BRITISH JOURNAL OF NEUROSURGERY, 2017

VOL. 31, NO. 3, 296–299

http://dx.doi.org/10.1080/02688697.2017.1317719

SHORT REPORT

Hydrocephalus and trigeminal neuralgia: exploring the association and

management options
Yuuki Charlie Barke Naa , Shyam Vijay Pujarab, Ahmad Abdelhai Moussab, Amy Charlotte Gerrishc and
Surajit Basub
a
School of Medicine, Queen’s Medical Centre, University of Nottingham, Nottingham, UK; bDepartment of Neurosurgery, Queen’s Medical Centre,
Nottingham University Hospitals, Nottingham, UK; cDepartment of Radiology, Queen’s Medical Centre, Nottingham University Hospitals,
Nottingham, UK

ABSTRACT ARTICLE HISTORY

We report the successful surgical management of three patients with trigeminal neuralgia and hydroceph- Received 1 November 2016
alus. MRI revealed no neurovascular contact at the trigeminal root entry zone. Trigeminal neuralgic symp- Accepted 23 March 2017
toms were controlled following alleviation of hydrocephalus. We hypothesize that trigeminal nerve
traction, secondary to hydrocephalus, as the cause for their trigeminal neuralgia.
KEYWORDS
Trigeminal neuralgia;
hydrocephalus; traction;
endoscopic third
ventriculostomy; ventriculo-
peritoneal shunt

Introduction
The aetiology of Trigeminal Neuralgia (TGN) is unknown and
speculative. Various authors have highlighted neurovascular/
tumour compression, demyelination or inflammatory plaques at
the root entry zone (REZ) as the main aetiology leading to
hyper-excitability of the nerve.1 Some researchers have suggested
physical contact or distortion of the nerve resulting in traction at
the REZ as a possible cause of TGN.2
TGN associated with hydrocephalus has limited reports across
the world.3,4 Similar reports have been described in individuals
with Chiari malformation and Dandy-Walker syndrome present-
ing with hydrocephalus and TGN.2 We present the management
of three cases and explore the likely pathophysiology in our
discussion.
History
Case one
This 31 year old male was diagnosed with right sided (V2/V3)
TGN. He presented with a 3 month history of severe intermittent
right-sided facial pain that was described as electrical shock. On
examination, he had preserved touch, pinprick and temperature
sensation. The patient had been tried on Carbamazepine,
Pregabalin and Gabapentin without adequate symptom control;
reporting significant neuralgic pain interfering with daily living.
He was referred for consideration of surgical management
options, including exploration of right cerebello-pontine angle.
MRI revealed no major vascular compression or other com-
pressive pathology at right REZ of right trigeminal nerve.
Moreover, MRI revealed hydrocephalus secondary to obstruction
of aqueduct of Sylvius, with marked dilation of the third and lat-
eral ventricles. This hydrocephalus was clinically asymptomatic in
the patient. However, establishment of CSF circulation to the
posterior fossa was thought to be a reasonable first step, due to
the potential risk of transtentorial herniation of brain following
posterior fossa access in the presence of aqueduct stenosis.
The patient underwent endoscopic third ventriculostomy. His
neuralgic symptoms gradually resolved and he became pain-free
without medication at 6 months. Post-operative MRI demonstrated
movement of the brainstem (Figure 1(a,b)) as well as reduced taut-
ness and a wider right trigeminal nerve (Figure 1(c,d)), measuring
2.2 mm pre-operatively and 2.7 mm post-operatively.
Case two
A 45 year old male was diagnosed with left sided (V2/V3) TGN.
His history of pain and the examination findings were analogous
to the case above. Despite maximal dose of Carbamazepine
(1200mg daily), there was no significant pain relief.
MRI revealed incidental finding of triventriculomegaly, in
keeping with significant arrested supratentorial hydrocephalus
with a web in aqueduct. Moreover, the gentleman was clinically
asymptomatic in relation to the hydrocephalus. There was no evi-
dence of microvascular compression or inflammatory plaques in
the REZ of the trigeminal nerve. The patient was offered endo-
scopic third ventriculostomy to treat hydrocephalus as the initial
step.
There was an immediate improvement of his neuralgic pain.
Post-operative imaging confirmed patent third ventriculostomy
and decompressed ventricles. This radiological and clinical find-
ing remained evident at a two month MRI scan. Furthermore,

CONTACT Yuuki Charlie Barke Na mzycb8@nottingham.ac.uk School of Medicine, Queen’s Medical Centre, University of Nottingham, B81a B floor,
Nottingham, United Kingdom, NG7 2UH
ß 2017 The Neurosurgical Foundation
 BRITISH JOURNAL OF NEUROSURGERY 297

Figure 1. MRI sagittal CISS sections pre-operatively (a) and post-operatively (b) for case one. The distance between the jugum sphenoidale and dorsum sellae bone struc-
tures was measured to ensure consistency of location between the scans. Movement of the brainstem pre- and post-operatively was demonstrated by a comparative differ-
ence in the angle between the jugum sphenoidale–dorsum sellae line and dorsum sellae–midbrain pontine junction line. Furthermore, comparison between pre-operative
(c) and post-operative (d) reconstructed MRI sagittal FIESTA sections revealed reduced tautness and increased width of the right trigeminal nerve post-operatively.

postsurgical MRI revealed movement of the brainstem
(Figure 2(a,b)), with reduced tautness and a wider left trigeminal
nerve (Figure 2(c,d)), measuring 2.5 mm pre-operatively and
2.9 mm post-operatively.
The patient later developed a facial pain of a neuropathic
characteristic, localized only to the angle of his mouth and cheek
that was dissimilar to his previous TGN. The neuropathic pain
was controlled with pharmaceuticals.
Case three
50 year old female presented a 5 year history of right sided TGN
with a radiologically proven vascular compression of the REZ.
Patient had right microvascular decompression (MVD) with com-
plete symptom control.
Two years later, she developed recurrent attacks of disequilib-
rium, nausea and vomiting as well as recurrence of neuralgic
(electric shock like pain) symptoms. The recurrence of neuralgia
was reported to be similar to pre-MVD state. MRI revealed a
communicating hydrocephalus and excluded recurrence of any
vascular contact or displacement of the Teflon placed at the REZ.
A programmable ventriculoperitoneal (VP) shunt (pressure set at
1.5) was placed to manage symptoms of hydrocephalus. Patient’s
symptoms of neuralgia settled following CSF diversion.
Interestingly, this patient had a relapse of hydrocephalus and
neuralgic pain symptoms following a shunt dysfunction 2 years
later, with relief of symptoms following revision of the shunt.
298 Y. C. B. NA ET AL.

Figure 2. MRI sagittal CISS sections pre-operatively (a) and post-operatively (b) for case two. The distance between the jugum sphenoidale and dorsum sellae bone struc-
tures was measured to ensure consistency of location between the scans. Movement of the brainstem pre- and post-operatively was demonstrated by a comparative dif-
ference in the angle between the jugum sphenoidale–dorsum sellae line and dorsum sellae–midbrain pontine junction line. Furthermore, comparison between pre-
operative (c) and post-operative (d) reconstructed MRI sagittal FIESTA sections revealed reduced tautness and increased width of the left trigeminal nerve post-operatively.

Discussion Similar reports have been documented in individuals with

TGN associated with hydrocephalus is rare, with limited reports
in current literature.3,4 Findler and Feinsod4 described a case
exhibiting right sided facial pain and hydrocephalus due to aque-
ductal stenosis. The symptoms resolved following insertion of a
VP shunt. Remarkably, shunt malfunction was accompanied by
relapse, and shunt revision was followed by relief of symptoms.
In another study, Tucker et al.3 reported two patients with
unilateral TGN associated with aqueductal stenosis induced
hydrocephalus. Hydrocephalus and TGN were relieved following
CSF diversion, and relapsed later on shunt dysfunction. Tucker
et al. suggested brain stem shift may have caused traction on tri-
geminal nerve sensory root prompting unilateral TGN.
Chiari malformation and Dandy-Walker syndrome presenting
with hydrocephalus and TGN.2 Vince et al.2 described a patient
with bilateral TGN associated with Chiari’s type 1 malformation
and hydrocephalus. Interestingly, stretching of the trigeminal
nerve due to caudally displaced brainstem was noted intra-
operatively.
The observations in our article as well as the previous reports
suggest the association of hydrocephalus with neuralgic symp-
toms in these patients are more than by chance. In two of our
patients, the trigeminal nerve looked and measured wider on
postsurgical reconstructed MRI taking into account fallacies when
comparing scans undertaken at different times (Figures 1(c,d)
and 2(c,d)). The MRI appearance of the nerve was not
 BRITISH JOURNAL OF NEUROSURGERY 299

commented on in previous reports. The comparative thinness Funding

observed in pre-operative scans can be explained as a marker of
No external funding was involved in the development of this study.
stretch, and in susceptible patients be the mechanism of inducing
neuralgic symptom. Interestingly, other traction cranial neuropa-
thies associated with hydrocephalus have been reported.5
On MRI sections, shift in brainstem were demonstrated for Ethical approval
both case one and case two (Figures 1(a,b) and 2(a,b)). We Ethical approval was not sought.
hypothesize that hydrocephalus alters the dynamics of CSF in the
basal cisterns causing traction of the pre-pontine segment of tri-
geminal nerve, which is anchored at the porus trigeminus.
Submission approval
Treatment of hydrocephalus modifies the pressure gradient along
neuraxis and can potentially reverse any micro-shift of the neural The institution accepts the submission of the paper.
structures.
ORCID
Conclusions Yuuki Charlie Barke Na http://orcid.org/0000-0002-4618-9940
Approximately 5% of TGN patients are reported without vascular
compression at REZ. Hydrocephalus or other factors that may
contribute to traction at REZ should be considered a plausible
cause. This article is a reminder that non-vascular analogous aeti- References
ologies causing traction or distortion can cause symptomatic 1. Nurmikko TJ, Eldridge PR. Trigeminal neuralgia-pathophysiology,
TGN in susceptible patients that one may encounter in clinical diagnosis and current treatment. Br J Anaesthesia 2001;87:117–32.
practice. 2. Vince GH, Bendszus M, Westermaier T, et al. Bilateral trigeminal neur-
algia associated with Chiari's type I malformation. Br J Neurosurg
2010;24:474–6.
Acknowledgements 3. Tucker WS, Fleming R, Taylor FA, Schultz H. Trigeminal neuralgia in
aqueduct stenosis. Can J Neurol Sci 1978;5:331–3.
None. 4. Findler G, Feinsod M. Reversible facial pain due to hydrocephalus with
trigeminal somatosensory evoked response changes. Case report. J
Neurosurg 1982;57:267–9.
Disclosure statement 5. Cultrera F, D'Andrea M, Battaglia R, Chieregato A. Unilateral oculo-
motor nerve palsy: unusual sign of hydrocephalus. J Neurosurg Sci
Y.N., S.P., A.M., A.G. and S.B. declare that they have no conflict of interest. 2009;53:67–70.
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