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.

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.

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.

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.