Craniopharyngiomas: Challenges and Controversies.

Despite its benign histopathology, the treatment of craniopharyngioma remains one of the most formidable challenges faced by skull base surgeons. The technical challenges of tackling these complex central skull base lesions are paralleled by clinical challenges related to their unique tumor biology and the often-complex decision making required. In this article, we critically appraise the most recent literature to explore the challenges and controversies surrounding the management of these lesions. The role of curative resections and the shift in the surgical paradigm toward the multidisciplinary goal-directed management approach are discussed.

Current Results of Surgical Treatment of Craniopharyngiomas: The Impact of Endoscopic Endonasal Approaches.

Surgery is the main treatment option for the management of craniopharyngiomas. Transcranial microsurgical approaches, such as pterional and subfrontal approaches, have constituted the classic operative strategy for resection of these tumors. However, the development of endoscopic endonasal approaches has revolutionized the treatment of craniopharyngiomas in the last 15 years, and endoscopic resection is favored for most craniopharyngiomas. In this article, we discuss our experience with the management of craniopharyngiomas and review the current results of the surgical treatment of those tumors, including discussion of goals of surgery, complications, recurrences, and the role of adjuvant treatment.

Creative and Innovative Methods and Techniques for the Challenges in the Management of Adult Craniopharyngioma.

Craniopharyngioma remains a major challenge in daily clinical practice. The pathobiology of the tumor is still elusive, and there are no consensus or treatment guidelines on the optimal management strategy for this relatively rare tumor. However, recent technical and scientific advances, including genomic and radiomic profiling, innovation in surgical approaches, more precise radiotherapy protocols, targeted therapy, and restoration of lost functions all have the potential to significantly improve the outcome of patients with craniopharyngioma in the near future. Although many of these innovative tools in the new armamentarium of the clinician are still in their infancy, they could reduce craniopharyngioma-related morbidity and mortality and improve the patients' quality of life. In this article, we discuss these creative and innovative approaches that may offer solutions to the obstacles faced in treating craniopharyngioma and future possibilities in improving the care of these patients.

Surgical Anatomy Applied to the Resection of Craniopharyngiomas: Anatomic Compartments and Surgical Classifications.

BACKGROUND: Resective surgery remains the main treatment option for most patients with craniopharyngiomas. Understanding of the microsurgical anatomy of the sella and suprasellar region and its relationship with these tumors is necessary to achieve effective surgical treatment and minimize complications. In this article, we review the surgical anatomy related to craniopharyngiomas and divide it in 5 compartments according to tumor extension. METHODS: Endoscopic and microsurgical dissection were performed in 3 freshly injected cadaver heads at the Weill Cornell Surgical Innovations Laboratory (New York, New York, USA) and at the Surgical Skills Center at Mount Sinai Hospital (Toronto, Ontario, Canada). Tumor extension was classified as 1) inferomedial or sellar, 2) superomedial or suprasellar, 3) lateral or sylvian, 4) posterior or interpeduncular/prepontine, and 5) intraventricular. The selection of surgical approaches is discussed based on the anatomic nuances of each these regions. In addition, we reviewed the literature regarding previous anatomic classifications for resection of craniopharyngiomas. RESULTS: Different approaches should be considered according to tumor extension into different compartments. Purely sellar tumors are amenable to endoscopic transsellar approaches, whereas those with a suprasellar extension require an extended transtuberculum approach. In some of those patients, a narrow chiasm-pituitary window may block access to the tumor and a transcranial translamina terminalis approach may be favored. Tumors occupying the interpeduncular fossa may pose a significant challenge for an endoscopic endonasal approach and transcranial approaches. Transcavernous approaches and anterior and posterior clinoidectomies may be required for adequate exposure in such patients. Translamina terminalis and/or transcallosal approaches are recommended for resection of purely intraventricular tumors. Tumors extending into the lateral compartment should be considered for transcranial frontotemporal approaches. CONCLUSIONS: The understanding of such anatomic nuances aids in the selection of the most appropriate surgical approach and in the prevention of potential complications. Because most craniopharyngiomas are midline lesions, the endoscopic endonasal approach represents an excellent approach for most of those tumors. However, transcranial approaches should be considered for tumors with extension into the lateral compartment and for selected tumors involving the ventricular compartment (purely intraventricular tumors and those with extension to the foramen of Monro and/or lateral ventricles).

Short Course Hypofractionated Radiotherapy for Frail or Elderly Patients With Meningioma.

Purpose/Objective(s) The incidence of intracranial meningiomas increases with age. The standard of care treatment is complete surgical excision, followed by radiation therapy (RT) if indicated. However, six weeks of RT can be challenging for elderly or frail patients. The purpose of this study was to determine if short course RT is safe and effective in elderly patients with meningioma. Materials/Methods We performed a retrospective analysis of patients with meningioma treated with short course beam RT (5-15 fractions) at a single institution. Seventeen patients (94%) received 4005 cGy over 15 fractions and one patient (6%) received 2500 cGy over five fractions. Study endpoints were treatment toxicity (edema), progression-free (PFS) and overall survival (OS). Results Eighteen patients with histologically proven (n = 12) or radiologically presumed meningioma (n = 6, presumed grade I) were identified. Median age at treatment was 85 years (66-95 years). There were eight, eight and two patients with grade I, II and III tumours, respectively. Eight patients (44%) had radiologic edema prior to RT. Six (33%) required dexamethasone treatment during RT and the dose was increased during RT for two patients. Fourteen patients had reduced or no edema post-RT and 13 patients had stable or improving symptoms post-RT. Six patients had disease progression (five in-field, one out-of-field). Median PFS was 3.3 and 0.9 years for grade I and II/III tumours, respectively (p = 0.014). Median OS was 3.3 and 2.5 years for grade I and II/III tumours, respectively (p = 0.12). Conclusion Short course RT for elderly patients with meningioma is well-tolerated and can offer disease control for some patients, particularly those with grade I tumours.

Long-term outcomes of transsphenoidal surgery for management of growth hormone-secreting adenomas: single-center results.

OBJECTIVE: Transsphenoidal surgery is advocated as the first-line management of growth hormone (GH)-secreting adenomas. Although disease control is defined by strict criteria for biochemical remission, the length of follow-up needed is not well defined in literature. In this report, the authors present their long-term remission rate and identify various predictive factors that might influence the clinical outcome. METHODS: The authors conducted a single-institute retrospective analysis of all transsphenoidal procedures for GH-secreting adenomas performed from January 2000 to June 2016. The primary outcome was defined as biochemical remission according to the 2010 consensus criteria and measured at the 1-year postoperative mark as well as on the last recorded follow-up appointment.Secondary variables included recurrence rate, patterns of clinical presentation, and outcome of adjuvant therapy (including repeat surgery). Subgroup analysis was performed for patients who had biochemical or radiological "discordance"-patients who achieved biochemical remission but with incongruent insulin-like growth factor 1 (IGF-1)/GH or residual tumor on MRI. Recurrence-free survival analysis was conducted for patients who achieved remission at 1 year after surgery. RESULTS: Eighty-one patients (45 female and 36 male) with confirmed acromegaly treated with transsphenoidal surgery were included. In 62 cases the patients were treated with a pure endoscopic approach and in 19 cases an endoscopically assisted microscopic approach was used.Primary biochemical remission after surgery was achieved in 59 cases (73%) at 1 year after surgery. However, only 41 patients (51%) remained in primary surgical remission (without any adjuvant treatment) at their last follow-up appointment, indicating a recurrence rate of 31% (18 of 59 patients) over the duration of follow-up (mean 100 ± 61 months). Long-term remission rates for pure endoscopic and endoscopically assisted cases were not significantly different (48% vs 52%, p = 0.6). Similarly, no significant difference in long-term remission was detected between primary surgery and repeat surgery (54% vs 33%, p = 0.22).Long-term remission was significantly influenced by extent of resection, cavernous sinus invasion (radiologically as well as surgically reported), and preoperative and early postoperative GH and IGF-1 levels (within 24-48 hours after surgery) as well as by clinical grade, with lower remission rates in patients with dysmorphic features and/or medical comorbidities (grade 2-3) compared to minimally symptomatic or silent cases (grade 1). CONCLUSIONS: The long-term surgical remission rate appears to be significantly less than "early" remission rates and is highly dependent on the extent of tumor resection. The authors advocate a long-term follow-up regimen and propose a clinical grading system that may aid in predicting long-term outcome in addition to the previously reported anatomical factors. The role of repeat surgery is highlighted.

Subthalamic deep brain stimulation under general anesthesia and neurophysiological guidance while on dopaminergic medication: comparative cohort study.

OBJECTIVES: The authors have previously reported on the technical feasibility of subthalamic nucleus deep brain stimulation (STN DBS) under general anesthesia (GA) with microelectrode recording (MER) guidance in Parkinsonian patients who continued dopaminergic therapy until surgery. This paper presents the results of a prospective cohort analysis to verify the outcome of the initial study, and report on wider aspects of clinical outcome and postoperative recovery. METHODS: All patients in the study group continued dopaminergic therapy until GA was administered. Baseline characteristics, intraoperative neurophysiological markers, and perioperative complications were recorded. Long-term outcome was assessed using selective aspects of the unified Parkinson's disease rating scale motor score. Immediate postoperative recovery from GA was assessed using the "time needed for extubation" and "total time of recovery." Data for the "study group" was collected prospectively. Examined variables were compared between the "study group" and "historical control group" who stopped dopaminergic therapy preoperatively. RESULTS: The study group, n = 30 (May 2014-Jan 2016), were slightly younger than the "control group," 60 (51-64) vs. 64 (56-69) years respectively, p = 0.043. Both groups were comparable for the recorded intraoperative neurophysiological parameters; "number of MER tracks": 60% of the "study group" had single track vs. 58% in the "control" group, p = 1.0. Length of STN MER detected was 9 vs. 7 mm (median) respectively, p = 0.037. A trend towards better recovery from GA in the study group was noted, with shorter "total recovery time": 60 (50-84) vs. 89 (62-120) min, p = 0.09. Long-term improvement in motor scores and reduction in L-dopa daily equivalent dose were equally comparable between both groups. No cases of dopamine withdrawal or problems with immediate postop dyskinesia were recorded in the "on medications group." The observed rate of dopamine-withdrawal side effects in the "off-medications" group was 15%. CONCLUSIONS: The continuation of dopaminergic treatment for patients with PD does not affect the feasibility/outcome of the STN DBS surgery. This strategy appears to reduce the risk of dopamine-withdrawal adverse effects and may improve the recovery in the immediate postoperative period, which would help enhance patients' perioperative experience.

The effect of dopaminergic therapy on intraoperative microelectrode recordings for subthalamic deep brain stimulation under GA: can we operate on patients 'on medications'?

OBJECTIVES: Microelectrode recording (MER) plays an important role in target refinement in deep brain stimulation (DBS) of the subthalamic nucleus (STN) for Parkinson's disease (PD). Traditionally, patients were operated on in the 'off-medication' state to allow intraoperative assessment of the patient response to direct STN stimulation. The development of intraoperative microelectrode recording (MER) has facilitated the introduction of general anaesthesia (GA). However, the routine withdrawal of dopaminergic medications has remained as standard practice. This retrospective review examines the effect of continuing these medications on intraoperative MER for subthalamic DBS insertion under GA and discusses the clinical implication of this approach. METHODS: Retrospective review of PD patients who had bilateral STN DBS insertion was conducted. A cohort of seven patients (14 STN microelectrodes) between 2012 and 2013, who inadvertently underwent the procedure while 'on medication', was identified. This 'on-medication' group was compared to all other patients who underwent the same procedure between 2012 and 2013 and had their medications withdrawn preoperatively, the 'off-medication' group, n = 26 (52 STN DBS). The primary endpoint was defined as the number of microelectrode tracks required to obtain adequate STN recordings. A second endpoint was the length of MERs that was finally used to guide the DBS lead insertion. The Reduction of the levo-dopa equivalent daily dose (LEDD) was also examined as a surrogate marker for clinical outcome 12 months postoperatively for both groups. For the on-medication group further analysis of the clinical outcome was done relying on the change in the motor examination at 12 months following STN DBS using the following parameters (Hoehn and Yahr scale, the number of waking hours spent in the OFF state as well as the duration of dyskinesia during the ON periods). RESULTS: The on-medication group was statistically comparable in all baseline characteristics to the off-medication group, including age at operation 57 ± 9.9 years vs. 61.5 ± 9.2 years, p = 0.34 (mean ± SD); duration of disease (11.6 ± 5 years vs. 11.3 ± 4 years, p = 0.68); gender F:M ratio (1:6 vs. 9:17, p = 0.40). Both groups had similar PD medication regimes preoperatively expressed as levodopa equivalent daily dose (LEDD) 916 mg (558-1850) vs. 744 mg (525-3591), respectively, p = 0.77. In the on-medication group, all seven patients (14 STN electrodes) had satisfactory STN recording from a single brain track versus 15 out of 26 patients (57.7 %) in the off-medication group, p = 0.06. The length of MER was 4.5 mm (3.0-5.5) in the on-medication group compared to 3.5 mm (3.0-4.5) in the off-medication group, p = 0.16. The percentage of reduction in LEDD postoperatively for the on-medication group was comparable to that in the off-medication group, 62 % versus 58 %, respectively, p > 0.05. All patients in the on-medication group had clinically significant improvement in their PD motor symptoms as assessed by the Hoehn and Yahr scale; the number of hours (of the waking day) spent in the OFF state dropped from 6.9 (±2.3) h to 0.9 (±1.6) h; the duration of dyskinesia during the ON state dropped from 64 % (±13 %) of the ON period to only 7 % (±12 %) at 12 months following STN DBS insertion. CONCLUSION: STN DBS insertion under GA can be performed without the need to withdraw dompaminergic treatment preoperatively. In this review the inadvertent continuation of medications did not affect the physiological localisation of the STN or the clinical effectiveness of the procedure. The continuation of dopamine therapy is likely to improve the perioperative experience for PD patients, avoid dopamine-withdrawal complications and improve recovery. A prospective study is needed to verify the results of this review.

'Goose bumps' as presenting feature of intraventricular glioblastoma multiforme.

'Goose-bumps' seizures are rare manifestations of epilepsy. They are rarely reported by patients and can be easily dismissed by clinicians. Clinically, it carries some diagnostic localising value especially with unilateral onset. In this report, we present a case of intraventricular glioblastoma multiforme with ipsilateral goose bumps and review the literature.

The role of the vascular surgeon in anterior lumbar spine surgery.

OBJECTIVES: Advances in spinal fusion techniques have led to an increase in the need for safe access to the lumbar spine anteriorly. The aim of this study is to examine the procedure-related complications of anterior lumbar inter-body fusion (ALIF) or anterior lumbar disc replacement (ALDR) when performed jointly by a vascular-surgeon and a neurosurgeon in a single centre. METHODS: A retrospective cohort analysis was conducted for all patients who underwent ALIF or ALDR between 2004 and 2010. Operative notes were examined to identify any procedure-specific complications. In-hospital postoperative complications were recorded. Outpatients' records were reviewed to record any late-onset postoperative complications. RESULTS: A total of 121 patients (68 female and 53 males) were included. Mean age was 44 years (range of 25-76). Eighty patients (66%) had ALIF while 24 patients (20%) underwent ALDR. The remaining 17 patients (14%) had combined procedure for multilevel disease. In all patients, a transperitoneal approach was performed by vascular surgeon. The main indication (88%) for performing surgery was degenerative lumbar disc disease. No visceral or 'major vascular' complications were reported in any patients. Only three patients had 'minor vascular' injuries. The only significant postoperative complication was self-limiting paralytic ileus affecting 18 patients (14.8%). Hospital stay ranged from 4 to 9 days (median of 5 days). CONCLUSIONS: The anterior lumbar approach is not generally favoured by many neurosurgeons, despite its many advantages, due to the significant risk of vascular injuries as reported in the literature. This risk is especially acknowledged by the emerging generation of neurosurgeons with very little general surgical exposure during the training years. Adopting a combined vascular and neurosurgical approach has been reported to reduce the risk of vascular injury in anterior lumbar surgery acceptably low. This team approach provides an excellent opportunity to preserve some key 'general' surgical skills for neurosurgeons and ensure safe outcome for the patients.

Steal phenomenon in the lower limb: presentation of a case with osseous metastases secondary to renal cell carcinoma and review of the literature.

Renal cell carcinoma is reported to have potent angiogenic activity with a high microvascular density in both primary and metastatic sites compared with other adenocarcinomas. Angiogenesis can lead to the formation of abnormal arteriovenous shunts that can, in patients with peripheral vascular disease, result in worsening of the degree of ischaemia by producing a vascular steal-like phenomena. Nevertheless, steal phenomena secondary to malignancies are extremely rare. We report a case of distal critical limb ischaemia in a patient with peripheral vascular disease exacerbated by massive arteriovenous shunting due to tibial metastases from renal cell carcinoma.