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Maya Harary, Aislyn C. DiRisio, Hassan Y. Dawood, John Kim, Nayan Lamba, Charles H. Cho, Timothy R. Smith, Hasan A. Zaidi and Edward R. Laws Jr.

OBJECTIVE

Loss of pituitary function due to nonfunctional pituitary adenoma (NFPA) may be due to compression of the pituitary gland. It has been proposed that the size of the gland and relative perioperative gland expansion may relate to recovery of pituitary function, but the extent of this is unclear. This study aims to assess temporal changes in hormonal function after transsphenoidal resection of NFPA and the relationship between gland reexpansion and endocrine recovery.

METHODS

Patients who underwent endoscopic transsphenoidal surgery by a single surgeon for resection of a nonfunctional macroadenoma were selected for inclusion. Patients with prior pituitary surgery or radiosurgery were excluded. Patient characteristics and endocrine function were extracted by chart review. Volumetric segmentation of the pre- and postoperative (≥ 6 months) pituitary gland was performed using preoperative and long-term postoperative MR images. The relationship between endocrine function over time and clinical attributes, including gland volume, were examined.

RESULTS

One hundred sixty eligible patients were identified, of whom 47.5% were female; 56.9% of patients had anterior pituitary hormone deficits preoperatively. The median tumor diameter and gland volume preoperatively were 22.5 mm (interquartile range [IQR] 18.0–28.8 mm) and 0.18 cm3 (IQR 0.13–0.28 cm3), respectively. In 55% of patients, endocrine function normalized or improved in their affected axes by median last clinical follow-up of 24.4 months (IQR 3.2–51.2 months). Older age, male sex, and larger tumor size were associated with likelihood of endocrine recovery. Median time to recovery of any axis was 12.2 months (IQR 2.5–23.9 months); hypothyroidism was the slowest axis to recover. Although the gland significantly reexpanded from preoperatively (0.18 cm3, IQR 0.13–0.28 cm3) to postoperatively (0.33 cm3, IQR 0.23–0.48 cm3; p < 0.001), there was no consistent association with improved endocrine function.

CONCLUSIONS

Recovery of endocrine function can occur several months and even years after surgery, with more than 50% of patients showing improved or normalized function. Tumor size, and not gland volume, was associated with preserved or recovered endocrine function.

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Ali O. Jamshidi, André Beer-Furlan, Daniel M. Prevedello, Ronald Sahyouni, Mohamed A. Elzoghby, Mina G. Safain, Ricardo L. Carrau, John A. Jane Jr. and Edward R. Laws

OBJECTIVE

The endoscopic endonasal approach has been proposed as a primary surgical strategy for select craniopharyngiomas. However, those tumors that arise from the sella have not been classified with the other craniopharyngioma subtypes in terms of surgical nuances, intraoperative findings, and postoperative outcomes. The authors describe their experience with a select subtype of craniopharyngioma arising within the sella subjacent to the diaphragma sellae and refer to these tumors as type 0.

METHODS

After obtaining IRB approval, three institutions retrospectively reviewed their data collected from 2005 to 2017. Patients eligible for inclusion in the study were those who had tumors that originated from the sella inferior to the diaphragma sellae. Demographic, clinical, radiological, surgical, and follow-up data were examined and analyzed.

RESULTS

Twenty-eight patients (average age 19.3 years, range 3–60 years) were included in this study. Sixteen patients (57%) were younger than 18 years of age. All patients had characteristic imaging features of an expanded sella. Seventy-five percent of the patients presented with some form of visual field deficit (89% had radiographic optic apparatus compression) and 39% with hypopituitarism. The average maximal tumor diameter in the axial, coronal, or sagittal plane was 3.1 cm. Gross-total resection was achieved in 82% of the patients. Twenty-one percent of patients experienced an iatrogenic complication, and there were only two cases (7%) of postoperative cerebrospinal fluid (CSF) leakage. Only two patients (7%) required the use of a nasoseptal flap as part of their original reconstruction. Pathology was uniformly the adamantinomatous subtype. Postoperative objective visual outcomes were improved in 71% of the patients with visual symptoms or visual field deficits on presentation and stable in 24%. Mean follow-up was 45.1 months (range 3–120 months) with an 18% recurrence rate at a mean of 44.4 months (range 10–84 months). One patient was lost to follow-up. Thirty-six percent of patients received postoperative radiation to treat recurrence or residual tumor. Endocrine data are also presented.

CONCLUSIONS

Craniopharyngiomas that originate within the sella below the diaphragma sellae are a select subtype characterized by 1) an enlarged sella, 2) an intact diaphragma sellae at surgery, and 3) an adamantinomatous pathology. These tumors can be treated transnasally without the absolute need for neurovascular flap reconstruction, as there is a low risk of CSF leakage.

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Alexandra M. Giantini Larsen, David J. Cote, Hasan A. Zaidi, Wenya Linda Bi, Paul J. Schmitt, J. Bryan Iorgulescu, Michael B. Miller, Timothy R. Smith, M. Beatriz Lopes, John A. Jane Jr. and Edward R. Laws Jr.

OBJECTIVE

The authors report the diagnosis, management, and outcomes of 6 cases of spindle cell oncocytoma (SCO) in an effort to guide clinical diagnosis and management of these uncommon lesions.

METHODS

This study is a retrospective review of cases involving adult patients who underwent resection of pituitary lesions at the authors’ institutions between January 2000 and October 2017. The authors identified patients with histopathological confirmation of SCO and collected clinical data, including preoperative, perioperative, and postoperative management, complications, and outcomes.

RESULTS

Six patients with SCO were identified. Clinical findings at initial presentation included visual disturbances, dizziness, and headache. All patients underwent resection. Four resections were initially performed by the transsphenoidal approach, and 2 resections were performed by craniotomy at an outside institution with subsequent transsphenoidal reoperations. Neither necrosis nor increased mitotic activity was seen in the tumor samples. All samples stained positive for S100 protein and thyroid transcription factor 1 and negative for glial fibrillary acidic protein and pituitary hormones. Five of the samples stained positive for epithelial membrane antigen. The average MIB-1 index was 8.3% (range 2–17). Postoperatively, 3 of the 6 patients received further treatment for progression of residual tumor or for recurrence, 2 have stable residual tumor, and 1 has had no recurrence after gross-total resection. Two patients developed postoperative complications of transient sixth cranial nerve palsy and diplopia. There were no other complications.

CONCLUSIONS

SCO poses both a diagnostic and therapeutic challenge. These tumors are often initially misdiagnosed as nonfunctional pituitary adenomas because of their sellar location and nonspecific symptomatology. Postoperatively, SCO must also be distinguished from other neoplasms of the posterior pituitary gland through histopathological examination. Resection of SCO can be challenging, given its highly vascular and adherent nature. Long-term follow-up is critical, as the tumor is associated with higher recurrence and progression rates compared to other benign neoplasms of the sella.

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Colin J. Przybylowski, Robert F. Dallapiazza, Brian J. Williams, I. Jonathan Pomeraniec, Zhiyuan Xu, Spencer C. Payne, Edward R. Laws and John A. Jane Jr.

OBJECTIVE

The object of this study was to compare the outcomes of primary and revision transsphenoidal resection (TSR) of nonfunctioning pituitary macroadenomas (NFPMAs) using endoscopic methods.

METHODS

The authors retrospectively reviewed the records of 287 consecutive patients who had undergone endoscopic endonasal TSR for NFPMAs at their institution in the period from 2005 to 2011. Fifty patients who had undergone revision TSR were retrospectively matched for age, sex, and duration of follow-up to 46 patients who had undergone primary TSR. Medical and surgical complications were documented, and Kaplan-Meier analysis was performed to assess rates of radiological progression-free survival (PFS).

RESULTS

The median follow-up periods were 45 and 46 months for the primary and revision TSR groups, respectively. There were no significant differences between the primary and revision groups in rates of new neurological deficit (0 in each), vascular injury (2% vs 0), postoperative CSF leak (6% vs 2%), transient diabetes insipidus (DI; 15% vs 12%), chronic DI (2% vs 2%), chronic sinusitis (4% vs 6%), meningitis (2% vs 2%), epistaxis (7% vs 0), or suprasellar hematoma formation (0 vs 2%). However, patients who underwent primary TSR had significantly higher rates of syndrome of inappropriate antidiuretic hormone (SIADH; 17% vs 4%, p = 0.04). Patients who underwent primary operations also had significantly higher rates of gross-total resection (GTR; 63% vs 28%, p < 0.01) and significantly lower rates of adjuvant radiotherapy (13% vs 42%, p < 0.01). Radiological PFS rates were similar at 2 years (98% vs 96%) and 5 years (87% vs 80%, p = 0.668, log-rank test).

CONCLUSIONS

Patients who underwent primary TSR of NFPMAs experienced higher rates of SIADH than those who underwent revision TSR. Patients who underwent revision TSR were less likely to have GTR of their tumor, although they still had a PFS rate similar to that in patients who underwent primary TSR. This finding may be attributable to an increased rate of adjuvant radiation treatment to subtotally resected tumors in the revision TSR group.

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Hussam Abou-Al-Shaar, Muhammad M. Abd-El-Barr, Hasan A. Zaidi, Eleanor Russell-Goldman, Rebecca D. Folkerth, Edward R. Laws Jr. and E. Antonio Chiocca

There is a wide group of lesions that may exist in the sellar and suprasellar regions. Embryologically, there is varying evidence that many of these entities may in fact represent a continuum of pathology deriving from a common ectodermal origin. The authors report a case of a concomitant suprasellar craniopharyngioma invading the third ventricle with a concurrent frontal lobe cystic dermoid tumor. A 21-year-old man presented to the authors' service with a 3-day history of worsening headache, nausea, vomiting, and blurry vision. Magnetic resonance imaging depicted a right frontal lobe lesion associated with a separate suprasellar cystic lesion invading the third ventricle. The patient underwent a right pterional craniotomy for resection of both lesions. Gross-total resection of the right frontal lesion was achieved, and subtotal resection of the suprasellar lesion was accomplished with some residual tumor adherent to the walls of the third ventricle. Histopathological examination of the resected right frontal lesion documented a diagnosis of dermoid cyst and, for the suprasellar lesion, a diagnosis of adamantinomatous craniopharyngioma. The occurrence of craniopharyngioma with dermoid cyst has not been reported in the literature before. Such an association might indeed suggest the previously reported hypothesis that these lesions represent a spectrum of ectodermally derived epithelial-lined cystic lesions.

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Timothy R. Smith, David J. Cote, John A. Jane Jr. and Edward R. Laws Jr.

OBJECTIVE

The object of this study was to establish recurrence rates in patients with craniopharyngioma postoperatively treated with recombinant human growth hormone (rhGH) as a basis for determining the risk of rhGH therapy in the development of recurrent tumor.

METHODS

The study included 739 pediatric patients with craniopharyngioma who were naïve to GH upon entering the Genentech National Cooperative Growth Study (NCGS) for treatment. Reoperation for tumor recurrence was documented as an adverse event. Cox proportional-hazards regression models were developed for time to recurrence, using age as the outcome and enrollment date as the predictor. Patients without recurrence were treated as censored. Multivariate logistic regression was used to examine the incidence of recurrence with adjustment for the amount of time at risk.

RESULTS

Fifty recurrences in these 739 surgically treated patients were recorded. The overall craniopharyngioma recurrence rate in the NCGS was 6.8%, with a median follow-up time of 4.3 years (range 0.7–6.4 years.). Age at the time of study enrollment was statistically significant according to both Cox (p = 0.0032) and logistic (p < 0.001) models, with patients under 9 years of age more likely to suffer recurrence (30 patients [11.8%], 0.025 recurrences/yr of observation, p = 0.0097) than those ages 9–13 years (17 patients [6.0%], 0.17 recurrences/yr of observation) and children older than 13 years (3 patients [1.5%], 0.005 recurrences/yr of observation).

CONCLUSIONS

Physiological doses of GH do not appear to increase the recurrence rate of craniopharyngioma after surgery in children, but long-term follow-up of GH-treated patients is required to establish a true natural history in the GH treatment era.

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Edward R. Laws, Judith M. Wong, Timothy R. Smith, Kenneth de los Reyes, Linda S. Aglio, Alison J. Thorne, David J. Cote, Felice Esposito, Paolo Cappabianca and Atul Gawande

OBJECT

Approximately 250 million surgical procedures are performed annually worldwide, and data suggest that major complications occur in 3%–17% of them. Many of these complications can be classified as avoidable, and previous studies have demonstrated that preoperative checklists improve operating room teamwork and decrease complication rates. Although the authors’ institution has instituted a general preoperative “time-out” designed to streamline communication, flatten vertical authority gradients, and decrease procedural errors, there is no specific checklist for transnasal transsphenoidal anterior skull base surgery, with or without endoscopy. Such minimally invasive cranial surgery uses a completely different conceptual approach, set-up, instrumentation, and operative procedure. Therefore, it can be associated with different types of complications as compared with open cranial surgery. The authors hypothesized that a detailed, procedure-specific, preoperative checklist would be useful to reduce errors, improve outcomes, decrease delays, and maximize both teambuilding and operational efficiency. Thus, the object of this study was to develop such a checklist for endonasal transsphenoidal anterior skull base surgery.

METHODS

An expert panel was convened that consisted of all members of the typical surgical team for transsphenoidal endoscopic cases: neurosurgeons, anesthesiologists, circulating nurses, scrub technicians, surgical operations managers, and technical assistants. Beginning with a general checklist, procedure-specific items were added and categorized into 4 pauses: Anesthesia Pause, Surgical Pause, Equipment Pause, and Closure Pause.

RESULTS

The final endonasal transsphenoidal anterior skull base surgery checklist is composed of the following 4 pauses. The Anesthesia Pause consists of patient identification, diagnosis, pertinent laboratory studies, medications, surgical preparation, patient positioning, intravenous/arterial access, fluid management, monitoring, and other special considerations (e.g., Valsalva, jugular compression, lumbar drain, and so on). The Surgical Pause is composed of personnel introductions, planned procedural elements, estimation of duration of surgery, anticipated blood loss and fluid management, imaging, specimen collection, and questions of a surgical nature. The Equipment Pause assures proper function and availability of the microscope, endoscope, cameras and recorders, guidance systems, special instruments, ultrasonic microdoppler, microdebrider, drills, and other adjunctive supplies (e.g., Avitene, cotton balls, nasal packs, and so on). The Closure Pause is dedicated to issues of immediate postoperative patient disposition, orders, and management.

CONCLUSIONS

Surgical complications are a considerable cause of death and disability worldwide. Checklists have been shown to be an effective tool for reducing preventable errors surrounding surgery and decreasing associated complications. Although general checklists are already in place in most institutions, a specific checklist for endonasal transsphenoidal anterior skull base surgery was developed to help safeguard patients, improve outcomes, and enhance teambuilding.

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Timothy R. Smith, M. Maher Hulou, Sandra C. Yan, David J. Cote, Brian V. Nahed, Maya A. Babu, Sunit Das, William B. Gormley, James T. Rutka, Edward R. Laws Jr. and Robert F. Heary

OBJECT

Recent studies have examined the impact of perceived medicolegal risk and compared how this perception impacts defensive practices within the US. To date, there have been no published data on the practice of defensive medicine among neurosurgeons in Canada.

METHODS

An online survey containing 44 questions was sent to 170 Canadian neurosurgeons and used to measure Canadian neurosurgeons’ perception of liability risk and their practice of defensive medicine. The survey included questions on the following domains: surgeon demographics, patient characteristics, type of physician practice, surgeon liability profile, policy coverage, defensive behaviors, and perception of the liability environment. Survey responses were analyzed and summarized using counts and percentages.

RESULTS

A total of 75 neurosurgeons completed the survey, achieving an overall response rate of 44.1%. Over one-third (36.5%) of Canadian neurosurgeons paid less than $5000 for insurance annually. The majority (87%) of Canadian neurosurgeons felt confident with their insurance coverage, and 60% reported that they rarely felt the need to practice defensive medicine. The majority of the respondents reported that the perceived medicolegal risk environment has no bearing on their preferred practice location. Only 1 in 5 respondent Canadian neurosurgeons (21.8%) reported viewing patients as a potential lawsuit. Only 4.9% of respondents would have selected a different career based on current medicolegal risk factors, and only 4.1% view the cost of annual malpractice insurance as a major burden.

CONCLUSIONS

Canadian neurosurgeons perceive their medicolegal risk environment as more favorable and their patients as less likely to sue than their counterparts in the US do. Overall, Canadian neurosurgeons engage in fewer defensive medical behaviors than previously reported in the US.

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Hasan A. Zaidi, Kenneth De Los Reyes, Garni Barkhoudarian, Zachary N. Litvack, Wenya Linda Bi, Jordina Rincon-Torroella, Srinivasan Mukundan Jr., Ian F. Dunn and Edward R. Laws Jr.

OBJECTIVE

Endoscopic skull base surgery has become increasingly popular among the skull base surgery community, with improved illumination and angled visualization potentially improving tumor resection rates. Intraoperative MRI (iMRI) is used to detect residual disease during the course of the resection. This study is an investigation of the utility of 3-T iMRI in combination with transnasal endoscopy with regard to gross-total resection (GTR) of pituitary macroadenomas.

METHODS

The authors retrospectively reviewed all endoscopic transsphenoidal operations performed in the Advanced Multimodality Image Guided Operating (AMIGO) suite from November 2011 to December 2014. Inclusion criteria were patients harboring presumed pituitary macroadenomas with optic nerve or chiasmal compression and visual loss, operated on by a single surgeon.

RESULTS

Of the 27 patients who underwent transsphenoidal resection in the AMIGO suite, 20 patients met the inclusion criteria. The endoscope alone, without the use of iMRI, would have correctly predicted extent of resection in 13 (65%) of 20 cases. Gross-total resection was achieved in 12 patients (60%) prior to MRI. Intraoperative MRI helped convert 1 STR and 4 NTRs to GTRs, increasing the number of GTRs from 12 (60%) to 16 (80%).

CONCLUSIONS

Despite advances in visualization provided by the endoscope, the incidence of residual disease can potentially place the patient at risk for additional surgery. The authors found that iMRI can be useful in detecting unexpected residual tumor. The cost-effectiveness of this tool is yet to be determined.

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Ryan Brewster, Wenya Linda Bi, Timothy R. Smith, William B. Gormley, Ian F. Dunn and Edward R. Laws Jr.

Baseball maintains one of the highest impact injury rates in all athletics. A principal causative factor is the “beanball,” referring to a pitch thrown directly at a batter’s head. Frequent morbidities elicited demand for the development of protective gear development in the 20th century. In this setting, Dr. Walter Dandy was commissioned to design a “protective cap” in 1941. His invention became widely adopted by professional baseball and inspired subsequent generations of batting helmets. As a baseball aficionado since his youth, Walter Dandy identified a natural partnership between baseball and medical practice for the reduction of beaning-related brain injuries. This history further supports the unique position of neurosurgeons to leverage clinical insights, inform innovation, and expand service to society.