Second-look surgery after pediatric brain tumor resection - Single center analysis of morbidity and volumetric efficacy.

Introduction: Postoperative residual tumor can occur for intentional or unintentional reasons. Decision-making regarding second-look surgery has to weigh molecular biology, probability of total resection and prognostic relevance against potential additional morbidity. In interdisciplinary tumor boards the neurosurgeon has to estimate risk and efficacy of second-look surgery in individual cases, based on precise data. Research question: Aim of this study was to provide such data by analyzing morbidity and volumetric efficacy of second-look surgery at a designated pediatric neuro-oncology unit. Material and methods: Children who received second-look surgery in 2007-2018 after incomplete resections were analyzed retrospectively. Measurements were performed on early postoperative magnetic resonance imaging, comparing axial diameter-based measurement as well as computer-assisted volumetric analysis. Results: 59 patients (37% of the overall cohort; 21 female; mean age: 8 â€‹± â€‹5 years) received a subtotal (n â€‹= â€‹35) or near total (n â€‹= â€‹24) resection. After interdisciplinary case review, 12 of these patients received second-look surgery mainly for residual ependymoma. This led to further tumor volume reduction in all cases (new degrees of resection: subtotal â€‹= â€‹2, near total â€‹= â€‹6, gross total â€‹= â€‹4). No new permanent morbidity or perioperative mortality was observed. Discussion and conclusion: Second-look surgery did not increase mortality and permanent morbidity, had an 8% rate of transient morbidity and achieved tumor volume reduction above 95% in 75% of selected cases, with 4 additional gross total resections. Second-look surgery is safe and effective with regard to volumetric outcome parameters even in cases with good initial resections, although the role of second-look surgery regarding oncological outcome has to be further investigated in times of personalized molecular medicine.

Structural damage burden and hypertrophic olivary degeneration in pediatric postoperative cerebellar mutism syndrome.

Cerebellar mutism syndrome (CMS) occurs in one out of four children after posterior fossa tumor surgery, with open questions regarding risk factors, pathophysiology, and prevention strategies. Because of similarities between several cerebellar syndromes, a common pathophysiology with damage to the dentato-thalamo-cortical and dentato-rubro-olivary pathways has been proposed. Hypertrophic olivary degeneration (HOD) is an imaging correlate of cerebellar injury observed for instance in stroke patients. Aim of this study was to investigate whether the occurrence and severity of CMS correlates with the extent of damage to the relevant anatomical structures and whether HOD is a time-dependent postoperative neuroimaging correlate of CMS. We performed a retrospective single center study of CMS patients compared with matched non-CMS controls. CMS occurred in 10 children (13% of the overall cohort) with a median age of 8 years. Dentate nucleus (DN) injury significantly correlated with CMS, and superior cerebellar peduncle (SCP) injury was associated by tendency. HOD was observed as a dynamic neuroimaging phenomenon in the postoperative course and its presence significantly correlated with CMS and DN injury. Children who later developed HOD had an earlier onset and tended to have longer persistence of CMS. These findings can guide surgical measures to protect the DN and SCP during posterior fossa tumor resections and to avoid a high damage burden (i.e., bilateral damage). Development of intraoperative neuromonitoring of the cerebellar efferent pathways as well as improved preoperative risk stratification could help to establish a patient-specific strategy with optimal balance between degree of resection and functional integrity.

Functional tracts of the cerebellum-essentials for the neurosurgeon.

The cerebellum is historically implicated in motor coordination, but accumulating modern evidence indicates involvement in non-motor domains, including cognition, emotion, and language. This correlates with the symptoms observed in postoperative cerebellar mutism syndrome (CMS). Profound knowledge of cerebellar functional topography and tractography is important when approaching cerebellar tumors, as surgical trauma to relevant structures of cerebellar pathways plays a role in the pathogenesis of CMS. The aim of this systematic review is to provide a concise overview of relevant modern neuroimaging data and cerebellar functional tracts with regard to neurosurgical procedures.

Barrel Stave Osteotomy Decompression for Acute Brain Injury in Infants: Technical Note.

Decompressive craniectomy (DC) is rarely required in infants, but when performed several aspects should be considered: These youngest patients are vulnerable to blood loss and cranial reconstruction can be challenging due to skull growth and bone flap resorption. On the other hand, infants have thin and flexible bone and osteogenic potential. The authors propose a technique which makes use of these unique aspects by achieving decompression with the craniofacial method of barrel stave osteotomy, aiming to achieve adequate DC, limit perioperative risks and facilitate subsequent cranial reconstruction.

Calcifying Pseudoneoplasm of Neuraxis (CAPNON) in the Posterior Third Ventricle-Challenge for Neuroendoscopy.

BACKGROUND: We report the first case of a purely intraventricular calcifying pseudoneoplasm of neuraxis (CAPNON) in the posterior third ventricle. CASE DESCRIPTION: A 63-year-old male without any previous medical history presented with Hakim triad. Imaging showed a calcified lesion of the posterior third ventricle with hydrocephalus. An endoscopic third ventriculostomy was performed. Endoscopic removal or debulking of the lesion was impossible due to its rock-hard consistency, and thus the procedure was aborted after biopsy. CONCLUSIONS: When encountering such calcified lesions within the ventricular system, especially in proximity to eloquent regions, the decision making process should include the hard consistency and parenchymal adhesions as obstacles to neuroendoscopic removal. Even for biopsy, a higher morbidity rate compared with typical soft tumors should be assumed. Although data on intraventricular CAPNON is limited, biopsy of the lesion and treatment of associated hydrocephalus appear to be the primary neurosurgical goals, followed by imaging surveillance.

From decompressive craniectomy to cranioplasty and beyond-a pediatric neurosurgery perspective.

PURPOSE: Decompressive craniectomy (DC) is an established neurosurgical emergency technique. Patient selection, optimal timing, and technical aspects related to DC and subsequent cranioplasty remain subjects of debate. For children, the overall degree of evidence is low, compared with randomized controlled trials (RCTs) in adults. METHODS: Here, we present a detailed retrospective analysis of pediatric DC, covering the primary procedure and cranioplasty. Results are analyzed and discussed in the light of modern scientific evidence, and conclusions are drawn to stimulate future research. RESULTS: The main indication for DC in children is traumatic brain injury (TBI). Primary and secondary DC is performed with similar frequency. Outcome appears to be better than that in adults, although long-term complications (especially bone flap resorption after autologous cranioplasty) are more common in children. Overt clinical signs of cerebral herniation prior to DC are predictors of poor outcome. CONCLUSIONS: We conclude that DC is an important option in the armamentarium to treat life-threatening intracranial hypertension, but further research is warranted, preferentially in a multicenter prospective registry.

Decompressive craniectomy for acute ischemic stroke.

Malignant stroke occurs in a subgroup of patients suffering from ischemic cerebral infarction and is characterized by neurological deterioration due to progressive edema, raised intracranial pressure, and cerebral herniation. Decompressive craniectomy (DC) is a surgical technique aiming to open the "closed box" represented by the non-expandable skull in cases of refractory intracranial hypertension. It is a valuable modality in the armamentarium to treat patients with malignant stroke: the life-saving effect has been proven for both supratentorial and infratentorial DC in virtually all age groups. This leaves physicians with the difficult task to decide who will require early or preemptive surgery and who might benefit from postponing surgery until clear evidence of deterioration evolves. Together with the patient's relatives, physicians also have to ascertain whether the patient will have acceptable disability and quality of life in his or her presumed perception, based on preoperative predictions. This complex decision-making process can only be managed with interdisciplinary efforts and should be supported by continued research in the age of personalized medicine.

Risk factors for in-brain local progression in elderly patients after resection of cerebral metastases.

Intracranial metastases are the most frequent brain tumor with recurrence rates after treatment of around 40-60%. Age is still considered a determinant of treatment and prognosis in this pathology. Recent studies analyzing the impact of metastasectomy in elderly patients focused on reporting perioperative mortality and morbidity rates but not on the evaluation of oncological outcome parameters. Aim of this study is to determine risk factors for in-brain local recurrence after brain surgery in this sub-population. From October 2009 until September 2016 all patients aged 65 years and above with histopathologically confirmed metastasis after surgical resection were retrospectively studied. Clinical, radiological and perioperative information was collected and statistically analysed. Follow-up consisted of clinical and radiological assessment every 3-months following surgery. 78 patients were included, of these 50% were female (39 patients). Median age was 71 years (66-83). Early postoperative-MRI verified a complete surgical resection in 41 patients (52.6%) and showed a tumor-remnant in 15 patients (19.2%). In 22 patients the MRI result was inconclusive (28.2%). None of the patients experienced severe complications due to surgery. The median postoperative NIHSS was adequate 1 ± 1.4 (0-6), nonetheless, insignificantly improved in comparison to the preoperative NIHSS (p = 0.16). A total of 20 patients (25.6%) presented local recurrence. The only statistically significant factor for development of local in-brain recurrence after resection of cerebral metastases in patients above 65 years of age was a tumor-remnant in the early postoperative MRI (p = 0.00005). Median overall survival was 13 months. Local in-brain recurrence after surgical resection of a cerebral metastasis in patients above 65 years of age was 25.6%. In our analysis, tumor-remnant in early postoperative MRI is the only risk factor for local in-brain recurrence. Oncological parameters in the present cohort do not seem to differ from recent phase III studies with non-geriatric patients. Nevertheless, controlled studies on the impact of metastasectomy in elderly patients delivering high quality reliable data are required.

Conservative and operative management of iatrogenic craniocerebral disproportion-a case-based review.

INTRODUCTION: Craniocerebral disproportion (CCD) can occur as a sequela after shunting in early infancy. It can be understood as a disorder closely related to slit ventricle syndrome and chronic overdrainage syndrome. Here, we present two exemplary cases and summarize the pathophysiological, diagnostic, and therapeutic approaches to CCD. CLINICAL PRESENTATION: Two premature babies underwent shunting for posthemorrhagic hydrocephalus and presented in later childhood with recurrent episodes of symptomatic raised intracranial pressure (ICP) at 2 and 8 years of age, respectively. DIAGNOSIS AND MANAGEMENT: Both patients had unchanged ventricular size on cranial imaging and fulfilled the clinical diagnostic criteria of CCD. After confirming shunt patency, ICP monitoring was performed to diagnose intermittent intracranial hypertension. Different treatment pathways were pursued: While readjustment of a programmable shunt valve was sufficient to alleviate the raised ICP in the first case, a cranial expansion surgery was necessary in the second case. OUTCOME AND CONCLUSIONS: Both children were treated successfully after thorough assessment and careful choice of treatment approaches. This review provides detailed insight into CCD and highlights the importance of individual and critical decision-making in these complex patients.

Is 5-ALA fluorescence of cerebral metastases a prognostic factor for local recurrence and overall survival?

BACKGROUND: 5-Aminolevulinic acid (5-ALA) fluorescence-guided resection technique was first introduced for malignant glioma. However, the impact of the 5-ALA fluorescence behaviour of cerebral metastases is still unclear. Aim of this study was to determine the impact of PpIX-fluorescence on the local progression-free and overall survival. MATERIALS AND METHODS: A secondary analysis was performed and included an updated follow-up of 136 patients comprised in two previous studies. Additionally, 82 new patients were included. All patients underwent surgical resection of cerebral metastasis and intraoperative estimation of 5-ALA-induced fluorescence. The 5-ALA fluorescence behaviour of cerebral metastases was correlated with the rate of local recurrences, the local progression-free and overall survival. RESULTS: 218 patients suffering from cerebral metastatic spread fulfilled the inclusion criteria and were analysed: complete surgical resection could be achieved in 123/218 patients (56.4%). Dichotomised degree of surgical resection (complete vs. incomplete or questionable complete resection) was not related to dichotomized 5-ALA fluorescence of cerebral metastases (p = 0.66). 51 patients (23.4%) developed a local in-brain progression within or at the border of the resection cavity. Of these, 8 patients showed a PpIX-fluorescent metastasis. There was a trend towards a correlation between a higher local in-brain progression in PpIX-non-fluorescent metastases (p = 0.03). Median time to local in-brain progression was 4 ± 11 months. PpIX-fluorescent and PpIX-non-fluorescent metastases showed a significantly different progression-free survival (p = 0.01). PpIX-positive and -negative metastases showed a significantly different overall survival (20 and 14 months respectively; p = 0.006). CONCLUSION: The 5-ALA fluorescence behaviour was related to the local progression-free and the overall survival in the present retrospective series and might be considered a prognostic marker. Further studies are required to appreciate the oncological impact of the 5-ALA induced fluorescence behaviour of cerebral metastases.

Endoscopic Lavage of Extensive Chronic Subdural Hematoma in an Infant After Abusive Head Trauma: Adaptation of a Technique From Ventricular Neuroendoscopy.

Subdural fluid collections are frequently encountered in young children after non-accidental injury. In a subset of patients, these collections progress in size and ultimately require permanent drainage, which is commonly achieved with subdural-peritoneal shunts. However, excessive protein and cellular contents in the fluid are potential risk factors for shunt failure. Here, we describe the adaptation of an endoscopic lavage technique established for ventricular endoscopy with the aim of improving fluid condition prior to shunting. We present a case of subdural fluid collections secondary to non-accidental injury, where permanent shunting was required but could not be performed due to excessive protein and cellular levels in the subdural fluid despite conventional burr hole drainage. A two-month-old male infant presented with a bulging and tense fontanel, a reduced level of consciousness, bradycardia, and significant macrocephaly. Computed tomography (CT) demonstrated massive bilateral, low attenuation subdural fluid collections, reaching a diameter of 4.5 cm. Emergency burr hole washout and insertion of subdural drains was performed. Despite prolonged drainage over 10 days, the protein level remained at 544 mg/dl and the mean erythrocyte count at 6,493/µl. Continuous drainage was required to avoid clinical deterioration due to raised intracranial pressure; however, the fluid condition was still considered incompatible with permanent subdural-peritoneal shunting. We, therefore, performed an endoscopic subdural lavage with a careful evacuation of residual blood deposits. No complications were encountered. Postoperatively, mean protein level was 292 mg/dl and mean erythrocyte count was 101/µl. Endoscopic lavage could be safely performed in a case of extensive subdural low attenuation fluid collections, where conventional burr hole drainage failed to improve protein and cellular contents as a prerequisite for successful permanent shunting. We conclude that adaptation of this technique can be helpful in selected cases as an alternative procedure.

Can the combination of hyperthermia, seizures and ion channel dysfunction cause fatal post-ictal cerebral edema in patients with SCN1A mutations?

A 21-year-old male with an SCN1A mutation died of cerebral herniation 3 h after a seizure occurring during physical activity. Cases of fatal cerebral edema in patients with SCN1A mutations after fever and status epilepticus have been recently reported raising the question whether sodium channel dysfunction may contribute to cerebral edema and thereby contribute to the increased premature mortality in Dravet Syndrome. We report on our patient and discuss whether the combination of hyperthermia and ion channel dysfunction may not only trigger seizures but also a fatal pathophysiological cascade of cerebral edema and herniation leading to cardiorespiratory collapse.

First Clinical Applications of a High-Definition Three-Dimensional Exoscope in Pediatric Neurosurgery.

The ideal visualization tools in microneurosurgery should provide magnification, illumination, wide fields of view, ergonomics, and unobstructed access to the surgical field. The operative microscope was the predominant innovation in modern neurosurgery. Recently, a high-definition three-dimensional (3D) exoscope was developed. We describe the first applications in pediatric neurosurgery. The VITOM 3D exoscope (Karl Storz GmbH, Tuttlingen, Germany) was used in pediatric microneurosurgical operations, along with an OPMI PENTERO operative microscope (Carl Zeiss AG, Jena, Germany). Experiences were retrospectively evaluated with five-level Likert items regarding ease of preparation, image definition, magnification, illumination, field of view, ergonomics, accessibility of the surgical field, and general user-friendliness. Three operations were performed: supratentorial open biopsy in the supine position, infratentorial brain tumor resection in the park bench position, and myelomeningocele closure in the prone position. While preparation and image definition were rated equal for microscope and exoscope, the microscope's field of view, illumination, and user-friendliness were considered superior, while the advantages of the exoscope were seen in ergonomics and the accessibility of the surgical field. No complications attributed to visualization mode occurred. In our experience, the VITOM 3D exoscope is an innovative visualization tool with advantages over the microscope in ergonomics and the accessibility of the surgical field. However, improvements were deemed necessary with regard to field of view, illumination, and user-friendliness. While the debate of a "perfect" visualization modality is influenced by personal preference, this novel visualization device has the potential to become a valuable tool in the neurosurgeon's armamentarium.

Cerebral amyloidoma is characterized by B-cell clonality and a stable clinical course.

Amyloidomas are rare amyloid-containing lesions, which may also occur in the central nervous system. Etiology, pathogenesis and clinical course are poorly understood. To gain more insight into the biology of cerebral amyloidoma, they aimed to characterize its histopathological, molecular and clinical features in a retrospective series of seven patients. FFPE tissue specimens were examined using immunohistochemistry, chromogenic in situ hybridization (CISH) for light chains kappa and lambda as well as an IgH gene clonality analysis. Follow-up information was gathered by reviewing patient records and imaging results. Median age of the three males and four females was 50 years (range: 35-53 years). All cerebral amyloidomas were located supratentorially and were classified as lambda light chain amyloidosis (AL-λ; n = 6) and kappa light chain amyloidosis (AL-κ; n = 1) on immunohistochemistry and CISH. B-cell clonality was confirmed by IgH gene clonality assay in all cases examined. After a median follow-up of 21 months, all patients were alive and showed stable disease. No progression to systemic disease was observed. In conclusion, their data suggest that cerebral amyloidoma is a local disease characterized by B-cell clonality and associated with a stable clinical course.

Various shades of red-a systematic analysis of qualitative estimation of ALA-derived fluorescence in neurosurgery.

5-Aminolevulinic acid (5-ALA)-fluorescence-guided resection is well established in many neuro-oncologic centers. Different classifications of 5-ALA-induced fluorescence have been reported. The aim of the systematic analysis was to evaluate the frequency of graduations, definitions, and designations of 5-ALA-induced fluorescence qualities. A systematic database search of PubMed was performed to identify studies reporting (1) on 5-ALA fluorescence-guided either spinal or cranial surgery, (2) on qualitative estimation and/or categorization of 5-ALA-induced fluorescence, (3) in English, and (4) were published as peer-reviewed original studies. Totally, 93 studies were identified. Different classification systems of 5-ALA-induced fluorescence were found. Over 60 % of the included studies used a dichotomized categorization of 5-ALA-induced fluorescence and 27.5 % of studies distinguished two different intensities of 5-ALA fluorescent tissue in addition to non-fluorescing tissue. More than 50 % of studies explicitly defined criteria for categorization of 5-ALA-induced fluorescence. The major limitation of the present analysis might be that it mainly comprises data from retrospective, uncontrolled, non-randomized trials. However, a precise definition of each 5-ALA-induced fluorescence quality is essential. Although dichotomized classification is the most common and simple graduation system, it may not be suitable for every clinical or scientific task. A three-level 5-ALA-induced fluorescence classification with precise definition of each fluorescence quality and their correlation with histological features would be more useful and reproducible in these cases.

Predictors for a further local in-brain progression after re-craniotomy of locally recurrent cerebral metastases.

Treatment of recurrent cerebral metastases is an emerging challenge due to the high local failure rate after surgery or radiosurgery and the improved prognosis of patients with malignancies. A total of 36 patients with 37 metastases who underwent surgery for a local in-brain progression of a cerebral metastasis after previous metastasectomy were retrospectively analyzed. Degree of surgical resection on an early postoperative MRI within 72 h after surgery was correlated with the local in-brain progression rate and overall survival. Complete surgical resection of locally recurrent cerebral metastases as confirmed by early postoperative MRI could only be achieved in 37.8%. Detection of residual tumor tissue on an early MRI following recurrent metastasis surgery correlated with further local in-brain progression when defining a significance level of p = 0.05 but not after Sidák or Bonferroni significance level correction for multiple testing: However, definite local tumor control could finally be achieved in 91.9% after adjuvant therapy. Overall survival after recurrent metastasectomy was significantly higher as predicted by diagnosis-specific graded prognostic assessment (12.9 ± 2.3 vs. 8.4 ± 0.7 months; p < 0.0001). However, our series involved a limited number of heterogeneous patients. A larger, prospective, and controlled study is required. Considering the adequate local tumor control achieved in the vast majority of patients, surgery of recurrent metastases may represent one option in a multi-modal treatment approach of patients suffering from locally recurrent cerebral metastases.

Outcome following surgical treatment of chronic subdural hematoma in the oldest-old population.

As a result of the demographic shift in western societies, the mean age at presentation of patients suffering from chronic subdural hematomas (cSDH) is increasing. Therapeutic strategies, surgical and non-surgical, need to be reevaluated and adapted accordingly. Age is considered to be a positive risk factor for a higher perioperative morbidity and mortality. The purpose of this study is to determine if old age (≥85 years) should be seen as a contraindication for surgical treatment. Two groups (56 patients each) with cSDH over and below 85 years of age from a single neurosurgical department with well-defined surgical treatment guidelines were retrospectively analyzed. Clinical characteristics of the patients, localization, treatment, prior medication, and complications were compared. Outcome was measured by clinical improvement postoperatively and by the Glasgow Outcome Scale (GOS) at 1 month after surgery. Age ≥85 years was associated with higher GOS 1 month after surgery (p = 0.038). 51.8% (58) of all patients had a complete neurological recovery postoperatively, and 74% (43) of these patients were ≥85 years. Elderly patients suffered from a significantly higher complication rate (p < 0.001) with odds of having a complication 18.3 times higher (p < 0.001) compared to patients <85 years. Both groups had a comparable mean hospitalization time (9.8 days for patients ≥85 years and 9.5 days for patients <85 years). Old age has no negative impact on overall outcome after surgical therapy of cSDH. Despite significantly higher complication rate in elderly patients, the outcome assessed by the GOS at 1 month after surgery was significantly better in comparison to patients younger than 85 years. Old age does therefore not seem to be a contraindication for surgical treatment of cSDH.

Recurrent spinal metastasis of a sporadic medullary carcinoma of the thyroid after radiation therapy: a case report and review of the literature.

Sporadic Medullary Carcinoma of the Thyroid is a relatively uncommon entity and at the time of diagnosis, most already present loco-regional metastasis. Therapy should be aggressive to reduce recurrence and mortality. Follow-up period should continue lifelong and should also include calcium/pentagastrin infusion test, as well as 6-month interval diagnostic imaging.

Early surgical occipitocervical stabilization for plasma cell neoplasms at the craniocervical junction: systematic review and proposal of a treatment algorithm.

BACKGROUND CONTEXT: Plasma cell neoplasms (PCNs) of the craniocervical junction (CCJ) are rare. Because of their destructive growth, PCNs may induce spinal instability and harbor the risk of sudden death. Therefore, PCNs at the CCJ require special consideration. Although the commonly used primary treatment of PCN is radiotherapy (RT), treatment guidelines are inexistent for CCJ occurrences. PURPOSE: This study aimed to conduct a systematic review of the literature, evaluate the benefit of early and extended surgical treatment followed by RT, and outline a treatment algorithm based on the data gathered. STUDY DESIGN/SETTING: Case series and systematic review of all reported cases in the English, Spanish and German medical literature were carried out. CASE SERIES: retrospective clinical study, tertiary care center (2004-2014). Patients with a lesion of the CCJ (C0-C2) were identified. Clinical charts, imaging data, operative reports, and follow-up data were analyzed. REVIEW: a systematic literature review was performed using PubMed. Further manuscripts were identified by the web search engine Google. RESULTS: Our series comprised four patients (one female, three males), mean age 58 years. There was one lesion of C1 and three of C2. Two patients with neck pain received vertebroplasty (C1 and C2, respectively) and RT as primary management. Both developed secondary instability of the CCJ after 12 and 5 months, respectively, and required occipitocervical stabilization (OCS). The other two patients underwent OCS and required no additional surgery and no signs of instability at follow-up. Forty-nine cases of OCS were published previously. Spinal stability was achieved significantly more frequently by OCS than by less invasive or medical interventional treatment options (p=.001; two-sided Fisher exact test). CONCLUSIONS: Plasma cell neoplasms are highly radiosensitive. However, at the CCJ, a life-threatening instability may occur early and require surgical treatment. Based on personal experience, we favor OCS in this location. A systematic review of the literature supports this approach. We present a summary of our findings in a concise treatment algorithm for PCN of the CCJ.