CLINICAL ARTICLE

J Neurosurg Pediatr 27:52–61, 2021

Fourth ventricle tumors in children: complications and

influence of surgical approach
Sebastian M. Toescu, MBChB(Hons), MRCS,1,2 Gargi Samarth, MBBS,1
Hugo Layard Horsfall, MBBS,1,3 Richard Issitt, DClinP,4 Ben Margetts, PhD,4
Kim P. Phipps, BSc(Hons),1 Noor-ul-Owase Jeelani, MBA, MPhil (Medical Law), FRCS(SN),1
Dominic N. P. Thompson, MBBS, FRCS(SN),1 and Kristian Aquilina, MD, FRCS(SN)1
1
Department of Neurosurgery, Great Ormond Street Hospital for Children, London; 2Developmental Imaging and Biophysics
Section, UCL GOS Institute of Child Health, London; 3Department of Neurosurgery, Addenbrooke’s Hospital, Cambridge; and
4
Digital Research Environment, Great Ormond Street Hospital for Children, DRIVE Office, London, United Kingdom

OBJECTIVES The goal of this study was to characterize the complications and morbidity related to the surgical man-
agement of pediatric fourth ventricle tumors.
METHODS All patients referred to the authors’ institution with posterior fossa tumors from 2002 to 2018 inclusive were
screened to include only true fourth ventricle tumors. Preoperative imaging and clinical notes were reviewed to extract data
on presenting symptoms; surgical episodes, techniques, and adjuncts; tumor histology; and postoperative complications.
RESULTS Three hundred fifty-four children with posterior fossa tumors were treated during the study period; of these,
185 tumors were in the fourth ventricle, and 167 fourth ventricle tumors with full data sets were included in this analysis.
One hundred patients were male (mean age ± SD, 5.98 ± 4.12 years). The most common presenting symptom was
vomiting (63.5%). The most common tumor types, in order, were medulloblastoma (94 cases) > pilocytic astrocytoma (30
cases) > ependymoma (30 cases) > choroid plexus neoplasms (5 cases) > atypical teratoid/rhabdoid tumor (4 cases),
with 4 miscellaneous lesions. Of the 67.1% of patients who presented with hydrocephalus, 45.5% had an external ven-
tricular drain inserted (66.7% of these prior to tumor surgery, 56.9% frontal); these patients were more likely to undergo
ventriculoperitoneal shunt (VPS) placement at a later date (p = 0.00673). Twenty-two had an endoscopic third ventricu-
lostomy, of whom 8 later underwent VPS placement. Overall, 19.7% of patients had a VPS sited during treatment.
Across the whole series, the transvermian approach was more frequent than the telovelar approach (64.1% vs 33.0%);
however, the telovelar approach was significantly more common in the latter half of the series (p < 0.001). Gross-total
resection was achieved in 70.7%. The most common postoperative deficit was cerebellar mutism syndrome (CMS;
28.7%), followed by new weakness (24.0%), cranial neuropathy (18.0%), and new gait abnormality/ataxia (12.6%). Use of
intraoperative ultrasonography significantly reduced the incidence of CMS (p = 0.0365). There was no significant differ-
ence in the rate of CMS between telovelar or transvermian approaches (p = 0.745), and multivariate logistic regression
modeling did not reveal any statistically significant relationships between CMS and surgical approach.
CONCLUSIONS Surgical management of pediatric fourth ventricle tumors continues to evolve, and resection is in-
creasingly performed through the telovelar route. CMS is enduringly the major postoperative complication in this patient
population.
https://thejns.org/doi/abs/10.3171/2020.6.PEDS2089
KEYWORDS cerebellar mutism syndrome; telovelar; complications; posterior fossa tumors; fourth ventricle; oncology;
surgical technique

T
umors of the fourth ventricle in children pose a superior cerebellar peduncle—and the floor of the fourth
particular surgical challenge due to the proximity ventricle, formed by the brainstem, contains several cra-
of eloquent neural parenchyma in the region. The nial nerve nuclei on its surface. Surgical access to tumors
roof and walls of the fourth ventricle contain key elements occupying or invading the fourth ventricle has, since the
of the efferent cerebellar pathway—the dentate nuclei and time of Cushing in the 1920s,1 been performed through

ABBREVIATIONS CMS = cerebellar mutism syndrome; CPA = cerebellopontine angle; CUSA = Cavitron ultrasonic surgical aspirator; EOR = extent of resection; ETV =
endoscopic third ventriculostomy; EVD = external ventricular drain; GTR = gross-total resection; STR = subtotal resection; VPS = ventriculoperitoneal shunt.
SUBMITTED February 11, 2020. ACCEPTED June 10, 2020.
INCLUDE WHEN CITING Published online October 23, 2020; DOI: 10.3171/2020.6.PEDS2089.

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 Toescu et al.

a direct transvermian route. The telovelar approach, first When urgent CSF diversion was required prior to de-
described in 1992,2 makes use of the natural corridor from finitive tumor resection, patients underwent either inser-
the cisterna magna to the fourth ventricle along the cer- tion of an external ventricular drain (EVD) or endoscopic
ebellomedullary fissure, and thus spares transgression of third ventriculostomy (ETV). Otherwise, children were
the cerebellar vermis. This has been thought to influence temporized with glucocorticoid administration before
the development of postoperative truncal ataxia and cer- tumor resection. Resective surgery of fourth ventricle tu-
ebellar mutism syndrome (CMS).3 Reports of neurological mors was performed by consultant neurosurgeons.
morbidity following fourth ventricle tumor resection are Demographic data for patients included in the cohort
largely restricted to small pediatric4–9 or combined adult were extracted from our institution’s clinical databases by
and pediatric series,10–12 and describe patient groups that using a direct SQL (Structured Query Language) inquiry.
are heterogeneous with respect to preoperative comorbidi- Data processing and analysis were carried out in R version
ties, tumor location within the posterior fossa, and tumor 3.5.0,14 and graphics were created using the ggplot2 pack-
histology and grade. age.15 To test for differences in proportions of categorical
The objective of this retrospective single-center series variables, the chi-square test was used, or Fisher’s exact
of consecutive cases was to critically assess early, surgery- test was used where numbers were small or proportions
related morbidity in pediatric fourth ventricle tumors over extreme, with an a priori α level of 0.05. The Shapiro-Wilk
2 decades, with particular reference to surgical approach. test was used to check normality of continuous variables
prior to hypothesis testing with either a t-test or Wilcoxon
signed-rank test. Bonferroni multiple comparison correc-
Methods tion was used within families of tests, represented in this
This study was registered under local research and paper as separate tables. Multivariate logistic regression
development protocols, and ethical approval was granted models were built to explore relationships between binary
from the institutional review board. Prospectively main- explanatory and response variables.
tained dedicated neuro-oncology databases were retro-
spectively interrogated to identify all children referred to
our academic tertiary referral center with a new poste- Results
rior fossa tumor between January 1, 2002, and Decem- Presentation and Demographics
ber 31, 2018, inclusive. Preoperative axial imaging of Three hundred fifty-four children with posterior fossa
these patients was reviewed by the authors (S.M.T., G.S., tumors were seen during the study period (20.8/year).
H.L.H., K.A.) to screen for true fourth ventricle tumors. After screening of preoperative imaging, 185 fourth
This included tumors in the fourth ventricle with exten- ventricle tumors were identified; 167 fourth ventricle
sions into the vermis or medial cerebellar hemispheres, tumors with full data sets were included in this analy-
cerebellopontine angle (CPA) via foramina of Luschka, sis (Fig. 1). The mean age at presentation was 5.98 years
and exophytic midbrain or medullary tumors. Tumors (SD 4.12 years); 100 patients were male (59.9%). Hydro-
purely in the cerebellar hemispheres, CPA, and nonexo- cephalus was seen in 112 patients (67.1%) at presentation.
phytic intrinsic brainstem tumors were excluded from the The most common presenting symptom was vomiting
cohort. Tumor volumes were estimated using the ABC/2 (106/167, 63.5%), followed by ataxia (86/167, 51.5%),
method.13 headache (81/167, 48.5%), visual change (57/167, 34.1%),
Clinical notes were reviewed to provide dichotomized long tract signs (31/167, 18.6%), and cranial neuropathy
data points on presenting symptoms (and their duration in (27/167, 16.2%). Symptoms were present for a median of
days); surgical episodes, techniques, and adjuncts; tumor 28 days prior to admission (range 1–270 days). Differ-
histology; and postoperative complications. Extent of re- ences were observed in the frequency of ages at which
section (EOR) was graded as gross-total resection (GTR), different symptoms occurred (Fig. 2 left). The average
subtotal resection (STR), or biopsy only, by reviewing age of children presenting with ataxia was significantly
operative notes and postoperative imaging. The clini- younger (p = 0.0384), whereas visual change (p < 0.001)
cal course of patients included in the study was assessed and headache (p < 0.001) were significantly more likely
over the first 30 postoperative days. Postoperative pedi- to be present in older children. Clustering analysis (Fig. 2
atric CMS was defined as present or absent based on the right) revealed that symptoms of raised intracranial pres-
description of mutism in the contemporaneous medical sure (vomiting and headache) and more “global” neu-
notes by the attending neurosurgeon or multidisciplinary rological phenomena such as ataxia and hydrocephalus
staff, who covered pediatric neurology, physiotherapy, and tended to group together. A separate cluster was observed
speech and language therapy. Postoperative weakness was for more focal symptoms, such as cranial neuropathy and
defined as new postoperative focal motor weakness or visual change.
long tract signs such as hypotonia or hyperreflexia. The
presence of new postoperative ataxia, diplopia, and cranial Hydrocephalus Management
nerve palsies, where these were not present preoperatively, Figure 1 depicts the management of children with hy-
was also noted. Further data points were collected on the drocephalus. Fifty-one patients had an EVD inserted; 34
presence of CSF leak, infection, hemorrhage (and subse- of these devices were sited prior to definitive tumor resec-
quent evacuation), stroke, and mortality. Patients without tion, 9 were sited during the operation to resect the tumor
valid data points for surgical approach were excluded (with 9 more having a transparietal ventricular tap per-
from the cohort by listwise deletion. formed intraoperatively), and 8 were sited after tumor re-

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Toescu et al.

FIG. 1. Flowchart of patient inclusion and hydrocephalus management. The p values are derived from chi-square or Fisher’s exact
tests; see Hydrocephalus Management for details.

FIG. 2. Left: Density plot showing frequency of age at presentation for different symptoms. Right: Heat map showing clustering
analysis of symptoms. Each column represents 1 patient, each row 1 symptom; darker bars indicate where a symptom was pres-
ent. Linked lines on the left of the plot indicate clusters of symptoms that tended to group together in an individual patient. CN =
cranial nerve.

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 Toescu et al.

TABLE 1. Tumor histology and postoperative morbidity in 167 in which a craniectomy was undertaken. The intradural
patients with fourth ventricle tumors surgical approach to the fourth ventricle was transverm-
Characteristic No. % ian in 107 (64.1%) and telovelar in 55 (33.0%) patients.
Five patients (3.0%) were treated via other approaches: 3
Histology underwent biopsy only, and in 2 the tumor was accessed
Medulloblastoma 94 56.3 directly on dural opening as it protruded through the fo-
Ependymoma 30 18.0 ramen of Magendie (GTR was achieved in both of these
Pilocytic astrocytoma 30 18.0 cases). In the early epoch, the telovelar approach was
used in 10/81 (12.3%) cases; in the late epoch, this fig-
Choroid plexus neoplasm 5 2.99
ure was 45/81 (55.6%; difference in proportions 43.2%,
ATRT 4 2.40 95% CI 29.2%–54.9%, p < 0.001). Larger tumors tended
Other 4 2.40 to be approached by the transvermian route (39.1 cm3 vs
Postop morbidity 31.7 cm3 for the telovelar group), although this result did
CMS 48 28.7 not survive multiple comparison correction. Intraopera-
Weakness 40 24.0 tive neuronavigation and neurophysiological monitoring
were used in a minority of cases (both were used in 5/167,
Cranial neuropathy 30 18.0
3.0%), whereas intraoperative ultrasonography was used
Infection 21 12.6 in 49/167 (29.3%). A Cavitron ultrasonic surgical aspirator
Gait abnormality 21 12.6 (CUSA) was used in 98/167 (58.7%), and fixed retractors
Diplopia 14 8.38 were used in only 1 case.
CSF leak 7 4.19 EOR was graded as GTR in 117/167 (70.1%), STR in
Hemorrhage 5 2.99 47/167 (28.1%), and biopsy only in 3 (1.8%) cases. Table
Sensory deficit 4 2.40
1 shows tumor histology; medulloblastoma was the most
common (94/167, 56.3%). The “other” category comprised
30-day mortality 4 2.40 1 embryonal tumor with abundant neuropil and true ro-
Evacuation of hemorrhage 0 0 settes (ETANTR), 1 teratoma, 1 juvenile xanthogranulo-
Stroke 0 0 ma, and 1 high-grade glioma.
ATRT = atypical teratoid/rhabdoid tumor.
Postoperative Morbidity and Mortality
Forty-nine patients (29.3%) had no postoperative com-
plications documented. The remainder had at least one
section, due to either pseudomeningocele or late postoper- postoperative complication documented (Table 1). The
ative hydrocephalus. Twenty-nine EVDs were transfrontal most common was CMS, which occurred in 48/167 pa-
and 22 were transparietal. There was a significantly higher tients (28.7%) overall across the cohort. Table 2 shows pa-
proportion of patients who underwent eventual ventricu- tient features that were explored for their association with
loperitoneal shunt (VPS) insertion after EVD placement CMS. Children who developed CMS tended to be younger
(17/51, 33.3%) compared with those who did not have a (mean age 5.21 vs 6.29 years) and to have tumor volumes
prior EVD (16/116, 13.8%; difference in proportions that were statistically significantly larger (42.0 cm3 vs 34.1
19.5%, 95% CI 6.0%–34.2%, p = 0.00673). Eight of 22 cm3), although this relationship did not survive multiple
patients (36.4%) undergoing ETV later underwent VPS in- comparison correction. The proportion of children devel-
sertion, although there was no statistically significant dif- oping CMS was not related to whether they had presented
ference in the proportion receiving a shunt compared with with hydrocephalus (p = 0.741). The rate of CMS in any
those who had not had an ETV (25/145, 17.2%; difference given year did not appear to be associated with utiliza-
in proportions 19.1%, 95% CI 1.1%–40.5%, p = 0.0701). tion of different surgical approaches in that year (Fig. 3).
Five patients (all aged < 1 year) had a CSF reservoir placed Nor was there any association between surgical approach
during treatment, with one undergoing eventual VPS in- and CMS (p = 0.745) across the whole series. Multivariate
sertion (Fisher’s test, p = 1.0). Overall, 33 patients (19.8%) logistic regression modeling including age at presentation,
underwent VPS placement during this series; 8 were de surgical approach, and hydrocephalus at presentation did
novo, with the remainder having undergone prior CSF di- not reveal any additional statistically significant relation-
versionary procedures. ships between these variables and the development of
CMS.
Surgical Techniques In addition, the presence of CMS was not related to in-
Eighty-one of 167 patients (48.5%) were placed in the traoperative neuronavigation use (p = 0.625), CUSA use
sitting position; 86/167 patients (51.5%) were positioned (p = 0.482), or EOR (p = 0.601). The use of intraopera-
prone. The cohort was divided into two broadly equal tive ultrasound imaging, however, was found to be protec-
epochs: early (2009 or earlier, n = 84) and late (2010 or tive against the development of postoperative CMS (p =
later, n = 83). In the early cohort, the sitting position was 0.0365), although this result did not survive Bonferroni
used in 57/84 cases (67.9%); in the late epoch, this figure correction.
was 24/83 (28.9%; difference in proportions 38.9, 95% Table 3 demonstrates the association of surgical ap-
CI 24.0%–51.4%, p < 0.001). The posterior fossa was ex- proach and all elements of postoperative neurologi-
posed via a suboccipital craniotomy in all but one case, cal morbidity. Forty of 167 patients (24.0%) had new or

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TABLE 2. Association of patient features with CMS

Characteristic CMS + CMS − Difference (95% CI) p Value
Age at presentation, yrs 5.21 6.29 1.08 (0.41, 1.75) 0.121
Mean tumor volume, cm3 42.0 34.1 7.9 (6.37, 9.43) 0.0139
Hydrocephalus at presentation
Yes 34/48 (70.8%) 78/119 (65.5%)
5.3% (−10.8, 19.4) 0.634
No 14/48 (29.2%) 41/119 (34.5%)
Intraop CUSA*
Yes 24/98 (24.5%) 18/58 (31.0%)
6.5% (−7.5, 21.3) 0.482
No 74/98 (75.5%) 40/58 (69.0%)
Intraop ultrasonography†
Used 8/47 (17.0%) 41/117 (35.0%)
18.0% (2.6, 30.1) 0.0365
Not used 39/47 (83.0%) 76/117 (65.0%)
Intraop neuronavigation†
Used 2/47 (4.26%) 3/117 (2.56%)
1.7% (−3.9, 11.8) 0.625
Not used 45/47 (95.7%) 114/117 (97.4%)
EOR
GTR 35/48 (72.9%) 83/119 (69.7%)
STR 12/48 (25.0%) 34/119 (28.6%) NA 0.601
Biopsy 1/48 (2.08%) 2/119 (1.68%)
NA = not applicable.
* No data for 11 patients.
† No data for 3 patients. Bonferroni significance level 0.05/7 = 0.00714.

FIG. 3. Bar plot of CMS rate and rate of usage of different surgical approaches across the current cohort. The yearly CMS rate
trend was plotted in a solid line using a locally weighted regression method, locally estimated scatterplot smoothing (Loess
method), with error bars (standard error of proportion) shown in gray. The dashed line shows the series-wide average CMS rate.

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TABLE 3. Association of surgical approach and postoperative neurological morbidity

Characteristic Telovelar Transvermian Difference (95% CI) p Value
Mean tumor volume, cm 3
31.72 39.13 7.41 (5.94, 8.88) 0.0766
CMS 17/55 (31.0%) 29/107 (27.1%) 3.8% (−10.2, 18.9) 0.745
Cranial neuropathy 11/55 (20.0%) 19/107 (17.8%) 2.2% (−9.6, 16.0) 0.893
Diplopia 7/55 (12.7%) 7/107 (6.5%) 6.2% (−2.8, 18.0) 0.302
Gait abnormality 10/55 (18.2%) 11/107 (10.3%) 7.9% (−2.9, 20.8) 0.242
Sensory deficit 2/55 (3.6%) 2/107 (1.9%) 1.8% (−3.6, 10.6) 0.605
Weakness* 18/55 (32.7%) 21/107 (19.6%) 13.1% (−0.7, 27.8) 0.0984
* One patient developed weakness after another surgical approach. Bonferroni significance level 0.05/7 = 0.00714.

worsened weakness postoperatively. As the second most Missing Data

commonly encountered neurological sequela of fourth In the set of 18 patients who were excluded due to miss-
ventricle tumor surgery, this appeared to be more frequent ing data on surgical approach, the mean age was 5.40
following telovelar surgery than transvermian surgery, years (SD 4.08 years), and this was not statistically sig-
although this result was not statistically significant after nificantly different from the mean age in the included co-
Bonferroni multiple comparison correction (difference in hort described above (p = 0.572). Thirteen patients were
proportions 13.1%, 95% CI −0.7% to 27.8%, p = 0.0984). female. Overall, 5 cases of CMS (27.8%) were seen in this
New or worsened cranial neuropathy occurred in 30/167 cohort, which was almost identical to the proportion seen
patients (18.0%). There was no significant difference in its in the included cohort described above. The distribution
incidence between the telovelar and transvermian groups of tumor histology seen in this group spanned the same
(p = 0.893). New gait abnormality and diplopia were the range as that of the included cohort (5 medulloblastoma,
next most common neurological sequelae seen, at 12.6% 4 pilocytic astrocytoma, 7 ependymoma, 2 choroid plexus
and 8.38%, respectively, and there were no significant dif- papilloma). There was a bimodal distribution of year of
ferences in their incidence between the telovelar and trans- presentation in this set of patients, with 5 cases being ex-
vermian groups (p = 0.242 and 0.302, respectively). New cluded from 2005 and 3 from 2014; however, there were no
sensory deficit postoperatively was rare, occurring in just other patterns seen in the missing set of data. Therefore,
4/167 patients (2.40%). these were assumed to be missing completely at random,16
Overall, 21/167 patients (12.6%) developed an infec- thus justifying the use of listwise deletion.
tive complication during their early postoperative course.
There were 7 surgical site infections; 3 incidences of CSF Discussion
infection (2 Staphylococcus aureus, 1 Enterococcus spp.); This retrospective single-institution series demon-
4 lower respiratory tract infections; 4 urinary tract infec- strates that resection of fourth ventricle tumors in children
tions; 1 gastrointestinal infection; and 2 device infections carries a high risk of neurological morbidity in the early
(1 percutaneous endoscopic gastrostomy site, 1 tunneled postoperative period. One hundred sixty-seven patients
vascular line). were included in this analysis, and this comprises the larg-
CSF leaks occurred postoperatively in 7 cases (4.19%). est cohort of fourth ventricle tumors, either adult or pedi-
Two of these underwent wound debridement and resutur- atric, reported in the literature. Surgical approach evolved
ing; 2 had a lumbar puncture performed alongside resutur- significantly over the course of the series, with more em-
ing; 1 had a temporary external lumbar drain placed; in 1, phasis on prone positioning and the telovelar approach.
the associated pseudomeningocele was tapped; and 1 was Only 49/167 patients studied across a recent 17-year time
managed conservatively with oral antibiotics. There were span had no documented complications. The most com-
5 incidences of postoperative hemorrhage in this series mon complication of fourth ventricle tumor surgery re-
(2.99%), none of which required evacuation. mains CMS; its incidence has remained relatively con-
There were 4 deaths (2.40%) within 30 days of opera- stant throughout the series (28.7% overall), and we found
tion in the patient cohort. Two patients presented in ex- no evidence that this is influenced by favoring a telovelar
tremis, in coma or cardiorespiratory arrest. One was an over a transvermian intradural approach.
8-year-old girl who had presented with hemorrhage into Presenting symptoms in this series were most com-
a medulloblastoma, whose pupils were fixed and dilated monly found to be vomiting and ataxia, which is in keep-
preoperatively. Another was a 2-year-old boy who, despite ing with other pediatric fourth ventricle tumor series.4–6
resection of an anaplastic ependymoma, suffered poste- Younger patients were more likely to present with ataxia,
rior circulation infarction and hypoxic-ischemic midbrain which is plausible because older children have better-
injury during cardiorespiratory arrest at presentation pre- developed cerebellar motor function. Older patients were
ceding surgery. Two patients with metastatic medulloblas- more likely to describe visual changes and headache on
toma developed early hydrocephalus following resective presentation, owing to greater language capacity. The
surgery and, in view of their extensive disease burden, clustering analysis revealed that children tended to present
were subsequently managed palliatively. with either “global” neurological deficits (such as hydro-
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cephalus or symptoms thereof) or more focal ones (such as

Approach (%) GTR (%) CMS (%) VPS (%) 30-Day Mortality (%)
cranial neuropathy). In the 112 patients (67.1%) who pre-
sented with hydrocephalus, 44 underwent EVD, and this
group had a significantly higher rate of undergoing VPS
NR

NR

NR
4.5

0
0

0
insertion at a later date (p = 0.00789). This finding (which
has been previously reported11) is not unexpected; patients
undergoing EVD were thought to have been at a higher
risk of raised intracranial pressure. However, almost two-
3.85 thirds of patients with EVDs did not go on to receive a
24.6‡

NR

NR
NR
VPS (29/44, 65.9%), suggesting that the hydrocephalus
100

30
often resolves once tumor resection is completed. Twen-
ty-one patients underwent ETV, of whom 8 later needed
2.5
10.8

13.3
13.6

a VPS. The proportion of patients receiving shunts was

7.7
30

0
higher compared with those who had not had a prior ETV
(24/144, 16.7%; difference in proportions 21.4%, 95% CI
2.7%–43.1%, p = 0.0429), although this did not reach a
81.2
93.3
84.6
84.1
NR

45

70

level of statistical significance after multiple comparison

correction. These findings are similar to those previously
reported17 and indicate the need for careful patient selec-
Telovelar

tion for preresection ETV.

<53.8†
100
100
100
100
100
100

Overall, 33/167 patients (19.8%) underwent VPS inser-

tion in this series. There is a wide variation in this figure in
posterior fossa tumor series reported in the literature, from
10%18,19 to 100%;4 many lie in the range of 20%–30%.8,10–12
Medulloblastoma (%) Ependymoma (%) Astrocytoma (%)
Pilocytic

18.2
20.5
22.5

Surgical Techniques
0
0

10
0

The telovelar approach takes advantage of the cerebel-

lomedullary fissure to arrive at and divide the membra-
nous, noneloquent tela choroidea and inferior medullary
TABLE 4. Published case series of purely pediatric fourth ventricle tumors undergoing resection

velum20 between the lateral surface of the uvula and the

medial surface of the tonsil. In so doing, the fourth ven-
17.5
15.9

36.3
6.7

tricle can be accessed without any transgression of neu-

0

100
15

ral tissue. Since the first surgical case series describing its
use in 11 pediatric fourth ventricle tumors,9 several more
have been published in pediatric,4–8 adult,12 or mixed10,21
cohorts. Table 4 shows an exploration of common features
across the published pediatric series.
Very large fourth ventricle tumors tend to compress
52.5

93.3
36.3
61.4
100*

0
70

the vermis. In these situations, a transvermian approach

through an attenuated vermis is more straightforward than
a bilateral telovelar approach. This is reflected in our re-
sults, which show a larger mean tumor volume for transver-
mian compared with telovelar approaches. The telovelar ap-
3 pts <2 yrs, 12 pts <10 yrs

proach allows early identification of the floor of the fourth

7.3 (10 mos–12 yrs)
Mean Age (range)

* Posterior fossa medulloblastomas (not solely fourth ventricle).

8.7 (2.5–14 yrs)
6.05 (2–15 yrs)

ventricle, early control of large tumor blood vessels arising

‡ May include some temporary CSF diversionary procedures.
6.6 (1–16 yrs)
8 (1–17 yrs)

from the posterior inferior cerebellar artery, and excellent

8.1

exposure of the lateral recesses of the fourth ventricle. Al-

† Only frequency of transvermian approach reported.

though the entire fourth ventricle can be exposed through

the open cerebellomedullary fissure, tumor resection in the
rostral third of the fourth ventricle may be challenging; in a
study using image guidance to compare transvermian and
NR = data not reported; pts = patients.

telovelar approaches, the rostral half of the fourth ventricle

No. of
Pts

44
63

40
26
20
15
11

was more easily accessed via a transvermian approach.22

Prevailing opinion favors the telovelar approach over
the transvermian route; it has been postulated that the for-
Zaheer & Wood, 20108
Cobourn et al., 202025

Kellogg & Piatt, 19979

Rajesh et al., 20077

mer technique could reduce the incidence of postopera-

Atallah et al., 20194
Authors & Year

tive CMS. Our findings do not support this hypothesis, and

Qiu et al., 20165

indeed there is increasing evidence suggesting that there

Eissa, 20186

are factors in addition to surgical approach that may be

responsible for CMS. Ultimately, a randomized trial of
surgical approach for fourth ventricle tumors is required
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 Toescu et al.

to add definitive, high-quality evidence. However, this aspirator use;36 however, neither of these factors was found
is not a matter in which neurosurgeons will have clini- to be significant in our analysis.
cal equipoise, and decisions regarding surgical approach The data presented in this study suggest that the use
will probably continue to be made on an individualized of intraoperative ultrasonography significantly reduces the
basis depending on the size and rostrocaudal position of incidence of CMS (p = 0.0365). Depending on the operat-
the tumor in the fourth ventricle, as well as the intent of ing surgeon and the configuration of the tumor, ultraso-
resection and the experience of the surgeon. Anatomical nography was used to plan the ideal location of the cer-
particularities of such tumors may even mandate the use ebellar corticotomy—and therefore minimize disruption
of both approaches simultaneously. of the superficial and deep cerebellar tissue—and to assess
depth of resection within the ventricle and its margins, en-
Cerebellar Mutism Syndrome suring that no uninvolved tissue, particularly at the cer-
The overall incidence of CMS in this series was 28.7%. ebellar peduncles, was resected. Navigation by anatomical
This is broadly similar to that reported in several stud- landmarks was generally considered sufficient for fourth
ies over the past 20 years,23,24 although there is some evi- ventricle tumors across this series—hence the infrequent
dence that CMS is declining in incidence.25,26 Because use of neuronavigation software.
definitions differ across published series, it is important to
exercise caution in comparing incidence. There is a clear Other Postoperative Complications
need for standardized diagnostic criteria for CMS phe- A single-center series of fourth ventricle tumors in
nomenology and severity. Currently, there are only a few adults and children11 reported that 76% of patients had a
published scales in use,27,28 mostly lacking clinical valida- documented postoperative complication. Moreover, the
tion. In this study, we took a pragmatic approach to defin- deficits noted (motor and gait disturbance, speech and
ing CMS based on review of contemporaneous medical swallowing disturbance, cranial nerve deficit, and visual
notes by looking for the key criterion of mutism, although impairment) are similar in frequency to those presented
this probably included children with reduced speech out- here. An international retrospective series of midline—
put postoperatively, which is an inherently broad and het- though not solely fourth ventricle—pediatric posterior
erogeneous group. As the field develops, the constellation fossa tumors found that the most common postoperative
of symptoms and signs that can be ascribed to CMS will neurological deficit was bulbar cranial neuropathies, at
become more nuanced—and certainly nonbinary—than 14.1%37 (18.0% in the current series). Allowing for differ-
previously thought.29,30 ences in data collection and classification of deficits, these
The neuroanatomical basis of CMS is thought to lie in figures are similar to those presented in this series.
the efferent cerebellar pathway,31,32 and the insult to this Our data indicate the low incidence of postoperative
neural circuitry putatively arises at the time of surgery. neurosurgical complications requiring reoperation, such
Therefore, sparing surgical damage to cerebellar paren- as hemorrhage (2.99%), CSF leak (4.19%), and CSF infec-
chyma might be expected to reduce the incidence or sever- tion (1.79%). There were no incidences of postoperative
ity of CMS. In a series of 16 patients in whom the telovelar stroke seen in this patient cohort. The 30-day mortality
approach was used, none developed CMS,21 although this rate was 2.40%, although 2 of these 4 children had already
cohort was mostly composed of adult patients, in whom suffered irreversible neurological damage prior to surgery
CMS is not a typical postoperative finding. Other series due to the acute nature of their presentations.
have shown that the transvermian approach appears to be It is important to recognize that the potential complica-
a risk factor for CMS.33,34 A recent series of 63 medul- tions following resective tumor surgery in the fourth ven-
loblastomas (not solely confined to the fourth ventricle) tricle are not trivial. Taken together, these figures can help
found that vermian incision “bore a statistically significant provide realistic expectations to clinicians, relatives, and
relationship to CMS,” despite the simultaneous finding patients ahead of such surgery, and can be usefully quoted
that a transvermian approach did not lead to a significantly during the consent process.
higher rate of CMS.25
One key finding in this study, namely that the adoption Limitations
of a telovelar approach does not significantly reduce the Our study was retrospective in nature and therefore
incidence of CMS, is corroborated by the work of other is subject to the usual caveats of incomplete data and re-
groups.8,35 Despite this, there has been a popularization of call bias. The use of listwise deletion to remove subjects
the use of the telovelar approach in posterior fossa tumor with incomplete data sets assumes that data are missing
surgery, as reflected in the literature and in the results pre- completely at random,16 and although this assumption is
sented here, shown graphically in Fig. 3. The latter shows thought to be correct, it cannot be tested. Multiple impu-
that CMS rates have remained relatively constant despite tation would have been unsuitable to address the miss-
an increasing proportion, year on year, of fourth ventricle ing data, given that most variables collected were binary.
tumor resections carried out via the telovelar approach, Despite our best efforts at identifying all cases of fourth
and this reflects a granularity of data on this issue that has ventricle tumors treated at our institution to report on the
not been reported before. largest series of fourth ventricle tumors in the literature,
The persistence of CMS despite the increasing use of the numbers in this series remain small, and results may
the telovelar approach in this series may not be related have been affected by the missing cases. Furthermore, this
simply to the surgical approach itself, but rather to second- is a heterogeneous group in which a total of 10 consultant
ary insults, such as fixed retractor systems25 and ultrasonic neurosurgeons performed tumor resections. Although data
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Toescu et al.

collection forms were prespecified, there may have been without splitting the vermis:​the cerebellomedullary fissure
a number of unobserved confounding factors that make approach. Pediatr Neurosurg. 1997;​27(1):​28–33.
it difficult to make a causal association. Finally, the fo- 10. Tomasello F, Conti A, Cardali S, et al. Telovelar approach
to fourth ventricle tumors:​highlights and limitations. World
cus of this communication was on the early postsurgical Neurosurg. 2015;​83(6):​1141–1147.
phase in order to faithfully describe the range of patient 11. Ferguson SD, Levine NB, Suki D, et al. The surgical treat-
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diotherapy regimens; longer-term outcomes were therefore 12. Winkler EA, Birk H, Safaee M, et al. Surgical resection of
not part of this study. fourth ventricular ependymomas:​case series and technical
nuances. J Neurooncol. 2016;​130(2):​341–349.
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In this series of pediatric fourth ventricle tumors we 1304–1305.
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Acknowledgments drainage following posterior fossa tumor surgery. J Neuro-
Mr. Toescu is funded by Great Ormond Street Hospital Chil- surg. 2019;​132(1):​296–305.
dren’s Charity (award no. 174385) and is an Honorary Research 19. Rappaport ZH, Shalit MN. Perioperative external ventricular
Fellow of the Royal College of Surgeons of England. All research drainage in obstructive hydrocephalus secondary to infraten-
at the Great Ormond Street Hospital NHS Foundation Trust and torial brain tumours. Acta Neurochir (Wien). 1989;​96(3-4):​
UCL Great Ormond Street Institute of Child Health is made pos- 118–121.
sible by the NIHR Great Ormond Street Hospital Biomedical 20. Mussi ACM, Rhoton AL Jr. Telovelar approach to the fourth
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30. Schmahmann JD. Neuroanatomy of pediatric postoperative Author Contributions

cerebellar cognitive affective syndrome and mutism. Neurol- Conception and design: Aquilina, Toescu. Acquisition of data:
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Disclosures
The authors report no conflict of interest concerning the materi-
als or methods used in this study or the findings specified in this
paper.

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