J Neurosurg 88:962–968, 1998

Basilar invagination: a study based on 190 surgically treated

patients

ATUL GOEL, M.CH., MOHINISH BHATJIWALE, M.S., M.CH., AND KETAN DESAI, M.S., M.CH.
Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical
College, Parel, Bombay, India

Object. The authors analyzed the cases of 190 patients with basilar invagination that was diagnosed on the basis of
criteria laid down in 1939 by Chamberlain to assess the appropriate surgical procedure.
Methods. Depending on the association with Chiari malformation, the anomaly of basilar invagination was classified
into two groups. Eighty-eight patients who had basilar invagination but no associated Chiari malformation were
assigned to Group I; the remainder of the patients, who had both basilar invagination and Chiari malformation, were
assigned to Group II. The principal pathological characteristic was observed to be direct brainstem compression due to
odontoid process indentation in Group I and a reduction in posterior cranial fossa volume in Group II.
Conclusions. Despite the anterior concavity of the brainstem in both groups, transoral surgery was the most suitable
procedure for those patients in Group I and decompression of the foramen magnum was found to be appropriate for
patients in Group II. After surgical decompression, a fixation procedure was found to be necessary in most Group I
cases, but only in a small minority of Group II cases.

KEY WORDS • basilar invagination • syringomyelia • Chiari malformation •

craniovertebral anomaly

invagination, a primary developmental
B
ASILAR
anomaly,19,45 has been a subject of clinical interest
for some time.4,5,7,14,20,22,26,28,29,31,32,40,45–47 Lessons
learned during the management of 190 cases of basilar
invagination are analyzed in this report.
Clinical Material and Methods
Approximately 1800 patients with craniovertebral
anomalies were treated in the neurosurgery department at
King Edward Memorial Hospital between 1950 and 1996.
In the present study 190 cases of patients with basilar
invagination who were surgically treated between January
1987 and March 1997 have been reviewed and analyzed.
Analysis of cases treated before 1994 was retrospective;
analysis of cases treated after that time was prospective.
Included in the study were only those patients in whom
magnetic resonance (MR) imaging and/or computerized
tomography (CT) scanning in addition to conventional
radiography were performed. Magnetic resonance imag-
ing was available in 138 cases and CT scanning in 108
cases. In accordance with the criteria for diagnosis of basi-
lar invagination, the tip of the odontoid process was at
least 2.5 mm above Chamberlain’s line4 in each case. De-
pending on the presence or absence of Chiari malforma-
tion, the series was divided into two groups. Group I was
composed of 88 patients without associated Chiari mal-
formation (Fig. 1); Group II was composed of 102 patients
with associated Chiari malformation (Fig. 2). Follow-up
review ranged from 2 months to 9 years (average 43
months). Sixteen patients did not report for any follow-up
examination after discharge from the hospital and they
were evaluated on the basis of their hospital stay. Six pa-
tients communicated only through letters informing us of
their clinical condition.
Results
The clinical features of the cases are presented in Table
1. Trauma of varying severity was the principle precipitat-
ing factor in 48% of the Group I cases. In no case in Group
II did trauma play any role in initiating the symptoms.
Infections probably precipitated events in four patients in
Group I. Of these, one patient had cervical tuberculous
lymphadenitis, whereas the other three had chronic respi-
ratory tract infections.
Radiographic Studies
Measurements of structures based on radiological stud-
ies are shown in Table 2. Measurements were made on all
available studies and were averaged. The landmarks used
for the measurements of various indices were based on a
review of original papers by VanGilder, et al.,44 and are

962 J. Neurosurg. / Volume 88 / June, 1998

Basilar invagination

TABLE 1
Clinical characteristics of 190 patients surgically treated for
basilar invagination*
Group I Group II
Characteristic No. (%) No. (%)

total no. of patients 88 102

age (yrs)
0–10 13 (15) 3 (3)
11–20 51 (58) 20 (20)
21–30 11 (13) 45 (44)
31–40 7 (8) 24 (24)
41–50 4 (5) 8 (8)
51–60 2 (2) 2 (2)
duration of symptoms
6–12 mos 20 (23) 14 (14)
FIG. 1. Magnetic resonance image obtained in an 8-year-old boy 13–24 mos 11 (13) 33 (32)
with Group I basilar invagination. 25–36 mos 2 (2) 7 (7)
37–48 mos 0 (0) 8 (8)
49–60 mos 2 (2) 12 (12)
61 mos–15 yrs 6 (7) 16 (16)
shown in Fig. 3. On MR imaging, the Klaus height index22 presenting symptoms
was measured from the tip of the odontoid process to a neck pain 52 (59) 11 (11)
line drawn along the tentorium. The omega angle was hoarseness or nasal regurgitation 8 (9) 16 (16)
modified as shown in Fig. 4. The line of the palate was paresthesia 22 (25) 81 (79)
weakness 88 (100) 96 (94)
taken as a fixed parameter and a parallel line was drawn ataxia 0 (0) 48 (47)
from it, passing from the center of the base of the axis. The bowel & bladder disturbance 25 (28) 14 (14)
angle of the odontoid process was measured on this line. finding on CNS examination
Syringomyelia was present only in Group II; a syrinx was only posterior column 34 (39) 12 (12)
identified in 51 (50%) of these cases. Occipitalization of only spinothalamic tract 7 (8) 16 (16)
the atlas was seen in 72 patients (81%) in Group I and 81 posterior column & spinothalamic tract 16 (18) 57 (56)
normal sensations 31 (35) 17 (17)
patients (79%) in Group II. Fusion of C-2 and C-3 was localized sign
observed in 49 cases (56%) in Group I and 43 cases (42%) short neck 36 (41) 51 (50)
in Group II. Lateral or paramesial compression by the lat- low hairline 42 (48) 38 (37)
eral mass of the atlas or the occipital condyle was seen in webbed neck 41 (47) 39 (38)
21 patients (24%) in Group I and 18 patients (18%) in torticollis 61 (69) 15 (15)
Group II. restricted neck movements 52 (59) 31 (30)

Spinal Instability Factor. Spinal instability was defined
as the clear radiographic presence of mobile subluxation
with flexion resulting in an increase in the atlantodental or
clivodental interval, increased compromise of the canal
diameter, or reduction in the girth of the brainstem. Spinal
instability was seen in three patients (3%) in Group I and
one patient (1%) in Group II.
FIG. 2. Magnetic resonance image obtained in a 34-year-old
man with Group II basilar invagination. Extensive syringomyelia is
noted.
* CNS = central nervous system.
Surgical Procedures
Before surgery, 82 patients in Group I and 19 patients in
Group II were placed in cervical traction. A majority of
patients (68 or 82%) in Group I improved clinically fol-
lowing application of traction. The improvement was
almost instantaneous in most cases. One patient (5%) in
Group II improved as a result of traction. Following trac-
tion, the omega angle, Chamberlain’s line, atlantodental or
clivodental interval, and craniocervical angulation re-
duced toward normal values in Group I. There was no
alteration in these parameters in any patient in Group II.
Table 3 shows the surgical methods and Table 4 pro-
vides a summary of the results of those surgeries. In
Group II, dorsal foramen magnum bone decompression
and duraplasty were performed in 75 patients (74%) in the
earlier portion of the series (average duration of follow up
55 months). In most of these cases, the suboccipital bone,
the rim of the foramen magnum, and the dura overlying
the herniating tonsils were thin. The dura was either left
open or a dural homograft was applied. No attempt was
made to lyse adhesions around the herniating tonsils or to
manipulate the area of the obex. Bone decompression of
the foramen magnum alone was performed in 16 patients
(16%) treated after 1994 (average follow up 18 months).

J. Neurosurg. / Volume 88 / June, 1998 963

 A. Goel, M. Bhatjiwale, and K. Desai

TABLE 2
Radiographic measurements in 190 patients surgically treated
for basilar invagination*
Group I Group II
Measurement No. (%) No. (%)

total no. of patients 88 102

modified omega angle (˚)
30–40 2 (2) 0 (0)
41–50 8 (9) 1 (1)
51–60 37 (42) 2 (2)
61–70 29 (33) 26 (25)
71–80 9 (10) 47 (46)
81–90 3 (3) 26 (25)
effective foramen magnum diameter (mm)
0–4 12 (14) 0 (0)
5–8 36 (41) 2 (2)
9–12 20 (23) 4 (4)
13–16 16 (18) 24 (24)
17–20 3 (3) 41 (40)
21–24 1 (1) 18 (18) FIG. 3. Line drawing showing landmarks used for measure-
$25 0 (0) 13 (13) ments. b = basal line; c = Chamberlain’s line; t = tuberculum sel-
brainstem girth (mm) in 138 patients† lae–torcular herophili line; w = Wackenheim’s clival line. The
0–3 19 (32) 0 (0) basal angle is the angle between lines b and w. The Klaus height
4–6 28 (47) 3 (4) index on plain radiography is the distance from the tip of the odon-
7–9 8 (14) 21 (27) toid process to line t.
10–12 3 (5) 52 (66)
13–15 1 (2) 3 (4)
Wackenheim’s clival line–tip of dens (mm)
#0 0 (0) 88 (86) Simultaneously with the foramen magnum decompres-
0–4 4 (5) 11 (11) sion, lateral fixation was performed in eight patients.16,18 In
5–8 4 (5) 3 (3)
9–12 16 (18) 0 (0)
the 51 patients in whom a syrinx was identified, syringot-
13–16 30 (34) 0 (0) omy was performed in 38 (75%); this involved insertion
17–20 24 (27) 0 (0) of a Silastic draining tube that ran from the syrinx at its
21–24 10 (11) 0 (0) maximum dimension to the subarachnoid space. In 13 pa-
Chamberlain’s line–tip of dens (mm) tients (25%) no direct surgical intervention for the syrinx
2.5–4 9 (10) 0 (0) was undertaken. In the early portion of the series, such an
5–8 9 (10) 5 (5)
9–12 28 (32) 10 (10)
approach was adopted in seven patients in whom the
13–16 32 (36) 22 (22) syrinx was relatively small. In the later portion of the
17–20 6 (7) 24 (24) series, bone decompression of the foramen magnum alone
21–24 4 (5) 14 (14)
25–27 0 (0) 19 (19)
$28 0 (0) 8 (8)
Klaus height index (mm)
16–20 0 (0) 8 (8)
21–25 2 (2) 24 (24)
26–30 9 (10) 35 (34)
31–35 25 (28) 21 (21)
36–40 19 (22) 10 (10)
41–45 24 (27) 4 (4)
46–50 9 (10) 0 (0)
basal angle (˚)
120–130 11 (13) 5 (5)
131–140 36 (41) 18 (18)
141–150 27 (31) 44 (43)
151–160 12 (14) 31 (30)
161–170 2 (2) 4 (4)
odontoid tip–PM (mm) in 138 patients‡
0–5 0 (0) 24 (32)
6–10 0 (0) 21 (27)
11–15 13 (22) 8 (10)
16–20 13 (22) 22 (28)
21–25 25 (42) 3 (4)
$26 8 (14) 0 (0) FIG. 4. Line drawing showing the parameters for measurement
* PM = pontomedullary junction.
of the modified omega line. Line A is drawn along the hard palate.
† At the site of maximum compression on MR imaging (Group I, 59 Line B is parallel to line A and passes through the center of the base
patients; Group II, 79 patients). of the axis. Line C extends from the center of the base of the axis
‡ As observed on MR imaging (Group I, 59 patients; Group II, 79 along the tip of the odontoid process. The angle between line B and
patients). line C is the modified omega angle.

964 J. Neurosurg. / Volume 88 / June, 1998

Basilar invagination

TABLE 3 TABLE 4
Surgical method used in 88 Group I patients and 102 Group II Outcome in 190 patients surgically treated for basilar
patients with basilar invagination* invagination categorized by surgical procedure*
No. of Patients (%) Group I Group II
Outcome No. (%) No. (%)
Method Group I Group II
FMD only
FMD only 10 (11) 81 (79) improvement 3 (30) 55 (68)
TO only 69 (78) 11 (11) deterioration 4 (40) 1 (1)
FMD followed by TO 8 (9) 3 (3) no change 3 (30) 25 (31)
TO followed by FMD 1 (1) 7 (7) TO only
dorsal fixation 61 (69) 13 (13) improvement 64 (93) 5 (45)
* FMD = foramen magnum decompression; TO = transoral surgery. deterioration 3 (4) 4 (36)
no change 2 (3) 2 (18)
FMD followed by TO
improvement 4 (50) —
was performed, even in the presence of the syrinx (in six deterioration 3 (38) —
no change 1 (12) 3 (100)
patients). TO followed by FMD
Transoral surgery was performed in 99 patients (Group improvement — 4 (57)
I, 78 patients [89%]; Group II, 21 patients [21%]) (Table deterioration — 0 (0)
3). Following transoral surgery, in six cases homologous no change 1 (100) 3 (43)
bone graft was placed between the drilled portion of the * FMD = foramen magnum decompression; TO = transoral surgery; — =
vertebral body of the axis and the inferior part of the clivus not applicable.
to assist fusion. In three patients a transoral plate and
screw fixation of the clivus to the cervical vertebral body
was performed.17 The bone graft was placed underneath
the metal plate. These patients were placed in halo fixa- appropriate. It divided the anomaly into two discrete types
tion following the surgery. Problems of infection and that probably have a common embryological origin,30 but
rejection of the metal implant in one case and poor or no diverse patterns of clinical presentation, radiological fea-
visualization of the bone graft on postoperative follow-up tures, and management considerations.
imaging in other cases led to the abandonment of anterior Clinical Features and Radiological and Pathological
fixation. Dorsal fixation (Table 3) was performed in the Characteristics
same surgical session following a transoral surgical pro-
cedure in 18 Group I patients. In these cases the indication On the basis of radiological studies it appeared that in
for immediate fixation was relatively high mobility of Group I there was invagination of the cervical spine into
the cervical vertebral bodies during drilling. In 39 other the base of the skull. The definition of basilar invagina-
Group I patients, fixation was performed as a second- tion as being prolapse of the spine into the base of the
stage surgery. Excessive pain and spasm of the neck mus- skull, as suggested by von Torklus,46 was only appropriate
cles and suboccipital radicular pain formed the primary to Group I. In Group II, the clivus was invaginated along
indication for fixation in these patients. No patient wors- with the cervical spine into the posterior cranial fossa.
ened in motor function prior to second-stage fixation. In Analysis on the basis of Chamberlain’s line4,13,29,44 and on
this group fixation was performed after the initial surgery the distance from the odontoid tip to the pontomedullary
within 15 days in 16 patients, within 2 months in 11 pa- junction (Table 2) showed that basilar invagination was
tients, and between 2 and 6 months in 12 patients. In four much more severe in patients in Group II than in those in
additional Group I patients a posterior fixation procedure Group I. As judged by the atlantodental or clivodental
was performed in an apparently reduced position of the interval, there was an element of “fixed” atlantoaxial dis-
basilar invagination and the atlantoaxial dislocation fol- location in Group I. The tip of the odontoid process was
lowing cervical traction. However, all four patients need- significantly superior to Wackenheim’s clival line in
ed transoral surgery at a later stage because the reduced Group I; in Group II it remained below both Wacken-
position could not be maintained by the implant. In Group heim’s clival line and McRae’s line29 of the foramen mag-
II, a posterior fixation procedure was conducted following num. On the basis of the Klaus height index,27,30,39,43 poste-
transoral decompression in the same surgical sitting in one rior fossa height was only moderately affected or not
patient. In four patients fixation was performed within 2 affected at all in Group I, but was markedly reduced in
weeks after transoral surgery. No patient needed a fixation Group II. The superior position of the odontoid process in
procedure as a delayed measure. In six Group II patients, patients in Group II was associated with a more horizon-
no fixation was necessary, even after both anterior and tal angulation and shortening of the clivus and rostral
posterior decompressive operations. positioning of the plane of the foramen magnum.26,43
Platybasia was seen in both groups,6 but was relatively
less frequent and less severe in Group I (Table 2). In
Discussion Group II, it appears that platybasia was as important as
invagination of the odontoid process in causing the anteri-
Although other subclassifications of basilar invagina- or concavity of the brainstem and in reducing the volume
tion have been described,22,34,37,47 we found division on the of the posterior fossa. The anterior concavity of the brain-
basis of presence or absence of Chiari malformation to be stem formed a pattern of smooth curvature in Group II,

J. Neurosurg. / Volume 88 / June, 1998 965


whereas the concavity was acute in Group I, the angle
being formed by the tip of the odontoid process. The ante-
roposterior diameter of the foramen magnum was marked-
ly reduced in Group I, whereas it was only marginally
reduced, unaffected, or even greater than normal in Group
II. Although the brainstem girth was markedly reduced in
Group I, it was only marginally affected or unaffected in
Group II, indicating thereby that there was no direct brain-
stem compression as a result of the odontoid process in the
latter group. In this study a modified omega angle22 was
used because the line of the hard palate was unaffected by
the relative movement of the head and cervical spine dur-
ing neck movement in these “fixed” craniovertebral
anomalies. Facial hypoplasia or hard palatal abnormality
was not seen in any case in this series and did not affect
the measurements. The reduction in the omega angle in
Group I showed that the odontoid process had tilted
toward the horizontal plane and was posteriorly angulated
in Group I, whereas it was closer to the vertical plane in
Group II (Table 2).
The association of Chiari malformation, syringomyelia,
and basilar invagination has been the subject of various
studies.5,8,10,12,24,31,34 There was Grade I Chiari malformation
in all our Group II cases. The presence and extent of the
syrinx did not correlate with the severity of basilar invagi-
nation and tonsilar herniation.42
Age and Gender of Patients
A significant majority of Group I patients were adoles-
cents (Table 1). Patients in Group II were older at the time
of presentation.10 Male predominance22,24,29,36,48 was more
marked in Group I (72%) than in Group II (54%).
Analysis of Symptoms
Symptom presentation was relatively acute in patients
in Group I, whereas it was longstanding and slowly pro-
gressive in patients in Group II (Table 1). Although long
term use of the head to carry loads has been recorded as a
precipitating factor in some previous studies from India,1,38
it did not seem significant in this series. In Group I, py-
ramidal symptoms formed a dominant component. Kines-
thetic sensations were also affected in a large majority of
cases. In Group II, spinothalamic sensory dysfunction and
ataxia were equally significant to motor and deep sensory
system affection (Table 1). Neck pain and spasms of the
muscles of the nape of the neck were frequent findings in
Group I. These features suggested strain on the muscles
that was secondary to subtle spinal instability in the re-
gion. The fact that trauma influenced acute development
of symptoms in a large number of Group I cases (48%)
also pointed toward an element of spinal instability in the
craniovertebral region. Mere physical examination of pa-
tients with basilar invagination was of diagnostic value in
a majority of cases in both groups (Table 1). The associa-
tion of platybasia, occipitalization of the atlas, and fusion
of cervical vertebrae in both groups suggests a common
origin.4,19,29,40,45
The analysis of radiological and clinical features sug-
gests that the symptoms and signs in Group I were a result
of brainstem compression by the odontoid process, where-
as in Group II they were directly related to the crowding
of neural structures at the foramen magnum (Table 1). Al-
966
A. Goel, M. Bhatjiwale, and K. Desai
though the dimensions of the foramen magnum were large
and sometimes were even larger than normally found, the
volume of its contents and, probably, the longstanding
“pulsatile” compression of structures at the foramen mag-
num resulted in neurological symptoms.49
Surgical Intervention
Clinical and radiological improvement following trac-
tion was only obvious in Group I. This suggests that trac-
tion assisted in pulling the odontoid process away from
the brainstem. This feature also indicates the relative ver-
tical instability of the craniovertebral region in Group I
and the stability of the craniovertebral joints in Group II.
The surgical treatment of basilar invagination in the
presence of Chiari malformation has not been clearly out-
lined and is confused by the fact that some authors cur-
rently recommend anterior surgery, even for this group of
patients.3,7,23,30,41 From the analysis of results shown in
Table 4, it appears that patients in Group II benefited from
foramen magnum decompression. The procedure resulted
in significant amelioration of symptoms and at least an
arrest in the progression of the disability. None of the
patients in this group had delayed worsening of their neu-
rological condition following a foramen magnum decom-
pression. The suboccipital bone, posterior rim of the fora-
men magnum, and dura overlying the herniated cerebellar
tissue were thin in a significant number of these cases.14
This was probably related to chronic pressure changes
secondary to reduced posterior fossa volume. Dural de-
compression was performed in the earlier phase of the
series. In the later phase, Group II patients were only treat-
ed with bone decompression of the foramen magnum and
the adjoining suboccipital bone, irrespective of the state of
the dura. This policy was based on the understanding that
reduction in the volume of the posterior cranial fossa, sec-
ondary to abnormal development of bones anterior to the
neuraxis, was the primary pathological condition in these
cases.15,26,33,35 The clinical and radiological results in
patients who underwent the various treatment modalities
were not different, and satisfactory outcome was noted
uniformly. Although this experience is limited, we can
infer from the results that bone decompression of the fora-
men magnum alone may be sufficient, even in the pres-
ence of syringomyelia.2,12,21,24,25
Marked clinical worsening was observed in four of 10
Group I patients in whom only a posterior decompression
was performed and in three of eight patients in whom a
posterior decompression was performed prior to transoral
surgery.9 On the other hand, transoral decompression was
an effective and gratifying surgical procedure in 64 (93%)
of 69 of these patients. The clinical worsening following
transoral surgery in Group II (Table 4) was related to a
markedly difficult surgical procedure. This was the result
of the high position of the odontoid process and the inabil-
ity to pull it caudally by cervical traction. Additional
removal of the clivus appears to be necessary to achieve
complete obliteration of the anterior indentation of the
brainstem in these cases. In the earlier portion of this
series, transoral bone decompression was performed in 21
Group II patients. Later such a procedure was not pre-
ferred because our impression is that the decompressive
effect on the neural structures of the posterior fossa and at
J. Neurosurg. / Volume 88 / June, 1998
Basilar invagination
the level of the foramen magnum is satisfactory following
a posterior decompression.
The study of pathological characteristics and the analy-
sis of results suggests that anterior transoral decompres-
sive surgery is relevant in Group I and posterior foramen
magnum decompression is appropriate in Group II. The
necessity for opening the dura after bone decompression
of the foramen magnum in Group II needs to be evaluated
on the basis of a larger study.
Role of Fixation
The role of posterior craniovertebral fixation following
transoral surgery in patients with basilar invagination has
remained controversial.7,11,20,30 It was observed that the
craniovertebral anomaly associated with basilar invagina-
tion is of a relatively fixed variety and simultaneous dor-
sal fixation following a transoral decompression is not
mandatory in either group. In Group I, younger patients
needed fixation more frequently than older patients.30 It
appears that in Group I, fixation is required but can safely
be staged, whereas in Group II it can be avoided.
Acknowledgments
The authors acknowledge with gratitude the help of Dr. Ravi
Ramakant, Head, Department of Radiology, King Edward Me-
morial Hospital and Seth Gordhandas Sunderdas Medical College,
Bombay, India; Dr. Vijay Sheel Kumar, Consultant Neurosurgeon,
Apollo Hospital, Delhi, India; and Dr. Naina Goel in the preparation
of the manuscript. Dr. Sunil K. Pandya was our principal source of
encouragement.
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Manuscript received July 23, 1997.
Accepted in final form January 15, 1998.
Address reprint requests to: Atul Goel, M.Ch., Department of
Neurosurgery, King Edward Memorial Hospital and Seth Gord-
handas Sunderdas Medical College, Parel, Mumbai 400 012, India.
J. Neurosurg. / Volume 88 / June, 1998