Childs Nerv Syst (2010) 26:267–271
DOI 10.1007/s00381-009-1027-z
CASE REPORT
Chronic uncal herniation secondary to posterior fossa
shunting: case report and literature review
Suhas Udayakumaran & Liat Ben Sira &
Shlomi Constantini
Received: 7 October 2009 / Published online: 14 November 2009
# Springer-Verlag 2009
Abstract
Introduction Chronic herniation syndromes other than
tonsillar herniation are not well-recognized. Transtentorial
uncal herniation in its chronic form has been reported in
only few case reports (Horowitz et al., J Neuroimaging
12:78–79, 2002; Naidich et al., Radiology 158:431–434,
1986; Ng and Valiante, J Clin Neurosci 16:944, 2009; Ng
and Valiante, J Clin Neurosci 16:984, 2009). We hereby
illustrate a case with this rare finding, including MR
imaging, and analyze this phenomenon.
Case report A 15-year-old girl had a resection of a
cerebellar pilocytic astrocytoma at 5 years of age. Two
months later, she underwent a cystoperitoneal shunt for
persistent headache and pseudomeningocele, secondary to
an enlarging cyst at the tumor bed. Subsequently, her shunt
was upgraded to a more conservative valve following
clinical evidence of over drainage. This was also associated
with imaging compatible with bilateral uncal herniation.
After the procedure, she had relief of symptoms.
Conclusion We conclude that a negative pressure gradient
in the posterior fossa, relative to the supratentorial
compartment, is the etiology of the chronic uncal hernia-
S. Udayakumaran : S. Constantini (*)
Department of Paediatric Neurosurgery, Dana Children’s Hospital,
Tel Aviv Sourasky Medical Center, Tel Aviv University,
6 Weizman St.,
Tel Aviv 64239, Israel
e-mail: sconsts@netvision.net.il
L. Ben Sira
Paediatric Radiology, Dana Children’s Hospital,
Tel Aviv Sourasky Medical Center, Tel Aviv University,
6 Weizman St.,
Tel Aviv 64239, Israel
tion in our patient. Comparing the case reports in the
literature with our case, we postulate that chronic uncal
herniation is a complication of shunting of a posterior fossa
fluid cavity in children, many of them with Dandy–Walker
syndrome and/or other cerebellar cystic formations. The
treatment priority at presentation should be to rule out
shunt malfunction. In the event of association with clinical
over-drainage syndrome, there may be a role for changing
the shunt system into a more conservative drainage
combination.
Keywords Chronic uncal herniation . Tentorial herniation .
Herniation syndromes . Intracranial hypotension .
Posterior fossa shunt . Dandy–Walker syndrome .
Cystoperitoneal shunt
Introduction
Various cerebral herniation syndromes are well-known.
Transtentorial herniation in its acute form is a well-
recognized entity with dismal clinical implications. It is
usually related to a supratentorial downward driving
force and raised intracranial pressure (ICP). It may also
be related transiently to postcraniotomy hypovolemia
[6].
Chronic herniation syndromes, other than tonsillar herni-
ation in Chiari malformation, are not well-recognized.
Transtentorial herniation in its chronic form has been
reported only in a few case reports, with undefined
pathophysiology and clinical implications [5, 8, 12, 13].
We hereby present a case of chronic uncal herniation
where the pathophysiology seems clear. Implications for
surgical treatment will be discussed.
268
Fig. 1 Cranial CT demonstrating postoperative changes including the
collapsed peritumoral cyst in the right cerebellar hemisphere (arrow)
with a shunt catheter tip seen within the cavity
Case report
A 15-year-old girl had a resection of cerebellar cystic
pilocytic astrocytoma at 5 years of age. Postoperatively, she
developed symptoms secondary to an enlarging cyst at the
tumor bed. Two months later, she underwent a cystoper-
itoneal shunt (CPS), with a low-pressure valve, for
persistent headache and pseudomeningocele. Subsequently,
8 months later, her shunt was upgraded for symptoms of
headache associated with radiological evidence of over
drainage and bilateral uncal herniation. She had relief of her
symptoms.
At the age of 11 and 13 years, she had shunt revisions
for suspected shunt malfunction; one was an episode of
Fig. 2 A recent MR axial T2 and coronal T1-weighted images, showing bilateral parahippocampal herniation (arrows). The ventricles are nondilated
Childs Nerv Syst (2010) 26:267–271
proximal disconnection and the second a documented distal
malfunction.
In the last 2 years, she continues to present to us with
intermittent, transient symptoms of diplopia and headache.
Cranial computer tomography (CT) during these episodes
has revealed no evidence suggestive of shunt malfunction.
The ventricles remain undilated and show the posterior
fossa shunt with a collapsed cyst (Fig. 1). Repeated
fundoscopy during these episodes showed no papilloedema.
According to the patient, these episodes would disappear
with analgesics and by resting in a horizontal position.
A recent MR showed persistent bilateral uncal hernia-
tion. This was associated with undilated ventricles (Fig. 2).
The posterior fossa appeared “tight” with a slit-like fourth
ventricle and cystic cavity. It also showed venous engorge-
ment associated dural enhancement of posterior fossa
meninges (Fig. 3).
In view of her transient symptomatology and doubtful
clinical significance of the imaging finding, we are closely
following her and may intervene depending on her
progress.
Discussion
The earliest clear description of uncal herniation per se is
attributed to Adolf Meyer (1866-1950), who, in 1920,
described a supratentorial tumor causing tentorial hernia-
tion, which impinged on the posterior cerebral artery and
caused hemianopia. He reported: “The falx and tentorium
constitute an important protection against any sudden
impacts of pressure by keeping apart heavy portions of
the brain, but they also provide an opportunity for trouble
in case of swelling or need of displacement” [1]. Trans-
tentorial herniation in its acute form is well-recognized and
is usually related to a supratentorial driving force and raised
ICP. It may also be related transiently to postcraniotomy
Childs Nerv Syst (2010) 26:267–271 269

Fig. 3 Recent sagittal T1and

axial postgadolinium demonstrat-
ing herniation of left cerebellar
tonsil (arrow) with prominent
dural venous engorgement, “slit
like” fourth ventricle and tight
posterior fossa

hypovolemia [6]. Lumbar puncture in a patient with
unilateral high ICP may precipitate uncal herniation and
lead to a full-blown brainstem compression syndrome.
Transtentorial herniation in its chronic form has been
reported in a few case reports with somewhat confusing
clinical implications and unclear pathophysiology. To the
best of our knowledge, three other publications are
available, with a total of ten patients (including our patient),
in the English literature on this issue [5, 8, 12, 13]. Most of
these reported cases were in the pre-MR era. Table 1
represents a summary of the published case reports. A
combined meta-analysis of all the described patients with
this phenomenon highlights the following features:
& Age: The age of presentation is usually pediatric (eight
of ten patients). Two patients in the series were adults.
One of the adult patient aged 27 had his first clinical
presentation at 12 years of age. Early age of presenta-
tion may be related to the underlying condition that led
to shunting of the posterior fossa [Dandy–Walker
syndrome (DWS) and cerebellar cystic lesion] or to
increased deformability of brain tissue in children.
& Nine of ten patients had posterior fossa CPS, and eight
patients had DWS. Our patient had a cerebellar cyst
secondary to total removal of cerebellar astrocytoma. It
seems obvious from these facts that this complication is
specific to the presence of a draining shunt in the
posterior fossa.
The explained pathophysiology seems to be quite trivial
in these cases. The continuous drainage of cerebrospinal
fluid induced by the posterior fossa shunt results in a
chronic negative pressure gradient between the supra- and
infratentorial compartments. This negative pressure creates
a vector of force, leading to downward displacement of the

Table 1 Summary of published case reports

Patient series Age at Type of shunt Basic clinical Symptomatology Management Authors' explanation
presentation (CP/VP/Both) diagnosis for parahippocampal
herniation

Ng et al. [12, 13], 27 years CP Dandy–Walker Complex partial Resection of the Past history of
n=1 (2009) syndrome seizure (since herniated portion herniation
15 years of age)
Naidich et al. [8], Less than 13 3 CP 4 CP+VP Dandy–Walker 4 shunt malfunction 4 patients multiple 4 shunt malfunction
n=7 (1986) (2 infants) syndrome shunt revision
1 EVD in posterior 2 died 1 external drainage
fossa shunt of the cyst
2 unknown 2 unknown
Our patient 12 years CP Cerebellar cystic Headache Shunt upgrade and Shunt related
astrocytoma Vomiting observation hypotension
Diplopia
Horowitz M et al. 41 years No shunt Atypical Neuralgia Biopsy of the Embryologically-related
[5], n=1 (2002) trigeminal herniated portion anatomic variant
neuralgia
270
uncus. The anatomical presence of a rigid tentorium with a
hiatus in it may facilitate the development of this kind of
gradient, effective enough to create a chronic herniation.
This phenomenon appears analogous to that of an acquired
Chiari secondary to lumboperitoneal shunts [3].
Naidich et al. [8] pointed out that this phenomenon
might reflect shunt malfunction. Although their series did
not mention the objective evidence of a raised ICP, due to
shunt blockage leading to a chronic phenomenon, we
believe a negative pressure gradient with negativity toward
posterior fossa is a mandatory prerequisite of the chronic
finding. Hence, an association with malfunctioning lateral
ventricular shunt may be a contributory event in selected
cases, as in the Naidich et al. series [8]. Moreover, most
supratentorial shunt malfunctions will lead to an acute or
subacute clinical syndrome. If tentorial herniation occurs in
these instances, full-blown acute brainstem compression
will inevitably lead to coma and decerebration.
Ng et al. had postulated that the parahippocampal
herniation, which they observed, was related to herniation,
from the suspected history of viral encephalitis “aggravated”
by DWS [12, 13]. Unlike the patient described by Ng
et al., interestingly, our patient had no history suggestive
of herniation before the appearance of the radiological
finding and symptoms. Repeated cranial CT in episodes
of headache and diplopia showed no signs suggestive of
shunt malfunction, and fundoscopy during these episodes
did not show papilloedema.
Horowitz et al. reported a 41-year-old man who
presented with “atypical” trigeminal neuralgia [5]. Along
with parahippocampal herniation, the imaging demonstrated
Chiari malformation. There was also an extremely caudal
transverse sinus and a low tentorium. They postulated that
this combination might represent a congenital anatomical
variant [5].
With the exception of the case reported by Horowitz
et al. [5], the predisposition to chronic uncal herniation in
shunted DWS and other cerebellar cysts is obvious. This
complication being unreported in cerebellar astrocytoma
resection may be due to the low frequency of CPS in this
situation. Continuity of the shunted cystic space to fourth
ventricle may be another prerequisite for the development of
chronic uncal herniation, as is natural in DWS and
postoperative in our patient (Fig. 2). This may be the reason
that this complication has never been reported following
CPS in treatment of a retrocerebellar arachnoid cyst [11].
Why the complication has been unreported in other recent
series on treatment of DWS is unclear [7, 15]. Naidich et al.
[8] noticed chronic cerebral herniation in seven (27%) of 26
patients.
Can choosing a more conservative shunting system in
selected patients prevent this complication? Our patient
initially had a low-pressure valve with a partial positive
Childs Nerv Syst (2010) 26:267–271
response following upgrading. It is advised therefore to use
more restrictive hardware in similar cases.
The symptomatologies associated with chronic cerebral
herniation seem varied. Our patient had intermittent
headache, vomiting, and diplopia, which can be explained
by traction on pain-sensitive intracranial structures,
meninges, and the abducent nerve [4, 9]. The patient
reported by Ng et al. presented with a seizure. Ng et al.
have successfully treated the seizure disorder (by surgical
disconnection of hippocampus); shunt-related hypotension
as a cause of seizures has been reported, albeit in only a few
case reports [2]. One case reported by Horowitz et al.
presented with trigeminal neuralgia. Such a facial pain may
represent local irritation or traction on the fifth nerve.
Naidich et al. [8] reported a difficult clinical course marked
by multiple shunt revision.
The phenomenon may be self-limiting in view of the
limited volume of fourth ventricle. The implication here is
that a collapsed fourth ventricle (as in Fig. 3) may no longer
contribute to the phenomenon, thus relieving the clinical
features. This may explain the episodic nature of the
presentation as seen our patient.
Chronic transtentorial herniation may be misinter-
preted as a posterior fossa tumor [5] or a medial temporal
lobe or ambient cistern mass lesion [8]. They are usually
bilateral (80% in this series). Occasionally, high-resolution
imaging may show deviation of the posterior cerebral
artery around the herniation site [8]. The predisposing
causative pathology may be suggestive, such as a CPS
associated with DWS.
The MRI in our patient, apart from the findings of
uncal herniation, showed venous-engorgement-associated
enhancement of the posterior fossa meninx, a slit-like
fourth ventricle, and a relatively tight posterior fossa.
These findings are analogous to the corresponding
Fig. 4 Cranial CT done 5 years back showed the same finding of
parahippocampal herniation (arrows) without the presence of ventric-
ular dilatation
Childs Nerv Syst (2010) 26:267–271
components in an over-shunted supratentorial ventricle
with secondary intracranial hypotension syndrome [10,
14]. The slit-like fourth ventricle was probably secondary
to the fourth ventricle being in continuity with the cystic
space with a draining shunt tip (Fig. 3). These findings
probably corroborate our postulation of a posterior fossa
hypotension creating a secondary negative pressure gradient,
relative to the supratentorial space.
We noticed the phenomenon within a few months of
insertion of the posterior fossa shunt, and the finding has been
persistent (Fig. 4). Naidich et al. reported changes in the
severity of herniation, with it varying relative to the success
of the lateral ventricle decompression. They also reported
complete disappearance of herniation in one patient [8].
Can chronic uncal herniation predispose to acute life
threatening brainstem compression? A theoretical possibility
of catastrophic consequence remains. Two out of the seven
patients in Naidich et al. series died. It is unclear how
contributory chronic cerebral herniation was to the event.
Naidich et al. [8] opined that the finding might be a bad
prognostic sign.
We recommend that patients with such MR findings be
handled cautiously. If asymptomatic, they can be followed.
However, if symptoms persist or progress, treatment should
be initiated. Situations where a patient is shunted, both
supra- and infra-tentorially, and has uncal herniation can
prove dangerous. In such a situation, supra-tentorial shunt
malfunction may lead to increased downward displacement
and acute brainstem compression. Upgrading the posterior
fossa system according to the surgeon’s preference is a
possibility in instances associated with isolated posterior
fossa shunts.
Conclusions
We postulate that chronic uncal herniation is a complication
of posterior fossa shunting that occurs mostly in children
with DWS or other cystic cavities that are in communica-
tion with the fourth ventricle. There may be a role of
upgrading of the shunt system in symptomatic patients. In
271
instances where a lateral ventricular shunt is also present,
the priority should be to rule out shunt malfunction.
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