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
only 15 cases reported.1,3,5–7,11,12,14–17,19,20) Intracranial
schwannomas occur in a number of mixed and pure-
ly motor cranial nerves, especially in association
with neurofibromatosis type 2.4,8,11,16) Total removal
of a large tumor in the region associated with the
trochlear nerve requires retraction of the temporal
lobe to obtain an adequate operative field. There-
fore, the use of a modern cranial base approach is
thought to be preferable to a simple subtemporal ap-
proach.18) Previous cases involved a relatively mid-
sized tumor, so the subtemporal approach was used
to obtain total or subtotal removal. However,
removal of a large tumor sometimes leads to unex-
pected postoperative neurological deficits, possibly
due to the intraoperative compression of the tem-
poral lobe.13)
Here, we present an extremely rare case of a large
trochlear nerve schwannoma without neurofibro-
matosis treated by the presigmoid transpetrosal ap-
proach, which allowed complete removal.
Received June 25, 2001; Accepted October 11, 2001
A 61-year-old man presented in December 1997 with
left-sided weakness and hypesthesia, bilateral bulbar
pareses, and trochlear nerve paresis which had
persisted since September 1997. During the 4 weeks
before his admission, he had had difficulty in swal-
lowing with intake of liquid and needed support be-
cause of cerebellar ataxia. He had no family history
of neurofibromatosis.
Examination found no cutaneous stigmata of
neurofibromatosis. His visual fields were full. The
pupils were equal and reacted directly and indi-
rectly to light. He had diplopia on downward gaze to
the right, suggesting right trochlear nerve paresis.
Corneal reflexes were brisk bilaterally, and facial
sensation was normal. The functions of the VIIth to
XIIth cranial nerves were intact. He demonstrated
mild left-sided weakness of the upper motor neuron
type, with hyperreactive reflexes. There was no
convincing sensory loss. T1-weighted magnetic reso-
nance (MR) imaging with gadolinium revealed an
intensely enhanced, well-circumscribed lesion with
multicystic formation occupying the prepontine and
interpeduncular cisterns. The midbrain and upper
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
Fig. 3 Intraoperative photograph and sketch show-
ing that minimal retraction of temporal lobe
(Temp) and cerebellum (cerebelli) allowed
exposure of the important anatomical de-
tails of the cerebellopontine angle such as
the trochlear nerve (Tro), tumor (Tum),
branches of the superior cerebellar artery
(SCAb), petrosal vein (Pv), and the trigemi-
nal nerve (Tri). The tumor clearly originated
from the trochlear nerve. P: rongeured
petrous bone covered with cotton plugs, R1
and R2: Sugita retractors, S: sucker.
nated from the trochlear nerve. Histological exami-
nation of the tumor specimen showed compact areas
of spindle cells arrayed in bundles (Antoni A type),
alternating with areas of loosely structured tissue
(Antoni B type). The histological diagnosis was neu-
rinoma.
Immediately after the surgery, left hemiparesis
and dysphagia disappeared. Diplopia due to right
trochlear nerve paresis also disappeared with visual
correction by control of the head position during the
first year after surgery. Postoperative MR imaging
demonstrated complete removal of the tumor, and
almost 3 years later there was no evidence of recur-
Neurol Med Chir (Tokyo) 42, January, 2002
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
small iso-intense lesions present problems.8) The
present tumor appeared with the following charac-
teristics of meningioma and neurinoma. The mass
was iso-intense on T1- and T2-weighted MR images
and brightly enhanced with gadolinium.1,2,17) An ex-
tremely peculiar case of cystic trochlear nerve
neurinoma mimicked a brainstem tumor.7) General-
ly, trochlear nerve neurinoma is preoperatively
identified as trigeminal neurinoma, meningioma, or
epidermoid cyst.1,2,5,11,16,17) In almost all case, direct
surgery revealed trochlear nerve neurinoma.
Trochlear nerve paresis was present in eight of 16
reported cases, including this case, and five of these
16 cases manifested as involvement of other cranial
nerves (Table 1). The unilateral combination of
hemiparesis, cerebellar ataxia, and sensory distur-
bance in the presence of extraaxial mass at the ten-
torial notch is more strongly suggestive of trochlear
nerve neurinoma.5) However, these symptoms
remain silent until the tumor size exceeds more than
3–4 cm diameter. A trochlear nerve neurinoma of
1.5 cm diameter, although extremely rare, was
found at autopsy.10)
No preoperative trochlear nerve involvement was
present in at least 45% of previous cases. The most
frequently used surgical approach was the subtem-
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
(Table 1), and the tumor was almost totally excised.
However, postoperative complications included
temporary oculomotor and abducens nerve pareses,
temporary hemiparesis and expressive dysphasia
due to intraoperative compression of the temporal
lobe and pons, and/or subsequent contusion.13) Even
if long-term follow up indicated recovery of the
preoperative deficit, modern cranial base surgery is
preferable to the simple subtemporal approach,18)
but the presigmoid transpetrosal approach has never
been used, possibly due to the low incidence of
trochlear nerve schwannoma. The preoperative
diagnosis was large trigeminal nerve schwannoma,
so the presigmoid transpetrosal approach was per-
formed in the present case. At 2 weeks after surgery,
dysphagia and left-sided weakness had completely
disappeared. The presigmoid transpetrosal ap-
proach provides excellent exposure of the cerebel-
lopontine angle and pons, so removal of the tumor
compressing the pons is unlikely to cause neurologi-
cal deterioration, if the plane between the tumor and
the pons is successfully dissected. To remove bone
and preserve brain function, cranial base surgery
technique is essential, and the total removal of such
a large trochlear nerve schwannoma located in the
cisternal portion is a good indicator of the effective-
ness of this approach.
Oculomotor, trochlear, and abducens nerve
schwannomas generally arise far from the glial
Schwann sheath junction, at not more than 1 mm
from the neuraxis.5) Trochlear nerve schwannomas
arise almost exclusively from the cisternal segment.
Therefore, the tumor grows in the space occupied by

Neurol Med Chir (Tokyo) 42, January, 2002

 Large Trochlear Nerve Schwannoma
the PCA, SCA, BA, and perforators. Trochlear nerve
schwannoma in the basal cistern is likely to form se-
vere adherences to these anatomical structures,
making total removal more difficult.
We started to treat large lesions located in the
petroclival region employing the presigmoid trans-
petrosal approach in 1988 according to Hakuba et
al.9) Acoustic neurinoma could be basically removed
via a lateral suboccipital route, but such tumor with
severe upward compression of the tentorium cere-
belli and extension into the petroclival region was
treated via the presigmoidal route. Postoperatively,
MR imaging was repeated to detect recurrence of
the tumor every one year, and MR angiography was
performed to investigate the patency of the lateral
and sigmoid sinus in the operated site after more
than 3 years in 11 of a total of 30 patients. Although
four of the 11 examined patients showed obstruction
of the lateral and sigmoid sinuses, but did not
present with any symptoms due to deterioration of
venous drainage system, due to development of the
compensatory venous drainage system following
gradual obstruction of the sinuses. Thus, this ap-
proach is thought to be safe.
The possibility of total removal depends upon the
severity of adhesion of the tumor to the brainstem
and/or important vessels in the deep brain region,
and can be well predicted by preoperative MR
imaging, as the presence of thin low-intense areas on
T1-weighted MR imaging, suggesting cerebrospinal
fluid collection, between the tumor and brainstem.
Furthermore, T2-weighted MR imaging shows
whether the tumor involves and/or encases the
major arteries in the cranial base and severely com-
presses the upper brainstem. Such conditions re-
quire a wide working space where many important
anatomical structures such as the BA, PCA, SCA,
IIIrd to VIIth cranial nerves, and pons can be ex-
posed with minimal retraction of the brain.
The present case indicates that the presigmoid
transpetrosal approach is the first choice to treat a
patient with a large trochlear nerve schwannoma
located in the ambient, parapeduncular, interpedun-
cular, or prepontine cistern.
References
1) Abe T, Iwata T, Shimazu M, Iida M, Izumiyama H,
Matsumoto K, Mizutani T, Tanaka H, Aruga T: [Two
cases of trochlear nerve neurinoma]. No Shinkei Geka
22: 371–375, 1994 (Jpn, with Eng abstract)
2) Beppu T, Yoshida Y, Wada T, Arai H, Suzuki M,
Kuroda K, Ogawa A: Trochlear and abducens nerve
neurinomas accompanied by a cerebellopontine an-
gle meningioma. Case report. Neurol Med Chir
(Tokyo) 37: 416–421, 1997
Neurol Med Chir (Tokyo) 42, January, 2002
35
3) Boggan JE, Rosenblum ML, Wilson CB: Neurilemmo-
ma of the fourth cranial nerve. Case report. J Neu-
rosurg 50: 519–521, 1979
4) Brodsky MC, Boop AF: Primary trochlear nerve ne-
oplasm in a child with neurofibromatosis 1. J Pediatr
Ophthalmol Strabismus 33: 328–329, 1996
5) Celli P, Ferrante L, Acqui M, Mastronardi L, Fortuna
A, Palma L: Neurinoma of the third, fourth, and sixth
cranial nerves: a survey and report of a new fourth
nerve case. Surg Neurol 38: 216–224, 1992
6) Dolenc VV, Coscia S: Cystic trochlear nerve neurino-
ma. Br J Neurosurg 10: 593–597, 1996
7) Garen PD, Harper CG, Teo C, Johnston IH: Cystic
schwannoma of the trochlear nerve mimicking a
brain-stem tumor. Case report. J Neurosurg 67:
928–930, 1987
8) Gentry LR, Mehta RC, Appen RE, Weinstein JM: MR
imaging of primary trochlear nerve neoplasms. AJNR
Am J Neuroradiol 12: 707–713, 1991
9) Hakuba A, Nishimura S, Jang BJ: A combined
retroauricular and preauricular transpetrosal-
transtentorial approach to clivus meningiomas. Surg
Neurol 30: 108–116, 1988
10) Ho KL: Schwannoma of the trochlear nerve. Case
report. Neurosurgery 55: 132–135, 1981
11) Jackowski A, Weiner G, O'Reilly G: Trochlear nerve
schwannomas: a case report and literature review. Br
J Neurosurg 8: 219–223, 1994
12) King JS: Trochlear nerve sheath tumor. Case report. J
Neurosurg 44: 245–247, 1976
13) Lanotte M, Giordana MT, Forni C, Pagni CA:
Schwannoma of the cavernous sinus. Case report
and review of the literature. J Neurosurg Sci 36:
233–238, 1992
14) Leunda G, Vaquero J, Cavezudo J, Gacia-Uria J,
Bravo G: Schwannomas of the oculomotor nerve.
Reports of four cases. J Neurosurg 57: 563–565, 1982
15) Mauice-Williams RS: Isolated schwannoma of the
fourth cranial nerve. J Neurol Neurosurg Psychiatry
52: 1442–1443, 1989
16) Nadkarni TD, Goel A: Trochlear nerve neurinoma
presenting as pathological laughter. Br J Neurosurg
13: 212–213, 1999
17) Santoreneos S, Hanieh A, Jorgensen RE: Trochlear
nerve schwannomas occurring in patients without
neurofibromatosis: case report and review of the
literature. Neurosurgery 41: 282–287, 1997
18) Sekhar LN: Comments to Santoreneos S, et al.
Neurosurgery 41: 287, 1997
19) Tokuriki Y, Yamashita J, Kikuchi H, Asato R, Handa
H: Trochlear nerve neurinoma. Case report. Neurol
Med Chir (Tokyo) 28: 70–73, 1988
20) Yamamoto M, Jimbo M, Ide M, Kubo O: Trochlear
neurinoma. Surg Neurol 28: 287–290, 1987
Address reprint requests to: T. Matsui, M.D., Department
of Neurosurgery, Saitama Medical Center/School,
1981 Kamoda, Kawagoe, Saitama 350–8550, Japan.
e-mail: matsui＠ns2.saitama-med.ac.jp.