Development and application of a diagnostic and severity scale to grade post-operative pediatric cerebellar mutism syndrome.

The post-operative pediatric cerebellar mutism syndrome (CMS) affects about one-third of children and adolescents following surgical removal of a posterior fossa tumor (PFT). According to the Posterior Fossa Society consensus working definition, CMS is characterized by delayed-onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children, and is frequently accompanied by additional features such as hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to develop a diagnostic scale to grade CMS duration and severity. Thirty consecutively referred subjects, aged 1-17 years (median 8 years, IQR 3-10), were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in case of CMS, for 30 days after the onset (T0) or until symptom remission. At day 30 (T1), CMS was classified into mild, moderate, or severe according to the proposed scale. CMS occurred in 13 patients (43%, 95% C.I.: 25.5-62.6%), with mild severity in 4 cases (31%), moderate in 4 (31%), and severe in 5 (38%). At T1, longer symptom persistence was associated with greater severity (p = 0.01). Greater severity at T0 predicted greater severity at T1 (p = 0.0001). Children with a midline tumor location and those aged under 5 years at diagnosis were at higher risk of CMS (p = 0.025 and p = 0.008, respectively). In conclusion, the proposed scale is a simple and applicable tool for estimating the severity of CMS at its onset, monitoring its course over time, and providing an early prognostic stratification to guide treatment decisions.

Surgical management of pituitary adenomas: does age matter?

PURPOSE: Along with increased life expectancy and improvements in the diagnostic tools and techniques, the number of elderly patients with symptomatic pituitary tumors being evaluated for surgery is increasing. To date, contrasting results of the safety and effectiveness of transsphenoidal surgery in patients over 65 years old were published in the medical literature. An investigation concerning the extension of resection, post operative complications and, recovery rate from endocrinological and visual symptoms in elderly patients who underwent transsphenoidal surgery for pituitary adenoma was conducted. METHODS: A retrospective review, which included a series of 81 patients 65 years old or older who underwent endoscopic endonasal surgery for pituitary adenomas was analyzed. Pre operative and post operative data were collected and patients were then compared among four age groups: 65-69 years old, 70-74 years old, 75-79 years old and 80 years and over. The results were then compared with the findings from the medical literature review. RESULTS: The statistical analysis failed to highlight any difference in terms of clinical presentation or complications among the four age groups, however there seems to be a slightly higher risk of post operative hypopituitarism in patients over 74 years old. CONCLUSION: The results of the study seem to confirm that transsphenoidal surgery for PA is safe and effective among patients that are 65 and over. Moreover, a partial resection is preferred over a gross total one in order to reduce the surgical risk.

Cortical Bone Trajectory Screw Placement Accuracy with a Patient-Matched 3-Dimensional Printed Guide in Lumbar Spinal Surgery: A Clinical Study.

BACKGROUND: Cortical bone trajectory (CBT) screw is an attractive technique in terms of fixation strength and less invasiveness. However, the insertion of a pedicle screw penetrating cortical bone on the ideal trajectory is technically demanding. The use of 3-dimensional (3D) patient-matched guides may facilitate the use of this technique. In this technical note and case series, the use of a patient-matched 3D targeting guide for a circumferential fixation with CBT screws is described. METHODS: Eleven patients with a mean age of 49 years were treated. The MySpine MC (Medacta International SA, Castel San Pietro, Switzerland) technology was used to place CBT screws. A computed tomography (CT) scan-derived 3D model of the patient vertebra was created after the surgeons planned the best custom CBT screw trajectory. Then, scaffolds were printed and used during surgery to guide the screw through the patient pedicle. An intersomatic arthrodesis was also performed. RESULTS: The images of the planned trajectory were superimposed on the postoperative CT scan, confirming the accuracy of the trajectory. The mean deviation from the planned pedicle midpoint was 0.91 mm; 85.2% of the screws were placed within 2° from the planned trajectory. There were 2 grade A (<2 mm) and no grade B or C perforations. The actual entry point was always within 2 mm from the planned entry point. CONCLUSIONS: This technical note and case series is the first clinical description on the use of a patient-matched guide for posterior CBT screw placement. The use of these devices could also improve placement accuracy and decrease the risk of nerve damage.

Endoscopic Endonasal Approach for Urgent Decompression of Craniovertebral Junction in Syringobulbia.

BACKGROUND: Syringobulbia is an uncommon lesion that occurs in the central nervous system; it is often defined as a pathologic cavitation in the brainstem. The cases with partial blockage of the cerebrospinal fluid pathways at the level of the foramen magnum are more common and the most important group. The most common treatment of syringobulbia is craniovertebral decompression. CASE DESCRIPTION: This paper reports a case of a symptomatic syringobulbia in which an urgent endoscopic endonasal approach to the craniovertebral junction (CVJ) was done to limit bulbo-medullary compression and rapid neurologic deterioration. A 69-year-old man was admitted to the hospital because of acute onset of dysphonia, dysphagia, imbalance, and vomiting. Magnetic resonance imaging revealed a cystic lesion in the brainstem, suggestive of a syringobulbia in Klippel Feil syndrome with CVJ stenosis. CONCLUSIONS: This case report details the successful use of endoscopic endonasal anterior decompression to treat syringobulbia, and adds to the growing literature in support of the endonasal endoscopic approach as a safe and feasible means for decompressing the craniocervical junction, even in the setting of urgency. However, prudent patient selection, combined with sound clinical judgment, access to instrumentation, and intraoperative imaging cannot be overemphasized.

The Endoscopic Endonasal Approach to Craniovertebral Junction Pathologies: Surgical Skills and Anatomical Study.

INTRODUCTION: Surgical anterior decompression is the treatment of choice for symptomatic irreducible ventral craniovertebral junction (CVJ) compression. Along with the classic transoral approach, the endoscopic endonasal approach has evolved and is gaining growing success. MATERIALS AND METHODS: In this work we discuss the surgical technique, give a complete step-by-step description of dissection of the craniovertebral junction and report a specific case of endoscopic endonasal odontoidectomy with use of a high-definition (HD) three-dimensional (3D) endoscope. DISCUSSION: The extended endonasal approach exploits an anatomical corridor to the odontoid process, involving only a small incision in the nasopharynx and sparing palate integrity. The most important limitation of the technique is 2D visualization, which hinders correct recognition of anatomical structures. CONCLUSION: The endoscopic endonasal route to the odontoid process has proven to be a feasible, safe and well-tolerated procedure. Anatomical study is very important for better understanding of the 3D anatomy of the CVJ and relation of critical neurovascular structures to specific bony and muscular landmarks.

Intraoperative neurophysiological monitoring during spinal surgery: technical review in open and minimally invasive approaches.

Neurophysiological monitoring is of undoubted value for the intraoperative safety of neurosurgical procedures. Widely developed and used for cranial surgery, it is equally as effective, though perhaps less commonly employed, for spinal pathology. The most frequently used techniques for intraoperative monitoring during spinal surgery include somatosensory evoked potentials (SSEPs), motor evoked potentials (MEPs) and electromyography, which can either be spontaneous free-running (sEMG) or triggered (tEMG). The knowledge of the benefits and limitations of each modality is essential in optimising the value of intraoperative monitoring during spinal procedures. This review will analyse the single techniques, their anatomical and physiological basis, their use in spinal surgery as reliable indicators of functional injury, their limits and their application to specific procedures in minimally invasive surgery, such as the lateral transpsoas access for interbody fusion and the divergent trajectory for cortico-pedicular screws. In these particular techniques, because of reduced visual exposure, neuromonitoring is indeed essential to exploit the full potential of minimally invasive surgery, while avoiding damage to nervous structures.

Super Giant Growth Hormone-Secreting Pituitary Adenoma in Young Woman: From Ventricles to Nose.

BACKGROUND: Giant pituitary adenomas are rare tumors that can have a devastating impact on a patient's life. They require a well-studied therapeutic approach that often combines different strategies. CASE DESCRIPTION: A 29-year-old woman was diagnosed with the largest GH-secreting pituitary adenoma reported in the literature, to the best of the author's knowledge. The tumor was removed with a combined approach: endoscopic endonasal transsphenoidal and transcortical transventricular. All available acromegaly drugs were used. After 2 surgeries, a large part of the tumor was removed. Both postoperative courses were uneventful. Because the disease was still active, medical therapy was initiated. The combination of pasireotide, pegvisomant, and cabergoline permitted satisfactory control of hormonal levels. CONCLUSIONS: Giant adenomas, >4 cm, are rare pituitary tumors. Therefore in order to achieve the best clinical results, they require complex management that involves a multidisciplinary team of ear, nose, and throat surgeons; endocrinologists; radiation therapists; ophthalmologists; and neurosurgeons.

Nasal Functions in Three-Dimensional Endoscopic Skull Base Surgery.

OBJECTIVES: Endoscopic transnasal transsphenoidal surgery has become the standard procedure for the majority of skull base diseases, including sellar, parasellar, and clival pathologies. The aim of this study was the objective evaluation of nasal airflow resistances and olfactory function in 3-dimensional (3D) endoscopic transnasal transsphenoidal surgery. METHODS: One hundred patients who underwent 3D transnasal endoscopic surgery for sellar, parasellar, and clival diseases were enrolled. Active anterior rhinomanometry and Sniffin' Sticks tests were performed before endoscopic surgery and at 3 and 6 months postoperatively. RESULTS: No significant difference about nasal airflow resistance and olfactory function was observed between preoperative and postoperative subjective and objective scores. In the group of patients with sellar and parasellar diseases, a worst nasal respiratory function was seen when crusting was present, and a worst olfactory function was observed in patients with synechiae. Nasal functions returned to previous levels when crusting or synechiae solved. No statistically significant correlation was observed between the evaluated nasal functions and the reconstruction with flaps. CONCLUSIONS: The 3D endoscopic transnasal transsphenoidal surgery represents a more and more important tool in skull base surgery. It does not determine nasal respiratory and olfactory alterations after the treatment, without an increase in nasal complaints that could worsen quality of life.

"Live Cadaver" Model for Internal Carotid Artery Injury Simulation in Endoscopic Endonasal Skull Base Surgery.

BACKGROUND: Intraoperative injury of the internal carotid artery (ICA) is the most dreaded complication in endoscopic endonasal surgery (EES) of skull base. Training for ICA injury is practically impossible in live operative settings. OBJECTIVE: To evaluate a pulsatile perfusion-based live cadaveric model for ICA injury simulation in a laboratory setting. The major emphasis of the study was to evaluate various means of controlling acute bleeding and evaluating the practical utility of this model for training purposes. METHODS: Five embalmed, uninjected cadaveric heads were prepared for study by connecting to a pulsatile perfusion pump system filled with artificial blood solution. EES approaches were used to evaluate different types of ICA injuries similar to operative scenarios. Various methods of managing ICA injuries such as packing, clipping, and trapping, were evaluated. The educational advantages of the live cadaver model were assessed using questionnaires given to participants in a hands-on dissection course. RESULTS: The trainee was faced with several scenarios similar to those encountered during an actual intraoperative ICA injury. Packing, clipping, and trapping of the ICA injury were successfully achieved in all segments of the ICA. Clip-based reconstruction techniques were successfully developed. All trainees reported gaining new knowledge, learning new techniques. The responses to the questionnaire confirmed the significant educational value of this model. CONCLUSION: The live cadaver model presented here provides real-life experience with major vessel injury during EES in a laboratory setting. This model could significantly improve current training for the management of intraoperative vascular injuries during EES.

Is Pseudotumor Cerebri An Unusual Expression of Chiari Syndrome? A Case Report and Review of the Literature.

The Chiari I malformation (CM-I) is a developmental alteration of the posterior cranial fossa (PCF), radiographically defined as the descent of the cerebellar tonsils ≤ 5 mm below the foramen magnum (FM) inside the cervical canal. Headache is the most frequent symptom associated with CM-I. The association of CM-I and neurological symptoms configures with Chiari syndrome. A rare symptom associated with Chiari syndrome is intracranial hypertension syndrome with cephalea and papilloedema-the typical findings of pseudotumor cerebri (PTC). PTC is a syndrome characterized by signs and symptoms of increased intracranial pressure (ICP) in the absence of space-occupying masses and/or obstruction of the ventricular system detectable by neuroimaging. The most common symptoms are headache and visual disturbances. Literature reports that the association between CM-I and PTC has a prevalence of 2-6%. More recently, a prevalence of 11% has been described in a specific subset of obese or overweight female patients between 20 and 40 years old. Here we report the case of a 38-year-old woman who came to our observation with a clinical picture and neuroradiological examinations compatible with both CM-I and PTC. We discuss the clinical case and the significant improvement after surgical occipito-cervical decompression.

Scalp Metastasis from Glioblastoma Multiforme: A Case Report and Literature Review.

Glioblastoma multiforme (GBM) is a common malignant brain tumor that rarely metastasizes extracranially, despite its aggressive clinical course. This report details the case of a young man presenting with a single subcutaneous localization of GBM that arose six months after initial surgery and recurred after excision. Only six other cases of scalp metastasis of GBM following surgery have been described in the literature, each with peculiar features. Whenever feasible, surgery is the most effective way to obtain local control of disease. However, a correct approach must be carefully planned to minimize the risks of recurrence and wound dehiscence.

Detailed anatomy knowledge: first step to approach petroclival meningiomas through the petrous apex. Anatomy lab experience and surgical series.

Petroclival meningiomas are a challenge for neurosurgeons due to the complex anatomy of the region that is rich of vessels and nerves. A perfect and detailed knowledge of the anatomy is very demanding in neurosurgery, especially in skull base surgery. The authors describe the microsurgical anatomy to perform an anterior petrosectomy based on their anatomical and surgical experience and perform a literature review. The temporal bone is the most complex and fascinating bone of skull base. The apex is located in the angle between the greater wing of the sphenoid and the occipital bone. Removing the petrous apex exposes the clivus. The approach directed through the temporal bone in this anatomical area is referred to as an anterior petrosectomy. The area that must be drilled is the rhomboid fossa that is defined by the Kawase, premeatal, and postmeatal triangles. In Division of Neurosurgery - University of Turin, 130 patients, from August 2013 to September 2015, underwent surgical resection of intracranial meningiomas. In this group, we have operated 7 PCMs and 5 of these were approached performing an anterior petrosectomy with good results. In our conclusions, we feel that this surgery require an advanced knowledge of human anatomy and a specialized training in interpretation of radiological and microsurgical anatomy both in the dissection lab and in the operating room.

Craniovertebral junction 360°: A combined microscopic and endoscopic anatomical study.

OBJECTIVES: Craniovertebral junction (CVJ) can be approached from various corridors depending on the location and extent of disease. A three-dimensional understanding of anatomy of CVJ is paramount for safe surgery in this region. Aim of this cadaveric study is to elucidate combined microscopic and endoscopic anatomy of critical neurovascular structures in this area in relation to bony and muscular landmarks. MATERIALS AND METHODS: Eight fresh-frozen cadaveric heads injected with color silicon were used for this study. A stepwise dissection was done from anterior, posterior, and lateral sides with reference to bony and muscular landmarks. Anterior approach was done endonasal endoscopically. Posterior and lateral approaches were done with a microscope. In two specimens, both anterior and posterior approaches were done to delineate the course of vertebral artery and lower cranial nerves from ventral and dorsal aspects. RESULTS: CVJ can be accessed through three corridors, namely, anterior, posterior, and lateral. Access to clivus, foreman magnum, occipital cervical joint, odontoid, and atlantoaxial joint was studied anteriorly with an endoscope. Superior and inferior clival lines, supracondylar groove, hypoglossal canal, arch of atlas and body of axis, and occipitocervical joint act as useful bony landmarks whereas longus capitis and rectus capitis anterior are related muscles to this approach. In posterior approach, spinous process of axis, arch of atlas, C2 ganglion, and transverse process of atlas and axis are bony landmarks. Rectus capitis posterior major, superior oblique, inferior oblique, and rectus capitis lateralis (RCLa) are muscles related to this approach. Occipital condyles, transverse process of atlas, and jugular tubercle are main bony landmarks in lateral corridor whereas RCLa and posterior belly of digastric muscle are the main muscular landmarks. CONCLUSION: With advances in endoscopic and microscopic techniques, access to lesions and bony anomalies around CVJ is becoming easier and straightforward. A combination of microscopic and endoscopic techniques is more useful to understand this anatomy and may aid in the development of future combined approaches.

3D HD Endoscopy in Skull Base Surgery: From Darkness to Light.

The introduction of modern endoscopy in neurosurgery brought light to one of the darkest areas: the skull base. In particular, the trans-nasal route allowed skull base surgeons to visualize and dominate the entire midline skull base. We analyzed our surgical series of 199 patients that were affected by several pathologies (pituitary adenomas, clivalchordomas, craniopharyngioma, Rathke's cleft cysts, tuberculum sellaemeningiomas, and craniovertebral junction pathologies with bulbar compression) and treated each by using a 3D-HD endoscope between December 2012 and December 2015 and reviewed the literature. We present our results in terms of tumor resections and decompression inpatients affected by craniovertebral junction pathologies. Analyzing our direct experience, as well as the literature, we can assert that the amount and accuracy of necessary movements in order to achieve a determined target are affected by the screen clarity and image resolution of the device. Additionally, the experience of the surgeon has an important role in the surgical outcome. Moreover, depth perception is critical in order to obtain precise and accurate movements. Our observations and the experts' opinion indicate that this modality provides improved surgical dexterity by affording the surgeon with depth perception while manipulating tissue and maneuvering the endoscope in the endonasal corridor, which is especially crucial in reducing the learning curve of young neurosurgeons.

Nanofibrous Synthetic Dural Patch for Skull Base Defects: Preliminary Experience for Reconstruction after Extended Endonasal Approaches.

Setting One of the consequences of the widespread use of endoscopic endonasal approaches (EEA) to skull base pathologies is the management of complex skull base defects. Nowadays, the gold standard is a multilayer closure that reproduces the physiological tissue barriers. Several techniques have been described in the literature; however, skull base reconstruction after EEA still represents a matter of debate, especially after extended EEA. A watertight closure is paramount to prevent cerebrospinal fluid leak and meningitis. Design Regarding this issue, we present our experience with a new synthetic dural patch, ReDura (Medprin Biotech, La Mirada, California, United States), as a subdural inlay in three patients who underwent endoscopic endonasal removal of sellar and suprasellar lesions. Conclusions ReDura patch showed the same versatility of autologous iliotibial tract. A dural patch that easily adapts to all defects, revealed to be a useful tool for performing watertight closure, possibly in a short operative time, after endoscopic approaches.

Endoscopic Endonasal Approach to the Odontoid Pathologies.

BACKGROUND: Surgical anterior decompression represents the treatment of choice for symptomatic irreducible ventral craniovertebral junction (CVJ) compression. With the refinement of the endoscopic techniques, the endonasal route has been proposed as alternative to the classic transoral approach to CVJ. Some reports assess the effectiveness and safety of endoscopic endonasal approaches to CVJ pathologies. MATERIALS AND METHODS: From July 2011 to February 2014, 12 patients with symptomatic nonreducible ventral spinal cord compression underwent purely 3-dimensional endoscopic endonasal odontoidectomy in our department. The surgical technique is described. RESULTS: A good brainstem-medullary decompression was achieved in all patients. In 10 of 12 patients the endotracheal tube was removed just after the procedure with good recovery of the respiratory function. We report no cases of velopharyngeal insufficiency. In 5 of 12 patients the preservation of C1 anterior was achieved, without the need for posterior cervical fixation. DISCUSSION AND CONCLUSIONS: Endoscopic endonasal odontoidectomy has proven to be safe and effective in selected patients. Soft and hard palate preservation dramatically reduces the risk of postoperative velopharyngeal insufficiency. Moreover, the endonasal endoscopic approach provides a direct access to the dens. Three-dimensional high-definition endoscope, laser, and ultrasound bony curettes revealed to be useful tools for this approach that, however, remains a demanding one.

Three-Dimensional Endoscopic Endonasal Approach and Outcomes in Sellar Lesions: A Single-Center Experience of 104 Cases.

BACKGROUND: Pituitary tumors account for approximately 15% of primary intracranial neoplasms and often are diagnosed incidentally. Common sellar lesions are pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. Currently endoscopic transsphenoidal approaches are largely used, and many centers also use 3-dimensional (3D) technology, although additional results of long-term follow-up are still being accumulated. METHODS: We present a retrospective analysis of 104 patients with sellar lesions (57 male and 47 female, mean age 52 years) who underwent 3D endoscopic transsphenoidal surgery in our center from December 2011 to March 2015 and were followed up for a mean time of 18 months. RESULTS: A total of 16 patients were diagnosed with growth hormone-secreting adenomas, 9 with adrenocorticotropic hormone-secreting, 5 with prolactin-secreting, and 2 with thyroid-stimulating hormone-secreting tumors and 58 with nonsecreting pituitary adenomas. Five patients had Rathke cleft cysts, 5 craniopharyngiomas, 2 fibrous solitary tumors, 1 a metastasis, and 1 a chordoma. At baseline, 47 patients (45.2%) had hormonal changes and 52 (50%) had visual field changes. Complete resection was achieved in 73 patients (70.1%). Follow-up evaluation detected hormonal remission in 7 patients with Cushing disease (77.7%) and in 11 patients with acromegaly (68.7%). Complications were cerebrospinal fluid leak in 5 cases (4.8%) and transient diabetes insipidus in 6 cases (5.7%). A total of 55 patients (52.9%) were discharged less than 72 hours after surgery. CONCLUSIONS: The use of an endoscopic endonasal approach with 3D technology provides several advantages relating to patient length of stay, rate of complications, postoperative recovery, and novice surgeons' training. Advantages of 3D endoscopy and long-term follow-up still need further elucidation.

Three-dimensional endoscopy in transnasal transsphenoidal approach to clival chordomas.

BACKGROUND: The purpose of this prospective, observational study was to evaluate the management of skull base chordomas surgically resected via a 3D-endoscopic transnasal approach. METHODS: Thirteen consecutive patients were observed and only 9 were surgically treated using a 3D-endoscopic transnasal approach assisted by a novel 3D visualization system. RESULTS: Nine consecutive male patients (mean age, 57.4 years) underwent exclusive 3D-endoscopic transnasal transsphenoidal resection of clival chordomas; gross total resection was achieved in 66.6% of cases (6 of 9 patients), near-total resection in 11.2% (1 of 9 patients), and partial resection in 22.2% (2 of 9 patients). The complications observed were 2 cases of postoperative cerebrospinal fluid (CSF) leaks and 1 case of temporary VI cranial nerve palsy. CONCLUSION: No discomfort was recorded; when a dura opening was required, 3D vision allowed an accurate intradural sharp dissection and a precise repair of the skull base. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1814-E1819, 2016.