ACTA

Acta Neurochirurgica61, 111--126 (1982) NEUROCHIRURGICA

9 by Springer-Verlag1982

Department of Neurosurgery (Prof. H. Verbiest), University Hospital Utrecht,

The Netherlands

C o n g e n i t a l B l o c k V e r t e b r a e C2-Cs
in Patients with Cervical Myelopathy

By

R. de Graaff

With 7 Figures

Summary
One hundred and forty-five patients presenting with symptoms of cervical
myelopathy were treated surgically. Eight of these patients (5.50/0) showed
congenitalblock vertebrae C~-C3. The relationship betweenthis congenitalanomaly
and the occurrenceof spondyloticmyelo-radiculopathyis discussed.
Keywords: Congenital block vertebrae; cervical myelopathy; cervical
spondylosis; Klippel-FeiI syndrome.

Introduction
Congenital anomalies of the cervical spine are relatively common
in clinical radiology. According to Fried 8 one of the most commonly
encountered anomalies are blo& vertebrae C2-Ca. The presence of
blo& vertebrae is not necessarily accompanied by clinical symptoms,
although in certain circumstances they may themselves cause neuro-
logical disturbances or they may reinforce the effect of trauma. They
also predispose to early spondylosis is.
Radiological examination shows also that cervical spondylosis is
a common finding after middle age. In only a small proportion of
the population, cervical spondylosis adversely affects the spinal cord
and its roots. We now know that patients presenting with symptoms
of spondylotic myelopathy often show a marked decrease in the
initial sagittal diameter of the spinal canal as compared to patients with
asymptomatic spondylosis or spondylosis with only radiculopathy.
In their paper on "The neurological manifestation of cervical
spondylosis" 2, Brain, Northfield, and Wilkinson described four pa-

0001-6268/82/0061/0111/$ 03.20
112 R. de Graaff:
Table 1
Case Symptomatology
I Numbness and tingling of all dig-
female its. Impairment of fine finger
75 years movements, muscular weakness,
and wasting of the small muscles
of the hands. Ne& pain and pain
in both shoulders. Finally "stiff
legs", numbness of feet and in-
ability to walk.
II Numbness and tingling of all dig-
female its and finally of both hands.
62 years Neck pain and severe limitation of
all cervical spine movements.
Muscular weakness of the right
hand and shoulder musculature.
Numbness of the feet, spastic-
ataxic gait, and finally inability to
walk without aid.
III Tingling of thumb and index fin-
male ger, followed by muscular weak-
69 years ness and severe wasting of the
shoulder musculature on the right
side. Clumsiness of both hands.
No neck pain. Numbness of both
feet, and slight ataxia.
IV Tingling and muscular weakness of
male the left hand and fingers. Pro-
21 years gressive difficulty in walking due
to a spastic-ataxic paresis, more
marked on the left side. Numb-
ness and tingling of both feet.
Finally tetraparesis and inability
to walk.
V Neck pain radiating in both arms
male to the thumbs and index fingers,
44 years with tingling and numbness of
digits I, II, and III. Muscular
weakness and clumsiness of both
hands. Lhermitte's sign. Progres-
sive difficulty in walking. Numb-
ness of feet.
VI Tingling and numbness of both
male hands and all fingers, with mus-
53 years cular weakness and clumsiness of
the right hand. Progressive dif-
ficulty in walking due to a spastic-
ataxic paresis more marked on the
right side. Urgency of micturation.
Neurological examination
Severe /imitation of all cervical
spine movements. Atrophy of the
small hand muscles. Paresis of
both arms and legs. Spastic-ataxic
gait. Impairment of deep sensa-
tion of both hands and feet.
Atrophy of the small muscles of
the right hand. Quadriparesis with
a right-sided preponderance.
Spastic-ataxic gait. Slight impair-
ment of appreciation of light
touch, tactile discrimination, and
pin-prick over all the fingers. Loss
of vibration sense below the knee.
Atrophy of the Spinati, Deltoid,
Biceps, and Brachio-radialis on the
right side. Impairment of deep
sensation in both hands. Loss of
vibration sense below the iliac
crest. Hyperreflexia of knee and
ankle jerks. Plantar reflextensor.
Paresis of the left forearm and
small muscles of the hand. Slight
impairment of deep sensation of
left hand and leg. Hypalgesia of
the right leg. Spastic-ataxic
paresis, more marked on the right
side.
Limitation of all cervical spine
movements. Lhermitte's sign on
flexion of the spine. Inverted
radial reflexes. Slight impairment
of deep sensation of all fingers.
Loss of vibration sense below the
knees. Spastic-ataxic gait.
Slight atrophy of the small muscIes
of the right hand, with paresis of
right forearm and hand. Loss of
deep sensation of all digits.
Spastic-ataxic paresis of both legs,
more marked on the right side.
Loss of vibration sense below the
costal margins.
 Congenital Block Vertebrae C~-C8 113

Table 1 (continued)

Case Symptomatology Neurological examination

Vll Neck pain and left-sided shoulder
male pain, with numbness and tingling
44 years of all fingers, with a left-sided
preponderance. After chiropraxis
progressive spastic paraparesis, also
more marked on the left side.
Finally inability to walk without
aid.
VIII Pain in the neck, right shoulder,
female and hand with progressive wasting
66 years of shoulder musculature and weak-
ness of forearm and hand, fol-
lowed by progressive paresis of
both legs, numbness and tingling
of both hands and feet. Finally
bedridden.
Slight atrophy of Deltoid, Spinati
and Biceps. Paresis left forearms
and hand. Slight impairment of
deep sensation of all fingers.
Severe spastic-ataxic paraparesis,
more marked on the left side. Loss
of deep sensation below the knees.
Atrophy of Deltoid, Spinati, Bi-
ceps and Brachio-radialis, with
paresis of right forearm and hand.
Impairment of deep sensation of
both hands. Hypalgesia below the
T2-1evel. Severe spastic-ataxic
paraparesis. Incontinence.

tients with congenital abnormalities of the cervical spine. Three

patients showed fusion of vertebral bodies, and one patient showed
a fusion of spinous processes. T h e y found it probable that the
resulting disorder of mobility caused an excessive stress upon normal
joints and so caused or contributed to the cause of cervical spondy-
losis.
In this paper we describe eight patients with cervical myelo-
radiculopathy. All patients showed congenital block vertebrae C~-C~
on radiological examination. The purpose of this paper is to show
the relationship between a congenital anomaly, i.e., block vertebrae
C2-C 8, and spondylarthrosis of the upper cervical spine, leading to
the complex of signs and symptoms of myelo-radiculopathy due to
involvement of the cervical cord and its roots.

Clinical M a t e r i a l
Since 1962, 145 patients presenting with symptoms of cervical spondylotic
myelopathy were treated surgically in our clinic. Forty-one patients were female
(28.5~ and 104 patients were male (71.5~ Sex ratio: 1:2.5. Ages varied
between 21 and 77 years.
Patients presenting with radiologicaI evidence of anomalies in the cranio-
cervical junction, and patients with the "original" Klippel-Feil syndrome,
Type I ~, 9, 10, were excluded from the series.
Three types of operations were performed: 1. decompressive laminectomy,
whether extensive 1 or limited to three or four levels, with or without sectioning
of the dentieulate ligaments and dural grafting (112 patients), 2. antero-lateral
8 Acta Neurochirurgica, Voi. 61, Fast. 1--3
114 R. de Graaff:

Table 2

Case CSF Queckenstedt Operation Results

protein
f n e

I 1.20 g/l + laminectomy C~-C7 Follow up: 17 months.

Unchanged.
II 0.58 g/l + laminectomy C~-C7 Follow up: 4 years. Pa-
tient walks without aid.
Sensory symptoms and
signs improved.
III 0.72 g/l + laminectomy Cz-Cv Follow up: 21 months.
Sensory signs and symp-
toms slightly improved.
Musc. weakness un-
changed.
IV 0.95 g/1 + + -- laminectomy C1-C7 Follow up: 3.5 years.
Complete recovery, ex-
cept hyperreflexia.
V 1.50 g/1 + laminectomy C~-C7 Follow up: 8 years,
sensory signs and symp-
toms unchanged. Rest of
symptoms improved.
VI 0.49 g/l + + + laminectomy C2-C5 Follow up: 7.5 years.
Slight improvement of
sensory symptoms. Walks
without difficulty.
VII 0.52 g/l + + -- laminectomy C~-C7 Follow up: 19 months.
Walks without aid. The
remainder of symptoms
unchanged.
viii 0.96 g/1 + laminectomy C2-T1 Complete tetraplegia
after the operation. Pa-
tient died two months
postoperatively.

The result of Queckenstedt's test is negative when there was a complete

obstruction to the flow of CSF. f ~ flexion, n = neutral position, e = extension.

operation according to the method of Robinson and Smith l~, which Verbiest
modified by using wedge-shaped, autoge~ous tibial cortical bone grafts instead of
cancellous iliac bone (21 patients), 3. a lateral operation on the cervical spine as
described by Verbiest 15, 18 with or without vertebral fusion (12 patients).
Eight patients (5.5%) showed radiologically congenital block vertebrae C~-C3.
One of these patients also had a congenital block vertebra C5-C ~. In this group
three patients were female. The length of history up to the time of diagnosis
varied from three months to nine years. All patients underwent a decompressive
laminectomy. One patient died two months after the operation due to cardiac
 Congenital Block Vertebrae Ce-C z 115

failure. Sevenpatients were available for follow up. The follow-up periods varied
between 17 months and 8 years.
The clinical findings and other pertinent data are summarized in Tables 1
and 2.
Results
History and Examination
Since a C2-Ca block vertebra can cause excessive dynamic stress
on the lower cervical levels, and so lead to spondylarthrosis of C3-C~
and C4-C 5 levels, one should expect involvement of the upper cervical
cord and of the roots C 4 and C 5. In five patients, however, dys-
aesthesiae, i.e., "numbness and tingling" of the finger tips and
eventually of the digits and hands, often followed by clumsiness and
weakness of one or both hands, were prominent presenting symptoms.
All patients however showed also evidence of spondylosis C5-C6
with foraminal narrowing, which makes the presenting symptoms as
mentioned above understandable. Only three patients presented with
symptoms of flaccid paresis and atrophy of the spinati, deltoids,
biceps, rhomboids, and brachio-radiolis indicating some involvement
of the C~ and C~ roots. The presenting symptom in two patients was
neck pain radiating into one or both shoulders, and in one patient
neck pain radiating into both arms to the thumbs and index
fingers.
In most cases these signs and symptoms preceded the onset of
weakness and atrophy of the small muscles of the hand, which was
asymmetrical in most cases. They also preceded the spastic-ataxic
paraparesis with hyperreflexia, clonus and extensor plantar signs.
Five patients were unable to walk without aid, one patient was
bedridden.
Impairment of appreciation of light touch, tactile discrimination,
and pinprick over the fingers, and loss of vibration sense below the
iliac crest or below the knees, were found in every patient. One
patient showed Lhermitte's sign on flexion of the cervical spine. On
examination, only two patients showed a severe limitation of all
cervical spine movements. One patient had urgency of micturition,
and at the time of admission another patient was incontinent (see
also Table 1).
RadioIogical Examination
Plain lateral films showed congenital block vertebrae C2-C~ in all
patients. One patient showed also a C5-C 6 block vertebra (case VIII).
All patients showed segmentation failures of both articular facets
and neural arches. Five patients had "fused" spinous processes, and
one patient showed a spina bifida C6 (case IV). Six patients under-
8*
116 R. de Graaff:

went a radiological examination of the thoracic and lumbar spine as

well. None of these patients showed other congenital anomalies of
the spine.
Degenerative changes affecting the discs, neurocentral joints, and
articular facets of the apophyseal joints were found to exist at the
levels C~-C4 and C~-C5, with relative sparing of the joints at lower
levels. Uncovertebral arthrosis leading to foraminal narrowing due
to encroachment by osteophytes was seen to exist at lower levels.
In four cases (I, II, VI, VIII) the combination of uncovertebral
arthrosis and osteoarthrosis of the apophyseal joints led to severe
narrowing of the C4-C5 intervertebral foramen. All patients except
case IV showed foraminal narrowing C5-C6, and to a lesser extent
foraminal narrowing C6-C v. In one case gross foraminal narrowing
C3-C ~ was observed. The other cases showed some narrowing of the
C3-C ~ foramen (Fig. 1). In all cases the C2-C 3 foramina were round
or oval, and smaller than those at the levels of the moveable joints.
Large osteophyte C3-C 4 were present in cases I, II, VI, VII, and to
a lesser extent in case VIII. In five patients we were able to measure
the mid-sagittal diameter (MSD) of the spinal canal on plain films,
according to the method of Burrows 3 (cases II-VI). In these cases we
also measured the "minimum theco-periosteal diameter" (MTPD),
as described by Nurick 22. The results are shown in Fig. 2. Two
cases (III and IV) showed evidence of absolute stenosis with, at more
than one level, an MSD of 10 mm. Two cases (II and V) showed
evidence of a relative stenosis (MSD less than 13 mm), and one case
had small, but otherwise normal, MSD (case VI). In this case a large
osteophyte, C~-C 4 reduced the MTPD to 10 mm. In all patients,
flexion-extension studies showed an abnormal motion pattern. Due
to disc degeneration and osteophytosis practically no movement be-
tween C3-C4 was observed in cases I, II, VI, and VIII. These patients
showed disproportionate, compensatory movements at lower levels,
especially at C4-C5. The remainder of the patients showed com-
pensatory movements at C3-C~, and to a lesser extent at C~-C5, with
in some cases subluxation and retrolisthesis of C3 and C4 during
extension, giving rise to a so-called "pincer mechanism".
All patients underwent positive contrast myelography. In three
cases we used oil (I, V, and VIII) and in five a water-soluble contrast
medium (metrizamide). Complete obstruction to the flow of contrast,
not influenced by flexion or extension of the cervical spine, was
observed at the level C~-C~ in two cases (III and VIII). A partial
block to contrast flow was observed in the others, although in these
cases a marked influence of changing the position from extension to
flexion was noticed. Cases III, VII, and VIII showed definite "filling
 Congenital Bto& Vertebrae C2-C~ t17

Fig. L The foramen C=-C~ is round and shows no narrowing from osteopbytes
(single arrow). The foramen of C~-C4 is narrowed due to osteophytes (double
arrow)

30'
2;
2;
24
2~
26
18~
16~

12~

®O ®®
®

~l I I I l i i
C1 C2 C3 C4 C5 C6 C-~

Fig. 2. The midline sagittal diameter in five patients of this series (--) in relation
to the normal range o,f the sagittaI diameter in normal patients (shaded area).
(3 represents the "minimum theco-periosteal diameter"
118 R. de Graaff:

defects" of the root-pouches C~-C5; cases I, II, and V I - V I I I showed

also "filling defects" of the root-pouches Cs-Co, and to a lesser extent
of C6-C 7. These "filling defects" were fairly asymmetrical.
Cerebrospinal Fluid
The protein content of the cerebrospinal fluid (CSF) was raised
in all cases but one (case VI). This patient showed no obstruction to
the flow of CSF. The manometric findings were significantly in-
fluenced by flexion and extension of the cervical spine (see Table 2).
Operative Results
All patients underwent a laminectomy in the prone position. One
patient (case VIII) had a complete transverse lesion of the cord
immediately after the operation. Although there was a recovery of
function in the first week after the operation, she died two months
later due to cardiac failure. One patient did not show any improve-
ment after the operation. This patient happened to be the oldest
patient of the series (case I), she should have been treated earlier.
One patient (case IV) showed a practically complete recovery.
This happened to be the youngest patient in the series, showing an
absolute stenosis of the spinal canal and a "soft disc" herniation
C~-C5, reducing the MTPD to 8.5 mm (Fig. 3). The other patients
all showed some improvement of the neurological disorder. This
was especially true for the long-tract motor signs: after the operation
all these patients were able to walk without aid, although in most of
these cases hyperreflexia and pathological reflexes were still present.
In all cases the "numbness and tingling" of the fingers and hands
were resistant to therapy, and so were the atrophy of the shoulder
muscles and of the small muscles of the hand (see also Table 2).

Discussion
The nomenclature of the radiological diagnosis "block vertebra"
remains a matter of dispute. The term "block vertebra" is a descrip-
tive term, saying nothing about the aetiology of the anomaly. Block
vertebrae, in addition to being congenital may be acquired, as is the
case in juvenile rheumatoid arthritis (Still's disease), rheumatoid
spondylitis, trauma, tuberculosis, or other infective diseases.
Still there is some disagreement whether the lesion is a fusion or
a non-segmentation. Hadley prefers to describe congenital union of
vertebrae as non-segmentation rather than fusion, because separation
of the primary segments of the spine during the first eight weeks of
embryonic life does not occur. Not-segmentation is the preferable
designation since the condition results from failure of segmentation
 Congenital Block Vertebrae C~-C3 119

of the mesodermal tissues, leading to abnormal intervertebral disc

formation 1~
In the literature the usage of the term "Klippel-Feil syndrome"
is variable, and so it is difficult to discover what constitutes the
minimal requirements of this syndrome. Gray, Romaine, and

Fig. 3. Case IV: Myelography with metrizamide shows a poor filling of the sub-
arachnoid space due to stenosis of the spinal canal and a "soft disc herniation"
Ca-C~ (arrow), which reduces the MTPD to 8.5 ram. Note also the subluxation
CrCv and the anomalous bifid spinous process C6

Skandalakis 9 in their collective review said that congenital fusion of

the cervical vertebrae is the common denominator of a galaxy of
skeletal defects with poorly known interrelationships of which one
of the best known constellations is the Klippel-Feil syndrome. In
general, this term usually refers to any congenital "fusion" of the
cervical spine. Fell, in his theses 7, described three types of the
120 R. de Graaff:

syndrome. In type I there is a massive fusion of the cervical and

upper thoracic vertebrae, with in many cases associated anomalies
of other parts of the body. Type II shows block vertebrae of only
one or two cervical levels, and type III includes cervical block
vertebrae together with block vertebrae in the thoracic or lumbar
spine. The patients in our series all belonged to type II.

Fig. 4. Flexion-extension films show the disproportionate mobility and instability

at C4-C~. The mobility at C~-C4 is inhibited by the osteophytes. Note also the
typical appearance of the "ghost" disc space C2-Cs and of the foramen C~-C8,
which is small and round

The author, however, like many others, in these cases prefers to

use the term "congenital block vertebrae" with the suggestion that
the level and extent of the non-segmentation should also be defined
in association with that term. The term "Klippel-Feil syndrome" is
reserved for those cases that belong to type I. These patients were
excluded from the series.
 Congenital Block Vertebrae C~-C a 121

The radiological appearence of the congenital block vertebrae is

usually characteristic. Apart from the vertebral bodies, all other parts
of two adjacent vertebrae may be involved. This includes the zygo-
apophyseal joints, the neural arches, and the spinous processes.
Occasionally there may be a spina bifida, as in one of our patients
(case IV). With synostosis, remnants of the epiphyseal tissue may

Fig. 5. Very severe spondylarthrosis of the upper cervical spine (case I)

persist within the block vertebrae as a bony or cartilaginous plate

throughout life. Since the ring-epiphysis of the involved adjacent
vertebrae does not develop, very often the antero-posterior diameter
of the vertebrae at the site of the synostosis is decreased, compared
with the normal end-plate size. This also means that the AP diameter
of the spinal canal at the site of the synostosis is enlarged.
Another sign that differentiates congenital block vertebrae from
the acquired forms, is the absence of osteophyte formation at the
122 R. de Graaff:

involved level. The foramina between non-segmented vertebrae are

characteristically oval or round and in most instances, smaller than
normal. They do not alter in size by movement, nor is there foraminal
narrowing due to encroachment by osteophytes. Structures passing
through these foramina do not run the risk of being compressed.

Fig. 6. Midline tomography showing a complete stop to the flow of contrast at

C~-C4 in a patient (case III), with absolute stenosis of the spinal canal

Since no movement occurs between the segments of a block

vertebra, additional dynamic stress necessarily falls upon the struc-
tures of the adjacent joints. Invariably this leads to spondylarthrosis
of the adjacent cervical segments, and possibly to a greater incidence
of cervical myelopathy and radiculopathy, as is shown in this paper.
With a C2-C3 block vertebra additional dynamic stress primarily
 O

gl

b~

Fig. 7, Case VI: Extension-flexion films (A and B)show the disproportionate mobility at C4-C 5 and C5-C~. due
to a block vertebra C~-C3, spondylosis, and osteophytosis of C~-C4 and C~-Cv. Plain lateral film, six years after
operation, shows a fusion of C5, C6, and C 7. Although the greatest stress is now placed upon C4-C5, practically no
spondylotic changes are noticed at this level
124 R. de Graaff:

falls upon the C3-C4 level, and to a lesser extent upon the C4-C 5
level. Once a severe spondylarthrosis at the C3-C 4 level is established,
the movements at this level are inhibited by osteophytes of the
vertebral bodies, and by osteophytosis of the neurocentral and zygo-
apophyseal joints. This is the cause of extra dynamic stress upon the
C~-C 5 level (Fig. 4). In elderly people this can lead to very severe
degenerative changes of the upper cervical spine (Fig. 5), and to com-
pression of the upper cervical cord and its roots. In conjunction with
"the normal for age" degenerative changes of the lower cervical
levels, especially C5-C6, the cord may be compressed from C~ to T1.
It is a well-known fact that in most cases the development of
myelopathy is related to the initial size of the spinal canal 12. 23
Verbiest distinguished between patients with relative and patients
with absolute stenosis of the lumbar spinal canal 27. We used the
same nomenclature for stenosis of the cervical spinal canal, and so
did Epstein e t al. 6 in a recent article on traumatic myelopathy in
patients with cervical spinal stenosis. Although all our patients
showed evidence of a reduced initial sagittaI diameter, we were able
to make accurate measurements in only five cases. Two patients
showed an absolute stenosis with a sagittal diameter of 10 ram, and
two patients showed a relative stenosis with a diameter of 13 turn
or less. Osteophytes, and in case IV a "soft disc herniation" (Fig. 3),
further reduced the sagittaI diameter of the spinal canal, leading in
five cases to a "minimum theco-periosteal diameter" of less than
10 ram. This makes compression of the cord very likely, especially
on extension of the cervical spine. Patients with cervical spinal
stenosis are uniquely vulnerable to hyperextension injuries of the
cord ~. The abnormal mobility of the cervical levels below a block
vertebra C~-C3, with in some cases subluxation, add to the inter-
mittent compression of the cord. None of our patients however,
presented with a history of acute trauma.
The nerve roots C~ and C5, passing through the intervertebral
foramina C3-C 4 and C~-C5 respectively may be compressed in a
foramen, once this is narrowed by osteophytes. Only three patients
of this series showed a flaccid paresis and atrophy of the involved
muscles of the shoulder, although all patients showed foraminal
narrowing C3-C~ and C4-C 5.
In six cases an improvement of spastic-ataxic paraparesis was
noticed. These patients were able to walk without aid. The very
disabling sensory disturbances, for instance the paraesthesiae and
numbness of the fingers and hands, together with the atrophy of the
small muscles of the hand, leading to a loss of dexterity, remained
unchanged in most cases.
 Congenital Block Vertebrae C2-C8 125

Conclusions

Congenital block vertebrae C2-C 3 predispose to early spondyl-

arthrosis of the upper cervical spine. Very often block vertebrae may
be discovered only incidentally in radiographs obtained for injury,
neck pain, headache, shoulder pain, etc. Once the diagnosis has been
established, measurements of the initial size of the midline sagittal
diameter of the spinal canal should be made. The radiological
examination should also include flexion-extension films.
The combination of a C2-C~ block vertebra, with increased
mobility of the C3-C4 segment or even a subluxation at this level on
extension of the spine and a developmental stenosis of the spinal
canal, is a harmful condition, even in the absence of neurological
signs and symptoms, and even in the absence of spondylosis. These
patients should stay under careful medical attention in order to
prevent the development of cervical myelo-radiculopathy with its
very disabling symptoms and its poor prognosis.

Acknowledgement
I would like to thank Mr. J. A. Veiga Pires, M.D., Ph.D., F.R.C.R., (Consultant
radiologist and head of the division of Neuroradiology, University Hospital,
Utrecht, The Netherlands) for helping me with the manuscript, Mr. A. Wollenberg,
medical photographer, for making the photographs and Miss Sanne de Graaff for
her administrative assistance.

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Authors' address: R. de Graaff, Department of Neurosurgery, University

Hospital, Nic. Beetsstraat 24, Utrecht, The Netherlands.