SNOT-22 (19.6 vs 13.4; p = 0.12).

CONCLUSIONS

This report of validated site-specific QOL following endoscopic surgery for craniopharyngiomas shows an overall maintenance of postoperative compared with preoperative QOL. Better QOL could be seen in patients with GTR and radiation therapy, and worse QOL was found in patients with visual or endocrine deficits. Nevertheless, patients with craniopharyngiomas still had worse QOL than those undergoing similar surgery for pituitary macroadenomas, confirming the worse prognosis of craniopharyngiomas even when removed via a minimally invasive approach. These measures should serve as benchmarks for comparison with open transcranial approaches to similar tumors.