Neurol Med Chir (Tokyo) 42, 31¿35, 2002

Presigmoid Transpetrosal Approach for the Treatment

of a Large Trochlear Nerve Schwannoma
—Case Report—

Toru MATSUI, Eiharu MORIKAWA, Tadashi MORIMOTO, and Takao ASANO

Department of Neurosurgery, Saitama Medical Center/School, Kawagoe, Saitama

Abstract
A 61-year-old man presented with a rare, large trochlear nerve schwannoma manifesting as left-sided
weakness and hypesthesia, bilateral bulbar pareses, and trochlear nerve paresis persisting for 3 months.
T1-weighted magnetic resonance imaging with gadolinium revealed an intensely enhanced, well-
circumscribed lesion with multicystic formation occupying the prepontine and interpeduncular
cisterns and compressing the pons and midbrain with greater extension to the right. The mass was com-
pletely removed through the presigmoid transpetrosal approach with preservation of the posterior
cerebral, superior cerebellar, and basilar arteries and their branches. Neuroradiological examination
after 3 years demonstrated no recurrence. Enlargement of a tumor in the cisternal portion is inclined to
involve and/or encase the adjacent major arteries and their branches. The presigmoid transpetrosal
approach is one of the best surgical routes to remove a large trochlear nerve schwannoma safely and
completely.
Key words: trochlear nerve schwannoma, presigmoid transpetrosal approach

Introduction Case Report

Trochlear nerve sheath tumors are uncommon, with A 61-year-old man presented in December 1997 with
only 15 cases reported.1,3,5–7,11,12,14–17,19,20) Intracranial left-sided weakness and hypesthesia, bilateral bulbar
schwannomas occur in a number of mixed and pure- pareses, and trochlear nerve paresis which had
ly motor cranial nerves, especially in association persisted since September 1997. During the 4 weeks
with neurofibromatosis type 2.4,8,11,16) Total removal before his admission, he had had difficulty in swal-
of a large tumor in the region associated with the lowing with intake of liquid and needed support be-
trochlear nerve requires retraction of the temporal cause of cerebellar ataxia. He had no family history
lobe to obtain an adequate operative field. There- of neurofibromatosis.
fore, the use of a modern cranial base approach is Examination found no cutaneous stigmata of
thought to be preferable to a simple subtemporal ap- neurofibromatosis. His visual fields were full. The
proach.18) Previous cases involved a relatively mid- pupils were equal and reacted directly and indi-
sized tumor, so the subtemporal approach was used rectly to light. He had diplopia on downward gaze to
to obtain total or subtotal removal. However, the right, suggesting right trochlear nerve paresis.
removal of a large tumor sometimes leads to unex- Corneal reflexes were brisk bilaterally, and facial
pected postoperative neurological deficits, possibly sensation was normal. The functions of the VIIth to
due to the intraoperative compression of the tem- XIIth cranial nerves were intact. He demonstrated
poral lobe.13) mild left-sided weakness of the upper motor neuron
Here, we present an extremely rare case of a large type, with hyperreactive reflexes. There was no
trochlear nerve schwannoma without neurofibro- convincing sensory loss. T1-weighted magnetic reso-
matosis treated by the presigmoid transpetrosal ap- nance (MR) imaging with gadolinium revealed an
proach, which allowed complete removal. intensely enhanced, well-circumscribed lesion with
multicystic formation occupying the prepontine and
interpeduncular cisterns. The midbrain and upper
Received June 25, 2001; Accepted October 11, 2001 pons were compressed and shifted to the left by the

31
32 T. Matsui et al.

large extension of the tumor, and the tumor was

attached to the upper third clivus and petrous bone
(Fig. 1). T2-weighted MR imaging clearly demon-
strated involvement of the basilar artery (BA) and
the neighboring major arteries and their branches
(Fig. 1). Cerebral angiography demonstrated no
tumor blush. The preoperative diagnosis was
trigeminal neurinoma rather than petroclival menin-
gioma. Therefore, we decided to approach this
lesion via the presigmoid transpetrosal approach
with the goal of removing bone to preserve the
brain.18)
The patient was positioned for the right presig-
moid transpetrosal approach. A U-shaped skin
incision starting two fingerbreadths anterior to the
external auditory canal (EAC) was carried out su- Fig. 1 Preoperative T1-weighted magnetic
periorly and then curved posteriorly three finger- resonance images with gadolinium reveal-
breadths superior to the EAC and curved again ing an intensely enhanced, well-circum-
downward to the suboccipital area, two finger- scribed lesion with multicystic formation
breadths posterior to the mastoid process. A occupying the prepontine, ambient, and in-
temporo-occipito-suboccipital craniotomy with terpeduncular cisterns. The pons and mid-
mastoidectomy was then performed. The mastoidec- brain are extremely compressed with great-
tomy was designed preoperatively to preserve the er extension to the right, and an attachment
vestibulocochlear apparatus, based on computed to the upper third clivus and petrous bone.
tomography (CT) (Fig. 2). Postoperative hearing loss The medial portion of the tumor is attached
to the basilar artery, suggesting involve-
should thus be avoided through rongeuring off the
ment of its perforators.
mastoid bone. Following the craniotomy, the presig-
moid and temporal dura was incised, and then the
superior petrosal sinus and tentorium cerebelli were
coagulated and cut. Evacuation of cerebrospinal
fluid from the ambient cistern was helpful to obtain
a large space for surgery by mild retraction of the
temporal lobe and cerebellum (Fig. 3). First of all,
the arachnoid membrane surrounding the petrosal
vein was incised to expose the trigeminal nerve. The
trigeminal, facial, and acoustic nerves were easily
identified and dissected from the tumor, but the
branches of the superior cerebellar artery (SCA)
were overlying the tumor. The distal portion of the
trochlear nerve was identified on the inner aspect of
the tumor. A large mass was located at the ambient
portion of the trochlear nerve. Yellowish-green fluid
was evacuated from the tumor but the lumen was
multilobulated. Unexpectedly, the tumor was com-
posed of parenchymatous as well as cystic compo-
nents. The tumor was removed by piecemeal resec-
tion to preserve the small branches from the major
arteries such as the BA, posterior cerebral artery Fig. 2 Postoperative bone window computed
(PCA), and SCA. A large tumor located in the cister- tomography scan clearly showing preserva-
nal region is likely to involve and/or encase vascular tion of the vestibulocochlear apparatus.
components, so careful attention was paid to
preserving any vessels. Finally, complete excision of
the tumor including part of the trochlear nerve was region of the BA-PCA junction and in the ventral site
obtained. Subsequently, the oculomotor nerve and of the SCA and the trigeminal root, respectively.
the abducens nerve were identified in the ventral These observations suggested that the tumor origi-

Neurol Med Chir (Tokyo) 42, January, 2002

 Large Trochlear Nerve Schwannoma 33

Fig. 4 Postoperative T1-weighted magnetic

resonance images with gadolinium (left)
within a month after surgery revealing com-
plete resection, and (right) at 3 years after
surgery showing no recurrence of the tumor.
The brainstem dislocated by the large mass
has normalized within 3 years.

rence (Fig. 4).

Discussion
Preoperative localization of intraaxial or extraaxial
lesions located in the parapeduncular region was
very difficult before the development of high-
resolution CT and/or MR imaging, but now only
Fig. 3 Intraoperative photograph and sketch show- small iso-intense lesions present problems.8) The
ing that minimal retraction of temporal lobe present tumor appeared with the following charac-
(Temp) and cerebellum (cerebelli) allowed
teristics of meningioma and neurinoma. The mass
exposure of the important anatomical de-
tails of the cerebellopontine angle such as
was iso-intense on T1- and T2-weighted MR images
the trochlear nerve (Tro), tumor (Tum), and brightly enhanced with gadolinium.1,2,17) An ex-
branches of the superior cerebellar artery tremely peculiar case of cystic trochlear nerve
(SCAb), petrosal vein (Pv), and the trigemi- neurinoma mimicked a brainstem tumor.7) General-
nal nerve (Tri). The tumor clearly originated ly, trochlear nerve neurinoma is preoperatively
from the trochlear nerve. P: rongeured identified as trigeminal neurinoma, meningioma, or
petrous bone covered with cotton plugs, R1 epidermoid cyst.1,2,5,11,16,17) In almost all case, direct
and R2: Sugita retractors, S: sucker. surgery revealed trochlear nerve neurinoma.
Trochlear nerve paresis was present in eight of 16
reported cases, including this case, and five of these
nated from the trochlear nerve. Histological exami- 16 cases manifested as involvement of other cranial
nation of the tumor specimen showed compact areas nerves (Table 1). The unilateral combination of
of spindle cells arrayed in bundles (Antoni A type), hemiparesis, cerebellar ataxia, and sensory distur-
alternating with areas of loosely structured tissue bance in the presence of extraaxial mass at the ten-
(Antoni B type). The histological diagnosis was neu- torial notch is more strongly suggestive of trochlear
rinoma. nerve neurinoma.5) However, these symptoms
Immediately after the surgery, left hemiparesis remain silent until the tumor size exceeds more than
and dysphagia disappeared. Diplopia due to right 3–4 cm diameter. A trochlear nerve neurinoma of
trochlear nerve paresis also disappeared with visual 1.5 cm diameter, although extremely rare, was
correction by control of the head position during the found at autopsy.10)
first year after surgery. Postoperative MR imaging No preoperative trochlear nerve involvement was
demonstrated complete removal of the tumor, and present in at least 45% of previous cases. The most
almost 3 years later there was no evidence of recur- frequently used surgical approach was the subtem-

Neurol Med Chir (Tokyo) 42, January, 2002

34 T. Matsui et al.

Table 1 Previous cases of trochlear nerve schwannoma

Case Age/ n.IV

Author (Year) Other symptoms Tumor site Surgery Follow up
No. Sex sign*

1 King (1976)12) 55/F ＋ rt hyperesthesia, cisternal subtemporal and 18 mos

n.V and n.VII transtentorial
2 Boggan et al. 32/F ＋ lt hemiparesis, cisternal frontotemporal, 8 mos
(1979)3) amenorrhea, subtemporal, and
n.V and n.VII transtentorial
3 Leunda et al. 54/M ＋ headache cisternal subtemporal and 1 yr
(1982)14) transtentorial
4 16/F ＋ headache cisternal and subtemporal and 6 mos
cavernous transtentorial
5 Yamamoto et al. 37/F ＋ increased ICP, cisternal subtemporal and 5 yrs
(1987)20) temporal headache transtentorial
6 Garen et al. 18/F － diplopia, n.III cisternal subtemporal and ?
(1987)7) transtentorial
7 Tokuriki et al. 43/M － lt numbness, cisternal subtemporal and 1 mo
(1988)19) gait disturbance, transtentorial
n.V and n.VII
8 Mauice-Williams 56/M － rt hemiparesis, cisternal suboccipital 2 yrs
(1989)15) diplopia
9 Celli et al. 51/M ＋ lt hemiparesis, cisternal subtemporal and 5 yrs
(1992)5) lt cerebellar ataxia transtentorial
10 Jackowski et al. 26/F ＋ lt facial numbness cisternal transtemporal and 6 mos
(1994)11) transtentorial
11 Abe et al. 60/M － lt hemiparesis cisternal suboccipital ?
(1994)1)
12 57/M － lt facial numbness cisternal subtemporal and ?
transtentorial
13 Dolenc and Coscia 68/M － lt hemiparesis, cisternal and subtemporal and 2 mos
(1996)6) diplopia (transient) cavernous suboccipital
14 Santoreneos et al. 35/F － lt hemiparesis, cisternal subtemporal and 23 mos
(1997)17) bil bulbar pareses, transtentorial
emotional disorder,
n.VII
15 Nadkarni and Goel 48/F － pathological laughter cisternal subtemporal and 6 mos
(1999)16) transtentorial
16 Present case 60/M ＋ lt hemiparesis, cisternal presigmoid and 2.5 yrs
diplopia, dysphagia transpetrosal

*＋: present, －: none. ICP: intracranial pressure, n.III: oculomotor nerve, n.IV: trochlear nerve, n.V: trigeminal
nerve, n.VII: facial nerve.

poral approach combined with tentorial incision disappeared. The presigmoid transpetrosal ap-
(Table 1), and the tumor was almost totally excised. proach provides excellent exposure of the cerebel-
However, postoperative complications included lopontine angle and pons, so removal of the tumor
temporary oculomotor and abducens nerve pareses, compressing the pons is unlikely to cause neurologi-
temporary hemiparesis and expressive dysphasia cal deterioration, if the plane between the tumor and
due to intraoperative compression of the temporal the pons is successfully dissected. To remove bone
lobe and pons, and/or subsequent contusion.13) Even and preserve brain function, cranial base surgery
if long-term follow up indicated recovery of the technique is essential, and the total removal of such
preoperative deficit, modern cranial base surgery is a large trochlear nerve schwannoma located in the
preferable to the simple subtemporal approach,18) cisternal portion is a good indicator of the effective-
but the presigmoid transpetrosal approach has never ness of this approach.
been used, possibly due to the low incidence of Oculomotor, trochlear, and abducens nerve
trochlear nerve schwannoma. The preoperative schwannomas generally arise far from the glial
diagnosis was large trigeminal nerve schwannoma, Schwann sheath junction, at not more than 1 mm
so the presigmoid transpetrosal approach was per- from the neuraxis.5) Trochlear nerve schwannomas
formed in the present case. At 2 weeks after surgery, arise almost exclusively from the cisternal segment.
dysphagia and left-sided weakness had completely Therefore, the tumor grows in the space occupied by

Neurol Med Chir (Tokyo) 42, January, 2002

 Large Trochlear Nerve Schwannoma 35

the PCA, SCA, BA, and perforators. Trochlear nerve 3) Boggan JE, Rosenblum ML, Wilson CB: Neurilemmo-
schwannoma in the basal cistern is likely to form se- ma of the fourth cranial nerve. Case report. J Neu-
vere adherences to these anatomical structures, rosurg 50: 519–521, 1979
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al.9) Acoustic neurinoma could be basically removed cranial nerves: a survey and report of a new fourth
via a lateral suboccipital route, but such tumor with nerve case. Surg Neurol 38: 216–224, 1992
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Neurol Med Chir (Tokyo) 42, January, 2002