Clinical Neurology and Neurosurgery 125 (2014) 151–154

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Clinical Neurology and Neurosurgery
journal homepage: www.elsevier.com/locate/clineuro

Review

A review of cavernous malformations with trigeminal neuralgia夽
Kazuhide Adachi ∗ , Mitsuhiro Hasegawa, Takuro Hayashi, Shinya Nagahisa, Yuichi Hirose
Department of Neurosurgery, School of Medicine, Fujita Health University, 1-98, Kutsugake Dengakugakubo, Toyoake City, Aichi 470-1192, Japan

a r t i c l e

i n f o

Article history:
Received 15 April 2014
Received in revised form 16 July 2014
Accepted 20 July 2014
Available online 30 July 2014
Keywords:
Trigeminal neuralgia
Cavernous malformation
Anterior transpetrosal approach
Pons
Operative approach

a b s t r a c t
Cavernous malformation with trigeminal neuralgia is relatively rare; only 10 cases have been reported.
In deciding treatment strategies, it is helpful to classify cavernous malformation according to its origin, as
follows: in the Gasserian ganglion (Type G); between the cisternal and intra-axial portions of the trigeminal nerve root (Type C); in the intra-axial trigeminal nerve root in the pons (Type P); or in the spinal
tract of the trigeminal nerve root (Type S). A 62-year-old male presented with left trigeminal neuralgia (V2 area) and left facial hypoesthesia. Imaging studies revealed a cerebellopontine angle mass lesion
with characteristics of a cavernous malformation and evidence of hemorrhage. The lesion was completely
removed via a left anterior transpetrosal approach. The mass was attached to the trigeminal nerve root; it
was located between the cisternal and intra-axial portions of the nerve root, and feeding off microvessels
from the trigeminal nerve vascular plexus. Histological examination confirmed a cavernous malformation. In this case, the cavernous malformation was Type C. We review cases of cavernous malformation
with trigeminal neuralgia and discuss therapeutic strategies according to the area of origin.
© 2014 Elsevier B.V. All rights reserved.

Contents
1.
2.
3.
4.

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Case report . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1. Introduction
Idiopathic trigeminal neuralgia is mainly caused by microvascular compression. The causes of symptomatic trigeminal neuralgia
include herpes zoster and tumor compression or involvement. Neoplastic trigeminal neuralgia is generally caused by a schwannoma,
meningioma, or epidermoid. In addition, rare cases of trigeminal
neuralgia due to cavernous malformation (CM) have been reported.
CM of the central nervous system, also called cavernous angioma
or cavernoma, is found in approximately 0.4–0.9% of the population [1,2]. CM may be intra-axial, occurring in areas such as the
brain stem, cerebellum, and cerebral hemisphere [3], or extra-axial.

夽 This study was not supported by grants or other funding sources, and none of
the authors has industry affiliations related to the materials or procedures used in
this study.
∗ Corresponding author. Tel.: +81 562 93 2111; fax: +81 3 3354 8053.
E-mail address: kazu-adachi@rio.odn.ne.jp (K. Adachi).
http://dx.doi.org/10.1016/j.clineuro.2014.07.025
0303-8467/© 2014 Elsevier B.V. All rights reserved.

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Extra-axial CMs are believed to arise from the capillary plexus
related to the dura mater [4–6], and are generally associated with
the floor of the middle cranial fossa, especially in the cavernous
sinus [2–4,6]. CMs have been reported to involve cranial nerves
such as the optic nerves and chiasm, the third nerve, the seventh nerve in the temporal bone, the vestibular nerve [7], and the
seventh–eighth nerve complex in the internal auditory canal [1,8].
The presenting symptoms are related to the nerve involved in the
lesion.
2. Case report
A 62-year-old male patient was referred to our department with
a three-month history of left facial pain triggered by facial compression. He had consulted a neighborhood hospital, where magnetic
resonance imaging (MRI) revealed a mass lesion of the left cerebellopontine angle (CPA). Follow-up MRIs at our hospital showed
that the lesion was attached to the trigeminal nerves. The lesion

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K. Adachi et al. / Clinical Neurology and Neurosurgery 125 (2014) 151–154

Fig. 1. MRI findings showing a left cerebellopontine angle mass 3 cm in diameter in contact with the trigeminal nerve. Highintense axial T1-weighted images (A) and
isointense to highintense T2-weighted (B) and FLAIR-weighted (C) MRIs show a left trigeminal mass with hemosiderin rims. Axial CISS-weighted findings (D, E) show a
low-intense mass with a hemosiderin rim and DVA (arrowheads).

appeared hyperintense on T1-weighted MRI; heterogeneous, highintense, and isointense with a hemosiderin rim on T2-weighted
MRI; and as a mass with a hemosiderin rim and a developmental
venous anomaly (DVA) medially on CISS-weighted MRI (Fig. 1A–E).
Based on these findings, the patient was diagnosed with a CM of
the left trigeminal nerve, and was admitted for surgical treatment
because of hemorrhage-related symptoms.
Neurological examination revealed left facial hypoesthesia. The
patient had no particular previous medical history, and laboratory findings were normal. The operation was performed using a
left anterior transpetrosal approach. The mass was blue-gray in
color and was buried in the drawn-out area shown in Fig. 2A,
between the cisternal and intra-axial portions of the trigeminal
nerve. Hemosiderin depositions and xanthosis were observed in
the trigeminal nerve around the CM. The tumor was resected en
bloc, and feeder microvessels were treated. The DVA was allowed
to remain. Hemosiderin had caused degeneration in the trigeminal
nerve, although it was preserved morphologically (Fig. 2B). Microscopically, granulation tissue with hemosiderosis was observed.
There was no intervening neural or glial tissue (Fig. 3). These findings were compatible with a diagnosis of CM.
After the surgery, left facial numbness remained without
trigeminal neuralgia. Postoperative MRI revealed no residual CM
(Fig. 4). The postoperative course was uneventful, and the patient
was discharged.

the vascular plexus of the cranial nerve; and Types P and S develop
from the vascular plexus of the cerebral parenchyma.
CM was treated surgically in 8 of the 11 reported cases. Postoperatively, facial numbness remained but trigeminal neuralgia was
relieved in 7 of the 8 cases [9–12,16,17]. The patient complains of
dysesthesia in the left occipital area and sensory disturbance of the

3. Discussion
CM of the trigeminal nerve with trigeminal neuralgia was first
described in 1988 by Fehlings et al. [9]. Since then, 11 welldocumented cases of histologically confirmed CM with trigeminal
neuralgia have been reported in English, including the present case
(Table 1) [9–18].
We classified these cases into four types according to the origin
of the CM: Type G, which originates in the Gasserian ganglion (3
cases); Type C, which originates between the cisternal and intraaxial portions of the trigeminal nerve root (2 cases, including ours);
Type P, originating in the intra-axial trigeminal nerve root in the
pons (4 cases); and Type S, which originates in the spinal tract of
the trigeminal nerve root below the medulla oblongata (2 cases)
(Fig. 5). When discussing CM and its treatment, it is important to
consider these distinct areas of origin. Type G develops from the
vascular plexus of the dura or cranial nerve; Type C develops from

Fig. 2. Intraoperative photography. (A) The mass was blue-gray in color with
microvessels, and was buried in the trigeminal nerve, which is drawn out in the
photographs (white arrow); hemosiderin deposition and xanthosis were present.
(B) The tumor was completely removed from beneath the trigeminal nerve (white
arrow), which was preserved morphologically.

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153

Fig. 5. Schematic drawing of the trigeminal nerve root, lateral view. We classified
trigeminal root cavernous malformations into 4 types according to the location of
origin: Type G: Gasserian ganglion; Type C: between the cisternal portion and intraaxial trigeminal nerve root; Type P: the intra-axial trigeminal nerve root in the pons;
Type S: the origin of the spinal tract of the trigeminal nerve root below the medulla
oblongata.

Fig. 3. Photomicrographs showing clustered dilated empty vascular channels with
a single layer of endothelium, no muscular tissue in the walls, and no intervening
neural or glial tissue, with thrombi in some channels (H&E). Original magnification:
4× (A), 40× (B).

right side of the body in surgically treated Type S CM [17]. In one
case involving a Type P CM [13], the patient died from postoperative
bleeding.
In the remaining 3 cases that were not treated surgically, one
patient with a Type S CM [18] experienced spontaneous remission
of symptoms, and two patients with Type P CM [14,15] were able to
control the pain with medication. All the non-operated CMs were
tiny lesions that were entirely intra-axial.
Surgical resection should be considered for symptomatic brainstem CMs that abut a pial surface or are surrounded by a thin rim of
brainstem tissue. Indeed, the natural history is worse for CM in the
brainstem than for CM in other CNS locations. Brainstem CM has

a higher hemorrhage rate, and is more likely to hemorrhage again
than CMs located elsewhere [19]. Some authors have reported that
radiation-related complications (19–41%) [20,21] and hemorrhage
(12–40%) [22,23] occur frequently with stereotactic radiosurgery
for CM. Based on this information, resection should be the primary
management strategy for CM whenever it can be accomplished
with an acceptable risk of morbidity.
The surgical approach for CM with trigeminal neuralgia should
consider the type of CM. A suboccipital approach has been used
for Type S CM [17], which is appropriate for the tumor location.
Type G CM has been treated using a subtemporal [9], front-temporal
[10], or pterional approach [11]. The Type C CM in our present case
was resected using an anterior transpetrosal approach, although
another reported Type C case [12] was treated using a lateral suboccipital approach. Type P CMs have been treated with a lateral
suboccipital [16] or subtemporal approach [13]. In one case of a
Type P CM treated using a subtemporal approach, the patient died
of postoperative bleeding [13], which can be caused by incomplete excision. An anterior transpetrosal approach was suitable for
dissecting the tumor in that case. A Type P CM is located below
the tentorium, making dissection from a subtemporal approach
difficult. Cenzato et al. [16] reported that incomplete excision is

Fig. 4. Axial postoperative FLAIR-weighted (A) and T2-weighted MRI (B) scan verifying complete resection of the cavernous malformation (white arrow) and continuity of
the trigeminal nerve (arrowheads).

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Table 1
Reported cases of CM with trigeminal neuralgia.
Type

Year

Author

Other symptoms

Final treatment

Pain controlled

Type G

1988

Fehlings et al.

Surgery (subtemporal approach)

Good

Type C

2006
2009
2005

Mascarenhas et al.
Seckin et al.
Deshmukh et al.

Surgery (front-temporal approach)
Surgery (pterional approach)
Surgery (lateral suboccipital approach)

Good
Good
Good

2013

Our case

Hypoesthesia, V3
weakness
–
V3 anesthesia
Hearing loss,
anesthesia
Hypoesthesia

Good

1996
2000
2002
2010
1989

De Benedittis
Shimpo
Vitek and Tettenborn
Cenzato et al.
Saito et al.

Surgery (anterior transpetrosal
approach)
Surgery (subtemporal approach)
Carbamazepin
Gabapentin
Surgery (lateral suboccipital approach)
Surgery (lateral suboccipital approach)

Death
Good
Good
Good
Good

2007

Stellmann et al.

Spontaneously disappeared

Good

Type P

Type S

SUNCT
Anisocoria
–
–
Hyperpathia (lt C2
area), lt hypoesthesia
(C2–7 dermatome)
Ptosis, hypaesthesia

SUNCT: short-lasting, unilateral neuralgiform headache attacks with conjunctive injection and tearing.

probably more dangerous than abstention, since the risk of new
hemorrhage from the fragments left by incomplete surgical excision can be as high as 43%. Complete surgical excision, conducted
while facing the field of operation, may be especially important for
brainstem CM.
One Type C CM was approached suboccipitally, and the petrosal
vein was sacrificed to secure the operating field [12]. Sacrificing the
petrosal vein could cause venous congestion and brain swelling,
which would make dissection more difficult when using a lateral
suboccipital approach.
For Type C or P CMs, we believe an anterior transpetrosal
approach is better than a lateral suboccipital or subtemporal
approach, because it provides good exposure, and the surgeon
can face the pontine surface and trigeminal nerve root, although
the working axis of the lateral suboccipital approach, which is
essentially sagittal, uses the windows created by the petrous bone,
petrosal vein and cerebellum [24,25].
4. Conclusion
CM with trigeminal neuralgia is rare. Resection should be the
primary management strategy whenever it can be accomplished
with an acceptable risk of morbidity, although pain has been
controllable with medication in some cases, and a spontaneous
remission of symptoms was reported in one case. Surgery for CM
should be performed facing the field of operation, since incomplete
excision may be more dangerous than abstention due to the risk of
new hemorrhage from any fragments. With the exception of Type
G and Type S CMs, an anterior transpetrosal approach is usually
suitable for dissecting a CM causing trigeminal neuralgia, since this
approach faces the lesion in the center of trigeminal nerve.
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