Aeta Neuroehirurgica 31,201--221 (1975) 9 by Springer-Verlag 1975
Neurosmgieal and Neuroradiologieal Departments, Neurological Institute of Milan
Intracranial Neurinomas of the Nerves of the Jugular Foramen Report
o f 12 P e r s o n a l
Cases
By
F. Pluchino, G. Crivelli, and M. A. Yaghi ~u
7 Figures
Summary 12 patients, 10 women and 2 men, were operated on for removal of a jugular foramen neurinoma. Removal was total in 11 cases and operative mortality was 16.6~ As there are no clinical or instrumental signs that permit sure distinction between these tumours and those of the cerebellopontine angle, only a thorough radiologieal study of the jugular foramen allows correct diagnosis. Often, however, the diagnosis is made during the operation. Key words: Neurinoma--jugular logical diagnosis.
foramen
clinical diagnosis---radio-
Neurinomas of the intraeranial segment of the glossopharyngeal, vagus and accessory nerves, also called neurinomas of the jugular foramen nerves, are very rare t u m o u r s (Hensehen 1955), so m u c h so t h a t in all only 27 eases have been described in the literature, 4 of t h e m necropsy findings (Gerhardt 1878, Gierlieh 1908, Henschen 1955, Russel et al. 1959). Of the 23 cases operated on only t h a t of Pool and P a v a (1957) and the 3 cases of Revilla (1947) form part of large surgical series of neurinomas of the eerebellopontine angle, the m a j o r i t y of authors reporting isolated cases, m o s t l y single (Cohen 1937, Columella et al. 1959, De Martel et M. 1932, Fishgold et al. 1954, Giordano 1968, Gra~ 1952, Guillain et M. 1933, Mansuy et al. 1964, N a u n t o n et al. 1968, P o r t u 1954), oce~sionMly more t h a n one (Columell~ et aI. 1959, Svien et al. 1963, R u b e r t i and Carteri 1961, with 2, 3 and 4 cases respectively). Acta Neurochirurgica, Vo].31, Fasc. 3--4
14
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F. Pluchino et al. :
Intrigued by the rarity of these tumours, we decided to check through the surgical files of the Neurological Institute of Milan to find their frequency in our material. We found t h a t between 1946 and 1971 alongside over 400 turnouts of various types of the cerebellopontine angle (including 238 neurinomas) 12 neurinomas of the jugular foramen were operated on and checked histologically. After reviewing these cases and the da~a of the literature (Cohen 1937, De Martel et al. 1932, Guillain et al. 1933, Mansuy et al. 1964, Pool et al. 1957, Porta 1954, Revilla 1947), we have come to the conclusion t h a t a precise preoperative diagnosis of these tumours is difficult. The published case reports of jugular foramen neurinomas commonly describe a cerebellar and vestibular hemisyudrome on the side of the tumour, intracranial hypertension, lesions of the third, fourth, fifth, sixth, seventh, eighth, ninth, tenth, eleventh and twelth cranial nerves (Cohen 1937, Columella et al. 1959, Columella et al. 1959, De Martel et al. 1932, Graf 1952, Guillain 1933, Mansuy et al. 1964, Naunton et al. 1968, Pool et al. 1957, Por~a 1954, Revilla 1947, Ruberti et al. 1961, Svien et al. 1963). Especially frequent are lesions of the last four cranial nerves, total or severe deafness is frequent, lesions of the trigeminat or facial nerves less so or less evident. According to some workers (Columella et al. 1959, De Martel et al. 1932, Mansuy et al. 1964, Ruberti et al. 1961), paralysis or paresis of a vocal cord associated with weakness of the trapezius and sternocleidomastoid muscles and homolateral nuchal headache are of great diagnostic value; glossopharyngeal neuralgia m a y be present (Cohen 1937); in rare cases hearing is normal (Mansuy et al. 1964). None of the cases described in the literature seem to have been diagnosed preoperatively, partly because there are no instrumental signs t h a t differentiate these tumours from neurinomas of the acoustic nerve (Giordano 1968, Naunton et al. 1968). Hence preoperative diagnosis is chiefly radiological, and since investigations with contrast media and gammaencephalography indicate a space-occupying lesion of the cerebellopontine angle in these cases, the most reliable data come, in our view, from the skull X-rays using special views for the jugular foramen.
Radiology of Neurinomas Of the Jugular Foramen The views useful for this purpose are the symmetrical bilateral and the unilateral oblique views. Tomography m a y be used in either case. The standard basal projection does not give a good view of the jugular foramen unless t o m o g r a p h y is used (Fig. 1). Some workers describe modifications of the basal view, which m a y be symmetrical bilateral (Fig. 2) or oblique unilateral (Decker 1968, Eraso 1961, Giraud et al. 1961). Others describe techniques for transoral symmetrical bilateral
Intraerania] Neurinomas of the Nerves of the Jugular F o r a m e n
203
Fig. t.. Tomogram of normal jugular foramina (arrows) in the basal view
Fig. 2. Normal jugular foramina (arrows) in the symmetrical bilateral projection according to Eraso's technique (see text and references)
14"
204
F. Pluchino et al. :
projections (Fig. 3) or oblique unilateral projections (Chausse 1950, Seuk K y K i m et al. 1966, Strickler 1966). The most well-known of the oblique unilateral views is Rossand's transmaxillary projection (Porcher et al. 1952) (Fig. 4), which we use routinely. The jugular foramen presents a pars nervosa and a pars vascularis: a fibrous (or less often bony) septum which stretches between the jugular spine of the petrous bone and the jugular process of the occipital
Fig. 3. Normal jugular foramina (arrows) in the transoral symmetrical projection (Seuk Ky Kim et al. 1966, Strickler 1966) bone separates the posterior and lateral portions of the pars vascularis (Fig. 5). Normal variations in size were described by Di Chiro (1964), who tabled them, including the values reported by other workers. The jugular foramina are generally asymmetrical both in shape and size; more often t h a n not the right is larger than the left. According to the observations of Di Chiro (1964), the difference in size is attributable to the pars vascularis. An alteration of the jugular foramen m a y be secondary to an intrinsic lesion, t h a t is to a lesion of the structures within the foramen itself, or, more rarely, to an extrinsic lesion, i.e. to a lesion of contiguous structures. The most frequent intrinsic lesions are glomns jugulare tumours (chemodectomas or non chromaffin parangiomas). Neurinomas of the ninth, tenth and eleventh nerves are very rare.
[ntraeranial Neurinomas of the Nerves of ~he Jugular Forame~t
205
Fig. l. Normal jugular foramina (arrows) in Rossand's unilateral oblique projection (Percher et al. 1952) An erosion with blurred and irregular edges t h a t at first affects mainly the pars vascularis is characteristic of a glomus jugulare tumour; the search for other bone snd angiographic signs t h a t will clinch the diagnosis is m a n d a t o r y (Hawkins 1961, Rice et al. 1963). A grow-th chiefly affecting the pars nervosa with clearcut and often thickened edges, erosion of the jugular process and sometimes widening
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F. Pluchino el al. :
of the petro-oecipital fissure should suggest a neurinoma of the ninth, ten*h and eleventh nerves. Extrinsie lesions affeeting the jugular foramen may be: primary or metastatie turnouts of contiguous bone structures, rhinopharyngeal
Fig, 5. Basal tomogram: a pars nervosa and glossopharyngeal (IX) nerve; b pars vaseularis and vagus nerve (X), accessory nerve (XI), ~ internal jugular vein; e jugular process of the petrous bone tumom's or meningiomas of the petrous ridge (Hawkins 1961). These tumours are responsible for erosion with somewhat blurred and irregular edges and m a y be difficult to distinguish from advanced glomus jugulare tumours (Di Chiro et al. 1964, Hawkins 1961, l~ice et al. 1963, Rueker 1963). Intrapetrous eholesteatomas m a y also affect the jugular foramen; they are generally described as well-defined areas with eleareut margins in the region of the attic and of the mastoid antrum (Hawkins 1961).
Intraeranial Neurinomas of the Nerves of the Jugular Foramen
207
Case-Material
A. Age, Sex, and Side Of the 12 patients 10 were women and 2 men; the age range was 20 to 60 years with a peak in the sixth decade (5 cases); in 7 cases the t u m o u r was on the right and in 5 on the left side. The salient clinical, radiological and surgical data on these 12 patients are summarized in the table.
B. Symptoms The length of history varied widely: in one patient (case 8) as much as ten years before admission, in 2 (cases 2 and 3) seven years, in 5 (cases 1, 6, 9, 10, 11) two years, in 1 (case 12) about a year and in 3 (cases 4, 5, 7) less t h a n a year. I n 5 eases the first s y m p t o m s were hearing disturbances (cases 2, 3, 5, 8, 10), in 3 (cases 6, 9, 12) giddiness and balance disturbances, in 2 headache (4, 11) and in 2 (eases 1, 7) ocular disturbances; in no ease was the first s y m p t o m impairment of one of the nerves of the jugular foramen. Of the 5 patients whose first s y m p t o m s were hearing disturbances progressing to deafness, 4 (eases 3, 5, 8, 10) had the hearing loss on the same side as the turnout and 1 (ease 2) on both sides. I n eases 2 and 3 the hearing defect commenced five or six years before the onset of an intracranial hypertension syndrome and in ease 8 as much as nine years before the rapid decrease in visual acuity of the right eye, t h a t is the s y m p t o m s t h a t led to hospitalization. The interval between the onset of s y m p t o m s and hospitalization was shorter, viz. between 8 months and 2 years, in the other two patients of this group. On admission the first had intermittent double vision on looking to the right, dysphagia for fluids and mild dysarthria (ease 5) and the other had balance disturbances and an intracranial hypertension syndrome (ease 10). Of the 3 patients whose first s y m p t o m s were giddiness and balance disturbances 2 complained of severe unilateral hearing trouble (eases 6 and 9) and one of total deafness on the same side, as the turnout occasional diplopia and mild dysarthria (case 12). I n the 2 cases in which the first s y m p t o m was headache, this was oppressive, continuous or intermittent. I n one patient this was followed four m o n t h s later b y paresis of the rectus lateralis oculi muscle on both sides, t h o u g h more marked on the left, t h a t is homolateral, as was subsequently found, to the lesion, and ataxia of the lower limbs, which later prevented her from walking (ease 4); in the other headache was followed b y giddiness, equilibrium disturbances, diminished hearing on the right side and total deafness on the left (ease 11). Of the two patients who started with visual disturbances, one (case i)
208
F. Pluchino et al. :
had scintillating scotoma in both eyes followed, one year later, by attacks of violent horizontal nystagmus in rapid succession and by giddiness and subjective loss of hearing before admission; the other (case 7) had reduced visual acuity on both sides, but more on the tumour side, followed by amenorrhea. 4 patients had late symptoms (appearing 10 to 20 months after the onset of the disease) indicating impairment of the lower cranial nerves: one (case 5) had dysphagia for fluids and mild dysarthria, one (case 6) dysphagia for fluids and solids and dysphonia whilst 2 (cases 4 and 12) had only mild dysarthria. I n the period immediately preceding hospitalization 3 patients (cases 1, 3, 6) had psychic disturbances in the shape of mental confusion, disorientation in time and space, abulia and memory defects; in only one case (9) did the patient notice facial paralysis. One patient had bilateral hyposmia (case 3) followed b y anosmia, which came on six and a half years after the first symptom.
C. _Neurological and Instrumental Signs In one case (3) neurological examination elicited bilateral anosmia. 8 patients had ocular signs: one patient had anisocoria only, the pupil on the tumour side predominating (case 12); 7 had papilloedema, always associated with other signs: in 2 (cases 3, 7) with decreased visual acuity and field defects, in 2 with decreased visual acuity (cases 8, 10), in 2 with defects of the extrinsic muscles of the eyes, t h a t is, in case 4 with bilateral paresis of the sixth nerves, more marked on the tumour side, and with paresis of the third nerve on the turnout side; in case 5 with paresis of the sixth nerve on the turnout side; lastly, in one patient the choked disk was associated with anisocoria, the pupil on the eontralateral side predominating. 10 patients had defective trigeminal sensation on the tumour side: 2 had hypoesthesia throughout the distribution (cases 8 and 12), 2 had hypoesthesia confined to the first branch (cases 3 and 10), 1 had hypoesthesia confined to the second branch (case 6), 5 had only diminished or absent corneal reflexes (cases 2, 4, 5, 7 and 11), this s y m p t o m did not appear in case 7 until fifteen days after admission. Facial movements were impaired in 6 patients: in 4 cases (1, 4, 10, and 12) the impairment was confined to movements of the lower half of the face omolateral to the tumour, in 2 to weakness of the orbieularis oculi muscle, bilateral in case 5 and omolateral in case 6. Severe loss of hearing on the side of the turnout was proven in all 12 patients and audiometry showed total deafness in 8 (cases 4, 5, 6, 8, 9, 10, 11, 12); in cases 1, 2, 3, and 7 there was marked hearing loss of the
Intraeranial Neurinomas of the Nerves of the Jugular Foramen
209
perceptive or mixed type. I n only one ease (7) were there no clinical signs of vestibular distress; of the other l l patients, 9 were subject to severe attacks of giddiness b r o u g h t on b y m o v e m e n t s of the head and eyes whereas 2 suffered from less severe b u t continuous giddiness; in 9 patients the deviation of the t r u n k in the erect position or while walking with the arms outstretched was t o w a r d the side of the lesion (cases 2, 3, 4, 5, 6, 8, 10, 11, 12) and in 2 t o w a r d the opposite side (cases 1 and 9). Caloric vestibular examination was done in all but one patient (case 7): it in one patient (case 1) induced a spontaneous horizontal n y s t a g m u s and in the other 10 patients multiple spontaneous n y s t a g m u s on the n o n - t u m o u r side. I n all 11 patients the verdict of the vestibular examination was a cerebellopontine angle syndrome with m a r k e d signs of brainstem involvement, as seen in large acoustic nerve neurinomas (cases 2, 5, 10, 11, 12). Clinical evidence of damage to the lower cranial nerves was found in 8 patients. These signs are shown in the following table: Table 1
Case Pharyngeal Palatal Dysphonia Dysarthria reflex reflex 3 4 5 6 7 9 ll 12
§ unilat. z_ unilat. @ bilat. @ bilat. § unilat. § unilat.
SwMlowing disturb- Hypotrophy of tongue anees
§ § --§
bilat. bilat,. unilat. unilat.
§ -t-
§ §
+
§ § § §
T
I n all cases in which the s y m p t o m s were unilateral t h e y related to the t u m o u r side. 8 patients had a cerebellar hemisyndrome oll the side of the t u m o u r of varying severity, characterized b y dysmetria, ataxia, adiadochokinesia, asynergia, h y p o t o n i a and tremor (cases 2, 3, 4, 5, 6, 81 10, and 11); 5 of t h e m were incapable of remaining in the erect position because of falling backward and to the side (cases 2, 3, 4, 5, and 8). A B a b i n s k y reflex was elicited in 4 cases: bilateral in case 5, on the side controlateral to the turnout in ease 4, omolateral in case 9, bilateral and associated with omolateraly increased m y o t a t i c reflexes in case 10. I n case 12 there were only increased m y o t a t i c reflexes on the side of the t u m o u r .
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D. Radiological Signs a) Secondary sella. Changes in the sella turcica were found in only 4 out of the 12 cases. The pattern varied from slight decalcification of the dorsum sellae to extensive destruction of both dorsum and floor.
Fig. 6. Case 4. Neurinoma of the left jugular foramen. L concentric enlargement of the left jugular foramen and destruction of the jugular process. R normal right jugular foramen b) Widening o/the jugular ]ora~nen. I n 4 patients (cases 3, 4, 6, 11) studied ~4th special projections and/or tomography the lesion had a characteristic appearance. The diagnostic features chiefly involved the pars nervosa, which showed a clearcut and at times thickened margin also destruction of the interjugular process (Fig. 6). I n cases 3, 4, and 11 the lesion was such as to cause major erosion with sharp edges in the middle and lower portions of the petrous bone, clearly visible in the anteroposterior tomogram. In case 11 the bone lesion extended posteriorly as far as the edge of the foramen magnum (Fig. 7).
IntraeraniM Neurinomas of the Nerves of tile Jugular Foramen
211
Fig. 7. Uase 11. Neurinoma of the right jugular foramen. Cleareut erosion of the right jugular foramen extending as far as the contour of the great, oeeipitM foramen and affecting the petrous bone. A tomogram in the basal view ; B tomogram in tho frontal view I n 2 oases (5 a n d 10) the s t u d y of the base of the skull was confined to the basal projection, which revealed only a n a s y m m e t r y of size of the j u g u l a r f o r a m e n t h a t could be t e r m e d borderline normal. I n ease 12, studied tomographieMly, the j u g u l a r f o r a m i n a were within n o r m a l limits. I n the other 5 eases there was no view of the j u g u l a r f o r a m e n region.
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c) Air studies. Pneumoencephalography was performed in only 4 cases; in one of these, because the air failed to enter the ventricular system, a ventrieulogram was also done (case 2). The air study of case 1 showed the pattern of an expanding lesion of the left cerebellopontine angle t h a t occupied the corresponding cistern causing contralateral displacement of the fourth ventricle and aqueduct and distension of the homolateral cisterna ambiens. In case 2 the air study b y lumbar route yielded generic evidence of a space-occupying lesion of the posterior fossa with hydrocephalus above; ventriculography showed triventricular hydrocephalus and blockade of the aqueduct in the neighbourhood of the third ventricle with no lateralising signs. The air study of case 7 showed a space-occupying lesion t h a t displaced the vallecula and fourth ventricle, leaving its imprint on the right contour, and hydrocephalus of all four ventricles. I n case 9 the cistern of the right cerebellopontine angle was not injected and there was hydrocephalus of all four ventricles. d) Angiographicstudies. 5 cases were studied angiographically, 4 with retrograde injection via the left arm and 1 via the left. Cases 5 and 11 presented the typical pattern of an extra-axial tumour of the right cerebellopontine angle. The prepontine and lateropontine portions of the right superior cerebellar artery were raised and pushed toward the midline. In the lateral view the right superior cerebellar artery was raised and stretched. Cases 7 and 8 presented no appreciable vessel displacements, although in case 8 fine arterial branches disposed in an are seemed to circumscribe a tumour in the territory of the inferior posterior cerebellar artery. This finding has been described as characteristic of other types of cerebellopontine angle tumours, especially of acoustic neurinomas 15, ~a, 35 I n case 12 the 6nly sign t h a t angiography supplied was a backward displacement of the tonsillar circulation, assessed according to its projection on the Twining line (Leman et al. 1967). This finding was present in the other four cases as well. e) Radioisotope scanning. Only 4 patients (cases 8, 9, 10, and 12) were subjected to gammaencephalography: 2 with ~0aHg, one with 99mTc and one with both of these isotopes. In all there was a hot area in the tumour site. In conclusion, in 4 of our patients operation was performed on the basis of a correct clinical and radiological diagnosis, thanks to the demonstration of a typical alteration of the jugular foramen (cases 3, 4, 6, and 11). For 2 patients (eases 2 and 7) the diagnosis was a posterior fossa space-occupying lesion; in these eases the jugular foramen was not
IntraeraniM Neurinomas of the Nerves of the Jugular Foramen
213
studied and arteriography and encephalography did not supply a more precise site. I n the other 6 patients the operation was performed on the diagnosis of acoustic nerve nenrinoma: for cases 1, 9, and 5 the diagnosis was based on the nenroradiologieal investigations (angiography or pneumoeneephalography); for cases 8 and 12 it was based on angiography and brain scans; for case 10 the diagnosis was based on clinical examination and brain scans. I n 3 of these cases (1, 8, 9) the jugular foramen was not studied radiologically, in 2 (5, 10) insufficiently and in case 12 the tomographie findings were normal.
E. Surgical Treatment All the patients were operated on in the prone position using general anaesthesia with controlled respiration. I n 2 patients (eases 2 and 7) a suboceipital bimastoid craniectomy was performed, in the other 10 eases a suboeeipital hemicraniectomy. I n 11 eases the turnout was exposed by retracting the eerebellar hemisphere without need for a m p u t a t i o n ; in one case only the homolateral cerebellar tonsil had to be a m p u t a t e d because it had herniated into the occipital foramen (case 2). The approximate size and the macroscopieal structure of the tumours are given in Table 2. I n one ease it m a y be definitely said t h a t the t u m o u r was attached to the glossopharyngeal nerve (ease 7), in another on the vagus (ease 9) and in another on the accessory nerve (ease 11). I n the other 9 patients it was impossible to say to which of the jugular foramen nerves the t u m o u r had been attached. The lower cranial nerves were spared in toto in 11 eases, whereas in ease 1 t h e y were extensively d a m a g e d during the manoeuvers to dissect the turnout capsule from the border of the jugular foramen, to which it adhered tightly. Of the 11 cases in which it was possible to isolate the acoustic and facial nerves from the tumour, only one suffered circumscribed damage to the facial nerve (case 3); in 5 patients (cases 2, 3, 4, 10, and 11) the turnout had grown into the enlarged jugular foramen, where it was nesting, and in case 11 there was an extraeranial projection of the turnout t h a t came into contact with the terminal portion of the vertebral artery; these outgrowths were completely removed in every ease. I n 9 cases there were adhesions between the t u m o u r and the brainstem (eases 1, 2, 4, 5, 6, 7, 8, 9 and ll), which in 5 cases (2, 4, 5, 6, and 8) were rather close and which in ease 2 were associated with severe distortion of the medulla oblongata due to herniation of the turnout t h r o u g h the foramen m a g n u m .
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Table Clinical signs Case No.
Age
Sex
Side
Duration of symptoms on admission
Cranial nerves damaged
Papillary oedema
1 c,cl, 24,809
36
f
it.
2 years
VII
2 e.cl. 26,290
50
f
ft.
7 years
II
3 c.ci. 30,958
52
f
It.
7 years
I II VIII
4 c.cl. 31,911
58
f
lt.
10 months
II VI IX
5 c.cl. 40,193
20
m
rt.
7 months
II V YI VII VIII IX X XII
6 e.e]. 44,875
58
f
it.
2 years
V IX
VII VIII X
7 e.cl. 50,750
27
f
rt,
8 months
II IX
V X
VIII
present
8 c.cl. 51,020
60
f
ft.
10 years
II
V
VIII
present
9
41
f
rt.
20 months
VIII IX
X
10 c.d. 54,840
30
f
lt.
2 years
II V VIII
VII
11 e.e], 55,227
42
f
rt.
2 yeats
V VIII XI XII
12 e.cl. 56,306
47
m
rt.
1 year
III V VIII
c.cL 52,642
Cerebellar signs
Radiologica:
Pyramidal signs
Secondary sella
VIII
III V VIII
V IX
III V VII VIII XII
VII XII
Plain X - r a y of the jugular foramen
n o t studied
present
ataxia asynergia
n o t studied
present
ataxia hypotonia
enlarged with clcarcut margin~
present
hypotonia
right Babinski
present
ataxia hypotonia asynergia
bilateral Babinski
hypotonia
present
hypotonia
ataxia hypotonia
present
enlarged with clearcut m a r g i m
inadequate study
present
enlarged with clearcut margin~
present
not studied
present
no~ studied
right Babinski
n o t studied
bilateral Babinski increased reflexes on left
inadequate study
asynergia hypotonia
enlarged w i t h clearcut margini
increased reflexes on r i g h t
normal
Intra,cranial
Neurinomas
of the Nerves
of the Jugular
signs
Foramen
215
Surgical findings
Plain X-ray PEG or ventribones adjacent to jugular culography foramen diagnosis
Arteriography diagnosis
Gamma encephalography
Preoperative diagnosis
Tumour texture and size
Nerves to which it adhered
Result
left cerebello pontine angle space occupying lesion
C.P. angle tumour
solid IX X XI tangerine
living ]8 months after operation
posterior fossa space occupying lesion
posterior fossa tunmur
solied IX X X I tangerine
died 36 hours after operation from haemorrhagie itlfarction of medulla oblonga~a proven at necropsy
lesion of the middle-lower p e r s o n of the petrous bone
jugular foramen neurinoma
solid apricot
IX X XI
perfectly normal life 10 years after operation
lesion of the mlddie-lower p e r s o n of the petrous bone
jugular foramen neurinolua
cystic apricot
I X X XI
perfectly normal life 2 years after operation
C.P. angle tumour
cystic IX X XI hen's egg
perfectly normal life 4 years after operation
jugular foramen neurinoma
solid aprico t
perfectly normal life 2 years after operation
posterior fossa tumour
solid apricot
spaceoccnpying right cerebello pontine angle lesion
posterior fossa inadequate space occupying study lesion pathological circulation in right cerebello pontine angle right cerebello Dentine angle space occupying lesion
lesions of the medial ~ of the petrous bone and occipital condyle
space occupying right cerebello pontine angle lesion inadequate study
IX X XI
IX
perfectly normal life 2 years after operation
accumulation C.P. angle Hg 203 in tmnour right C.P. angle
solid walnut,
IX X XI
died on 6th day no necropsy
accumulation in right C.P. angle
C.P. angle turnout
solid walnut
X
perfectly normal life 18 months after operation
accumulation Hg 203 and Tc 99 m in left C.P. angle
C.P. angle tumour
cystic apricot
IX X XI
perfectly normal life 1 year after operation
jugular foramen neurinoma
solid apricot,
XI
perfectly normal life 1 year after operation
aecumuiation C.P. angle Tc 99 m in tunmur right C.P. angle
cystic I X X X[ hen's egg
perfectly normal life 4 months after operation
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F. Pluchino e~ al. :
The tumour was removed completely in all cases but one, in which, because of the tight adhesions between the anterior superior pole of the neurinoma and the acoustic-facial complex, intracapsular removal was performed to avoid injuring the right facial nerve and so causing facial diplegia in a patient who already had eontralateral peripheral paralysis of the seventh nerve for other reasons (case 9).
F. Postoperative Course and Results One patient remained conscious and lucid for the first 24 hours after the operation but t~hen went into coma and died 12 hours later (case 1): necropsy revealed a haemorrhagic infarct extending from the dentate nueleus as far as the lower portion of the superior cerebellar peduncle on the same side as the turnout. Another patient died on the sixth d a y after several serious gastric haemorrhages and permission for necropsy was not granted (case 8). 6 patients had an uneventful postoperative course (cases 3, 5, 7, 10, 11, 12) but in 2 cases (3,s~ 7) corneal anaesthesia necessitated partial tarsorrhaphy and in case 3 a peripheral facial paresis appeared after the operation. 4 patients (cases 2, 4, 6, and 9) had a stormy postoperative course. 3 patients had bronchopneumonia, which was slow to clear (cases 2, 4, and 6), and had to be fed by nasal tube for a long time; one patient (case 4) had a severe gastric haemorrhage on the fourth d a y and another (ease 6) had to undergo traeheostomy; ease 9 had late respiratory insufficiency necessitating traeheostomy and mechanical ventilation for ten days. These ten patients were discharged in good or fair general and neurological condition and all were leading a normal life after an interval ranging from four months to ten years from operation.
Discussion I n the few cases of jugular foramen neurinoma reported in the literature the patients are usually described as having a cerebellar and vestibular hemisyndrome omolaterM to the tumour, intracraniM hypertension and lesions of the third to twelfth cranial nerves (Cohen 1937, Columella et al. 1959, De Martel et al. 1932, Graf 1952, Guillain 1933, Mansuy et al. 1964, Naunton et al. 1968, Pool et al. 1957, Porta 1954, I~evilla 1947, Ruberti et al. 1961, Svien et al. 1963): especially frequent are lesions of the last four cranial nerves, total or severe deafness is frequent, lesions of the trigeminal and facial nerves are less frequent or less marked. According to some (Columella et al. 1959, De Martel et al. 1932 ; Mansuy et al. 1964, l~uberti et al. 1961), paralysis or paresis of a vocal cord associated with weakness of the trapezius and sternocleido-
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mastoid muscles and homolateral nuehal headache are of high diagnostic value; in some cases glossopharyngeal neuralgia m a y be present (Cohen 1937); in rare cases hearing is normal (Mansuy c t a l . 1964). On our evidence and that of other workers there seems to be no characteristic syndrome of neurinomas of the jugular foramen because these tumours very often give signs similar to those of acoustic neurinomas. In fact, in all our patients the acoustic nerve was damaged, even when the tumour was not very large (ease 8), so that there would seem to be justification for Naunton et al.'s (1968) assertion that from the otovestibular point, of view acoustic neurinomas cannot be distinguished from those of the jugular foramen. Furthermore, 4 of our patients (cases 1, 2, 8, 10) wesented without any impairment of the lower cranial nerves and in this connexion it is noteworthy that the accessory nerve was not damaged in any of our patients, not even in case ll, in which the turnout adhered closely to this nerve, from which it seemed to have originated. If we add that acoustic neurinomas may induce marked signs of damage of the lower cranial nerves, it is clear that the symptoms of such tumours closely resemble those of the jugular foramen. The only relatively reliable criterion is that in neurinomas of the jugular foramen the signs of trigeminal and facial nerve damage are later and/or milder than in acoustic neurinomas. However, this clue is not always available and so is far from sure. I n our experience a correct diagnosis can be made only in cases in which the clinical symptoms are accompanied by a bone change of the jugular foramen, which can be demonstrated by a thorough X-ray examination. The other neuroradiologieal investigations generally permit a generic diagnosis of a space-occupying lesion of the eerebellopontine angle. Intact jugular foramina do not, however, rule out the possibility of a neurinoma of the lowest cranial nerves (ease 12). None of our eases presented bone changes of the internal acoustic meatus. Naunton (1968) described changes in the appearance of the internal auditory foramen in a ease of neurinoma of the eleventh nerve, whilst Di Chiro (1964) found an abnormal enlargement of the jugular foramen in a case of neurinoma of the eighth nerve. The upshot of our findings is that the usual views of the skull are not sufficient for the radiologieal detection of intraeranial tumours of the jugular foramen and that the region must be studied with elective techniques. Moreover, since the clinical signs of these tumours often closely resemble those of acoustic neurinomas, it would be no bad thing for the jugular foramen region to be examined radiologieally in all patients with signs of damage to the lowest cranial nerves. From the surgical point, of view knowing that the turnout is a neurinoma of the jugular foramen rather than an unspecified tumour of the Acta NcurocMrurgiea, Vol. 31, Fase. 3--4
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cerebellopontine angle does not alter the surgical approach but it holds out greater hope in the first case of sparing the facial nerve, which is of some importance to the patient. Of the 23 operated cases described in the literature one was only explored (Pool et al. 1957) and one underwent removal of unspecified extent (Giordano 1968); 10 underwent partial or subtotal removal of the tumour (De Martel 1932, Guillain et al. 1933, Cohen 1937, Graf 1937, Porta 1954, 3 cases of Columella 1959, cases 2 and 4 of l~uberti and Carteri 1961) with only one death (Guillain 1933); 11 patients underwent apparently total removal of the tumour (Fischgold 1954, Mansuy et al. 1964, Naunton 1968, cases 1 and 3 of Ruberti and Carteri I961, 3 cases of Svien et al. 1963, 3 cases of Revilla 1947) and all the patients of this group survived the operation. The mortality in the literature is thus 10% for partial removal and zero for total removal. In our series (11 radical operations and 1 subtotal removal with 2 deaths) both the operative deaths occurred in radical operations and hence the mortality is 16.6%, or 18% if referred to the radical operations only. Our cases together with those of the literature make a total of 33 operations with a mortality of 9.1%, whether referring to the partial removals (11 operations, 1 death), to the radical removals (22 operations, 2 deaths) or to the total (33 operations, 3 deaths). In view of this, radical removal would seem to us to be the right course unless the surgeon considers the risk too great on grounds of age, general condition or anatomical condition discovered at operation (as in our case 9). As with neurinomas of the acoustic nerve, amputation of the outer third of the cerebellar hemisphere is in our view almost always unnecessary, simple retraction being usually sufficient especially if preceded by the administration of osmotics or b y ventricular puncture; damage to the seventh, eighth, ninth, tenth and eleventh nerves, rare and usually circumscribed but possible during the removal of very large neurinomas, can be avoided by a precise preoperative diagnosis and the use of an operating microscope. Conclusions This study and previous reports in the literature prompt the following conclusions : 1. E x a c t preoperative diagnosis of neurinomas of the jugular foramen, especially differential diagnosis from acoustic neurinomas, is difficult since the only clinical pointer is paralysis or paresis, which m a y be mild, of the lower cranial nerves, which is found more frequently t h a n in acoustic neurinoma, whereas loss of facial sensation and movement are found less often.
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2. H e n c e a eerebellopontine angle s y n d r o m e in which t h e trigeminM a n d facial n e r v e lesions are slight a n d lower cranial nerve lesions are m a r k e d or a n y w a y p r e s e n t should s y s t e m a t i c a l l y suggest n e u r i n o m a of t h e j u g u l a r f o r a m e n a n d p r o m p t t h o r o u g h X - r a y e x a m i n a t i o n of t h e base of t h e skull w i t h special views for t h e j u g u l a r foramen, because X - r a y c o n f i r m a t i o n in t h e shape of a eleareut widening of t h e j u g u l a r foramen, chiefly in t h e p a r s nervosa, clinches t h e diagnosis in t y p i c a l eases. O t h e r neuroradiological investigations are useful because t h e y s u p p l y i n f o r m a t i o n on t h e size of t h e t u m o u r a n d its connexions with i n t r a e r a n i a l structures. 3. T h e results of surgical t r e a t m e n t are satisfactory, w i t h a m o r t a l i t y of 16.6~o on t h e basis of our 12 o p e r a t i o n s a n d 9.1~ on t h e basis of t h e cases in t h e l i t e r a t u r e . As for acoustic neurinomas, r e m o v a l m u s t be radical, e x c e p t in special eases. A n a c c u r a t e p r e o p e r a t i v e diagnosis is v a l u a b l e because it enables t h e surgeon to p l a n t h e o p e r a t i o n in such a w a y as to spare t h e acoustic-facial complex c o m p l e t e l y or nearly, even in t h e presence of v e r y large t u m o u r s , like those of our eases.
References
1. Chauss6, C., Trois incidences pour l'examen du rocher. Acta Radiol. (Stockholm) 34 (1950), 274--287. 2. Cohen, H., Glosso-pharyngeal neuralgia. J. Laryng. 52 (1937), 527-536. 3. Columella, F., G . B . Delzanno e G. C. Nicola, Neurinoma extradurale dell'angolo ponto-cerebellare. Chirurgia 14 (i959), 699--794. 4. Les neurinomes des quatre dcrniers nerfs craniens. Neurochirurgie 5 (1959), 280--295. 5. Decker, K., Neuroradiologia clinica, pp. 16, 24, 28. P a d o v a : Picein Ed. 1968. 6. De MarteI, T., ct J. Guillaume, Neurinomes des nerfs mixtes. Operation. Gu6rison. l~ev. Neurologique 39, 1 (1932), 884--889. 7. Di Chiro, G., R . L . Fisher, and K . B . Nelson, The jugular foramen. J. Neurosurg. 21 (1964), 447--460. 8. Eraso, S. T., Rontgen and clinical diagnosis of glomus jugulare tumors. F o u r eases and a new radiographic technic. Radioldgy 77 (1961), 252-256. 9. Fishgold, I-I., J. Metzger et G. Korach, Tomographie de la rggion p6trosphgno-oeeipitale. Incidence des quatGre dernigres paires crgniennes. Acta Radiol. (Stockholm) 42 (1954), 56--64. 10. Frey, K . W., Die Tomographie der Glomustumoren des Ohres. Fortsch. R6ntgenstr. 107 (1967), 785--786. 11. Gerhardt, C., Zur Diagnostik multipler Neurombildung. Dtseh. Arch. Klin. Med. 21 (1878), 268--289. 12. Gierlich, Zur Symptomatologie der Tumoren des Kleinhirns und des Kleinhirnbrtickenwinkels. Dtsch. Med. Wschr. 34 (1908), 1800--1804. 15"
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13. Giraud, M., P. Bret, A. Aujon, L. Chollat et C. Feuillade, Exploration radiographique et tomographique du canal dechir6 post@rieur normal et pathologique. Ann. Radiol. (Paris) 4 (i961), 543--558. 14. Giordano, R., Sindrome dell'angolo ponto-cerebellare da neurinoma acustico e del foro lacero posteriore, meningioma e colosteatoma. Considerazioni cliniche. Riv. Oto-Neuro-Oftal. 43/4 (1968), 355--384. 15. Goree, J. A., G . T . Tindall, and G. L. Odom, Percutaneous retrograde brachial angiography in the diagnosis of acoustic neurinoma. Results in 4 cases. Amcr. J. Roentgenol. 92 (1964), 829--835. 16. Graf, K., Gesehwfilste des Ohres u n d des Kleinhirnbriickenwinkels, pp. 191 197. Stuttgart: G. Thieme. 1952. 17. Guillain, G., I. Bertrand et J. Lereboullet, Neurinome des nerfs mixtes: prolongement extracranien de la tumeur. Rcv. Neurol. 40, 2 (1933), 56--68. 18. Hawkins, T. 1)., Glomus jugulare and carotid body tumours. Clin. Radiol. 12 (1961), 199--2t3. 19. Henschen, F., Tumoren des Zentralnervensystems u n d seiner Hfillen, in : H e n k e - - L u b a r s c h - - R o s s l e : Handb. Spez. Path. Anat. ttistol. 13, 3, S. 865--866. Berlin-GSttingen-Heidelbcrg: Springer. 1955. 20. Leman, P., F. Comadon et C. Leifer, Description des trajets normaux des art6res de la fosse postgrieure. Ann. Radiol. 10 (1967), 781---790. 21. Mansuy, L., P. Girard et P. Bret, A p r o p o s de deux observations de tumeur du trou dgchir6 post6rieur. Rev. Oto-Neuro-Ophthal. 36/5 (1964), 249--254. 22. Naunton, R . F . , L. Proctor, and B.S. Elpern, The audiologic signs of n i n t h nerve neurinoma. Arch. Otolaryng. 87/8 (1968), 222--227. 23. Olsson, O., Vertebral angiography in the diagnosis of acoustic nerve tumors. Acts Radiol. 39 (1953), 265--272. 24. Pool, J . L . , and A . A . Pays, The early diagnosis and treatment of acoustic nerve tumors, p. 140. Springfield, Ill.: Ch. C Thomas. 1957. 25. Porcher, P., Y. Porot et L. Rossand, L'incidence trans-maxillaire (incidence facile et shre pour la raise en 6vidence de la pyramide p6treuse et du trou d6chir6 post6ricur). J. de Radiol. et d'Electrol. 33 (1952), 379--382. 26. Ports, C., Contributo della stratigrafia alla diagnostics dei tumori della base crania. Neurone 2 (1954), 57--71. 27. Rice, R . P . , and C. 13. Holman, Roentgengraphic manifestations of tumors of glomus jugulare (ehemodectoma). Amer. J. Roentgenol. 89 (1963), 1201--1208. 28. Revilla, A. G., Neurinomas of cerebellopontile recess. A clinical study of one hundred and sixty cases including operative mortality and end results. Johns Hopk. I-Iosp. Bull. 80 (1947), 254--296. 29. Ruberti, R., e A. Carteri, Neurinomi del forame lacero posteriore. G. Psichiat. Neuropat. 89 (1961), 1099--1128. 30. Rucker, T., Radiology of glomus jugulare tumors in the temporal bone. Radiology 81 (1963), 807--816. 31. Russel, 1). S., and L . J . Rubinstein, Pathology of tumours of the nervous system, p. 237. London: E. Arnold Ltd. 1959. 32. Seuk Ky I~im, and M. P. Capp, Jugular foramen and early roentgen diagnosis of glomus jugulare tumor. Amer. J. Roentgenol. 97 (1966), 597--600.
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33. Striekler, J. M., New and simple techniques for demonstration of the jugular foramen. Amer. J. Roentgenol. 97 (1966), 601--606. 34. Svien, H. J., H. L. Baker, and M. H. Rivers, Jugular foramen syndrome and allied syndromes. Neurology 13 (1963), 797--809. 35. Takahashi, M., G. Wilson, and S. Hanafee, The anterior inferior eerebellar artery: its radiographic a n a t o m y and significance in the diagnosis of extra-axial tumors of posterior fossa. Radiology 90 (1968), 281---287. Authors' address: Dr. F. Pluehino, Dr. G. Crivelli, Neurosurgieal Department, Dr. M. A. Vaghi, Neuroradiologieal Department, I s t i t u t o Neurologico di Milano, Via Giovanni Celoria, i1, 1-20133 Milano, Italy.
Neurosmgieal and Neuroradiologieal Departments, Neurological Institute of Milan
Intracranial Neurinomas of the Nerves of the Jugular Foramen Report
o f 12 P e r s o n a l
Cases
By
F. Pluchino, G. Crivelli, and M. A. Yaghi ~u
7 Figures
Summary 12 patients, 10 women and 2 men, were operated on for removal of a jugular foramen neurinoma. Removal was total in 11 cases and operative mortality was 16.6~ As there are no clinical or instrumental signs that permit sure distinction between these tumours and those of the cerebellopontine angle, only a thorough radiologieal study of the jugular foramen allows correct diagnosis. Often, however, the diagnosis is made during the operation. Key words: Neurinoma--jugular logical diagnosis.
foramen
clinical diagnosis---radio-
Neurinomas of the intraeranial segment of the glossopharyngeal, vagus and accessory nerves, also called neurinomas of the jugular foramen nerves, are very rare t u m o u r s (Hensehen 1955), so m u c h so t h a t in all only 27 eases have been described in the literature, 4 of t h e m necropsy findings (Gerhardt 1878, Gierlieh 1908, Henschen 1955, Russel et al. 1959). Of the 23 cases operated on only t h a t of Pool and P a v a (1957) and the 3 cases of Revilla (1947) form part of large surgical series of neurinomas of the eerebellopontine angle, the m a j o r i t y of authors reporting isolated cases, m o s t l y single (Cohen 1937, Columella et al. 1959, De Martel et M. 1932, Fishgold et al. 1954, Giordano 1968, Gra~ 1952, Guillain et M. 1933, Mansuy et al. 1964, N a u n t o n et al. 1968, P o r t u 1954), oce~sionMly more t h a n one (Columell~ et aI. 1959, Svien et al. 1963, R u b e r t i and Carteri 1961, with 2, 3 and 4 cases respectively). Acta Neurochirurgica, Vo].31, Fasc. 3--4
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Intrigued by the rarity of these tumours, we decided to check through the surgical files of the Neurological Institute of Milan to find their frequency in our material. We found t h a t between 1946 and 1971 alongside over 400 turnouts of various types of the cerebellopontine angle (including 238 neurinomas) 12 neurinomas of the jugular foramen were operated on and checked histologically. After reviewing these cases and the da~a of the literature (Cohen 1937, De Martel et al. 1932, Guillain et al. 1933, Mansuy et al. 1964, Pool et al. 1957, Porta 1954, Revilla 1947), we have come to the conclusion t h a t a precise preoperative diagnosis of these tumours is difficult. The published case reports of jugular foramen neurinomas commonly describe a cerebellar and vestibular hemisyudrome on the side of the tumour, intracranial hypertension, lesions of the third, fourth, fifth, sixth, seventh, eighth, ninth, tenth, eleventh and twelth cranial nerves (Cohen 1937, Columella et al. 1959, Columella et al. 1959, De Martel et al. 1932, Graf 1952, Guillain 1933, Mansuy et al. 1964, Naunton et al. 1968, Pool et al. 1957, Por~a 1954, Revilla 1947, Ruberti et al. 1961, Svien et al. 1963). Especially frequent are lesions of the last four cranial nerves, total or severe deafness is frequent, lesions of the trigeminat or facial nerves less so or less evident. According to some workers (Columella et al. 1959, De Martel et al. 1932, Mansuy et al. 1964, Ruberti et al. 1961), paralysis or paresis of a vocal cord associated with weakness of the trapezius and sternocleidomastoid muscles and homolateral nuchal headache are of great diagnostic value; glossopharyngeal neuralgia m a y be present (Cohen 1937); in rare cases hearing is normal (Mansuy et al. 1964). None of the cases described in the literature seem to have been diagnosed preoperatively, partly because there are no instrumental signs t h a t differentiate these tumours from neurinomas of the acoustic nerve (Giordano 1968, Naunton et al. 1968). Hence preoperative diagnosis is chiefly radiological, and since investigations with contrast media and gammaencephalography indicate a space-occupying lesion of the cerebellopontine angle in these cases, the most reliable data come, in our view, from the skull X-rays using special views for the jugular foramen.
Radiology of Neurinomas Of the Jugular Foramen The views useful for this purpose are the symmetrical bilateral and the unilateral oblique views. Tomography m a y be used in either case. The standard basal projection does not give a good view of the jugular foramen unless t o m o g r a p h y is used (Fig. 1). Some workers describe modifications of the basal view, which m a y be symmetrical bilateral (Fig. 2) or oblique unilateral (Decker 1968, Eraso 1961, Giraud et al. 1961). Others describe techniques for transoral symmetrical bilateral
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Fig. t.. Tomogram of normal jugular foramina (arrows) in the basal view
Fig. 2. Normal jugular foramina (arrows) in the symmetrical bilateral projection according to Eraso's technique (see text and references)
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projections (Fig. 3) or oblique unilateral projections (Chausse 1950, Seuk K y K i m et al. 1966, Strickler 1966). The most well-known of the oblique unilateral views is Rossand's transmaxillary projection (Porcher et al. 1952) (Fig. 4), which we use routinely. The jugular foramen presents a pars nervosa and a pars vascularis: a fibrous (or less often bony) septum which stretches between the jugular spine of the petrous bone and the jugular process of the occipital
Fig. 3. Normal jugular foramina (arrows) in the transoral symmetrical projection (Seuk Ky Kim et al. 1966, Strickler 1966) bone separates the posterior and lateral portions of the pars vascularis (Fig. 5). Normal variations in size were described by Di Chiro (1964), who tabled them, including the values reported by other workers. The jugular foramina are generally asymmetrical both in shape and size; more often t h a n not the right is larger than the left. According to the observations of Di Chiro (1964), the difference in size is attributable to the pars vascularis. An alteration of the jugular foramen m a y be secondary to an intrinsic lesion, t h a t is to a lesion of the structures within the foramen itself, or, more rarely, to an extrinsic lesion, i.e. to a lesion of contiguous structures. The most frequent intrinsic lesions are glomns jugulare tumours (chemodectomas or non chromaffin parangiomas). Neurinomas of the ninth, tenth and eleventh nerves are very rare.
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Fig. l. Normal jugular foramina (arrows) in Rossand's unilateral oblique projection (Percher et al. 1952) An erosion with blurred and irregular edges t h a t at first affects mainly the pars vascularis is characteristic of a glomus jugulare tumour; the search for other bone snd angiographic signs t h a t will clinch the diagnosis is m a n d a t o r y (Hawkins 1961, Rice et al. 1963). A grow-th chiefly affecting the pars nervosa with clearcut and often thickened edges, erosion of the jugular process and sometimes widening
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of the petro-oecipital fissure should suggest a neurinoma of the ninth, ten*h and eleventh nerves. Extrinsie lesions affeeting the jugular foramen may be: primary or metastatie turnouts of contiguous bone structures, rhinopharyngeal
Fig, 5. Basal tomogram: a pars nervosa and glossopharyngeal (IX) nerve; b pars vaseularis and vagus nerve (X), accessory nerve (XI), ~ internal jugular vein; e jugular process of the petrous bone tumom's or meningiomas of the petrous ridge (Hawkins 1961). These tumours are responsible for erosion with somewhat blurred and irregular edges and m a y be difficult to distinguish from advanced glomus jugulare tumours (Di Chiro et al. 1964, Hawkins 1961, l~ice et al. 1963, Rueker 1963). Intrapetrous eholesteatomas m a y also affect the jugular foramen; they are generally described as well-defined areas with eleareut margins in the region of the attic and of the mastoid antrum (Hawkins 1961).
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Case-Material
A. Age, Sex, and Side Of the 12 patients 10 were women and 2 men; the age range was 20 to 60 years with a peak in the sixth decade (5 cases); in 7 cases the t u m o u r was on the right and in 5 on the left side. The salient clinical, radiological and surgical data on these 12 patients are summarized in the table.
B. Symptoms The length of history varied widely: in one patient (case 8) as much as ten years before admission, in 2 (cases 2 and 3) seven years, in 5 (cases 1, 6, 9, 10, 11) two years, in 1 (case 12) about a year and in 3 (cases 4, 5, 7) less t h a n a year. I n 5 eases the first s y m p t o m s were hearing disturbances (cases 2, 3, 5, 8, 10), in 3 (cases 6, 9, 12) giddiness and balance disturbances, in 2 headache (4, 11) and in 2 (eases 1, 7) ocular disturbances; in no ease was the first s y m p t o m impairment of one of the nerves of the jugular foramen. Of the 5 patients whose first s y m p t o m s were hearing disturbances progressing to deafness, 4 (eases 3, 5, 8, 10) had the hearing loss on the same side as the turnout and 1 (ease 2) on both sides. I n eases 2 and 3 the hearing defect commenced five or six years before the onset of an intracranial hypertension syndrome and in ease 8 as much as nine years before the rapid decrease in visual acuity of the right eye, t h a t is the s y m p t o m s t h a t led to hospitalization. The interval between the onset of s y m p t o m s and hospitalization was shorter, viz. between 8 months and 2 years, in the other two patients of this group. On admission the first had intermittent double vision on looking to the right, dysphagia for fluids and mild dysarthria (ease 5) and the other had balance disturbances and an intracranial hypertension syndrome (ease 10). Of the 3 patients whose first s y m p t o m s were giddiness and balance disturbances 2 complained of severe unilateral hearing trouble (eases 6 and 9) and one of total deafness on the same side, as the turnout occasional diplopia and mild dysarthria (case 12). I n the 2 cases in which the first s y m p t o m was headache, this was oppressive, continuous or intermittent. I n one patient this was followed four m o n t h s later b y paresis of the rectus lateralis oculi muscle on both sides, t h o u g h more marked on the left, t h a t is homolateral, as was subsequently found, to the lesion, and ataxia of the lower limbs, which later prevented her from walking (ease 4); in the other headache was followed b y giddiness, equilibrium disturbances, diminished hearing on the right side and total deafness on the left (ease 11). Of the two patients who started with visual disturbances, one (case i)
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had scintillating scotoma in both eyes followed, one year later, by attacks of violent horizontal nystagmus in rapid succession and by giddiness and subjective loss of hearing before admission; the other (case 7) had reduced visual acuity on both sides, but more on the tumour side, followed by amenorrhea. 4 patients had late symptoms (appearing 10 to 20 months after the onset of the disease) indicating impairment of the lower cranial nerves: one (case 5) had dysphagia for fluids and mild dysarthria, one (case 6) dysphagia for fluids and solids and dysphonia whilst 2 (cases 4 and 12) had only mild dysarthria. I n the period immediately preceding hospitalization 3 patients (cases 1, 3, 6) had psychic disturbances in the shape of mental confusion, disorientation in time and space, abulia and memory defects; in only one case (9) did the patient notice facial paralysis. One patient had bilateral hyposmia (case 3) followed b y anosmia, which came on six and a half years after the first symptom.
C. _Neurological and Instrumental Signs In one case (3) neurological examination elicited bilateral anosmia. 8 patients had ocular signs: one patient had anisocoria only, the pupil on the tumour side predominating (case 12); 7 had papilloedema, always associated with other signs: in 2 (cases 3, 7) with decreased visual acuity and field defects, in 2 with decreased visual acuity (cases 8, 10), in 2 with defects of the extrinsic muscles of the eyes, t h a t is, in case 4 with bilateral paresis of the sixth nerves, more marked on the tumour side, and with paresis of the third nerve on the turnout side; in case 5 with paresis of the sixth nerve on the turnout side; lastly, in one patient the choked disk was associated with anisocoria, the pupil on the eontralateral side predominating. 10 patients had defective trigeminal sensation on the tumour side: 2 had hypoesthesia throughout the distribution (cases 8 and 12), 2 had hypoesthesia confined to the first branch (cases 3 and 10), 1 had hypoesthesia confined to the second branch (case 6), 5 had only diminished or absent corneal reflexes (cases 2, 4, 5, 7 and 11), this s y m p t o m did not appear in case 7 until fifteen days after admission. Facial movements were impaired in 6 patients: in 4 cases (1, 4, 10, and 12) the impairment was confined to movements of the lower half of the face omolateral to the tumour, in 2 to weakness of the orbieularis oculi muscle, bilateral in case 5 and omolateral in case 6. Severe loss of hearing on the side of the turnout was proven in all 12 patients and audiometry showed total deafness in 8 (cases 4, 5, 6, 8, 9, 10, 11, 12); in cases 1, 2, 3, and 7 there was marked hearing loss of the
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perceptive or mixed type. I n only one ease (7) were there no clinical signs of vestibular distress; of the other l l patients, 9 were subject to severe attacks of giddiness b r o u g h t on b y m o v e m e n t s of the head and eyes whereas 2 suffered from less severe b u t continuous giddiness; in 9 patients the deviation of the t r u n k in the erect position or while walking with the arms outstretched was t o w a r d the side of the lesion (cases 2, 3, 4, 5, 6, 8, 10, 11, 12) and in 2 t o w a r d the opposite side (cases 1 and 9). Caloric vestibular examination was done in all but one patient (case 7): it in one patient (case 1) induced a spontaneous horizontal n y s t a g m u s and in the other 10 patients multiple spontaneous n y s t a g m u s on the n o n - t u m o u r side. I n all 11 patients the verdict of the vestibular examination was a cerebellopontine angle syndrome with m a r k e d signs of brainstem involvement, as seen in large acoustic nerve neurinomas (cases 2, 5, 10, 11, 12). Clinical evidence of damage to the lower cranial nerves was found in 8 patients. These signs are shown in the following table: Table 1
Case Pharyngeal Palatal Dysphonia Dysarthria reflex reflex 3 4 5 6 7 9 ll 12
§ unilat. z_ unilat. @ bilat. @ bilat. § unilat. § unilat.
SwMlowing disturb- Hypotrophy of tongue anees
§ § --§
bilat. bilat,. unilat. unilat.
§ -t-
§ §
+
§ § § §
T
I n all cases in which the s y m p t o m s were unilateral t h e y related to the t u m o u r side. 8 patients had a cerebellar hemisyndrome oll the side of the t u m o u r of varying severity, characterized b y dysmetria, ataxia, adiadochokinesia, asynergia, h y p o t o n i a and tremor (cases 2, 3, 4, 5, 6, 81 10, and 11); 5 of t h e m were incapable of remaining in the erect position because of falling backward and to the side (cases 2, 3, 4, 5, and 8). A B a b i n s k y reflex was elicited in 4 cases: bilateral in case 5, on the side controlateral to the turnout in ease 4, omolateral in case 9, bilateral and associated with omolateraly increased m y o t a t i c reflexes in case 10. I n case 12 there were only increased m y o t a t i c reflexes on the side of the t u m o u r .
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D. Radiological Signs a) Secondary sella. Changes in the sella turcica were found in only 4 out of the 12 cases. The pattern varied from slight decalcification of the dorsum sellae to extensive destruction of both dorsum and floor.
Fig. 6. Case 4. Neurinoma of the left jugular foramen. L concentric enlargement of the left jugular foramen and destruction of the jugular process. R normal right jugular foramen b) Widening o/the jugular ]ora~nen. I n 4 patients (cases 3, 4, 6, 11) studied ~4th special projections and/or tomography the lesion had a characteristic appearance. The diagnostic features chiefly involved the pars nervosa, which showed a clearcut and at times thickened margin also destruction of the interjugular process (Fig. 6). I n cases 3, 4, and 11 the lesion was such as to cause major erosion with sharp edges in the middle and lower portions of the petrous bone, clearly visible in the anteroposterior tomogram. In case 11 the bone lesion extended posteriorly as far as the edge of the foramen magnum (Fig. 7).
IntraeraniM Neurinomas of the Nerves of tile Jugular Foramen
211
Fig. 7. Uase 11. Neurinoma of the right jugular foramen. Cleareut erosion of the right jugular foramen extending as far as the contour of the great, oeeipitM foramen and affecting the petrous bone. A tomogram in the basal view ; B tomogram in tho frontal view I n 2 oases (5 a n d 10) the s t u d y of the base of the skull was confined to the basal projection, which revealed only a n a s y m m e t r y of size of the j u g u l a r f o r a m e n t h a t could be t e r m e d borderline normal. I n ease 12, studied tomographieMly, the j u g u l a r f o r a m i n a were within n o r m a l limits. I n the other 5 eases there was no view of the j u g u l a r f o r a m e n region.
212
F. Pluchino e~ al. :
c) Air studies. Pneumoencephalography was performed in only 4 cases; in one of these, because the air failed to enter the ventricular system, a ventrieulogram was also done (case 2). The air study of case 1 showed the pattern of an expanding lesion of the left cerebellopontine angle t h a t occupied the corresponding cistern causing contralateral displacement of the fourth ventricle and aqueduct and distension of the homolateral cisterna ambiens. In case 2 the air study b y lumbar route yielded generic evidence of a space-occupying lesion of the posterior fossa with hydrocephalus above; ventriculography showed triventricular hydrocephalus and blockade of the aqueduct in the neighbourhood of the third ventricle with no lateralising signs. The air study of case 7 showed a space-occupying lesion t h a t displaced the vallecula and fourth ventricle, leaving its imprint on the right contour, and hydrocephalus of all four ventricles. I n case 9 the cistern of the right cerebellopontine angle was not injected and there was hydrocephalus of all four ventricles. d) Angiographicstudies. 5 cases were studied angiographically, 4 with retrograde injection via the left arm and 1 via the left. Cases 5 and 11 presented the typical pattern of an extra-axial tumour of the right cerebellopontine angle. The prepontine and lateropontine portions of the right superior cerebellar artery were raised and pushed toward the midline. In the lateral view the right superior cerebellar artery was raised and stretched. Cases 7 and 8 presented no appreciable vessel displacements, although in case 8 fine arterial branches disposed in an are seemed to circumscribe a tumour in the territory of the inferior posterior cerebellar artery. This finding has been described as characteristic of other types of cerebellopontine angle tumours, especially of acoustic neurinomas 15, ~a, 35 I n case 12 the 6nly sign t h a t angiography supplied was a backward displacement of the tonsillar circulation, assessed according to its projection on the Twining line (Leman et al. 1967). This finding was present in the other four cases as well. e) Radioisotope scanning. Only 4 patients (cases 8, 9, 10, and 12) were subjected to gammaencephalography: 2 with ~0aHg, one with 99mTc and one with both of these isotopes. In all there was a hot area in the tumour site. In conclusion, in 4 of our patients operation was performed on the basis of a correct clinical and radiological diagnosis, thanks to the demonstration of a typical alteration of the jugular foramen (cases 3, 4, 6, and 11). For 2 patients (eases 2 and 7) the diagnosis was a posterior fossa space-occupying lesion; in these eases the jugular foramen was not
IntraeraniM Neurinomas of the Nerves of the Jugular Foramen
213
studied and arteriography and encephalography did not supply a more precise site. I n the other 6 patients the operation was performed on the diagnosis of acoustic nerve nenrinoma: for cases 1, 9, and 5 the diagnosis was based on the nenroradiologieal investigations (angiography or pneumoeneephalography); for cases 8 and 12 it was based on angiography and brain scans; for case 10 the diagnosis was based on clinical examination and brain scans. I n 3 of these cases (1, 8, 9) the jugular foramen was not studied radiologically, in 2 (5, 10) insufficiently and in case 12 the tomographie findings were normal.
E. Surgical Treatment All the patients were operated on in the prone position using general anaesthesia with controlled respiration. I n 2 patients (eases 2 and 7) a suboceipital bimastoid craniectomy was performed, in the other 10 eases a suboeeipital hemicraniectomy. I n 11 eases the turnout was exposed by retracting the eerebellar hemisphere without need for a m p u t a t i o n ; in one case only the homolateral cerebellar tonsil had to be a m p u t a t e d because it had herniated into the occipital foramen (case 2). The approximate size and the macroscopieal structure of the tumours are given in Table 2. I n one ease it m a y be definitely said t h a t the t u m o u r was attached to the glossopharyngeal nerve (ease 7), in another on the vagus (ease 9) and in another on the accessory nerve (ease 11). I n the other 9 patients it was impossible to say to which of the jugular foramen nerves the t u m o u r had been attached. The lower cranial nerves were spared in toto in 11 eases, whereas in ease 1 t h e y were extensively d a m a g e d during the manoeuvers to dissect the turnout capsule from the border of the jugular foramen, to which it adhered tightly. Of the 11 cases in which it was possible to isolate the acoustic and facial nerves from the tumour, only one suffered circumscribed damage to the facial nerve (case 3); in 5 patients (cases 2, 3, 4, 10, and 11) the turnout had grown into the enlarged jugular foramen, where it was nesting, and in case 11 there was an extraeranial projection of the turnout t h a t came into contact with the terminal portion of the vertebral artery; these outgrowths were completely removed in every ease. I n 9 cases there were adhesions between the t u m o u r and the brainstem (eases 1, 2, 4, 5, 6, 7, 8, 9 and ll), which in 5 cases (2, 4, 5, 6, and 8) were rather close and which in ease 2 were associated with severe distortion of the medulla oblongata due to herniation of the turnout t h r o u g h the foramen m a g n u m .
214
F . P l u c h i n o e t al. :
Table Clinical signs Case No.
Age
Sex
Side
Duration of symptoms on admission
Cranial nerves damaged
Papillary oedema
1 c,cl, 24,809
36
f
it.
2 years
VII
2 e.cl. 26,290
50
f
ft.
7 years
II
3 c.ci. 30,958
52
f
It.
7 years
I II VIII
4 c.cl. 31,911
58
f
lt.
10 months
II VI IX
5 c.cl. 40,193
20
m
rt.
7 months
II V YI VII VIII IX X XII
6 e.e]. 44,875
58
f
it.
2 years
V IX
VII VIII X
7 e.cl. 50,750
27
f
rt,
8 months
II IX
V X
VIII
present
8 c.cl. 51,020
60
f
ft.
10 years
II
V
VIII
present
9
41
f
rt.
20 months
VIII IX
X
10 c.d. 54,840
30
f
lt.
2 years
II V VIII
VII
11 e.e], 55,227
42
f
rt.
2 yeats
V VIII XI XII
12 e.cl. 56,306
47
m
rt.
1 year
III V VIII
c.cL 52,642
Cerebellar signs
Radiologica:
Pyramidal signs
Secondary sella
VIII
III V VIII
V IX
III V VII VIII XII
VII XII
Plain X - r a y of the jugular foramen
n o t studied
present
ataxia asynergia
n o t studied
present
ataxia hypotonia
enlarged with clcarcut margin~
present
hypotonia
right Babinski
present
ataxia hypotonia asynergia
bilateral Babinski
hypotonia
present
hypotonia
ataxia hypotonia
present
enlarged with clearcut m a r g i m
inadequate study
present
enlarged with clearcut margin~
present
not studied
present
no~ studied
right Babinski
n o t studied
bilateral Babinski increased reflexes on left
inadequate study
asynergia hypotonia
enlarged w i t h clearcut margini
increased reflexes on r i g h t
normal
Intra,cranial
Neurinomas
of the Nerves
of the Jugular
signs
Foramen
215
Surgical findings
Plain X-ray PEG or ventribones adjacent to jugular culography foramen diagnosis
Arteriography diagnosis
Gamma encephalography
Preoperative diagnosis
Tumour texture and size
Nerves to which it adhered
Result
left cerebello pontine angle space occupying lesion
C.P. angle tumour
solid IX X XI tangerine
living ]8 months after operation
posterior fossa space occupying lesion
posterior fossa tunmur
solied IX X X I tangerine
died 36 hours after operation from haemorrhagie itlfarction of medulla oblonga~a proven at necropsy
lesion of the middle-lower p e r s o n of the petrous bone
jugular foramen neurinoma
solid apricot
IX X XI
perfectly normal life 10 years after operation
lesion of the mlddie-lower p e r s o n of the petrous bone
jugular foramen neurinolua
cystic apricot
I X X XI
perfectly normal life 2 years after operation
C.P. angle tumour
cystic IX X XI hen's egg
perfectly normal life 4 years after operation
jugular foramen neurinoma
solid aprico t
perfectly normal life 2 years after operation
posterior fossa tumour
solid apricot
spaceoccnpying right cerebello pontine angle lesion
posterior fossa inadequate space occupying study lesion pathological circulation in right cerebello pontine angle right cerebello Dentine angle space occupying lesion
lesions of the medial ~ of the petrous bone and occipital condyle
space occupying right cerebello pontine angle lesion inadequate study
IX X XI
IX
perfectly normal life 2 years after operation
accumulation C.P. angle Hg 203 in tmnour right C.P. angle
solid walnut,
IX X XI
died on 6th day no necropsy
accumulation in right C.P. angle
C.P. angle turnout
solid walnut
X
perfectly normal life 18 months after operation
accumulation Hg 203 and Tc 99 m in left C.P. angle
C.P. angle tumour
cystic apricot
IX X XI
perfectly normal life 1 year after operation
jugular foramen neurinoma
solid apricot,
XI
perfectly normal life 1 year after operation
aecumuiation C.P. angle Tc 99 m in tunmur right C.P. angle
cystic I X X X[ hen's egg
perfectly normal life 4 months after operation
216
F. Pluchino e~ al. :
The tumour was removed completely in all cases but one, in which, because of the tight adhesions between the anterior superior pole of the neurinoma and the acoustic-facial complex, intracapsular removal was performed to avoid injuring the right facial nerve and so causing facial diplegia in a patient who already had eontralateral peripheral paralysis of the seventh nerve for other reasons (case 9).
F. Postoperative Course and Results One patient remained conscious and lucid for the first 24 hours after the operation but t~hen went into coma and died 12 hours later (case 1): necropsy revealed a haemorrhagic infarct extending from the dentate nueleus as far as the lower portion of the superior cerebellar peduncle on the same side as the turnout. Another patient died on the sixth d a y after several serious gastric haemorrhages and permission for necropsy was not granted (case 8). 6 patients had an uneventful postoperative course (cases 3, 5, 7, 10, 11, 12) but in 2 cases (3,s~ 7) corneal anaesthesia necessitated partial tarsorrhaphy and in case 3 a peripheral facial paresis appeared after the operation. 4 patients (cases 2, 4, 6, and 9) had a stormy postoperative course. 3 patients had bronchopneumonia, which was slow to clear (cases 2, 4, and 6), and had to be fed by nasal tube for a long time; one patient (case 4) had a severe gastric haemorrhage on the fourth d a y and another (ease 6) had to undergo traeheostomy; ease 9 had late respiratory insufficiency necessitating traeheostomy and mechanical ventilation for ten days. These ten patients were discharged in good or fair general and neurological condition and all were leading a normal life after an interval ranging from four months to ten years from operation.
Discussion I n the few cases of jugular foramen neurinoma reported in the literature the patients are usually described as having a cerebellar and vestibular hemisyndrome omolaterM to the tumour, intracraniM hypertension and lesions of the third to twelfth cranial nerves (Cohen 1937, Columella et al. 1959, De Martel et al. 1932, Graf 1952, Guillain 1933, Mansuy et al. 1964, Naunton et al. 1968, Pool et al. 1957, Porta 1954, I~evilla 1947, Ruberti et al. 1961, Svien et al. 1963): especially frequent are lesions of the last four cranial nerves, total or severe deafness is frequent, lesions of the trigeminal and facial nerves are less frequent or less marked. According to some (Columella et al. 1959, De Martel et al. 1932 ; Mansuy et al. 1964, l~uberti et al. 1961), paralysis or paresis of a vocal cord associated with weakness of the trapezius and sternocleido-
Intraeranial Neurinomas of the Nerves of the Jugular Foramen
217
mastoid muscles and homolateral nuehal headache are of high diagnostic value; in some cases glossopharyngeal neuralgia m a y be present (Cohen 1937); in rare cases hearing is normal (Mansuy c t a l . 1964). On our evidence and that of other workers there seems to be no characteristic syndrome of neurinomas of the jugular foramen because these tumours very often give signs similar to those of acoustic neurinomas. In fact, in all our patients the acoustic nerve was damaged, even when the tumour was not very large (ease 8), so that there would seem to be justification for Naunton et al.'s (1968) assertion that from the otovestibular point, of view acoustic neurinomas cannot be distinguished from those of the jugular foramen. Furthermore, 4 of our patients (cases 1, 2, 8, 10) wesented without any impairment of the lower cranial nerves and in this connexion it is noteworthy that the accessory nerve was not damaged in any of our patients, not even in case ll, in which the turnout adhered closely to this nerve, from which it seemed to have originated. If we add that acoustic neurinomas may induce marked signs of damage of the lower cranial nerves, it is clear that the symptoms of such tumours closely resemble those of the jugular foramen. The only relatively reliable criterion is that in neurinomas of the jugular foramen the signs of trigeminal and facial nerve damage are later and/or milder than in acoustic neurinomas. However, this clue is not always available and so is far from sure. I n our experience a correct diagnosis can be made only in cases in which the clinical symptoms are accompanied by a bone change of the jugular foramen, which can be demonstrated by a thorough X-ray examination. The other neuroradiologieal investigations generally permit a generic diagnosis of a space-occupying lesion of the eerebellopontine angle. Intact jugular foramina do not, however, rule out the possibility of a neurinoma of the lowest cranial nerves (ease 12). None of our eases presented bone changes of the internal acoustic meatus. Naunton (1968) described changes in the appearance of the internal auditory foramen in a ease of neurinoma of the eleventh nerve, whilst Di Chiro (1964) found an abnormal enlargement of the jugular foramen in a case of neurinoma of the eighth nerve. The upshot of our findings is that the usual views of the skull are not sufficient for the radiologieal detection of intraeranial tumours of the jugular foramen and that the region must be studied with elective techniques. Moreover, since the clinical signs of these tumours often closely resemble those of acoustic neurinomas, it would be no bad thing for the jugular foramen region to be examined radiologieally in all patients with signs of damage to the lowest cranial nerves. From the surgical point, of view knowing that the turnout is a neurinoma of the jugular foramen rather than an unspecified tumour of the Acta NcurocMrurgiea, Vol. 31, Fase. 3--4
15
218
F. Pluchino et al. :
cerebellopontine angle does not alter the surgical approach but it holds out greater hope in the first case of sparing the facial nerve, which is of some importance to the patient. Of the 23 operated cases described in the literature one was only explored (Pool et al. 1957) and one underwent removal of unspecified extent (Giordano 1968); 10 underwent partial or subtotal removal of the tumour (De Martel 1932, Guillain et al. 1933, Cohen 1937, Graf 1937, Porta 1954, 3 cases of Columella 1959, cases 2 and 4 of l~uberti and Carteri 1961) with only one death (Guillain 1933); 11 patients underwent apparently total removal of the tumour (Fischgold 1954, Mansuy et al. 1964, Naunton 1968, cases 1 and 3 of Ruberti and Carteri I961, 3 cases of Svien et al. 1963, 3 cases of Revilla 1947) and all the patients of this group survived the operation. The mortality in the literature is thus 10% for partial removal and zero for total removal. In our series (11 radical operations and 1 subtotal removal with 2 deaths) both the operative deaths occurred in radical operations and hence the mortality is 16.6%, or 18% if referred to the radical operations only. Our cases together with those of the literature make a total of 33 operations with a mortality of 9.1%, whether referring to the partial removals (11 operations, 1 death), to the radical removals (22 operations, 2 deaths) or to the total (33 operations, 3 deaths). In view of this, radical removal would seem to us to be the right course unless the surgeon considers the risk too great on grounds of age, general condition or anatomical condition discovered at operation (as in our case 9). As with neurinomas of the acoustic nerve, amputation of the outer third of the cerebellar hemisphere is in our view almost always unnecessary, simple retraction being usually sufficient especially if preceded by the administration of osmotics or b y ventricular puncture; damage to the seventh, eighth, ninth, tenth and eleventh nerves, rare and usually circumscribed but possible during the removal of very large neurinomas, can be avoided by a precise preoperative diagnosis and the use of an operating microscope. Conclusions This study and previous reports in the literature prompt the following conclusions : 1. E x a c t preoperative diagnosis of neurinomas of the jugular foramen, especially differential diagnosis from acoustic neurinomas, is difficult since the only clinical pointer is paralysis or paresis, which m a y be mild, of the lower cranial nerves, which is found more frequently t h a n in acoustic neurinoma, whereas loss of facial sensation and movement are found less often.
Intraeranial Neurinomas of the Nerves of the Jugular F o r a m e n
219
2. H e n c e a eerebellopontine angle s y n d r o m e in which t h e trigeminM a n d facial n e r v e lesions are slight a n d lower cranial nerve lesions are m a r k e d or a n y w a y p r e s e n t should s y s t e m a t i c a l l y suggest n e u r i n o m a of t h e j u g u l a r f o r a m e n a n d p r o m p t t h o r o u g h X - r a y e x a m i n a t i o n of t h e base of t h e skull w i t h special views for t h e j u g u l a r foramen, because X - r a y c o n f i r m a t i o n in t h e shape of a eleareut widening of t h e j u g u l a r foramen, chiefly in t h e p a r s nervosa, clinches t h e diagnosis in t y p i c a l eases. O t h e r neuroradiological investigations are useful because t h e y s u p p l y i n f o r m a t i o n on t h e size of t h e t u m o u r a n d its connexions with i n t r a e r a n i a l structures. 3. T h e results of surgical t r e a t m e n t are satisfactory, w i t h a m o r t a l i t y of 16.6~o on t h e basis of our 12 o p e r a t i o n s a n d 9.1~ on t h e basis of t h e cases in t h e l i t e r a t u r e . As for acoustic neurinomas, r e m o v a l m u s t be radical, e x c e p t in special eases. A n a c c u r a t e p r e o p e r a t i v e diagnosis is v a l u a b l e because it enables t h e surgeon to p l a n t h e o p e r a t i o n in such a w a y as to spare t h e acoustic-facial complex c o m p l e t e l y or nearly, even in t h e presence of v e r y large t u m o u r s , like those of our eases.
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33. Striekler, J. M., New and simple techniques for demonstration of the jugular foramen. Amer. J. Roentgenol. 97 (1966), 601--606. 34. Svien, H. J., H. L. Baker, and M. H. Rivers, Jugular foramen syndrome and allied syndromes. Neurology 13 (1963), 797--809. 35. Takahashi, M., G. Wilson, and S. Hanafee, The anterior inferior eerebellar artery: its radiographic a n a t o m y and significance in the diagnosis of extra-axial tumors of posterior fossa. Radiology 90 (1968), 281---287. Authors' address: Dr. F. Pluehino, Dr. G. Crivelli, Neurosurgieal Department, Dr. M. A. Vaghi, Neuroradiologieal Department, I s t i t u t o Neurologico di Milano, Via Giovanni Celoria, i1, 1-20133 Milano, Italy.
