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

2021 ◽  
Author(s):  
Federica S. Ricci ◽  
Rossella D’Alessandro ◽  
Alessandra Somà ◽  
Anna Salvalaggio ◽  
Francesca Rossi ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Tumor Location ◽  
Posterior Fossa Tumor ◽  
Surgical Removal ◽  
Cerebellar Mutism ◽  
Severity Scale ◽  
Working Definition ◽  
Prognostic Stratification ◽  
Oropharyngeal Dysfunction

AbstractThe 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. What is Known:• Post-operative pediatric Cerebellar Mutism Syndrome (CMS) is a complex phenomenon with a wide spectrum of symptoms that may manifest in children undergoing the resection of a posterior fossa tumor (PFT) and that can result into long-term impairment. What is New:• This study developed and pilot-tested an easily applicable diagnostic and severity scale to grade the duration and the severity of symptoms of the CMS.• The proposed scale was found to be a sensitive instrument to identify even mild CMS presentations.• By scoring not only the duration but also the severity of symptoms the scale allows a more accurate prognostic stratification for an optimal planning of clinical and rehabilitative interventions.

scholarly journals Unmet Needs in Clinical Practice: A Proposal and Application of A Diagnostic and Severity Scale to Grade Cerebellar Mutism Syndrome

2021 ◽  
Author(s):  
Federica Ricci ◽  
Rossella D'Alessandro ◽  
Alessandra Somà ◽  
Anna Salvalaggio ◽  
Francesca Rossi ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Unmet Needs ◽  
Tumor Location ◽  
Surgical Removal ◽  
Cerebellar Mutism ◽  
Severity Scale ◽  
Working Definition ◽  
Prognostic Stratification ◽  
Oropharyngeal Dysfunction ◽  
4Th Ventricle

Abstract BACKGROUND: 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, with additional common features that include hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to propose a diagnostic scale to grade CMS duration and severity.METHOD: Thirty consecutive subjects, aged 1-17 years (median 8 years, IQR 7) were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in cases of CMS, for the next 30 days after the onset (T0) or until symptom remission. At day 30th (T1), CMS was classified into mild, moderate, or severe according to the proposed Scale.RESULTS: CMS occurred in 13 patients (43.3%, 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).CONCLUSIONS: 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.

Cerebellar mutism after posterior fossa tumor resection in children: a multicenter international retrospective study to determine possible modifiable factors

2019 ◽  
Vol 36 (6) ◽  
pp. 1159-1169 ◽  
Author(s):  
Bryan Renne ◽  
Julia Radic ◽  
Deepak Agrawal ◽  
Brittany Albrecht ◽  
Christopher M. Bonfield ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Posterior Fossa ◽  
Tumor Resection ◽  
Posterior Fossa Tumor ◽  
Cerebellar Mutism ◽  
Modifiable Factors

Long-term cognitive deficits following posterior fossa tumor resection: A neuropsychological and functional neuroimaging follow-up study.

2009 ◽  
Vol 23 (6) ◽  
pp. 694-704 ◽  
Author(s):  
Hyo Jung De Smet ◽  
Hanne Baillieux ◽  
Peggy Wackenier ◽  
Mania De Praeter ◽  
Sebastiaan Engelborghs ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Cognitive Deficits ◽  
Functional Neuroimaging ◽  
Tumor Resection ◽  
Posterior Fossa Tumor ◽  
Follow Up Study

Factors predicting “postoperative cerebellar mutism syndrome’’ after posterior fossa tumor excision in pediatric patients

2022 ◽  
Vol 0 (0) ◽  
pp. 0
Author(s):  
KamleshSingh Bhaisora ◽  
Aanchal Datta ◽  
Ashutosh Kumar ◽  
Suyash Singh ◽  
ArunKumar Srivastava ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Pediatric Patients ◽  
Posterior Fossa Tumor ◽  
Cerebellar Mutism ◽  
Tumor Excision

A novel grading system for the prediction of the need for cerebrospinal fluid drainage following posterior fossa tumor surgery

2020 ◽  
Vol 132 (1) ◽  
pp. 296-305 ◽  
Author(s):  
Sae-Yeon Won ◽  
Florian Gessler ◽  
Daniel Dubinski ◽  
Michael Eibach ◽  
Bedjan Behmanesh ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
External Ventricular Drain ◽  
Tumor Location ◽  
Burr Hole ◽  
University Hospital ◽  
Posterior Fossa Tumor ◽  
Grading System ◽  
Perilesional Edema ◽  
Csf Drainage ◽  
The University

OBJECTIVEProphylactic placement of an external ventricular drain (EVD) is often performed prior to resection of a posterior fossa tumor (PFT); however, there is no general consensus regarding the indications. The purpose of this study was to establish a novel grading system for the prediction of required CSF drainage due to symptomatic elevated intracranial pressure (ICP) after resection of a PFT to identify patients who require an EVD.METHODSThe authors performed a retrospective analysis of data from a prospective database. All patients who had undergone resection of a PFT between 2012 and 2017 at the University Hospital, Goethe University Frankfurt, were identified and data from their cases were analyzed. PFTs were categorized as intraparenchymal (iPFT) or extraparenchymal (ePFT). Prior to resection, patients underwent EVD placement, prophylactic burr hole placement, or neither. The authors assessed the amount of CSF drainage (if applicable), rate of EVD placement at a later time point, and complication rate and screened for factors associated with CSF drainage. By applying those factors, they established a grading system to predict the necessity of CSF drainage for elevated ICP.RESULTSA total of 197 patients met the inclusion criteria. Of these 197, 70.6% received an EVD, 15.7% underwent prophylactic burr hole placement, and 29.4% required temporary CSF drainage. In the prophylactic burr hole group, 1 of 32 patients (3.1%) required EVD placement at a later time. Independent predictors for postoperative need for CSF drainage due to symptomatic intracranial hypertension in patients with iPFTs were preoperative hydrocephalus (OR 2.9) and periventricular CSF capping (OR 2.9), whereas semi-sitting surgical position (OR 0.2) and total resection (OR 0.3) were protective factors. For patients with ePFTs, petroclival/midline tumor location (OR 12.2/OR 5.7), perilesional edema (OR 10.0), and preoperative hydrocephalus (OR 4.0) were independent predictors of need for CSF drainage. According to our grading system, CSF drainage after resection of iPFT or ePFT, respectively, was required in 16.7% and 5.1% of patients with a score of 0, in 21.1% and 12.5% of patients with a score of 1, in 47.1% and 26.3% of patients with a score of 2, and in 100% and 76.5% of patients with a score ≥ 3 (p < 0.0001). The rate of relevant EVD complications was 4.3%, and 10.1% of patients were shunt-dependent at 3-month follow-up.CONCLUSIONSThis novel grading system for the prediction of need for CSF drainage following resection of PFT might be of help in deciding in favor of or against prophylactic EVD placement.

scholarly journals A Paravermal Trans-Cerebellar Approach to the Posterior Fossa Tumor Causes Hypertrophic Olivary Degeneration by Dentate Nucleus Injury

Cancers ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 258
Author(s):  
Martin A. Schaller-Paule ◽  
Peter Baumgarten ◽  
Volker Seifert ◽  
Marlies Wagner ◽  
Eike Steidl ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Posterior Fossa ◽  
Dentate Nucleus ◽  
Clinical Syndrome ◽  
Surgical Removal ◽  
Posterior Fossa Surgery ◽  
Tumor Surgery ◽  
Cerebellar Mutism ◽  
Brain Tumor Surgery ◽  
Hypertrophic Olivary Degeneration

Background: In brain tumor surgery, injury to cerebellar connectivity pathways can induce a neurodegenerative disease called hypertrophic olivary degeneration (HOD), along with a disabling clinical syndrome. In children, cerebellar mutism syndrome (CMS) is another consequence of damage to cerebello–thalamo–cortical networks. The goal of this study was to compare paravermal trans-cerebellar to other more midline or lateral operative approaches in their risk of causing HOD on MR-imaging and CMS. Methods: We scanned our neurosurgical database for patients with surgical removal of pilocytic astrocytoma, ependymoma and medulloblastoma in the posterior fossa. Fifty patients with a mean age of 22.7 (±16.9) years were identified and analyzed. Results: HOD occurred in n = 10/50 (20%) patients within four months (median), always associated with contralateral dentate nucleus (DN)-lesions (p < 0.001). Patients with paravermal trans-cerebellar approach significantly more often developed HOD (7/11; 63.6%) when compared to other approaches (3/39; 7.7%; p < 0.001). Injury to the DN occurred more frequently after a paravermal approach (8/11 vs. 13/39 patients; p < 0.05). CMS was described for n = 12/50 patients (24%). Data indicated no correlation of radiological HOD and CMS development. Conclusions: A paravermal trans-cerebellar approach more likely causes HOD due to DN-injury when compared to more midline or lateral approaches. HOD is a radiological indicator for surgical disruption of cerebellar pathways involving the DN. Neurosurgeons should consider trajectories and approaches in the planning of posterior fossa surgery that spare the DN, whenever feasible.

Bromocriptine for the treatment of postoperative cerebellar mutism syndrome in pediatric patients: Three case reports

2020 ◽  
pp. 107815522098204
Author(s):  
Miguel Á Amor-García ◽  
Cecilia M Fernández-Llamazares ◽  
Silvia Manrique-Rodríguez ◽  
Álvaro Narrillos-Moraza ◽  
Marina García-Morín ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Low Dose ◽  
Pediatric Patients ◽  
Case Reports ◽  
Tumor Resection ◽  
Posterior Fossa Tumor ◽  
Cerebellar Mutism ◽  
Common Complication ◽  
Minimum Effective Dose ◽  
Biological Origin

Introduction Cerebellar mutism syndrome (CMS) is a common complication after posterior fossa tumor resection. It is characterized by a significant lack or loss of speech. Its biological origin remains unclear and there are no standardized treatments. However, bromocriptine seems to be a possible treatment for this condition. Case report In this paper, we present three cases of pediatric patients (4, 5, and 17-year old) who developed CMS after posterior fossa tumor surgery. They were treated with bromocriptine to improve neurological symptoms. Management and outcome: Bromocriptine was started at a low dose and was progressively increased to reach the minimum effective dose. After four months of treatment, a normal and fluid speech was observed in the three patients. No discontinuation due to adverse events were reported. Discussion Bromocriptine has shown to be an effective and safe treatment for CMS in pediatric patients after posterior fossa tumor resection.

scholarly journals QOL-07. CORTICAL VOLUME AND THICKNESS IN ADULT SURVIVORS OF CHILDHOOD POSTERIOR FOSSA TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii432-iii432
Author(s):  
Charlotte Sleurs ◽  
Jurgen Lemiere ◽  
Jeroen Blommaert ◽  
Sabine Deprez ◽  
Karen Van Beek ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Cortical Thickness ◽  
Inferior Frontal Gyrus ◽  
Peak Level ◽  
Adult Survivors ◽  
Posterior Fossa Tumor ◽  
Cranial Radiotherapy ◽  
Cortical Volume ◽  
The Right

Abstract PURPOSE A brain tumor treatment including cranial radiotherapy has previously been associated with long-term neurocognitive sequelae. Since underlying neurological mechanisms remain inconclusive, we investigated cortical features in childhood posterior fossa tumor survivors. METHODS T1-weighted MRI (MPRAGE, resolution=.98x.98x1.2mm) was acquired to investigate the cortical structure in adult survivors of childhood infratentorial tumors (n=19, 15males) (16.4–34.8 years old, &gt;2years after treatment). These scans were compared to age- and gender- matched controls. Supratentorial cortical volume and thickness were investigated using voxel-based morphometry (VBM) and surface-based morphometry (SBM), respectively. We compared patients and controls, irradiated (n=13) versus non-irradiated patients, and investigated the age at radiotherapy (peak level: p&lt;.001). RESULTS Lower GM volumes were encountered in multiple brain areas of patients compared to controls, with the largest clusters in the right and left occipital fusiform gyri. Irradiated patients showed lower GM volumes then non-irradiated patients in the superior and middle frontal gyri, the right supramarginal gyrus and precuneus. Age at radiotherapy was associated with GM volume in the inferior frontal gyrus. SBM yielded larger cortical thickness in patients in the left precuneus, inferior temporal and fusiform gyrus. The opposite effect was only marginally significant, in the left temporal lingual gyrus. Age at radiotherapy was not associated with cortical thickness, but radiotherapy was associated with lower thickness of the left pars opercularis. CONCLUSION Widespread differences in cortical volumes and thickness were observed in posterior fossa tumor survivors. Both radiotherapy and age at radiotherapy could be suggested as risk factors for long-term cortical development.

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