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J Neurosurg 82:924–932, 1995

Surgical treatment of jugular foramen schwannomas

M ADJID S AMII, M.D., R AMESH P. B ABU, M.D., M ARCOS TATAGIBA, M.D., AND ABOLGHASSEM SEPEHRNIA, M.D.

Department of Neurosurgery, Nordstadt Hospital, Hannover, Germany

Sixteen patients with schwannomas of the jugular foramen were operated on in the Department of Neurosurgery of the Nordstadt Hospital in Hannover, Germany, between 1986 and 1992. Patients with neurofibromatosis were excluded. The records of the 16 patients were retrospectively reviewed. There were five women and 11 men (mean age 43 years) with a symptom duration ranging from 6 months to 20 years (mean 5 years). The predominant symptoms were hearing loss, hoarseness, and cerebellar symptoms. Computerized tomography (CT), magnetic resonance (MR) imaging, and angiogra- phy were performed in all 16 patients. Depending on the radiological and surgical features, the tumors were classified into four types: Type A, a tumor primarily at the cerebellopontine angle with minimal enlargement of the jugular foramen (eight cases); Type B, a tumor primarily at the jugular foramen with intracranial extension (two cases); Type C, a primarily extracranial tumor with extension into the jugular foramen (one case); and Type D, a dumbbell-shaped tumor with both intra- and extracranial components (five cases). A retromastoid suboccipital craniectomy was performed for Type A tumors, and a combined cervical-mastoidectomy for Types B, C, and D. Total tumor removal was achieved in all cases. There was no operative mortality. Postoperative complications were cerebrospinal fluid leakage in one patient and mastoiditis in two patients. The follow-up period ranged from 12 to 42 months (mean 22 months). All patients were alive at the last follow-up review, and CT and/or MR imaging showed no tumor recurrence.

KEY WORDS

jugular foramen

neurinoma

retromastoid craniectomy

schwannoma

T WENTY-FIVE percent of all schwannomas are found in the head and neck region. 33 Intracranial schwan- nomas constitute approximately 8% of all primary

brain tumors. 55 Schwannomas arising from the ninth, 10th, and 11th cranial nerves without associated neurofibro-

matosis are relatively uncommon, 2,5–9,15,19,20,25,27,32,34,35,39–42, 44–46,48–50,53,54,59,61,63,64,67 and comprise only 2.9% of all intra-

cranial schwannomas. 67 Less than 120 such cases have been reported. 15,25,35,38,45,59,67 There is some confusion as to the total number of cases reported as some authors tend to include hypoglossal schwannomas in their series because of anatomical and clinical similarity as well as the diffi- culty in locating the nerve of origin. 7,61,70 In this communication, we report 16 cases of schwan- nomas of the jugular foramen operated on at the Neu- rosurgery Department of Nordstadt Hospital, Hannover, Germany, between 1986 and 1992. In this retrospective study, the clinical and radiological features are discussed, various surgical approaches are briefly reviewed, and the operative approach we preferred in the cases is outlined.

Clinical Material and Methods

Patient Population

Between 1986 and 1992, 23 patients with schwannomas of the jugular foramen and hypoglossal canal were oper-

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ated on at our institution. These 23 cases accounted for 1% of 2200 intracranial tumors, and 4% of 570 intracra- nial schwannomas resected during the same time period in our department. The ratio of jugular foramen schwanno- mas to acoustic schwannomas during this time was 1:24. Five patients who were diagnosed as having neurofibro- matosis type 2 were excluded from this study, as were two patients with solitary schwannomas of the hypoglossal canal. Sixteen patients had solitary schwannomas of the jugular foramen, and the clinical records, radiological ex- aminations, and operative notes of these patients were retrospectively reviewed. There were five women and 11 men in this series (Table 1). The average age was 43 years (range 20 to 71 years). The initial symptom for which they sought medical help was hearing loss (50%), headaches (66%), hoarseness (38%), and dysphagia (38%). One patient had shoulder weakness and one patient had facial paresthesias at the time of presentation. On examination, sensorineural hear- ing loss was seen in 75% of cases, involvement of the hypoglossal nerve with weakness of the tongue in 31%, facial palsy in 25%, diminished sensation in the trigemi- nal nerve distribution in two patients (13%), and cerebel- lar dysfunction in 44% (Table 2). All patients had pre- and postoperative computerized tomography (CT) using 1.5-mm slices with bone algo-

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Jugular foramen schwannomas

Jugular foramen schwannomas rithms; all underwent four-vessel angiography and mag- netic resonance (MR) imaging. Cerebral

rithms; all underwent four-vessel angiography and mag- netic resonance (MR) imaging. Cerebral angiography was performed to study the tumor vascularity and the patency of the jugular bulb. In the 11 most recent cases, MR angiography was performed. All patients have been regularly followed postoperatively in the Neurosurgery

been regularly followed postoperatively in the Neurosurgery F IG . 1. Drawing showing tumor Types A

FIG. 1. Drawing showing tumor Types A to D, classified according to tumor extension. Type A, tumors primarily at the cerebellopontine angle with minimal enlargement of the jugular foramen; Type B, tumors primarily in the jugular foramen with intracranial extension; Type C, primarily extracranial tumors with extension into the jugular foramen; and Type D, dumbbell-shaped tumors with both intra- and extracranial components.

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Department by CT and/or MR studies to detect any tumor recurrence.

Clinical Staging

At least two types of classification have been developed for jugular foramen schwannomas, depending on the site and extent of the tumors. The classification system helps to decide on the operative approach. Franklin, et al., 19 classified these tumors into Classes A, B, and C, based on the categories used for glomus jugular tumors. Kaye, et al., 35 developed a similar but simpler grading system, as follows: Type A tumors were primarily intracranial with minimal extension into the bone; Type B tumors were pri- marily within the bone with or without an intracranial component; and Type C tumors were primarily extracra- nial with only a minor extension into the bone or into the posterior fossa. Pellet, et al., 53 added another type, Type D, which is a saddlebag-shaped Type B tumor with intracra- nial and extracranial components linked via the jugular foramen. In our series, tumor extension was classified depending on the radiological and surgical features into Types A, B, C, and D (Fig. 1), as follows: Type A, tumors primarily at the cerebellopontine angle with minimal enlargement of

B, C, and D (Fig. 1), as follows: Type A, tumors primarily at the cerebellopontine angle

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F IG . 2. Schematic diagram showing the neurovascular contents of the jugular (jug.) foramen.

FIG. 2. Schematic diagram showing the neurovascular contents of the jugular (jug.) foramen. Inf. petr. sinus = inferior petrosal sinus; sig. sinus = sigmoid sinus; j. vein = jugular vein; roman numerals denote cranial nerves. Three configurations are shown.

the jugular foramen; Type B, tumors primarily in the jugu- lar foramen with intracranial extension; Type C, primarily extracranial tumors with extension into the jugular fora- men; and Type D, dumbbell-shaped tumors with both in- tra- and extracranial components.

Anatomical Study

The jugular foramen is situated between the lateral part of the occipital bone and the petrous part of the temporal bone. DiChiro, et al., 11 described the foramen as a canal coursing anteriorly, inferiorly, and laterally from an in- tracranial to an extracranial opening. The foramen is di- vided into two or rarely three compartments. 4 The two compartments are separated by a bony fibrous septum. Di- Chiro, et al., observed that occasionally the separation was incomplete, and called this the “intrajugular process,” which may be supplemented by a fascial ligament. 4 The anteromedial compartment is called the “pars nervosa,” and the posterolateral compartment is referred to as the “pars venosa” or “pars vascularis.” The pars nervosa con- tains the inferior petrosal sinus, vena canaliculi cochleae, and glossopharyngeal nerve, whereas the pars venosa con- tains the vagus and accessory nerves and the proximal part of the jugular bulb (Fig. 2). Kveton and Cooper 37 observed that the jugular bulb was not truly compartmentalized in the pars nervosa and pars venosa. Schwaber, et al., 58 in their cadaver dissections, noted that the vascular system was contained within the endothelial-lined lumen and that the cranial nerves were covered by fibrous tissue and lay outside the lumen of the jugular bulb. Anatomists divide the jugular foramen into anterior, posterior, and intermediate positions, where the interme- diate position contains the ninth, 10th, and 11th cranial nerves. There is also no universal agreement regarding the position of cranial nerves in the jugular foramen. Arenberg and McCreary 2 and Gejrot 21 believed that the ninth, 10th, and 11th cranial nerves are located in the pars nervosa, but Mountjoy, et al., 44 and Bossy 4 placed only the 10th and 11th nerves in the pars nervosa. There is a com- mon dural sheath for the 10th and 11th cranial nerves. The glossopharyngeal nerve has a separate dural covering

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has a separate dural covering 9 2 6 M. Samii, et al. F IG . 3.

FIG. 3. Magnetic resonance images showing jugular foramen schwannomas. A: Sagittal view of a Type A tumor. B: Axial view of a Type B tumor.

as it passes through the foramen. The meningeal branches of the occipital and ascending pharyngeal arteries pass through the intermediate portion and they are responsible for the vascular supply to tumors in this region. Jugular foramen schwannomas can arise from the prox- imal or distal part of the ninth, 10th, and 11th cranial nerves, presenting as either intracranial or extracranial masses. Dumbbell-shaped tumors with both intracra- nial and extracranial extension have been known. 3,12,59,62 After reviewing the available literature in 1975, Hakuba, et al., 25 found that the tumor most commonly arose from the ninth–11th nerve complex, and that the accessory nerve was involved the least. 6,34,41,46

Surgical Technique

The choice of surgical approach was determined by the type of tumor extension identified. 56,57 Type A jugular foramen schwannomas (Fig. 3A) were approached via a lateral suboccipital route with patients in a semisitting (lounging) position and the head fixed in a head holder, flexed slightly, and turned 30˚. For Types B (Fig. 3B), C, and D (Fig. 4) tumors, a cervical–transmastoid approach was used. The patients were operated on in the supine position with the head turned 50˚ to the opposite side, ele- vated 10˚ from the horizontal plane, and flexed 15˚ after fixation in a Mayfield head holder.

Type A Jugular Foramen Neurinomas. The

skin is

opened with a vertical incision behind the mastoid notch. The 8- to 10-cm incision extends from the top of the external ear down toward the neck. The neck muscles are divided vertically and retracted. A lateral suboccipital craniectomy is performed, exposing the transverse sinus superiorly and the sigmoid sinus laterally. The dura mater is then opened as a C-shaped flap a few millimeters away from the transverse and sigmoid sinuses. The dura and the cerebellum are retracted medially, the cerebellopontine cistern is opened, and the intracranial aspect of the tumor is exposed at the cerebellopontine angle. If necessary, the dorsal part of the jugular foramen is opened extradurally, and the dural incision is extended to the jugular foramen

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Jugular foramen schwannomas F IG . 4. A and B: Axial magnetic resonance image (A) and

FIG. 4. A and B: Axial magnetic resonance image (A) and cor- responding coronal computerized tomography (CT) scan (B) showing a Type D tumor. C: Enlargement of the jugular foramen and the bone destruction is seen in the bone-window CT scan.

to facilitate an intraluminal tumor removal. Microsurgical piecemeal tumor debulking and meticulous dissection of the cranial nerves should be done, while respecting the arachnoid planes. After tumor removal, the dura is closed in watertight fashion and the mastoid air cells are sealed with muscle and fibrin glue.

Type B to D Jugular Foramen Neurinomas. A retroauric-

ular skin incision is made, extending along the anterior border of the sternocleidomastoid muscle to the level of the hyoid bone. The mastoid is exposed after the mobi- lization of both the sternocleidomastoid muscle and the posterior belly of the digastric muscle. The caudal cranial nerves are identified in the neck along with the internal jugular vein and followed cranially to the skull base. The main stem of the facial nerve is exposed anterior to the origin of the digastric muscle, in the depth of the tympa- nomastoid fissure, inferior to the stylomastoid foramen. A part of the occipital squama is removed lateral to the occipitomastoid suture. A lateral suboccipital craniectomy then exposes the transverse and sigmoid sinuses. The sigmoid sinus is mobilized from its bone groove caudally down to the jugular foramen. The mastoid tip is removed and, to further extend the exposure, the posterior part of the occipital condyle may be removed, thus open-

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ing the jugular foramen dorsolaterally. Through this ap- proach, the sigmoid sinus, the jugular bulb, and the inter- nal jugular vein are exposed. The petrous bone is further drilled away anteromedial to the fallopian canal up to the styloid process, and inferior to the labyrinth. The dura is opened anterior to the sigmoid sinus, extending the incision to the jugular foramen. The transverse sinus is kept intact. The dura, transverse and sigmoid sinuses, and jugular bulb are retracted medially. The cerebellum is then gently retracted, and the cerebellopontine cistern is opened, releasing cerebrospinal fluid (CSF). The in- tracranial tumor portion is exposed. The extracranial portion of the tumor is resected first, using microsurgical technique. The intracranial portion of the tumor is then removed through the widened jugular foramen and the suboccipital craniectomy. The cranial nerves ventral to the tumor are identified if possible and separated from the tumor. It is important to recognize that schwannomas of the jugular foramen usually displace the jugular bulb dorsally. After tumor removal, the jugular bulb is replaced to its correct site within the jugular fossa and the dura is sealed in watertight fashion. In order to seal the dural defect at the jugular foramen, we use a piece of lyophilized dura and fibrin glue, reinforcing it with the posterior belly of the digastric muscle. The sternocleido- mastoid muscle is sutured back at its insertion on the mas- toid process. To avoid CSF leakage, lumbar drainage is placed. Prophylactic antibiotic medications are given for 7 to 10 days.

Results

Preoperative CT scans and MR images revealed iso- dense or isointense lesions, respectively, with enhance- ment after intravenous administration of contrast medium. Eight tumors belonged to Type A, two to Type B, one to Type C, and five to Type D. All the patients with 12th nerve involvement had dumbbell tumors (Type D). In two cases, the tumors presented with marked cystic de- generation. Cerebral angiography was remarkable for mild to mod- erate vascularity, differentiating these lesions from glo- mus jugulare tumors. The jugular bulb was patent in seven cases, partially occluded in four, and completely occluded in five. Tumors with extension into the cerebellopontine angle (11 cases) showed displacement of the anterior infe- rior cerebellar artery, posterior inferior cerebellar artery (PICA), and superior cerebellar artery (SCA), similar to acoustic tumors. Because none of these tumors was high- ly vascularized, preoperative embolization was not per- formed. Tumors at the cerebellopontine angle (Type A) were approached via a retromastoid suboccipital craniectomy (Table 3). For Type B to D tumors, a single-stage com- bined cervical and suboccipitomastoid approach with opening of the jugular foramen was used. The surgical findings and operative results are summarized in Table 3. There was no operative mortality. Two patients had mastoiditis, which was treated with antibiotic medica- tions. One patient had CSF leakage despite lumbar drainage, and needed surgical intervention for closing the dural defect at the region of the jugular bulb. In the imme-

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M. Samii, et al. diate postoperative period, the temporary cranial nerve morbidity rate was 38% (Table

diate postoperative period, the temporary cranial nerve morbidity rate was 38% (Table 4). Three patients had facial palsy, one had abducent palsy, one had swallowing problems, and one had tongue weakness as a new deficit. In all other patients, the cranial nerve dysfunction re- mained the same as preoperatively. The mean follow-up period was 22 months (range 12 to 42 months). All cranial nerve dysfunction had improved considerably at the last follow-up examination.

Discussion

Presentation

Schwannomas arising from purely motor nerves and particularly those arising from the lower cranial nerves are rare. To our knowledge, less than 120 cases have been reported in the literature. 15,25,35,38,45,59,67 These tumors arise from the ninth, 10th, and 11th cranial nerves. With the available neurodiagnostic armamentarium, a preoperative diagnosis of jugular foramen schwannoma is almost cer-

diagnosis of jugular foramen schwannoma is almost cer- 9 2 8 tain. However, it is often

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tain. However, it is often not possible to determine the nerve of origin of these tumors. 9 Jugular foramen schwannomas are more common in females; the age at diagnosis ranges from 14 to 63 years (average 37 years). 19,25,26,35,54,67 Although the youngest patient recorded was a 4-year-old boy, 59 these tumors are infrequent in the first decade and should raise the suspi- cion of neurofibromatosis type 2. 40 There seems to be no tumor predilection for the left or the right side. Symptoms do not manifest until the tumor attains a fairly large size, and hence the symptoms vary from very subtle to severe and may extend over years. Tan, et al., 67 noted the range of symptoms to be 4 months to 10 years and Hakuba, et al., 25 observed a range of 3 months to 13 years, with an average of 2.7 years. Intracranial and bone tumors have the longest duration as opposed to extracranial tumors, which have a shorter duration. 35 The origin as well as the size of the tumor at the jugular foramen defines the clini- cal presentation and determines the surgical approach. If the tumor arises more proximally, it will have a main- ly intracranial extension, presenting as a posterior fossa or cerebellopontine angle mass. The more distal lesions manifest as extracranial, cervical, or skull base tumors. Tumors arising in the midregion will expand the temporal

bone. 14,36,39,65

The early symptoms of presentation depend on the nerve of origin; however, this may not be true in cases of jugular foramen tumors, because the compartment is so small that all cranial nerves may be affected at the same time. Horn, et al., 27 have observed that, although these tumors present as a jugular foramen syndrome, palsies of the ninth, 10th, or 11th cranial nerves are not always pres- ent; 2,32,54,63,64 however, Tan, et al., 67 reported involvement of the nerves of the jugular foramen in eight of their 14 patients. Hakuba, et al., 25 reviewed the world literature in 1979 and found that more than half of the patients had blurred vision with decreased visual acuity or diplopia, and they noted dysphagia in half of the cases. Patients with larger

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tumors present with 12th nerve involvement, increased intracranial pressure, papilledema, cerebellar mass, and/or brainstem signs. 67

Differential Diagnosis

Historically, jugular foramen schwannomas are difficult to differentiate from acoustic tumors. Patients frequently present with seventh and eighth nerve deficits by virtue of the proximity of the jugular foramen to the internal acoustic canal. This has been observed more frequently in cases of Type A tumors; 35 but patients with Type B tumors more often exhibit tinnitus, deafness, middle ear mass, or involvement of the hypoglossal nerve. 35 The deafness seen with Type B tumors may be sensorineural or conductive or of a mixed type. Type C tumors present as a neck mass or mass of the lateral pharyngeal wall, 39,43,50 or can present with a variety of jugular foramen syndromes, includ- ing Vernet, Sicard–Collet, Schmidt, Avellis, Tapia, or Vil- laret. 65 Dumbbell-shaped tumors frequently present as a neck mass 59 or associated with difficulty in swallowing. A variety of tumors arising from the structures within or adjacent to the jugular foramen are encountered in the cerebellopontine angle. Of prime importance in the differ- ential diagnosis of jugular foramen schwannomas are the acoustic schwannoma and glomus jugulare tumors. However, other tumors that involve the cerebellopon- tine angle, namely meningiomas, choroid plexus papillo- mas, chordomas, exophytic pontine gliomas, chondromas, cerebellar hemangioblastomas, myxofibrosarcomas, epi- dermoid cysts, carcinomas of the tympanic cavity, and aneurysms of the internal carotid artery should be consid-

ered. 1,5,21,24,31,65

Even if the clinical presentation of these tumors mis- leads the clinician into making an erroneous diagnosis, like acoustic tumors or glomus jugulare tumors, radiolog- ical investigations often pinpoint the pathology of these cerebellopontine angle tumors. Plain x-ray films, which were essential until a decade ago, have become obsolete since the invention of high-resolution CT (with soft-tissue and bone algorithms), and MR imaging. 42,69 Variations of jugular foramen measurements, views, and projections on plain x-ray films have been well described. 10,13,22,54 DiChiro, et al., 11 have described enlargement of the jugu- lar foramen involving predominantly the pars nervosa showing smooth, indistinct sclerotic margins with or with- out destruction of the internal jugular process. In Type B tumors, considerable bone destruction has been noted. In cases of glomus jugulare tumors, irregular bone destruc- tion of the jugular foramen is frequently identified. Naunton, et al., 47 described changes in the appearance of the internal acoustic meatus in 11th nerve tumors, and DiChiro, et al., found enlargement of the jugular foramen in acoustic tumors. All of these findings are better appreciated in high-res- olution CT scans with bone windows. On CT scans with- out contrast enhancement, the tumor appears isodense or slightly hyperdense, and shows moderate enhancement after administration of contrast medium. 70 This is in con- tradistinction to meningiomas and glomus tumors, where enhancement is marked. If the tumor is of considerable size, even the fourth ventricular displacement is seen. 45 The invention of MR imaging and MR angiography has

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revolutionized the management of these tumors. The soft- tissue details, vascular supply to the tumors, relationship of major vessels, as well as the patency of the jugular bulb are well appreciated in MR imaging with gadolinium en- hancement and MR angiography. In T 1 -weighted images, these tumors are isointense or hypointense, becoming hyperintense in T -weighted images. 42 They show signifi- cant enhancement after intravenous gadolinium adminis- tration. Absence of a tumor tail within the acoustic meatus differentiates them from acoustic schwannomas. En- hancement is considerably greater in meningiomas, and signal-void areas, indicating the vascularity, are more prominent in glomus jugular tumors. 42,45,69 It may be some time before conventional angiography can be dispensed with altogether, because MR angiogra- phy is not yet fully developed. As mentioned earlier, the tumor derives its vascular supply from meningeal branch- es of occipital and/or ascending pharyngeal arteries. Oc- casional tumors have been described as being moderately vascular 35 but not as much as the highly vascular glomus jugular tumors. Various changes are noted on angiog- raphy, including ventral convex displacement of the cervi- cal portion of the internal carotid artery, displacement of the PICA to the opposite side, displacement of the SCA backward, and displacement of the tonsillar circula- tion. 25,51,66 In larger tumors, compression or obstruction of the jugular bulb is seen. 2,23 Sometimes the tumor can mimic a brainstem tumor on angiography. 41 Angiography is also useful in performing embolization if the tumor is very vascular, 9 although this procedure is not absolutely necessary.

Surgical Management

Until recently, some of these tumors were diagnosed as glomus jugular tumors 20 and have been subjected to non- surgical treatment like irradiation, because glomus jugular tumors were considered to be inoperable. Because jugular foramen schwannomas are benign, the goal of surgical treatment is to achieve complete resection. Constantly evolving surgical approaches to the petrous bone, a better understanding of skull base anatomy, improved electro- physiological monitoring, and microsurgical techniques have made the total removal of these tumors a possibility without causing major morbidity or mortality. We consid- er success at the first attempt most important, because reoperation in this difficult location causes significant cra- nial nerve deficits due to scarring from earlier surgery. Historically, there has been a constant quest for better and wider exposure of this area in order to deal with these difficult tumors. In 1904, Panse 52 gained access to the internal auditory canal through the mastoid air cells. In

1964 and 1968, House 28,29 modified a posterior petrosec-

tomy approach allowing exposure of the external two-

thirds of the cerebellopontine angle for acoustic tumors. In

1976 House and Hitselberger 30 operated on a tumor of the

clivus and acoustic canal via the transcochlear approach. Later in 1977 Fisch 16 improved on Gallard and Rebattu’s cervicomastoid approach, calling it the “infratemporal approach.” Various approaches have been described to re- sect these tumors depending on the tumor location and extension. Tumors confined to the upper cervical soft tis- sue are frequently dealt with via a conventional lateral

2

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neck approach; 3 occasionally a modified parotidectomy incision with cervical extension is performed if the tumor reaches into the jugular foramen. This latter approach helps to mobilize the facial nerve. 19 These tumors can al- so be managed through a mastoid–hypotympanic neck approach employed for glomus jugulare tumors. 7,8 In Type A tumors, a suboccipital craniectomy gives a good exposure. In Type C tumors, the “infratemporal ap- proach” described by Fisch 16 is suitable, because it pro- vides direct access to the jugular foramen and neck, providing good control of neighboring major blood ves-

sels. 1618 If the intradural tumor extension is more than 2 cm, this approach does not offer full view of the brainstem and cerebellum on which the tumor encroaches and hence,

in these cases, the Type A infratemporal approach is used

with a suboccipital craniectomy. 19 If the tumor is extradu- ral, even if it is less than 2 cm in size, the Type A infratem- poral approach is adequate and safe.

In Type B tumors, suboccipital craniectomy with either

a translabyrinthine transcochlear or an infralabyrinthine

(retrolabyrinthine) approach has been advocated, depend- ing on the function of the eighth nerve. Pellet, et al., 53 have described the widened transcochlear approach for volumi- nous Type B tumors with both intra- and extradural exten- sion, their so-called “Type D” tumors. Kinney, et al., 36 and Crumley and Wilson 9 have advocated a two-stage com- bined neurootological approach for their patients. Horn, et al., 27 resected these tumors in a single stage. Arenberg and McCreary 2 and Neely 48 used a suboccipital approach for these tumors. Gacek 20 performed a transmastoid approach, achieving only subtotal removal. Call and Pulec 5 believed that a transmastoid approach is better than a posterior fossa craniectomy for intracranial tumors to avoid poten- tial complications associated with posterior fossa surgery. Vellutini, et al., 71 reported improved hearing following removal of schwannomas arising from the lower cranial nerves through a retromastoid craniectomy. This has also been our experience, and we do not recommend any approach that primarily destroys the inner ear, causing loss of hearing. In all of our cases, the Type A tumors were operated on through a retromastoid craniectomy and Types B, C, and D tumors through a single-stage cervical approach combined with mastoidectomy. Even though several approaches exist and can be used to remove these tumors with intracranial and extracranial extensions, we believe that a single-stage modified cervicomastoid approach is the method of choice. The advantages are as follows: 1) the facial nerve is left in its bone canal; 2) drilling of petrous bone inferior to the labyrinth and cochlea allows preservation of hearing and vestibular function; 68 3) in case of injury to spinal accessory nerve during cervical tumor resection, reconstruction can be per- formed using a nerve graft; and 4) mobilizing the sigmoid and transverse sinuses and the jugular bulb medially allows better exposure and avoids division of the sigmoid sinus.

Complications

The major complications with this type of surgery include CSF leakage, meningitis and cranial nerve deficits. CSF Leakage. Thorough watertight dural closure pri-

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marily or with fascia lata or lyophilized dura is essential. Recently, fibrin glue has been found useful in decreasing CSF leakage. In all patients we used lumbar drainage for 1 week postoperatively. With the infratemporal approach

a muscle rotation flap may be created to reinforce closure in the infratemporal fossa.

Cranial Nerve Deficit and Swallowing. Worsening of

lower cranial nerve deficits resulting in breathing difficul- ty, dysphagia, and hoarseness are expected complications in resection of sizeable tumors. Severe, life-threatening complications such as aspiration pneumonia may accom- pany caudal cranial nerve injuries. The significance of neurological deficit depends on the type and number of cranial nerve deficits; they usually do not produce major disability. However, the presence of two or more neu- ropathies, especially in combination with the 10th nerve, may cause aspiration, which is potentially dangerous. We have observed that patients with slowly progressive deficits of the ninth, 10th, and 11th cranial nerves com- pensate for the altered mechanism of swallowing. Those patients who have normal cranial nerve function preoper- atively do not tolerate well postoperative deficits of acute onset. Intensive patient observation and care must be stressed in the immediate postoperative period. The moment of extubation is a critical instant, at which time a thorough neurological evaluation is mandatory. A nasogastric tube is kept in place. If the swallowing function remains im- paired over 1 week when there has been intraoperative cranial nerve preservation, or if cranial nerves are injured at surgery, temporary tracheostomy with a cuffed tube is performed. We maintain the tracheostomy until the patient is able to begin swallowing rehabilitation, which requires

a team of different professionals. For intractable aspira-

tion, or if a prolonged recovery period is anticipated, a gastrostomy is performed. In some cases, if aspiration of saliva remains a problem, surgical intervention with laryngeal closure is advocated. 14 In cases of unilateral 10th nerve injury, Ey and Denecke 14 recommended glottal repair with a cartilage implant into the vocal cords and resection of the paralyzed pharyngeal wall. For temporary vocal cord paresis, injection of Gelfoam or Teflon into vocal cords is employed. 60

Follow-Up Monitoring

Complete excision of the lesion is essential because the tumors tend to recur rapidly 35 and reoperation is likely to increase cranial nerve deficits secondary to scarring. Tan, et al., 67 reported that three of their patients with intracap- sular subtotal removal had suffered tumor recurrence 5, 15, and 16 years, respectively, after the original operation. Hence, patients need to be followed routinely with MR imaging, and any recurrence detected must be operated on immediately before it involves other cranial nerves. Early diagnosis, adopting the right surgical approach, and total removal of these benign tumors would result in excellent surgical results and long-term tumor-free interval or cure.

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Arenberg IK, McCreary HS: Neurilemmoma of the jugular

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575–715, 1968

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Bossy J: Atlas of Neuroanatomy and Special Sense Organs.

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1978

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chiatry 36:592–595, 1973

30. House WF, Hitselberger WE: The transcochlear approach to the skull base. Arch Otolaryngol 102:334–354, 1976

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Philadelphia: WB Saunders, 1970, p 285

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Manuscript received March 9, 1994. Accepted in final form September 12, 1994. Address for Dr. Babu: New York University Medical Center, New York, New York. Address reprint requests to: Madjid Samii, M.D., Neurochirur- gische Klinik, Krankenhaus Nordstadt, Haltenhoffstrasse 41, 30167 Hannover, Germany.

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