Original Article
Cerebellar Mutism Syndrome After Posterior Fossa Tumor Surgery in Children—A
Retrospective Single-Center Study
Stephanie Schmidt1, Edina Kovacs1, Diren Usta2, Rouven Behnisch4, Felix Sahm3,5, Daniel Haux1, Olaf Witt2, Till Milde2,
Andreas Unterberg1, Ahmed El Damaty1
- OBJECTIVE: Cerebellar mutism syndrome (CMS) is a
well-known complication after posterior fossa tumor sur-
gery in pediatric patients. We evaluated the incidence of
CMS in our institute and analyzed its association with
multiple risk factors, such as tumor entity, surgical
approach, and hydrocephalus.
- METHODS: All pediatric patients who had undergone intra-
axial tumor resection in the posterior fossa between January
2010 and March 2021 were included in the retrospective
analysis. Various data points, including demographic, tumor-
associated, clinical, radiological, surgery-associated, compli-
cations, and follow-up data, were collected and statistically
evaluated for an association with CMS.
- RESULTS: A total of 63 surgeries in 60 patients were
included. The median patient age was 8 years. Pilocytic
astrocytoma was the most common tumor type (50%), fol-
lowed by medulloblastoma (28%) and ependymomas (10%).
Complete, subtotal, and partial resection was achieved in
67%, 23%, and 10%, respectively. A telovelar approach had
been used the most often (43%) compared with a trans-
vermian approach (8%). Of the 60 children, 10 (17%) had
developed CMS and showed marked improvement but with
residual deficits. The significant risk factors were a
transvermian approach (P [ 0.03), vermian splitting when
added to another approach (P [ 0.002), an initial
Key words
- Cerebellar mutism
- Hydrocephalus
- Medulloblastoma
- Posterior fossa
- Telovelar
Abbreviations and Acronyms
CM: Cerebellar mutism
CMS: Cerebellar mutism syndrome
DTC: Dentatothalamocortical
EPN: Ependymoma
EVD: External ventricular drain
MB: Medulloblastoma
MRI: Magnetic resonance imaging
PA: Pilocytic astrocytoma
PFS: Posterior fossa syndrome
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presentation with acute hydrocephalus (P [ 0.02), and
hydrocephalus present after tumor surgery (P [ 0.004).
- CONCLUSIONS: Our CMS rate is comparable to those
described in the literature. Despite the limitations of the
retrospective study design, we found that CMS was not
only associated with a transvermian approach but was
also associated with a telovelar approach, although to a
lesser extent. Acute hydrocephalus at the initial presen-
tation necessitating urgent management was significantly
associated with a greater incidence of CMS.
INTRODUCTION
C erebellar mutism (CM) is a complication in children first
described by Rekate et al.1 in 1985 as a transient loss of
speech after tumor resection in the posterior fossa. CM
syndrome (CMS) comprises a complex set of neurologic and
neurocognitive disorders for which the cardinal and central
component is an initially profound, but usually reversible,
speech disorder.2 Other symptoms such as ataxia, cranial nerve
deficits, neurobehavioral changes, emotional lability, and urinary
retention or incontinence can occur. Posterior fossa syndrome
(PFS) describes a broader symptomatology complex, for which
CMS or CM is an integral part.3,4 Thus, CMS should be
VP: Ventriculoperitoneal
WHO: World Health Organization
From the 1Neurosurgery Department, 2Pediatric Neurooncology Department, and
3
Department of Neuropathology, Heidelberg University Hospital; 4Institute of Medical
Biometry, Heidelberg University; and 5CCU Neuropathology, German Consortium for
Translational Cancer Research, German Cancer Research Center, Heidelberg, Germany
To whom correspondence should be addressed: Ahmed El Damaty, M.D., Ph.D.
[E-mail: ahmed.eldamaty@med.uni-heidelberg.de]
Stephanie Schmidt and Edina Kovacs are co-first authors.
Citation: World Neurosurg. (2023) 173:e622-e628.
https://doi.org/10.1016/j.wneu.2023.02.117
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STEPHANIE SCHMIDT ET AL.
considered when the neurological deficits are related to cerebellar
function, and PFS should be considered when the symptoms are
related to cranial nerve or brainstem deficits. A similar
condition, cerebellar cognitive affective syndrome, has been
described in adults with cerebellar lesions. Cerebellar cognitive
affective syndrome is characterized by speech difficulties,
personality changes, executive function impairment, and spatial
cognition.5
The incidence of CMS varies from 10% to 30%.6 The onset is
typically within 1 week after posterior fossa surgery. The
symptoms will usually improve over a period of weeks or months,
although many children will continue to experience persistent
neurological deficits, typically so-called ataxic dysarthria.7,8 The
exact cause of CMS remains unclear. Although many risk factors
have been discussed, only a midline tumor location and
medulloblastoma (MB) pathology have been reproducibly
identified.9 Other reported risk factors include tumor size,
brainstem invasion, molecular MB subtype, hydrocephalus, and
factors associated with surgery, such as a transvermian approach,
usage of ultrasonic cavitation devices, and postoperative
infection.10,11 Surgical experience was also identified as a major
modifiable contributor to the development of PFS, as shown by a
significantly higher PFS incidence in a low-volume surgery center.9
Surgical disruption of the dentatothalamocortical (DTC)
pathway has been suggested to be associated with CMS.10 Because
CMS usually occurs a few days, rather than immediately, after
surgery, secondary injury mechanisms, such as ischemia,
traction, and thermal damage from ultrasonic cavitation devices,
could be the cause.12 In addition, bilateral hypertrophic olivary
degeneration has been postulated as a sensitive and specific
indicator of PFS when found by magnetic resonance imaging
(MRI) of patients after surgery.13
METHODS
We performed a retrospective, single-center study. Our institu-
tional ethics committee approved the data collection for the pre-
sent study. The parents or guardians provided written informed
consent before data collection, and the data were collected
retrospectively from the medical records. We included all pediatric
patients aged <18 years who had undergone posterior fossa tumor
surgery in our department between March 2010 and March 2021.
We excluded patients with extra-axial tumors with a far lateral
location because a retrosigmoid approach via an extra-axial route
had been used most often. Also, patients who had undergone only
a biopsy were excluded because the risk of CMS was limited owing
to the minimal manipulation. The following data points were
collected: gender, symptoms before surgery, postoperative clinical
course, handedness, hydrocephalus (including method and time
of treatment), tumor location, radiological tumor morphology
(including volume and contrast enhancement), histopathological
findings (including molecular genetics), and surgical approach.
Routine evaluation of neurological status and speech function
were performed preoperatively, immediately postoperatively, at
3 months postoperatively, and at the last follow-up.
Tumor volume was calculated after a review of MRI studies by
measuring the largest craniocaudal, anteroposterior, and
transverse diameters using the formula for ellipsoid masses:
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ORIGINAL ARTICLE
CEREBELLAR MUTISM IN PEDIATRIC PATIENTS
(length
width
height)/2. The extent of resection was
evaluated and classified from the operative surgical report and
early postoperative MRI findings within 48 hours after surgery
into 3 categories: partial resection (<90% tumor volume
reduction), subtotal resection (>90% tumor volume reduction),
and gross total resection (no residual tumor found on early
postoperative MRI). We also analyzed the surgical approach,
including whether vermian splitting and ultrasonic cavitation
had been used. We divided the timing of surgery into 3 groups:
elective, emergency (within 48 hours of admission), and urgent
(within a few hours after radiological imaging).
The tumors were classified according to location as midline,
midline with a lateral preference, or lateral. Involvement of the vermis
and brainstem infiltration were documented separately. The
histopathological findings were classified using the 2016 World
Health Organization (WHO) classification system.14 For all patients,
conventional microscopic analysis, immunohistochemistry,
comprehensive molecular profiling, including whole exome
sequencing, DNA methylation analysis 450k or 850k, RNA
sequencing, and microarray gene expression, were performed,
depending on the diagnostic requirements and tissue availability.
In the present study, CMS was defined as a postoperative
speech impairment. We observed all other related symptoms,
including ataxia, emotional lability, behavioral disorders, cranial
nerve deficits, and brainstem dysfunction. The data were pooled
for descriptive statistical analysis. All variables were analyzed with
a special focus on their relation to the occurrence of mutism with
or without other symptoms and/or influencing their intensity or
course. Gender, handedness, hydrocephalus present before and
after surgery, radiological tumor morphology (e.g., contrast
enhancement), vermis invasion, brainstem invasion, residual tu-
mor in the brainstem, vermis splitting, postoperative infection,
postoperative chemotherapy, and radiation were coded as binary
(yes vs. no) outcomes. Patient age, tumor volume, and hospital
length of stay were considered continuous variables. To compare
the 2 groups, the c2 test was used for categorical variables and the
Welch 2-sample t test for continuous variables. To identify
possible risk factors for CMS, univariate and multivariate logistic
regression analyses were performed. All analyses were performed
using SPSS, version 28.0 (IBM Corp., Armonk, New York, USA),
and R, version 4.1.1 (R Foundation for Statistical Computing,
Vienna, Austria), for Windows and Mac.
RESULTS
Cohort Description
Sixty patients fulfilled the inclusion criteria. The male/female ratio
was 1:1, and the median patient age was 8  5.48 years. The median
follow-up was 36 months (range, 12e130 months). Complete
resection, subtotal resection, and partial resection was achieved for
40 patients (67%), 14 patients (23%), and 6 patients (10%),
respectively. The surgical approach used was the classic telovelar
approach for 26 patients (43%), the transvermian approach for 5
patients (8%), and the supracerebellar median and transcerebellar
lateral approaches for 29 patients (48%). Three patients (5%) had
required a second resection, two for MB recurrence and one for
pilocytic astrocytoma (PA). Thus, 60 patients had undergone 63
surgeries. DNA methylation analysis was performed for 41 samples:
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STEPHANIE SCHMIDT ET AL.
850k for 32 (51%) and 450k for 9 (14%). For 22 samples, no DNA
methylation analysis was performed. Of the 60 children, 10 (17%)
had developed postoperative impaired speech and were included in
the CMS group, with 50 patients (83%) in the non-CMS group. For 2
patients (20%), CMS had developed, in addition to a preoperative
speech disorder. All 10 patients with mutism also had ataxia. In
addition, 70% showed irritability and 50% behavioral disturbances.
All patients with CMS had experienced marked improvement over
time but with residual neurological symptoms found at follow-up,
mainly ataxic dysarthria (Table 1).
Integrated Diagnosis
Of the 31 patients with PA grade 1, 4 had had a histological diag-
nosis of PA WHO grade 1. The diagnosis was corrected after mo-
lecular analysis to dysembryoplastic neuroepithelial tumor WHO
grade 1, ganglioglioma WHO grade 1, high-grade astrocytoma with
piloid features WHO grade 3, and angiocentric glioma WHO grade
1 with MYB-QKI fusion for 1 patient each. In addition, 1 patient had
had a histological diagnosis of glioblastoma WHO grade 4 that was
corrected after molecular analysis to PA grade 1. A total of 19 pa-
tients had had MB WHO grade 4, including 2 with WNT-activated
MB, 5 with SHH-activated MB, 3 with group 3, 5 with group 4, 1
with desmoplastic MB, and 3 with MB, not otherwise classified. Five
patients had had anaplastic ependymoma (EPN) grade 3. One pa-
tient had had tanycytic EPN histologically that had been corrected
after molecular analysis to a low-grade glial tumor with a PTCH-1
mutation, WHO grade 2. Finally, one patient each had had the
following: ganglioglioma WHO grade 1, atypical teratoid rhabdoid
tumor, embryonal tumor with multilayered rosettes, diffuse midline
glioma of the brainstem WHO grade 4 with H3K27 mutation,
hemangioblastoma WHO grade 1, plexus papilloma WHO grade 1,
and meningioma WHO grade 1 (Figure 1).
Risk Factors
The following risk factors were found on univariate analysis to be
significant: use of a transvermian approach (P ¼ 0.03) and vermis
splitting when added to another approach (P ¼ 0.002). The
presence of hydrocephalus per se was found to be relevant: 19
patients (32%) had presented initially with acute hydrocephalus
necessitating urgent external ventricular drain (EVD) insertion
before tumor surgery the next day. Of these 19 patients, 7 (37%)
had developed CMS postoperatively (P ¼ 0.02). An additional 21
patients (35%) had received an EVD at tumor resection because of
preexisting hydrocephalus. However, only 2 of these patients
(10%) had later developed CMS. Finally, 20 patients (33%) had not
presented with hydrocephalus initially, and only 1 patient (5%)
had developed CMS after surgery. A total of 19 patients (32%) had
required implantation of a ventriculoperitoneal (VP) shunt after
removal of the EVD because of the presence of hydrocephalus
despite tumor surgery. Of these 19 patients, 7 (37%) had developed
CMS postoperatively (P ¼ 0.004). Of the 60 patients, 41 (68%) had
not required VP shunt placement after tumor removal and only 3 of
these patients (7%) had developed CMS.
Our multivariate logistic regression analysis of the risk factors
(i.e., gender, initial presentation with acute hydrocephalus,
persistence of hydrocephalus postoperatively, 3 main pathological
entities [MB, EPN, PA], operative approach, resection extent) and
their association with CMS yielded 2 statistically significant
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ORIGINAL ARTICLE
CEREBELLAR MUTISM IN PEDIATRIC PATIENTS
associations: acute hydrocephalus necessitating urgent insertion
of an EVD before surgery the next day (P ¼ 0.048) and the use of a
transvermian surgical approach (P ¼ 0.03), confirming the uni-
variate risk analysis results (Table 2 and Figure 2). Of the 5 patients
who had undergone surgery via a transvermian approach, 3 (60%)
had developed CMS. In contrast, 4 of 26 patients (15%) had
developed CMS after a telovelar approach.
Of the 10 patients, with postoperative CMS, 6 (60%) had had a
diagnosis of MB, 3 (30%) a diagnosis of PA, and 1 (10%) a diag-
nosis of anaplastic EPN (Figure 1A). The diagnosis of MB did not
reach statistical significance but exhibited a strongly suggestive
trend toward a correlation with CMS. The tumor location in the
posterior fossa was in the midline for 67%, the midline with a
lateral preference for 22%, and laterally for 12% (P ¼ 0.277).
The average tumor volume was 28  25.1 cm3. Contrast
enhancement on MRI was found in 55 cases (92%). Tumor
invasion of the vermis and the brainstem was present in 16
patients (27%) and 17 patients (29%), respectively.
The nonsignificant risk factors using the c2 test for CMS were
handedness (P ¼ 0.072), tumor location (P ¼ 0.209), vermis in-
vasion (P ¼ 0.068), brainstem invasion (P ¼ 0.928), tumor size
(P ¼ 0.214), radiological contrast enhancement (P ¼ 0.296),
timing of surgery (P ¼ 0.258), use of a Cavitron ultrasonic surgical
aspirator (P ¼ 0.794), residual tumor in the brainstem (P ¼
0.275), postoperative infection (P ¼ 0.427), intensive care unit
length of stay (P ¼ 0.221), inpatient length of stay (P ¼ 0.051),
postoperative chemotherapy (P ¼ 0.338), postoperative radiation
(P ¼ 0.558).
DISCUSSION
In the present study, we evaluated our cohort of 60 patients after
intra-axial tumor resection in the posterior fossa during an 11-year
period. Of the 60 patients, 3 had required a second operation for
tumor recurrence, resulting in 63 surgical procedures. The 17%
incidence of CMS for our study is within the previously reported
range of 10% to 30%.4,6
Patients diagnosed with MB have been reported to have a higher
incidence of CMS,15 which was confirmed in our study. Of our 17
patients with MB, 6 (35%) had developed CMS compared with 3 of
30 patients with PA (10%), and 1 of 6 patients with EPN (16.7%).
Jabarkheel et al.11 reported an incidence of CMS in 370 MB patients
of 23.8%. The incidence varied according to the molecular subtype
of MB, with 35% in group 4, 31% in group 3, 21% in WNT, and 7%
in SHH. The molecular subtype could not be validated for our 6
patients with MB (2 with group 4, 2 with WNT, 1 with group 3,
and 1 with SHH) who had developed CMS owing to the limited
number of MB patients in our cohort (n ¼ 17), and those
without detailed molecular analysis data available from the
earlier years of the study. However, we found a higher incidence
in those with group 4 MB and those with WNT MB. These 2
subtypes both have an excellent prognosis, necessitating every
effort to avoid the occurrence of CMS for these patients.
A midline tumor location, infiltration of the brainstem, and
tumor size were identified as risk factors across various
studies.9,10,15,16 Our cohort showed a higher percentage of CMS
cases among those with midline tumors and those with
infiltration of the brainstem; however, neither difference was
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STEPHANIE SCHMIDT ET AL.

Table 1. CMS Cohort
Deficits
Symptom ICU Hospital Present
Pt. Age Surgery Preoperative Tumor Vermis Brainstem Volume Histological Surgical Ultrasonic Resection Behavioral Emotional Onset LOS LOS at Last
No. Gender (years) Year Findings Hydrocephalus Location Invasion Invasion (mL) Type EVD Approach Cavitation Extent CMS Ataxia Changes Lability (days) (days) (days) Follow-Up
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1 M 4 2014 No abnormality No Median Yes No 8.8 PA No Transvermian Yes GTR Yes Yes No Yes 6 7 18 Ataxia, mild
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detected coordination deficit

2 M 7 2014 Diplopia, ataxia, Yes Median Yes Yes 18.5 MB In Transvermian No STR Yes Yes Yes Yes 2 12 39 Dysarthria, ataxia,
headache, situ fine motor deficit
nausea,
vomiting

3 F 14 2015 Fine motor Yes Median No No 31 MB In Telovelar Yes GTR Yes Yes No No 4 5 77 Dysarthria, ataxia,
deficit, vertigo, situ abducens paresis
headache,
nausea,
vomiting

4 F 2 2016 Ataxia, Yes Median No Yes 38.3 MB In Telovelar Yes STR Yes Yes No No Preoperatively 5 15 Residual left
developmental situ hemiparesis,
delay, speech strabismus
impairment,
right
hemiparesis

5 F 2 2016 Ataxia, Yes Median No No 43.4 MB Yes Telovelar No GTR Yes Yes No Yes 1 14 65 Dysarthria, ataxia,
developmental strabismus, facial
delay paresis

6 M 4 2016 Ataxia, Yes Median No No 53.9 MB In Transvermian Yes STR Yes Yes No Yes 1 6 20 Severe ataxia,
headache, situ hypotonia
nausea,
vomiting
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7 M 7 2016 Ataxia, Yes Median Yes No 14.4 MB In Telovelar No GTR Yes Yes Yes Yes 3 3 20 Ataxic dysarthria,
headache, situ nystagmus, ataxia,
nausea, dysmetria
vomiting

8 F 12 2020 Headache, Yes Mediolateral Yes No 30 PA Yes Transcortical Yes GTR Yes Yes Yes Yes 6 1 22 Mild ataxia,
nausea, paramedian dysdiadochokinesia

CEREBELLAR MUTISM IN PEDIATRIC PATIENTS

vomiting, fine
motor
dysfunction

9 M 4 2020 Headache, Yes Median Yes Yes 105 EPN In Transcortical Yes GTR Yes Yes Yes No 4 4 15 Mild ataxia, ataxic
nausea, situ paramedian dysarthria,
vomiting, motor coordination
developmental deficit, dysmetria,
delay, hypotonia dysdiadochokinesia

10 M 3 2021 Speech Yes Median No No 52.8 PA In Supracerebellar Yes GTR Yes Yes Yes Yes Preoperatively 3 10 Speech arrest
impairment, situ transcortical
ataxia, squint

CMS, cerebellar mutism syndrome; Pt. No., patient number; EVD, external ventricular drain; ICU, intensive care unit; LOS, length of stay; M, male; PA, pilocytic astrocytoma; GTR, gross total resection; MB, medulloblastoma; STR, subtotal

ORIGINAL ARTICLE
resection; F, female; EPN, ependymoma.
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 ORIGINAL ARTICLE
STEPHANIE SCHMIDT ET AL. CEREBELLAR MUTISM IN PEDIATRIC PATIENTS

Figure 1. Distribution of tumor entity in (A) cerebellar mutism syndrome (CMS) and (B) non-CMS groups.

statistically significant. Tumor size also did not differ significantly,
although a volumetric measurement cannot be compared across
children of different ages. Other studies, however, have shown
that a tumor size >5 cm in diameter significantly increased the
risk of CMS.10 A midline location has been described as a risk
factor for CMS. The midline location has anatomical structures
considered relevant for CM, such as the vermis, fastigial nuclei,
and parts of the DTC pathway.17-20 The DTC pathway also
passes through the brainstem and, as such, is at risk of damage
caused by brainstem infiltration and resection.
Grønbæk et al.21 performed a prospective study of 500 children
in their study and suggested that a midline tumor location,
younger patient age, and high-grade tumor histological findings
all increase the risk of speech impairment after posterior fossa
tumor surgery. They could not find any evidence to recommend a
telovelar versus transvermian surgical approach in relation to the
risk of developing a postoperative speech impairment. Correlating
with other reports, CMS not only occurs after a transvermian
approach, but also after a telovelar approach, although the inci-
dence has been comparatively lower.12,22 However, in our cohort, 1
patient who had developed CMS after surgery using a telovelar
approach was reported to have received a partial incision of the
lower vermis. Another patient who had developed CMS after a
midline supracerebellar approach had also received a partial
incision of the upper vermis. Thus, the CMS cases in our cohort
were significantly associated with a vermian incision, even if
partial. Therefore, we believe that an incision in the region of
the vermis carries more risk of injury to the structures possibly
involved in the pathophysiology and development of CMS (i.e.,
DTC tract within the superior cerebellar peduncles and fastigial
nuclei).10,20,23
Although it has been suggested that treatment of hydrocephalus
might decrease the rate of CMS, studies have reported conflicting
results.24 Cerebrospinal fluid diversion through an EVD or

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STEPHANIE SCHMIDT ET AL. CEREBELLAR MUTISM IN PEDIATRIC PATIENTS

had occurred after 7 of 37 surgeries (19%) compared with only 3

Table 2. Distribution of Statistically Significant Variables cases after 26 surgeries (11.5%) in the last 5 years. In addition, in
CMS Non-CMS Total the first 6 years, the first 37 surgeries had been performed by 6
Variable (n [ 10) (n [ 50) (n [ 60) P Value different surgeons, of whom only 2 had performed >50 brain
tumor surgeries in the pediatric population within the previous 5
Initial acute hydrocephalus with 7 (70) 12 (24) 19 (32) 0.01 years before our study. In contrast, within the last 5 years of the
EVD insertion present study, the 26 surgeries had only been performed by 2
Transvermian surgical approach 3 (30) 2 (4) 5 (8) 0.03 neurosurgeons who had fulfilled that criterion. The value of
Vermian splitting 4 (40) 3 (6) 7 (12) 0.002
accumulated surgeon experience at high-volume surgery centers
and development of the surgical technique were significant in
Postoperative hydrocephalus 7 (70) 12 (24) 19 (32) 0.004 relation to the incidence of CMS.9 Although the reduction was not
with implantation of VP shunt
statistically significant (P ¼ 0.5), more patients were saved from
Data presented as n (%). the disabling complication of CMS, especially for patients with a
CMS, cerebellar mutism syndrome; EVD, external ventricular drain; VP, good prognosis (e.g., those with WNT-MB or PA).
ventriculoperitoneal. Ahmadian et al.25 reported after a systematic review that
cerebral perfusion after posterior fossa tumor resection was
reduced in CMS patients compared with patients without CMS.
Pettersson et al.26 also reported after a systematic review about
endoscopic third ventriculostomy has been suggested to cause less the risk factors for the development of CMS. They found that
mechanical traction during surgery, which, in turn, could decrease brainstem invasion, fourth ventricle invasion, superior cerebellar
the incidence of CMS. In our study, acute hydrocephalus with peduncle invasion, a diagnosis of MB, MB >50 mm, left-
clinical decompensation in the form of a disturbed handedness, and a vermis incision were associated with a
consciousness level at the initial presentation that necessitated higher incidence of CMS. We reviewed the MRI studies of our
urgent EVD insertion before tumor resection was associated patients with CMS. We noted that in most cases, signs of ischemia
with a significantly higher risk of CMS. This might have resulted were present in the region dorsal to the fourth ventricular roof. We
from the higher rate of acute hydrocephalus in patients with assumed these signs indicated injury to the dentate and/or fasti-
aggressive tumors (i.e., MB), who, in turn, have a higher risk of gial nuclei, with consequent gray matter deficits found on follow-
CMS. Also, patients who had required VP shunt insertion after up MRI. However, we could not found signs of hypertrophic oli-
EVD insertion and tumor surgery had had a higher risk of CMS, vary degeneration, as described by Patay et al.13 A further study
which we correlated with tumor progression and leptomeningeal with our neuroradiologists will be reported describing in detail
spread in patients with MB. the MRI findings for CMS patients versus a control group of
We noted a decline in CMS frequency in our cohort during the patients without CMS.
last 5 years of our 11-year study. In the first 6 years, we found CMS
Study Limitations
Because our study was retrospective, our data relied on clinical
documentation, which could have been incomplete. Thus,
although handedness has been discussed as a risk factor for CM, it
had not been sufficiently documented for our cohort. Further-
more, mild cases of CMS could have been underreported. Exten-
sive preoperative speech tests were not performed in our cohort
owing to the frequent acute onset of symptoms and urgent need
for surgery. Preoperative speech testing could be useful to detect
speech impairments because those patients might have a higher
risk of developing CMS.

Figure 2. Forest plot showing odds ratio of multivariable logistic regression
for occurrence of mutism. Risk variable indicated in brackets. evd, external
ventricular drain; op, operative; preop, preoperatively.
CONCLUSIONS
Within the limitations of a retrospective, single-institutional series,
we have presented our institutional experience with CMS after
posterior fossa tumor surgery in a pediatric population. Our overall
CMS rate of 17% compares with the reported incidence. CMS was
associated with a transvermian approach but also developed after a
telovelar approach, although with a significantly lower incidence.
Adding a partial vermian incision to other approaches also carried a
significant risk for the development of postoperative CMS. Our
findings have validated that acute hydrocephalus necessitating ur-
gent management at the initial presentation is a significant risk
factor for CMS. Also, the permanent need for a VP shunt after

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STEPHANIE SCHMIDT ET AL. CEREBELLAR MUTISM IN PEDIATRIC PATIENTS

tumor surgery could be interpreted as a negative factor, retrospec-
tively, in the prediction of CMS after tumor surgery. Although
symptom improvement had occurred in almost all patients with
CMS, residual deficits, including dysarthria and neurocognitive
problems, were still present in a substantial number at 1 year after
surgery. Restricting the surgery in this vulnerable group to only
neurosurgeons with experience treating pediatric brain tumors is
advised, because we found a clear difference in the incidence of
CMS. A better understanding of the role of the normal cerebellum
and its connections in the early development of a child’s central
nervous system is required to avoid such complications and develop
rehabilitation strategies for patients with persistent cognitive,
behavioral, and motor deficits.
CRediT AUTHORSHIP CONTRIBUTION STATEMENT
Stephanie Schmidt: Investigation, Writing e original draft, Review
of final version. Edina Kovacs: Investigation, Writing e original
draft, Review of final version. Diren Usta: Investigation, Writing e
original draft, Review of final version. Rouven Behnisch: Writing e
review & editing, Statistics, Review of final version. Felix Sahm:
Writing e review & editing, Review of final version. Daniel Haux:
Writing e review & editing, Review of final version. Olaf Witt:
Writing e review & editing, Review of final version. Till Milde:
Writing e review & editing, Methodology, Review of final version.
Andreas Unterberg: Writing e review & editing, Review of final
version. Ahmed El Damaty: Methodology, Writing e original draft,
Writing e review & editing, Review of final version.

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Conflict of interest statement: The authors declare that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 2 December 2022; accepted 24 February 2023
Citation: World Neurosurg. (2023) 173:e622-e628.
https://doi.org/10.1016/j.wneu.2023.02.117
Journal homepage: www.journals.elsevier.com/world-
neurosurgery
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