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Rafael Uribe-Cardenas, Andre E. Boyke, Justin T. Schwarz, Peter F. Morgenstern, Jeffrey P. Greenfield, Theodore H. Schwartz, James T. Rutka, James Drake, and Caitlin E. Hoffman

OBJECTIVE

Early surgical intervention for pediatric refractory epilepsy is increasingly advocated as surgery has become safer and data have demonstrated improved outcomes with early seizure control. There is concern that the risks associated with staged invasive electroencephalography (EEG) in very young children outweigh the potential benefits. Here, the authors present a cohort of children with refractory epilepsy who were referred for invasive monitoring, and they evaluate the role and safety of staged invasive EEG in those 3 years old and younger.

METHODS

The authors conducted a retrospective review of children 3 years and younger with epilepsy, who had been managed surgically at two institutions between 2001 and 2015. A cohort of pediatric patients older than 3 years of age was used for comparison. Demographics, seizure etiology, surgical management, surgical complications, and adverse events were recorded. Statistical analysis was completed using Stata version 13. A p < 0.05 was considered statistically significant. Fisher’s exact test was used to compare proportions.

RESULTS

Ninety-four patients (45 patients aged ≤ 3 [47.9%]) and 208 procedures were included for analysis. Eighty-six procedures (41.3%) were performed in children younger than 3 years versus 122 in the older cohort (58.7%). Forty-two patients underwent grid placement (14 patients aged ≤ 3 [33.3%]); 3 of them developed complications associated with the implant (3/42 [7.14%]), none of whom were among the younger cohort. Across all procedures, 11 complications occurred in the younger cohort versus 5 in the older patients (11/86 [12.8%] vs 5/122 [4.1%], p = 0.032). Two adverse events occurred in the younger group versus 1 in the older group (2/86 [2.32%] vs 1/122 [0.82%], p = 0.571). Following grid placement, 13/14 younger patients underwent guided resections compared to 20/28 older patients (92.9% vs 71.4%, p = 0.23).

CONCLUSIONS

While overall complication rates were higher in the younger cohort, subdural grid placement was not associated with an increased risk of surgical complications in that population. Invasive electrocorticography informs management in very young children with refractory, localization-related epilepsy and should therefore be used when clinically indicated.

Open access

Mina M. Gerges, Brett Youngerman, Vijay K. Anand, Jeffrey P. Greenfield, and Theodore H. Schwartz

An 8-year-old child presented with fatigue, weight loss, and visual deterioration. MRI demonstrated a craniopharyngioma with compression of the optic chiasm and extensive edema on the hypothalamus and optic radiations. The tumor was completely removed via an endoscopic endonasal approach. Postoperatively, vision improved and hypothalamic edema completely resolved within 5 days. This video demonstrates the technical nuances of the surgery and discusses the impact of surgery on the hypothalamic nuclei in pediatric patients.

The video can be found here: https://youtu.be/wxkBmhTPi6c.

Free access

Iyan Younus, Mina M. Gerges, Rafael Uribe-Cardenas, Peter F. Morgenstern, Mahmoud Eljalby, Abtin Tabaee, Jeffrey P. Greenfield, Ashutosh Kacker, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Endoscopic endonasal approaches (EEAs) to the skull base have evolved over the last 20 years to become an essential component of a comprehensive skull base practice. Many case series show a learning curve from the earliest cases, in which the authors were inexperienced or were not using advanced closure techniques. It is generally accepted that once this learning curve is achieved, a plateau is reached with little incremental improvement. Cases performed during the early steep learning curve were eliminated to examine whether the continued improvement exists over the “tail end” of the curve.

METHODS

A prospectively acquired database of all EEA cases performed by the senior authors at Weill Cornell Medicine/NewYork-Presbyterian Hospital was reviewed. The first 200 cases were eliminated and the next 1000 consecutive cases were examined to avoid the bias created by the early learning curve.

RESULTS

Of the 1000 cases, the most common pathologies included pituitary adenoma (51%), meningoencephalocele or CSF leak repair (8.6%), meningioma (8.4%), craniopharyngioma (7.3%), basilar invagination (3.1%), Rathke’s cleft cyst (2.8%), and chordoma (2.4%). Use of lumbar drains decreased from the first half to the second half of our series (p <0.05) as did the authors’ use of fat alone (p <0.005) or gasket alone (p <0.005) for dural closure, while the use of a nasoseptal flap increased (p <0.005). Although mean tumor diameter was constant (on average), gross-total resection (GTR) increased from 60% in the first half to 73% in the second half (p <0.005). GTR increased for all pathologies but most significantly for chordoma (56% vs 100%, p <0.05), craniopharyngioma (47% vs 0.71%, p <0.05) and pituitary adenoma (67% vs 75%, p <0.05). Hormonal cure for secreting adenomas also increased from 83% in the first half to 89% in the second half (p <0.05). The rate of any complication was unchanged at 6.4% in the first half and 6.2% in the latter half of cases, and vascular injury occurred in only 0.6% of cases. Postoperative CSF leak occurred in 2% of cases and was unchanged between the first and second half of the series.

CONCLUSIONS

This study demonstrates that contrary to popular belief, the surgical learning curve does not plateau but can continue for several years depending on the complexity of the endpoints considered. These findings may have implications for clinical trial design, surgical education, and patient safety measures.

Free access

Iyan Younus, Mina M. Gerges, Rafael Uribe-Cardenas, Peter F. Morgenstern, Mahmoud Eljalby, Abtin Tabaee, Jeffrey P. Greenfield, Ashutosh Kacker, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Endoscopic endonasal approaches (EEAs) to the skull base have evolved over the last 20 years to become an essential component of a comprehensive skull base practice. Many case series show a learning curve from the earliest cases, in which the authors were inexperienced or were not using advanced closure techniques. It is generally accepted that once this learning curve is achieved, a plateau is reached with little incremental improvement. Cases performed during the early steep learning curve were eliminated to examine whether the continued improvement exists over the “tail end” of the curve.

METHODS

A prospectively acquired database of all EEA cases performed by the senior authors at Weill Cornell Medicine/NewYork-Presbyterian Hospital was reviewed. The first 200 cases were eliminated and the next 1000 consecutive cases were examined to avoid the bias created by the early learning curve.

RESULTS

Of the 1000 cases, the most common pathologies included pituitary adenoma (51%), meningoencephalocele or CSF leak repair (8.6%), meningioma (8.4%), craniopharyngioma (7.3%), basilar invagination (3.1%), Rathke’s cleft cyst (2.8%), and chordoma (2.4%). Use of lumbar drains decreased from the first half to the second half of our series (p <0.05) as did the authors’ use of fat alone (p <0.005) or gasket alone (p <0.005) for dural closure, while the use of a nasoseptal flap increased (p <0.005). Although mean tumor diameter was constant (on average), gross-total resection (GTR) increased from 60% in the first half to 73% in the second half (p <0.005). GTR increased for all pathologies but most significantly for chordoma (56% vs 100%, p <0.05), craniopharyngioma (47% vs 0.71%, p <0.05) and pituitary adenoma (67% vs 75%, p <0.05). Hormonal cure for secreting adenomas also increased from 83% in the first half to 89% in the second half (p <0.05). The rate of any complication was unchanged at 6.4% in the first half and 6.2% in the latter half of cases, and vascular injury occurred in only 0.6% of cases. Postoperative CSF leak occurred in 2% of cases and was unchanged between the first and second half of the series.

CONCLUSIONS

This study demonstrates that contrary to popular belief, the surgical learning curve does not plateau but can continue for several years depending on the complexity of the endpoints considered. These findings may have implications for clinical trial design, surgical education, and patient safety measures.

Full access

Edgar G. Ordóñez-Rubiano, Jonathan A. Forbes, Peter F. Morgenstern, Leopold Arko, Georgiana A. Dobri, Jeffrey P. Greenfield, Mark M. Souweidane, Apostolos John Tsiouris, Vijay K. Anand, Ashutosh Kacker, and Theodore H. Schwartz

OBJECTIVE

Gross-total resection (GTR) of craniopharyngiomas (CPs) is potentially curative and is often the goal of surgery, but endocrinopathy generally results if the stalk is sacrificed. In some cases, GTR can be attempted while still preserving the stalk; however, stalk manipulation or devascularization may cause endocrinopathy and this strategy risks leaving behind small tumor remnants that can recur.

METHODS

A retrospective review of a prospective cohort of patients who underwent initial resection of CP using the endoscopic endonasal approach over a period of 12 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital, was performed. Postresection integrity of the stalk was retrospectively assessed using operative notes, videos, and postoperative MRI. Tumors were classified based on location into type I (sellar), type II (sellar-suprasellar), and type III (purely suprasellar). Pre- and postoperative endocrine function, tumor location, body mass index, rate of GTR, radiation therapy, and complications were reviewed.

RESULTS

A total of 54 patients who had undergone endoscopic endonasal procedures for first-time resection of CP were identified. The stalk was preserved in 33 (61%) and sacrificed in 21 (39%) patients. GTR was achieved in 24 patients (73%) with stalk preservation and 21 patients (100%) with stalk sacrifice (p = 0.007). Stalk-preservation surgery achieved GTR and maintained completely normal pituitary function in only 4 (12%) of 33 patients. Permanent postoperative diabetes insipidus was present in 16 patients (49%) with stalk preservation and in 20 patients (95%) following stalk sacrifice (p = 0.002). In the stalk-preservation group, rates of progression and radiation were higher with intentional subtotal resection or near-total resection compared to GTR (67% vs 0%, p < 0.001, and 100% vs 12.5%, p < 0.001, respectively). However, for the subgroup of patients in whom GTR was achieved, stalk preservation did not lead to significantly higher rates of recurrence (12.5%) compared with those in whom it was sacrificed (5%, p = 0.61), and stalk preservation prevented anterior pituitary insufficiency in 33% and diabetes insipidus in 50%.

CONCLUSIONS

While the decision to preserve the stalk reduces the rate of postoperative endocrinopathy by roughly 50%, nevertheless significant dysfunction of the anterior and posterior pituitary often ensues. The decision to preserve the stalk does not guarantee preserved endocrine function and comes with a higher risk of progression and need for adjuvant therapy. Nevertheless, to reduce postoperative endocrinopathy attempts should be made to preserve the stalk if GTR can be achieved.

Full access

Jonathan A. Forbes, Matei Banu, Kurt Lehner, Malte Ottenhausen, Emanuele La Corte, Andrew F. Alalade, Edgar G. Ordóñez-Rubiano, Jeffrey P. Greenfield, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Epidermoid cysts (ECs) commonly extend to involve the ventral cisterns of the cranial base. When present, symptoms arise due to progressive mass effect on the brainstem and adjacent cranial nerves. Historically, a variety of open microsurgical approaches have been used for resection of ECs in this intricate region. In recent years, the endoscopic endonasal approach (EEA) has been proposed as an alternative corridor that avoids crossing the plane of the cranial nerves. To date, there is a paucity of data in the literature regarding the safety and efficacy of the EEA in the treatment of ECs of the ventral cranial base.

METHODS

The authors reviewed a prospectively acquired database of EEAs for resection of ECs over 8 years at Weill Cornell, NewYork-Presbyterian Hospital. All procedures were performed by the senior authors. Standardized clinical and radiological parameters were assessed before and after surgery. Statistical tests were used to determine the impact of previous surgery and tumor volume on extent of resection and recurrence as well as the method of closure on rate of CSF leak.

RESULTS

Between January 2009 and February 2017, 7 patients (4 males and 3 females; age range 16–70 years) underwent a total of 8 surgeries for EC resection utilizing the EEA. Transplanum and transclival extensions were performed in 3 and 5 patients, respectively. Methods of closure incorporated a gasket seal in 6 of 8 procedures and a nasoseptal flap in 7 of 8 procedures. Gross-total resection (GTR) was achieved in 43% of patients, and near-total resection (> 95%) was obtained in another 43%. Complications included diabetes insipidus (n = 2), postoperative CSF leak (n = 2), transient third cranial nerve palsy (n = 1), and epistaxis (n = 1). With a mean follow-up of 43.5 months, recurrence has been observed in 2 of 7 patients. In 1 case, reoperation for recurrence was required 71 months following the initial surgery. Use of the gasket-seal technique with nasoseptal flap coverage significantly correlated with the absence of postoperative CSF leakage (p = 0.018). GTR was achieved in 25% of the patients who had prior surgeries and in 50% of patients without previous resections. The mean volume of cysts in which GTR was achieved (4.3 ± 1.8 cm3) was smaller than that in which subtotal or near-total resection was achieved (12.2 ± 11 cm3, p = 0.134).

CONCLUSIONS

The EEA for resection of ECs of the ventral cranial base is a safe and effective operative strategy that avoids crossing the plane of the cranial nerves. In the authors’ experience, gasket-seal closure with nasoseptal flap coverage has been associated with a decreased risk of postoperative CSF leakage.

Free access

Andrew F. Alalade, Elizabeth Ogando-Rivas, Jerome Boatey, Mark M. Souweidane, Vijay K. Anand, Jeffrey P. Greenfield, and Theodore H. Schwartz

OBJECTIVE

The expanded endonasal endoscopic transsphenoidal approach has become increasingly used for craniopharyngioma surgery in the pediatric population, but questions still persist regarding its utility in younger children, in recurrent and irradiated tumors, and in masses primarily located in the suprasellar region. The narrow corridor, incomplete pneumatization, and fear of hypothalamic injury have traditionally relegated this approach to application in older children with mostly cystic craniopharyngiomas centered in the sella. The authors present a series of consecutive pediatric patients in whom the endonasal endoscopic approach was used to remove craniopharyngiomas from patients of varied ages, regardless of the location of the tumor and previous treatments or surgeries, to ascertain if the traditional concerns about limitations of this approach are worth reevaluating

METHODS

Eleven consecutive pediatric patients (age ≤ 18 years) underwent surgery via an endoscopic transsphenoidal approach at NewYork-Presbyterian/Weill Cornell Medical Center from 2007 to 2016. The authors recorded the location, consistency, and size of the lesion, assessed for hypothalamic invasion radiographically, calculated skull base measurements, and assessed parameters such as extent of resection, visual function, endocrinological function, weight gain, and return-to-school status.

RESULTS

The average age at the time of surgery was 7.9 years (range 4–17 years) and the tumor sizes ranged from 1.3 to 41.7 cm3. Five cases were purely suprasellar, 5 had solid components, 4 were reoperations, and 5 had a conchal sphenoid aeration. Nevertheless, gross-total resection was achieved in 45% of the patients and 50% of those in whom it was the goal of surgery, without any correlation with the location, tumor consistency, or the age of the patient. Near-total resection, subtotal resection, or biopsy was performed intentionally in the remaining patients to avoid hypothalamic injury. Anterior pituitary dysfunction occurred in 81.8% of the patients, and 63.3% developed diabetes insipidus . Two patients (18%) had a greater than 9% increase in body mass index. Visual function was stable or improved in 73%. All children returned to an academic environment, with 10 of them in the grade appropriate for their age. There was a single case of each of the following: CSF leak, loss of vision unilaterally, and abscess.

CONCLUSIONS

The endoscopic transsphenoidal approach is suitable for removing pediatric craniopharyngiomas even in young children with suprasellar tumors, conchal sphenoid sinus, recurrent tumors, and tumors with solid components. The extent of resection is dictated by intrinsic hypothalamic tumor invasiveness rather than the approach. The endoscopic transsphenoidal approach affords the ability to directly inspect the hypothalamus to determine invasion, which may help spare the patient from hypothalamic injury. Irrespective of approach, the rates of postoperative endocrinopathy remain high and the learning curve for the approach to a relatively rare tumor is steep.

Full access

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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Sivashanmugam Dhandapani, Harminder Singh, Hazem M. Negm, Salomon Cohen, Mark M. Souweidane, Jeffrey P. Greenfield, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Craniopharyngiomas can be difficult to remove completely based on their intimate relationship with surrounding visual and endocrine structures. Reoperations are not uncommon but have been associated with higher rates of complications and lower extents of resection. So radiation is often offered as an alternative to reoperation. The endonasal endoscopic transsphenoidal approach has been used in recent years for craniopharyngiomas previously removed with craniotomy. The impact of this approach on reoperations has not been widely investigated.

METHODS

The authors reviewed a prospectively acquired database of endonasal endoscopic resections of craniopharyngiomas over 11 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital, performed by the senior authors. Reoperations were separated from first operations. Pre- and postoperative visual and endocrine function, tumor size, body mass index (BMI), quality of life (QOL), extent of resection (EOR), impact of prior radiation, and complications were compared between groups. EOR was divided into gross-total resection (GTR, 100%), near-total resection (NTR, > 95%), and subtotal resection (STR, < 95%). Univariate and multivariate analyses were performed.

RESULTS

Of the total 57 endonasal surgical procedures, 22 (39%) were reoperations. First-time operations and reoperations did not differ in tumor volume, radiological configuration, or patients' BMI. Hypopituitarism and diabetes insipidus (DI) were more common before reoperations (82% and 55%, respectively) compared with first operations (60% and 8.6%, respectively; p < 0.001). For the 46 patients in whom GTR was intended, rates of GTR and GTR+NTR were not significantly different between first operations (90% and 97%, respectively) and reoperations (80% and 100%, respectively). For reoperations, prior radiation and larger tumor volume had lower rates of GTR. Vision improved equally in first operations (80%) compared with reoperations (73%). New anterior pituitary deficits were more common in first operations compared with reoperations (51% vs 23%, respectively; p = 0.08), while new DI was more common in reoperations compared with first-time operations (80% vs 47%, respectively; p = 0.08). Nonendocrine complications occurred in 2 (3.6%) first-time operations and no reoperations. Tumor regrowth occurred in 6 patients (11%) over a median follow-up of 46 months and was not different between first versus reoperations, but was associated with STR (33%) compared with GTR+NTR (4%; p = 0.02) and with not receiving radiation after STR (67% vs 22%; p = 0.08). The overall BMI increased significantly from 28.7 to 34.8 kg/m2 over 10 years. Six months after surgery, there was a significant improvement in QOL, which was similar between first-time operations and reoperations, and negatively correlated with STR.

CONCLUSIONS

Endonasal endoscopic transsphenoidal reoperation results in similar EOR, visual outcome, and improvement in QOL as first-time operations, with no significant increase in complications. EOR is more impacted by tumor volume and prior radiation. Reoperations should be offered to patients with recurrent craniopharyngiomas and may be preferable to radiation in patients in whom GTR or NTR can be achieved.