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J Neurosurg 96:453–463, 2002

Trigeminal schwannomas: removal of dumbbell-shaped


tumors through the expanded Meckel cave and outcomes
of cranial nerve function

OSSAMA AL-MEFTY, M.D., SAMER AYOUBI, F.R.C.S.(I),


AND ESAM GABER, M.B.B.CH., M.CH., PH.D.

Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas

Object. As in patients with vestibular schwannomas, advances in surgical procedures have markedly improved
outcomes in patients with trigeminal schwannomas. In this article the authors address the function of cranial nerves
in a series of patients with trigeminal schwannomas that were treated with gross-total surgical removal. The authors
emphasize a technique they use to remove a dumbbell-shaped tumor through the expanded Meckel cave, and dis-
cuss the advantage of the extradural zygomatic middle fossa approach for total removal of tumor and preservation
or improvement of cranial nerve function.
Methods. Within an 11-year period (1989–2000), 25 patients (14 female and 11 male patients with a mean age
of 44.4 years) with benign trigeminal schwannomas were surgically treated by the senior author (O.A.) with the aim
of total removal of the tumor. Three patients had undergone previous surgery elsewhere. Trigeminal nerve dysfunc-
tion was present in all but two patients. Abducent nerve paresis was present in 40%. The approach in each patient
was selected according to the location and size of the lesion. Nineteen tumors were dumbbell shaped and extended
into both middle and posterior fossae. All 25 tumors involved the cavernous sinus. The zygomatic middle fossa ap-
proach was particularly useful and was used in 14 patients. The mean follow-up period was 33.12 months. In pa-
tients who had not undergone previous surgery, the preoperative trigeminal sensory deficit improved in 44%, facial
pain decreased in 73%, and trigeminal motor deficit improved in 80%. Among patients with preoperative abducent
nerve paresis, recovery was attained in 63%. Three patients (12%) experienced a persistent new or worse cranial
nerve function postoperatively. Fifth nerve sensory deficit persisted in one of these patients, sensory and motor dys-
function in another, and motor trigeminal weakness in the third patient. In all patients a good surgical outcome was
achieved. One patient died 2 years after treatment from an unrelated cause. In three patients the tumors recurred af-
ter an average of 22.3 months.
Conclusions. Preservation or improvement of cranial nerve function can be achieved through total removal of a
trigeminal schwannoma, and skull base approaches are better suited to achieving this goal. The zygomatic middle
fossa approach is particularly helpful and safe. It allows extradural tumor removal from the cavernous sinus, the in-
fratemporal fossa, and the posterior fossa through the expanded Meckel cave.

KEY WORDS • cranial nerve • cavernous sinus • Meckel cave •


trigeminal schwannoma • cystic tumor • brain neoplasm • skull base surgery

is possible of course that a method may some day be patients with vestibular schwannomas. Consequently, the
I
T
evolved whereby a Gasserian neurinoma or meningio- emphasis of treatment has shifted to the patient’s quality of
ma, even after it has crossed the ridge, may be safely life, with the aim to recover or preserve cranial nerve func-
approached and removed. Should this come to pass, it will tion. In this report we focus on cranial nerve function and
be another conquest for neurosurgery.—Harvey Cushing11 examine the eventual outcome of cranial nerve deficits,
whether they are present preoperatively or develop post-
THE treatment of trigeminal schwannomas is fast approach- operatively. Various surgical approaches have been used
ing the fulfillment of Dr. Cushing’s prediction. With the ad- for the treatment of these tumors; the proper selection of
vent of microsurgical techniques and skull base approach- the surgical approach minimizes postoperative deficits. We
es, a remarkable improvement has been seen in outcomes emphasize the advantage of using the zygomatic middle
in patients undergoing surgical treatment for trigeminal fossa approach for the extradural removal of even large,
schwannomas,12,13,31,35,38,42,48,49,52 a trend that parallels that of dumbbell-shaped tumors, which are known to be the most
challenging. By expanding the Meckel cave, a large portion
of the posterior fossa can be removed carefully through the
Abbreviations used in this paper: CT = computerized tomog- middle fossa without drilling the petrous apex. We present
raphy; MR = magnetic resonance; SPECT = single-photon emis- the anatomical basis of and the surgical techniques for this
sion CT. maneuver.

J. Neurosurg. / Volume 96 / March, 2002 453


O. Al-Mefty, S. Ayoubi, and E. Gaber

TABLE 1
Clinical findings in 25 patients with trigeminal schwannoma*
Signs
Symptoms
Corneal
Case DOS Numb- Sensory TM Reflex Cere- Brain-
No. (mos) Facial Pain ness Other Deficit Deficit Deficit Other CN bellar stem

1 12 tic douloureux   V2 N N   
2 12 V1, V2  headache V1 N N  ataxia 
3 3 tic douloureux V1–3 nausea, headache V1, V2     
4 12 periorbital  headache, diplopia N N N VI ataxia 
5 ? tic douloureux V2  V2 N N   
6† 12   diplopia V1–3 N   ataxia 
7 ?  V2 blind spot: uveitis V2 N N   
8† 4 retrobulbar  hemicrania N wasting N   
9 4   vertigo N N    
10† 36   diplopia V1–3   VI, IX, X, XII ataxia 
11 24  V1, V2 diplopia V1–3 N N VI  
12 96 tic douloureux V1–3 diplopia V1–3 atrophy N VI ataxia 
13 60   diplopia V1–3   VI  
14† 12  V1–3 diplopia V1–3 N N IV  
15 24   diplopia V2, V3 N  VI, VIII, IX, X  
16† 4   diplopia V1, V2 N  VI  
17 2  V2  V2 N    
18 5  tingling diplopia V2, V3   IV, VI  
19† 19   diplopia, visual V1–3 N N   
obscuration
20† ?   diplopia, headache, V2, V3 N N IV, VI nystag- 
blurred vision mus
21† 2   ear fullness, ringing N N N VIII  
22† 9 retroorbital  diplopia N N N IV, VI  
23 48   imbalance V2, V3 N N II, VIII ataxia 
24 3   dizziness N N N VIII  
25† 5    N N N   
total 18.5‡ 14 (56%) 19 (76%) 21 (84%) 18 (72%) 6 (24%) 9 (36%) 14 (56%) 7 (28%) 1 (4%)
* CN = cranial nerve; DOS = duration of symptoms; N = normal; TM = trigeminal motor;  = present; – = absent; ? = unknown.
† Cystic lesion.
‡ Value represents the mean duration of symptoms.

Clinical Material and Methods The postoperative evaluation and follow-up review of
Within an 11-year period (1989–2000), a series of 25 pa- patients involved general and neurological examinations. A
tients with histologically verified benign trigeminal nerve CT scan was obtained early after surgery. Within 1 week af-
schwannomas underwent surgery performed by the senior ter surgery MR imaging was usually performed to evaluate
author (O.A.). Data from some of these patients were in- the extent of tumor excision. Additional MR images were
cluded in a previous publication on the overall treatment obtained approximately 6 months after surgery and, later,
of tumors of the cavernous sinus.14 Three patients were re- annually to detect any recurrence. The pathological exami-
ferred to our department because they harbored a growing nation included intraoperative frozen section and formalin-
residual tumor after they had undergone a previous subtotal fixed specimens to which routine and special stains were
resection elsewhere. All patients underwent detailed gener- applied.
al, ophthalmological, and neurological examinations. Au-
diograms were obtained in 12 patients. Results
Neuroimaging evaluation of these patients included pre-
operative MR imaging, CT scanning, and, early in the se- This series consists of 14 female and 11 male patients.
ries, cerebral angiography with cross-compression studies The mean age of the patients was 44.4 years (range 11–80
or a balloon-occlusion test with SPECT scanning to evalu- years), and the peak incidence of tumor discovery occurred
ate cerebral collateral circulation. Magnetic resonance an- during the patient’s fourth decade. Table 1 lists the clinical
giography replaced angiography later in the series. findings in the 25 patients. The mean duration of symptoms
All patients underwent surgical excision aimed at total before treatment (known for 22 patients) was 18.5 months
removal of the tumor. Intraoperative monitoring included (range 2–96 months).
recording of brainstem auditory evoked responses and so- Signs and Symptoms
matosensory evoked potentials with median nerve stimula-
tion. Electromyographic recordings from the appropriate At the time of surgery, all but two patients manifested tri-
muscle were obtained for monitoring the third, fifth, sixth, geminal nerve dysfunction (sensory, motor deficit, or pain).
and seventh cranial nerves. When necessary, the lower cra- Fourteen patients experienced facial pain, four of whom
nial nerves were monitored using electromyography. displayed the typical tic douloureux. The other 10 patients

454 J. Neurosurg. / Volume 96 / March, 2002


Trigeminal schwannomas

described their pain as periorbital or retroorbital (three pa- balloon occlusion, SPECT was performed. Normal perfu-
tients), or as facial pain with undetermined characteristics sion was seen in all three cases. In one patient CT angiogra-
(seven patients). Nineteen patients reported having numb- phy was performed to distinguish the tumor from a throm-
ness along one or more divisions of the trigeminal nerve. bosed aneurysm.
During the physical examination, 21 (84%) of the 25 pa-
tients displayed trigeminal dysfunction. Eighteen experi- Surgical Procedure
enced sensory disturbances, namely, hypesthesia, dyses- The surgical approach was selected after a thorough
thesia, or hypalgesia, along one or more divisions of the study of each patient’s preoperative radiological findings
trigeminal nerve. One patient exhibited a reduced corneal and was tailored to each case.
reflex only, without reduced sensation in any of the three Because of the large size of the tumor in most patients,
dermatomes, and another patient only had only fifth nerve its extension into both middle and posterior fossae, and the
motor weakness. Nine patients (36%) displayed a de- involvement of the cavernous sinus, a skull-base approach
creased or absent corneal reflex. Decreased motor function, was deemed advantageous for most patients. The cranioor-
appearing as weakness or atrophy of the temporal or mas- bital zygomatic4 approach was used in three patients, the
seter muscles, was seen in six patients (24%). petrosal3 or extended petrosal2 in six patients, and the pter-
Thirteen patients (52%) complained of diplopia, which ional in one patient. One extensive tumor required a com-
was a presenting symptom in three patients. Four patients bination of the cranioorbital zygomatic and the petrosal
(16%) exhibited a deficit of the fourth cranial nerve, and 10 approaches (Fig. 1). We used the zygomatic middle fossa
patients (40%) a deficit of the sixth nerve. Two patients approach in 14 patients and found it particularly helpful
each (8%) displayed deficits of the ninth and 10th cranial and safe. It allowed extradural removal of tumor from the
nerves (one due to tumor and the other as a result of previ- cavernous sinus and removal of tumor extension in the in-
ous surgery), and one patient (4%) displayed a deficit of the fratemporal fossa. We removed extensions into the poste-
12th nerve (due to a previous surgery). One patient experi- rior fossa through the expanded Meckel cave without sec-
enced episodes of obscured vision associated with reduced tioning the tentorium or drilling the petrous apex. Only
visual acuity and restricted visual fields, and two patients large caudal extensions could not be reached in this way.
experienced visual symptoms due to an unrelated patho- Thus, the anatomical basis of the procedure and the zygo-
logical condition. Six patients displayed cerebellar signs. matic middle fossa approach deserve a description here.
Of the 12 patients who underwent audiography, five had Microsurgical Anatomy of the Meckel Cave. Johann Fried-
normal hearing, one had a positive decay reflex on the af- rich Meckel the Younger (1781–1833) described the dural
fected side, and five had high-frequency hearing loss. cleft that was named after him in the following manner:
Neuroimaging Evaluation . . . at the upper edge of the petrous portion of the temporal
bone it (the trigeminal nerve) enters a rounded and oblong
Preoperative CT scans were obtained in 18 patients and sheath of the dura matter, which generally is entirely separated
revealed bone erosion in six (33%). Contrast enhancement from the cavernous sinus. This sheath is at first loose, but after-
demonstrated a homogeneously enhanced lesion in 10 pa- wards is placed strongly on its surface. It thus goes from before
tients. In one patient, the tumor was not apparent on CT downward and from behind forward on the anterior face of the
scans and was only revealed by MR imaging studies. petrous portion of the temporal base.32
Magnetic resonance images were obtained routinely in The Meckel cave is a dural recess extending from the
all patients. The lesion was isointense or slightly hypoin- posterior fossa into the posteromedial portion of the middle
tense on T1-weighted images, hyperintense on T2-weight- cranial fossa, providing a natural pathway that allows ac-
ed images, and homogeneously enhanced after administra- cess from the middle fossa to the posterior fossa. Trigemi-
tion of contrast agent. In 10 tumors a cystic component was nal schwannomas expand the Meckel cave, allowing entry
identified. Magnetic resonance arteriography, which was to the posterior fossa (Fig. 2).
performed in 15 patients, revealed narrowing of the inter- The oval mouth (porus trigeminus) of the Meckel cave is
nal carotid artery in only one patient. Magnetic resonance situated just below the superior petrosal sinus. The cave
venography, which was performed in 10 patients, visual- is elongated forward, like a three-fingered glove, covering
ized the vein of Labbé and the exact venous sinus anatomy the roots, ganglion, and divisions of the fifth nerve until
in all 10 patients. they reach their respective foramina. The wrist of this glove
The results of the CT and MR imaging studies and the is located where the roots are transmitted. The cave extends
surgical findings helped us accurately diagnose the trigem- anteriorly to a level abreast of the anterior wall of the pitu-
inal schwannomas and clearly identify the tumors’ loca- itary fossa, laterally to the foramen ovale, and upward to
tions, extensions, and origins. The tumor was located in the half the height of the cavernous sinus. The trigeminal nerve
middle fossa in six patients (24%) and in both middle and carries its arachnoid sheath into the middle cranial fossa. A
posterior fossae in 19 patients (76%). The lesion extended probe inserted into the cave along the lateral wall may
to the orbit in one patient, and the maxillary sinus and in- reach these levels without much resistance.29 Due to this
fratemporal fossa were each involved in one patient. invagination of the dura of the posterior fossa under that
Early in the series, cerebral angiography was performed of the middle cranial fossa, a plane of cleavage is formed
in seven patients and a balloon occlusion test in six. One laterally between the two fused layers. This plane serves
patient could not tolerate the occlusion, and tumor blush as the anatomical basis for the extradural approach to the
was detected in another. In two patients angiography dem- Meckel cave.22,23
onstrated displacement of the nearby vessels. In three of the Surgical Procedure via the Zygomatic Middle Fossa Ap-
patients who had undergone angiography and tolerated the proach. The initial steps for the zygomatic middle fossa

J. Neurosurg. / Volume 96 / March, 2002 455


O. Al-Mefty, S. Ayoubi, and E. Gaber

FIG. 1. Preoperative (A and B) and postoperative (C and D) MR images demonstrating a giant trigeminal schwanno-
ma, the treatment of which necessitated a combination of the cranioorbital zygomatic and petrosal approaches. Note the
extensive cystic degeneration. E and F: Photographs showing that the patient has recovered from a preexisting sixth
cranial nerve dysfunction and a postoperative third cranial nerve dysfunction, but still has facial hypesthesia.

approach are similar to those of previously described pro- through the mouth of the Meckel cave without drilling the
cedures.1 The dura mater of the middle fossa is elevated petrous apex or sectioning the tentorium. The mouth can be
from the floor of the temporal fossa to expose the middle further enlarged by an upward small incision toward the su-
meningeal artery, which is coagulated and sectioned. The perior petrosal sinus, which might require coagulation and
foramen ovale and rotundum are identified. Posterior to sectioning of the most anterior portion of the superior pe-
the foramen ovale, the lesser and greater petrosal nerves trosal sinus. The tumor is carefully dissected from the fifth
are identified, and traction on the greater petrosal nerve is nerve rootlets and from the brainstem and the basilar artery
avoided. Proximal control of the cavernous carotid artery is and its branches by maintaining intraarachnoidal dissection
obtained in the petrous carotid artery canal, as previously (Figs. 4 and 5).
described.46 Tumors that extend into the infratemporal fossa or the
The sheaths of the second and third divisions of the tri- pterygopalatine fossa are extirpated after removal of the
geminal nerve are elevated to expose the lower cavernous floor of the middle fossa between the foramen ovale and ro-
sinus. The outer layer of the lateral wall of the cavernous si- tundum. Tumors that extend along the superior orbital fis-
nus is peeled medially and posteriorly to expose the tumor, sure are excised after removal of the floor of the middle
which usually displaces the gasserian ganglion, expands fossa between the rotundum and the opening of the superi-
the Meckel cave, and splays the divisions of the trigeminal or orbital fissure. For patients harboring large tumors, judi-
nerves (Fig. 3). The tumor capsule is entered between the cious placement of abdominal fat in the cavernous sinus
divisions of the trigeminal nerve or behind the ganglion. can prevent venous bleeding and a cerebrospinal fluid leak,
The lesion is debulked using suction and followed poste- and will facilitate follow-up MR imaging examinations.
riorly into the Meckel cave. It is then carefully dissected The temporal muscle is split and the partially vascularized
from the roots, ganglion, and divisions of the nerve to spare flap is laid on the floor of the middle fossa to support the
fascicles that are not the origin of the tumor in a manner closure with vascularized tissue.
similar to that used for other schwannomas. After the tumor
Surgical Complications and Outcome
is extirpated from the middle fossa and cavernous sinus
extradurally, it is followed into the cerebellopontine angle Twenty-seven surgical procedures were performed in 25

456 J. Neurosurg. / Volume 96 / March, 2002


Trigeminal schwannomas

patients. Three patients who had permanent cranial nerve


deficits after undergoing previous surgery elsewhere were
excluded, and their corresponding nerve functions were not
included in the analysis of recovery. Table 2 lists postoper-
ative improvements as they relate to preoperative findings.
Preoperative deficits of all other involved cranial nerves
resolved by 4 to 6 months after surgery, except for two of
the eight cases of abducent nerve deficit. All cerebellar and
brainstem manifestations resolved postoperatively.
The surgically induced cranial nerve dysfunction de-
scribed here includes new deficits or worsening of present-
ing deficits in these nerves. It occurred in a total of 11 pa-
tients (44%) in different combinations and was alleviated in
all but three patients (12%). No intraoperative complica-
tions occurred. In seven patients (28%; Cases 1, 2, 7, 8, 9,
14, and 21) trigeminal nerve function (sensory, motor, or
corneal reflex) worsened after surgery. Five patients (Cases
2, 7, 8, 9, and 21) experienced numbness of the face, al-
though two of them had not noted any numbness before
surgery. In one patient (Case 7) facial pain developed after
surgery. Two patients (Cases 1 and 8) displayed fifth crani-
al nerve motor weakness and four patients (Cases 1, 2, 14,
and 21) exhibited a decreased corneal reflex.
Sensory function returned to nearly normal in three of
five patients, and the corneal reflex returned to normal in
all but one patient. In two patients motor function of the
fifth cranial nerve, which was affected by surgery, recov-
ered in one and persisted mildly in the other.
Other cranial nerves were affected in four patients (Cas-
es 7, 9, 11, and 20). These included trochlear nerve deficits
in two patients (8%) that fully recovered 4 months postop-
eratively. Transient third nerve palsy in one patient and
facial palsy in another patient observed 4 days after surgery
recovered completely after 1 year. Function of the abducent FIG. 2. Upper: Photograph showing an anatomical model of a
nerve was not worsened by surgery. Cerebrospinal fluid dumbbell-shaped tumor that expands the orifice of the Meckel cave
rhinorrhea occurred in one patient (4%) and resolved after and compresses the trigeminal rootlet and ganglion. Lower: Con-
lumbar drainage. One patient experienced Dilantin intox- trast-enhanced axial MR images demonstrating a comparable ex-
ication, but recovered completely. Frontalis muscle weak- panded Meckel cave with a dumbbell-shaped schwannoma preop-
ness from the peripheral frontal branches of the facial nerve eratively (lower left) and 6 months postoperatively (lower right).
over the temporal muscle occurred in four patients, but re-
solved during the follow-up period. Table 3 lists postoper-
ative complications and follow-up clinical findings in all 25 changes, but we continue to follow them closely by per-
patients. forming MR imaging examinations.
Three patients (13%) experienced tumor recurrence after
a mean period of 22.3 months (range 13–36 months). In the
first patient regrowth was identified 18 months after total
tumor removal. This recurrence was detected on MR im- Discussion
ages and the patient underwent a repeated operation 4 years First reported by Dixon in 1846, schwannomas that arise
and 10 months after the first surgery. She was free from re- from the intracranial portion of the trigeminal nerve are
currence for 5 years after the second operation, but the tu- rare, accounting for 0.07 to 0.33% of intracranial tumors
mor again recurred. The second patient who experienced and 0.8 to 8% of intracranial schwannomas.5,10,26,38,52 In
tumor recurrence exhibited ataxia and diplopia 3 years after 1952, Cuneo and Rand10 published an excellent detailed
total resection of tumor. The recurrent tumor was extirpat- history on the treatment of these lesions and stated that, in
ed radically, and he was still free from recurrence 1 year 1927, Peet had found 63 cases in the literature. Subsequent
after surgery. In the third patient tumor recurrence was re- reviews have revealed approximately 402 cases to date.5,26,52
vealed by MR imaging 13 months after surgery. There were Because of the different origins and extensions of these
no clinical manifestations of the disease and, therefore, the tumors along the trigeminal nerve, several classification
patient was followed up for 2 years until he died of an un- systems have been suggested that have implications on the
related condition at the age of 82 years. In four patients, clinical findings, surgical approach, and outcomes of pa-
postoperative MR images demonstrated a small area of tients with these tumors. Jefferson20 classified these tumors
increased enhancement that did not change on subsequent into three types: Type A, tumors located mainly in the mid-
MR images. These areas are believed to be postoperative dle fossa that arise from the gasserian ganglion; Type B, tu-

J. Neurosurg. / Volume 96 / March, 2002 457


O. Al-Mefty, S. Ayoubi, and E. Gaber

FIG. 3. Photograph of an anatomical dissection (left) and artist’s illustrations (right and overlap) demon-
strating the surgeon’s extradural middle fossa view of a dumbbell-shaped trigeminal schwannoma with ex-
posure of the petrous carotid artery, three divisions and ganglion of the fifth cranial nerve, and entry into the
expanded Meckel cave.

mors located predominately in the posterior fossa that arise view of the literature, Sindou and Pelissou40 found that total
from the root of the trigeminal nerve; and Type C, tumors removal of the tumor had been achieved in only 50% of
with significant components in both middle and posteri- cases because of the close relationship of the lesion to the
or fossae, which he described as dumbbell-shaped or hour- cerebellopontine angle, petrous apex, cavernous sinus, and
glass tumors. Several modifications of this classification important cranial nerves. Despite the emphasis on total re-
have been described.26,38,52 moval in later series,31,45 this has not always been possible
because of risks to vital structures.8,31,35 In their review of
the literature, Samii and colleagues38 found that total or
Goal of Treatment near-total removal was achieved in approximately 70% of
Trigeminal schwannomas are overwhelmingly benign cases during the past 15 years. Our aim in treating patients
lesions; only a few malignant cases have been reported.6,10, with these tumors has been to achieve total removal, even
12,17,28
Even when large, they displace surrounding neuro- if the tumor is large and involves the cerebellopontine an-
vascular structures rather than engulf them. Therefore, total gle, petrous apex, or cavernous sinus (Table 4).
removal is believed to offer the best chance of cure,13,42 and Early attempts to remove these tumors were associated
subtotal removal is reported to be associated with higher with high rates of mortality.5 Hence, the emphasis on early
rates of recurrence.35 Subtotal removal is also reported to be detection of tumor is essential in preventing instances of
a source of postoperative bleeding from the tumor bed with morbidity and mortality.27,44 With the advent of microsur-
associated instances of morbidity and mortality.5,28 In a re- gical procedures, there has been a remarkable improve-

FIG. 4. Photograph of an anatomical dissection and an artist’s illustration demonstrating the surgeon’s view after the
posterior fossa extension of the tumor has been reached and removed through the Meckel cave, exposing the basilar artery
and the brainstem.

458 J. Neurosurg. / Volume 96 / March, 2002


Trigeminal schwannomas

FIG. 5. Operative photographs demonstrating extradural expo-


sure via the middle fossa and the removal of the posterior fossa
extension through an expanded Meckel cave. Upper Left: The
tumor (T) is exposed by peeling the outer dural wall (D) of the
cavernous sinus, and the stretched rootlets (arrow) can be seen.
Upper Right: View after the tumor has been dissected, with pres-
ervation of the trigeminal nerve including its divisions (V1,V2,
and V3), ganglion (G), and roots (arrowhead). The posterior fossa
arachnoid is seen through the mouth of the Meckel cave (arrow).
Lower: Posterior fossa neurovascular structures as seen through
the Meckel cave after tumor removal. B = basilar artery; P = pons.

ment in the ability to remove trigeminal schwannomas to- the sixth and seventh cranial nerves can be a complication
tally with low risks of mortality and morbidity.8,12,13,31,35,42,45,52 of surgery. Good health with only trigeminal nerve symp-
Fifth cranial nerve deficits that developed postoperatively toms is considered to be an excellent outcome.49
have been almost universal.26 The absence of mortality or Currently, the emphasis of treatment has shifted to the
major morbidity in our series corresponds to other excellent patient’s quality of life through the preservation or im-
results reported in recent series.12,13,42,52 provement of cranial nerve function, including that of the
Stereotactic radiosurgery has been used recently to treat
patients with trigeminal schwannomas.19 Tumor shrinkage
was achieved in 56% of treated patients and tumor growth TABLE 2
arrest in 44%. No neurological deficit was observed after a
mean follow-up period of 44 months. Although the authors Relationship between preoperative findings and
recommended that stereotactic radiosurgery be used as the postoperative improvement*
primary treatment in patients with small and moderate- Preop Clinical Findings No. of Cases W/ Postop Improvement (%)
sized trigeminal schwannomas, we reserve the use of radio-
surgery as adjuvant therapy for residual or recurrent tumors facial pain 8 of 11 (73)
that cannot be removed surgically, for patients unable to facial numbness 6 of 16 (38)
trigeminal motor deficit 4 of 5 (80)
undergo surgery and for patients who opt not to have sur- cerebellar signs 4 of 4 (100)
gery. We agree with others that the best treatment for tri- brainstem signs 1 of 1 (100)
geminal neurinomas is complete microsurgical removal of CN deficits
the lesion.13 II 1 of 1 (100)
IV 3 of 3 (100)
Cranial Nerve Function VI 6 of 8 (75)
VIII 3 of 4 (75)
Injury or permanent damage to the trigeminal branches IX 1 of 1 (100)
has been inevitable in many cases.49 Good health with tri- X 1 of 1 (100)
geminal nerve symptoms and other neurological deficits is * Three patients with prior surgically induced cranial nerve deficits were
considered to be a good outcome, even though injuries to excluded.

J. Neurosurg. / Volume 96 / March, 2002 459


O. Al-Mefty, S. Ayoubi, and E. Gaber

TABLE 3
Surgical intervention and follow-up results*
Follow Up

Case Surgical Duration Tumor


No. Approach Postop Complication Clinical Findings (mos) Recurrence (mos)

1 ZMF weak masseter muscle, mild fron- preop V2 & V3 numbness remained, trigeminal pain improved; 60 none
talis muscle weakness,† & de- postop corneal reflex & frontalis & masseter muscles re-
creased corneal reflex covered
2 pet‡ weak frontalis muscle,† decreased preop facial pain persisted; postop numbness, corneal deficit, & 29 none
V2, & decreased corneal reflex frontalis muscle recovered
3 ZMF none preop V2 & V3 numbness & corneal deficit persisted, pain & 75 none
masseter weakness improved
4 COZ phenytoin toxicity preop CN VI palsy & pain improved 62 stable on MR image
5 ZMF none preop facial pain persisted 134 18, reop at 5 yrs,
(2) 2nd recurrence 5
yrs later
6§ pet none preop facial pain recovered 48 36, reop; 1 yr
(2) free from recurrence
7 pet V1 & V2 numbness, facial preop V1 & V2 numbness improved; postop mild to moderate 42 none
pain, weak CN IV pain persisted, CN IV recovered completely
8† ZMF V2 & V3 numbness preop pain & masseter muscle weakness persisted; postop V2 & 10 none
V3 numbness persisted
9 ZMF V2 & V3 numbness, facial preop pain recovered; postop headache & facial palsy recovered, 4 none
palsy, headache numbness largely recovered
10§ COZ‡ none preop V2 & V3 numbness, decreased corneal reflex, & masseter 60 none
muscle weakness persisted; CN VI improved; lower CNs improved
11 pter CN IV dysfunction preop V1–3 numbness & CN VI weakness persisted; postop CN IV 24 13, died 2 yrs later
dysfunction resolved of other disease
12 COZ none preop V2 & V3 numbness persisted, pain & masseter muscle 60 none
improved, CN VI palsy improved after 8 yrs of paralysis preop
13 pet none preop numbness & pain improved, but retroorbital pain persisted 15 none
14§ pet dysphasia, decreased postop dysphasia & corneal reflex fully recovered 20 none
corneal reflex
15 pet CSF rhinorrhea preop V2 & V3 numbness improved, pain & CN VI dysfunction 58 none
resolved, CSF leak stopped
16† ZMF none preop CN V & VI improved 24 none
17 ZMF mild frontalis muscle preop numbness & corneal reflex improved; postop frontalis 37 none
weakness† branches improved
18 ZMF none preop CN V & VI weakness & corneal reflex recovered 12 none
19§ ZMF none preop pain improved 5 none
20§ COZ & transient CN III paresis preop ophthalmoplegia recovered, but CN V deficit persisted 24 none
pet‡
21§ ZMF weak frontalis muscle,† V1 preop CN V numbness increased; postop frontalis branches 5 none
numbness, decreased corneal improved
reflex
22§ ZMF none preop CN IV improved, persistent CN VI pain improved 7 none
23 ZMF none preop CN V & cerebellar function improved 6 none
24 ZMF none normal 3 none
25§ ZMF none preop V1 & V2 numbness; postop V2 numbness persisted 4 none
* Mean duration of follow up was 33.12 months during which three (15%) of 25 patients experienced tumor recurrence. The outcome in each case was
good. Abbreviations: COZ = cranioorbital zygomatic; CSF = cerebrospinal fluid; pet = petrosal; pter = pterional; ZMF = zygomatic middle fossa.
† From skin flap.
‡ Underwent previous surgery elsewhere.
§ Cystic lesion.

trigeminal nerve. Most patients have only a slight deficit 73%, and trigeminal motor deficit improved in 80% of pa-
in trigeminal function, which should remain the same13 or tients. The preoperative deficits of all other involved cra-
even improve. Our findings indicate that nontraumatic mi- nial nerves resolved by 4 to 6 months after surgery, except
crosurgical dissection of the remaining fibers of the trigem- for two of the eight cases of abducent nerve deficit. All cer-
inal roots, ganglion, and divisions effectively preserves or ebellar and brainstem manifestations resolved postopera-
even improves trigeminal nerve function. tively. Table 5 shows the effects of surgery on initial cranial
The clinical sequence of symptoms and cranial nerve de- nerve symptoms in major reported series. Cranial nerve de-
ficits is well described in the literature.5,12,13,15,18,24,31,35,38,39,42,45, ficits as a result of surgery and their clinical progress are
47,49,51,52
The tumors in our patients fit the usual pattern of listed in Table 6.
presentation. After surgery, preoperative facial numbness Our experience in this small series confirmed the report-
improved in 44% of patients, facial pain was alleviated in ed value of neurophysiological monitoring in skull-base

460 J. Neurosurg. / Volume 96 / March, 2002


Trigeminal schwannomas

TABLE 4 nerve dysfunction was seen in 70% of patients after con-


Total removal of tumor reported in the literature ventional approaches. Yoshida and Kawase52 also found
that rates of total tumor removal and clinical outcomes
Temporal Fossa Posterior Fossa Dumbbell Shape were significantly better in patients treated via a skull base
No. of Total No. of Total No. of Total
approach than in those treated via conventional approaches.
Authors & Year Cases Removal Cases Removal Cases Removal Skull base approaches were used in all but one of our pa-
tients, and gross-total resection was achieved in all patients.
McCormick, et al., 4 3 3 2 7 4 There was no incidence of mortality or major morbidity.
1988 Dumbbell-shaped tumors that involved both middle and
Pollack, et al., 1989 2 2 4 4 6 2
Yasui, et al., 1989 2 2 2 2 4 4 posterior fossae (Type C in Jefferson’s classification20 and
Samii & Matthies, 5 5 1 0 5 4 Type 4 in the classification of Lesoin and colleagues26) are
1997 distinct in their clinical and surgical considerations. Some
Day & Fukushima, 15 14 9 6 9 7 investigators have suggested that total removal of a dumb-
1998* bell-shaped tumor is more difficult when the retrosigmoid
Yoshida & Kawase, 9 6 5 3 12 10 and subtemporal approach is used.38 In most reported cases,
1999
present study 6 6 0 0 19 19 however, the ability to remove these tumors totally was not
total 43 38 (88%) 24 17 (71%) 62 50 (81%) significantly reduced (Table 4).
Dumbbell-shaped tumors were dominant in our series
* Excluding malignant cases in series.
(19 cases), constituting 76% of all tumors. Tumors in all
patients involved the cavernous sinus. Intraorbital, intra-
maxillary, intrasphenoidal, and infratemporal fossa exten-
surgery.34 It assists in locating cranial nerves displaced by sions appeared in our series and did not preclude total re-
tumor before they are inadvertently damaged and continu- moval of the tumor.
ally confirms their function during surgery.16,33,41,50 Electric As the literature shows, patients who underwent extra-
stimulation is also a useful predictor of postoperative func- dural surgery did well, even as early as the 1950s.5,10,27 In
tion.33,50 the 1980s, surgeons mainly used an intradural approach to
these tumors.6,8,31,35,49 Lesoin and colleagues26 advocated ex-
tradural approaches only for small tumors. More recently,
Role of Skull Base Approaches
the extradural approach has been used for extracranial tu-
Recently, authors have used skull base approaches to mors with intracranial extension,38 peripheral and gasserian
treat patients with trigeminal schwannomas.12,13,42,52 In a lesions,12,13 and small posterior fossa tumors when an ante-
comparison of conventional and skull base approaches, Ta- rior transpetrosal approach is added.52
ha and colleagues42 found that skull base approaches al- This extradural tumor removal is readily achieved
lowed better exposure of these tumors, multiple working through the zygomatic middle fossa approach from the cav-
angles with minimal brain retraction, and more complete ernous sinus and the infratemporal fossa. We add a zygo-
removal without increased morbidity. They found residual matic osteotomy to minimize retraction of the temporal
or recurrent tumors in 65% of patients who had undergone lobe and allow better access to the anterior temporal fossa.
conventional approaches compared with 10% of those who A posterior fossa extension is removed through the expand-
had undergone skull base approaches. In addition, cranial ed Meckel cave. Only large caudal extensions cannot be

TABLE 5
Literature review of effects of surgery on preoperative symptoms of trigeminal schwannoma*
CN V
CN CN CN Overall CN CN Lower
No. Pain Sensory Motor Overall III IV VI Diplopia VII VIII CNs
of
Authors & Year Cases N I N I N I N I N I N I N I N I N I N I N I

McCormick, et al., 14 8 7 11 0 4 0 — — 1 — 0— — — 2 3 2 3 2 4 3 — —
1988
Bordi, et al., 1989 6 — — — — — — 5 0 — — — — — — — — — — — — — —
Pollack, et al., 1989 16 7 ? 11 ? 3 ? — — 1 ? 1 ? 5 ? 5 ? 5 ? 7 ? 2 ?
Yasui, et al., 1989 8 3 ? 7 0 1 ? — — — — — — — — — — — — — — — —
Dolenc, 1994 44 3 3 — — — — 23 11 — — — — 5 5 5 5 — — — — — —
Samii, et al., 1995 12 2 ? 6 0 — — — — 3 ? 4 ? 4 ? 6 ? 2 ? — — — —
Taha, et al., 1995 15 3 100% 14 19% 5 — — — 2 — 2 — 2 — — 67% 1 — 3 — — —
Day & Fukushima, 38 13 11 32 2 — — — — 2 — — — 5 — 7 4 — — — — — —
1998
Yoshida & Kawase, 27 3 ? 14 ? 3 ? 21 ? 3 ? 2 ? 4 ? — — 2 ? 3 ? — —
1999
present study 22† 11 8 16 7 5 4 — — 0 0 3 3 8 6 9 6 0 0 4 3 1 1
* I = number of patients with improvement; N = total number of patients; — = data not available.
† Three patients with prior surgically induced cranial nerve deficits were excluded.

J. Neurosurg. / Volume 96 / March, 2002 461


O. Al-Mefty, S. Ayoubi, and E. Gaber

TABLE 6
Cranial nerve deficits after surgery for trigeminal schwannoma and their progress*
No. of Patients
CN V
W/ CN CN CN CN CN
Postop Sensory Motor Total III IV VI Diplopia VII VIII
Compli-
Authors & Year Total cations N P N P N P N P N P N P N P N P N P

Lesoin, et al., 1986 6 3 — — — — 1 1 — — — — — — — — 2 1 — —


McCormick, et al., 1988 14 12 — — — — 9 9 — — — — — — 3 1 3 1 4 1
Pollack, et al., 1989 16 14 7 — — 14 7 — — — — 4 1 4 1 3 1 2 0
Yasui, et al., 1989 8 8 7 7 — — 7 7 1 1 1 1 4 4 4 4 4 4 — —
Dolenc, 1994 44 — — — — 10 10 — — — — — — — — — — — —
Samii, et al., 1995 12 9 — — — — 8 8 — — — — — — — — 2 0 2 2
Taha, et al., 1995 15 70% 70% 56% 56% — — — — — — — — 63% 7% — — — —
Day & Fukushima, 1998 38 12 — — — — 11 11 — — — — — — 1 1 — — — —
Yoshida & Kawase, 1999 27 17 — — — — 10 10 3 2 — — 5 2 6 2 2 0 — —
present study 25 11 5 4 2 1 7 3 1 0 2 0 0 0 3 0 1 0 0 0
* N = total number of patients with postoperative cranial nerve dysfunction; P = number of patients with permanent deficits.

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