SURGICAL
TREATMENT
OF A.
Though
we
must
go back
THE
BONOLA,
KLIPPEL-FEIL
MODENA,
to Herodotus
for
SYNDROME
ITALY
the
earliest
account
of the
Acephala (Fig. 1), who were supposed to inhabit the western part of Lybia, Aldrovandi (1522-1605) the suggestion that the monstrosity consisted of the neck, the head and chest being so closely approximated that the eyes to the chest (Belloni 1950). There is no doubt that the ancient inventors seen an example of severe congenital deformity of the cervical spine. Hailer ( I 743) and Morgagni (1 746) described the anatomical features with reduction in number and fusion of the vertebrae. But it was not “
and
“
adequate
clinical
given by wrongly-as
Klippel and Feil, who a single well defined
anatomical
description the
race
of
in two such cases until 1912 that an
of
regarded entity.
mythical
we owe to Ulisses simply of absence seemed to belong of the myth had
this
anomaly
was
deformity-perhaps
The Klippel-Feil syndrome, or congenital osseous torticollis, studied later by numerous authors, and especially by Bertolotti (1920), who recognised the following essential features: 1) absence was
or
shortening
of the There
of the
neck; might
neck;
cranio-facial asymmetry, impaired jaw movement, occipital platycephaly; congenital deformities According to graphically include cervical I
FIG.
The Acephalus. ture by Nicholaus Cathedral of
A sculp-
in the Ferrara
These
diverse
Hitherto to treat the I believe attempt and four
that made
function. headings:
4) operations
in other Bertolotti fusion of
parts of the the anatomical the vertebrae
body. changes into one
hemivertebra,
440
seen mass,
platyspondyiy,
the accepted practice condition symptomatically.
has
cases the the cervical
I shall describe 1) operations on
partial to
entity, been With
policy of deformity
absence
believe but
of the
that
the
merely
sacrum,
Klippel-Feil
should be of improving
of the muscle;
total
absence should
of cervical
either to ignore the problem the advances that have been
the surgical treatment skin; 2) operations on
with the vertical of the
syndrome
as a condition
non-intervention with the object
radioeither atlanto-
and
Klippel-Feil 3) operations
deformity
of treatment or made in surgery discarded and both appearance
an
syndrome under on nerves; and
on bone. Klippel-Feil
syndrome
is often
ON
SKIN
encountered
in the
“status”
of
(Turner syndrome), the most striking feature of which is the “pterygium of asymmetrical creases extending from the mastoid process to the acromion, involving present
of movements
back, simian deformity of the scapulae. angular cervico-thoracic scoliosis, and and the condition might be associated with
OPERATIONS
The
absence
occipital fusion, assimilation of the third cervical vertebra axis, transverse segmentation of vertebral bodies and segmentation of bodies and arches, basal segmentation
as a single clearly defined other malformations.
in severe to correct
or
at the back of the neck (Fig. 2). features, such as thoracic and
flat
cervico-thoracic,
odontold process, of the coccyx. manifestations lead me
(Italy).
not be regarded with or without
or
2) limitation
and 3) a low hair line also be other minor
the the
muscles and fasciae as well as the skin. sex incidence distinguishes this syndrome,
Though neck which occurs THE
JOURNAL
OF
Bonnevie colli” and
Ullrich consisting sometimes
deformities are mainly in girls, BONE
AND
JOINT
often from SURGERY
SURGICAL
other
varieties
of pterygium Rosselli
of the colli
were
A patient
Klippel-Feil
(all
all
in females)
associated
with
TREATMENT
typical
OF
syndrome, that
with
Klippel-Feil
VOL.
movement 38
B,
NO.
2,
was MAY
1956
still
restricted
are
studied
deficiencies
disease
KLIPPEL-FEIL
which
I have
(right)
by the
compared
the
cervical
with
441
SYNDROME
commonest
from
of the
The operation of double z-plasty. with resection afforded permanent correction of the deformity though
THE
in boys.
records or
thoracic
a normal
The
of Professor
girl
cases
Sanvenero
vertebrae
of the
three
same
(Fig.
age.
of part of the muscle or fascia when necessary, and allowed greater freedom of neck movement, bone
deformities.
3).
442
A.
BONOLA
ON
OPERATIONS
In some is amenable posture and
of these
of the
is maintained and
muscles
for
is a bilateral
improved
which
frequently
divided
are
or
three
In one much
collar months.
and
tenotomy later
of pterygium
The
correction
or division
of other
Disorders
colli (syndrome of Bonnevie before and after plastic surgery
of the syndrome (Serafini
spinal and and
case
leaving
applied
obtained
muscles,
muscle clavicular
which
by
such
is dissected and sternal
scars. with
this
Correction
over-correction
method
as platysma
might
and
well
trapezius,
3
Ullrich) with congenital deformity (case of Dr Sanvenero Rosselli).
cord
and
ON
compression
they doubtless have Bertolotti), spastic
by operative
removal
of the cervical
spine
NERVES of nerve
roots
have
the same congenital hemiparesis (Sicard
pyramidal quadriplegia (Guillamme and Mollaret), pain is also frequent, as well as painful trophic after the twentieth year of life. Such cases have These radicular syndromes, surely connected bifid spines, or congenital deformities of either certainly be improved or foraminotomy.
muscle
contracted.
OPERATIONS Klippel-Feil syndromes
sternomastoid
to avoid
by a plaster
FIG.
An example
of the
case, in which I used Putti’s technique, the improved. Through a sub-mastoid incision
by subcutaneous
by stretching also
MUSCLE
contracture
the mastoid insertion of the sternomastoid three centimetres long is removed. The
by a Schanz
two
be further are
by the ear) a segment
at first
retained
there
to correction by surgery. mobility of the neck were
(the scar is hidden from the bone and heads
cases
been
origin. and
described
in the
Spasmo-cerebellar Sermayez), spastic
all with late onset, are examples. Brachial syndromes of the brachial plexus beginning not so far been treated surgically. with the existence of cervical ribs, hidden the radicular or plexus distribution, might
of supernumerary
THE
ribs
JOURNAL
or by hemilaminectomy
OF
BONE
AND
JOINT
SURGERY
SURGICAL
It
is in
deformities
fact
which
plexus
a
affecting
notion the
may
TREATMENT
now position
commonly or
be responsible
OF THE
for
accepted,
distribution severe
disease
associated
with
congenital
high
pain
its
roots and
sciatica,
The
upper
novelty,
can
scapula.
The
VOL.
same
38 B,
NO.
difficulty many 2,
MAY
would authors 1956
be greater. have given
show the child aged of the left scapula.
photographs
bony anomalies (spina bifida), or with disturbance discs; and these disorders have been treated successfully decompressive surgery could be attempted also in the
risk and Although
that found
congenital in
or
the
not
sacral they
are
4
associated
the
be
whether
before
with
The lower pictures lowering and fixation
despite nerve
443
SYNDROME
the age of four,
intervertebral
operation.
left
of
lumbar
FIG.
Klippel-Feil
KLIPPEL-FEIL
a detailed
description
nine,
of
show
five years
the
after
child
of the ligaments by operation. cervical spine; but the
pathological
at
operative
or there bony
444
A.
anatomy in the Klippel-Feil syndrome cord have surprisingly been ignored, roots which are certainly associated investigations in this direction should the
lumbo-sacral
region
of the
BONOLA
the associated congenital disturbances as have the anomalies in the distribution with the deformity in many cases. bring important results, as they have
of the spinal of the nerve Anatomical in disorders of
spine.
5 Radiographs
FIG.
Same
patient
as shown
in Figure OPERATIONS
4.
ON
before
and
after
operation.
BONES
congenital deformities of the shoulder girdle are often associated with the Klippel-Feil syndrome, among which the congenital high scapula is common. in fact this condition is nearly always associated with deformities, however slight, of the spinal column, and, especially when the scapula is fixed to the cervico-thoracic vertebrae through a bony bridge having the form of a rib, it may be responsible for severe deformities with limitation Various
THE
JOURNAL
OF
BONE
AND
JOINT
SURGERY
SURGICAL of
the
relief
given
satisfactory
OF
THE
KLIPPEL-FEIL
445
SYNDROME
and upper limb (Figs. 4 to 5). Operations that high scapula have followed Putti’s technique,
of the neck of congenital
movement
for
TREATMENT
results
consistent
with
the
degree
of deformity,
the
I have which
age
undertaken has always
of the
patient,
and
the complications that are sometimes present in severe cases, such as paresis of the brachial plexus. The steps of the operation may be summarised as follows. The incision curves along the vertebral margin of the scapula. The lower part of the trapezius and the upper part of the latissimus dorsi are divided. Below the rhomboid muscle the bony rib-like process that anchors the
scapula
to the
and severed fixed to the
cervico-thoracic
vertebrae
from its fibro-muscular fifth rib. The reduction
for some weeks the clavicle also
before movements had to be divided
Osteotomy
of the spine
is isolated
attachment. of the scapula
and
removed.
It is brought is maintained
are begun. In one patient before adequate function
as described
The
is freed
operated upon by Putti in 1908 of the scapula could be secured. et a!. (1945)
by Smith-Petersen
scapula
down until its lower angle is by a plaster shoulder spica
and
La Chapelle
(1934)
and by Delitala and Pais (1952) for the correction of deformities at sites involving less risk could be carried out with extreme caution also for bony torticollis and for the typical cervicothoracic flexion deformities which, according to some authors, can simulate the late results of spondylitis. Such an operation would sometimes be practicable because the intersomatic joints are often preserved was in fact carried out (1951)
but
the
Since
result
and interrupt successfully by
was
in most
the cervico-thoracic Herbert (1948)
an operation
on
the
minor
this
nature
ribs,
which
not
seriously
support
such
seems
ever
having
are
often
as low
perfectly been
tried
patient
was
implantation 1 was
before.
and
type. in
a woman
Radiographs
the
occiput,
from
the
and
posterior
cervical ribs rudimentary scoliosis
but
other
The
the
to
other
right.
and
the
6).
exposing
the
excised,
beginning
with
wound VOL.
the
lung,
was
closed
of
four
the
38 B,
NO.
2,
MAY
1956
that I have operation of
that
of
removal
through
the
upper
synostoses,
surgery
Klippel-Feil
posterior
no
more
vertebrae
was
carried
for
than were
able
to
syndrome
arch
of
the
a vestige, fused
would
tuberculosis
lent
of the classical
atlas
was
scarcely
into
a solid
assimilated differentiated
block.
Bilateral
with the first rib. There was to be the cause of a high thoracic
rotate
in order to
be
in layers.
the
posterior
first The
fused.
the
on
scapula,
The
The
out
paravertebral
of the ribs.
third.
is liable
completely
severe
seemingly fused which appeared
a right
margin
first
which
was
patient
was
cervical rib with which it was few centimetres from the joint apex
atlas
The operation record of an
the
head
through
a few
a
degrees,
abolished.
Through
medial
divided,
I believed interlinked
The
cervical
The
impossible
thoracic segment by of correcting angular
REPORT
with
the left one hemivertebra were
(Fig.
the
breasts. to find
of pulmonary
platybasia.
of
thoracoplasty
anaesthesia
processes
arch.
movements
Operation-Partial
local
body
were present, cervico-thoracic
convex
all
slight
the
of the unable
experience
of thirty-two
showed
the
Nevertheless
CASE
The
is technically
“
or congenitally
respiration,
view.
osteotomy by Law
In many examples of the Klippel-Feil syndromefirst four ribs are found much above the level of appearance that has been termed “cervical chest.” or bilateral cervical rib in these cases, as well as
rational.
supernumerary
impair
to this
block
of the first idea not only
“
variations
therefore
cervico-thoracic
of obtaining a cervicalisation thoracic thoracoplasty, with
deformity but also of forming a new neck. and always in the more severe cases-the the shoulder girdle, thus giving the typical There is also a high incidence of unilateral of other
A cervical undertaken
was
poor.
cases
I studied the possibility means of bilateral upper
devised
synostosis. another
and
rib
removed
ribs
were
not
to save
time
and
anchored
side
to the
on
midway
trapezius arches
was
right incision
and
of
the
May 10, between
rhomboid uppermost
together
posterior
the
the but
excessive costo-vertebral
under
spinous
muscles three
with
disarticulated,
to avoid
1954,
were
ribs
were
supernumerary were
divided
lowering angle.
a
of the The
446
A.
Progress-The was a noticeable
post-operative improvement
able
extend
to flex and
showed
the neck
a subcutaneous
2
/
C0574
course in the
was uneventful. Immediately shape of the neck on the right
for the first
air-bubble
BONOLA
and
time.
Radiographs
a haematoma
after side,
the and
a few days
above
the
lowered
after
collar
Four
the
left
months
The a
pulmonary
applied
side,
further
physiotherapy,
of the
pleura,
DE5TRA
that was
there was
the operation
dome
FIG. 6 Diagrams illustrating the operation of right partial thoracoplasty of the seventh cervical and third thoracic ribs. The diagrams on the right show tracings from radiographs of and (b) after right partial thoracoplasty. The diagrams below show the dimensions of removed. The seventh cervical and the first thoracic ribs were fused into one large rib and 4 centimetres broad. Six months later the same operation was performed on
evidence
operation the patient
the
patient
function
to mould
the
after
first
the
first,
second
was
discharged
month
she
with
not
operation
and
able massage and was
was
damaged.
regenerating the
third from to
ribs
ribs
daily
cervical
stage
resected
ten to
was
under
days
work.
weeks
improved
second
being
hospital return
Two
to the
after
In
suspension THE
the
after
operation
a plastic
contour. carried
local second
out
OF
successfully
on
anaesthesia. operation
the meantime she for half an hour. JOURNAL
and first, second the neck (a) before the costal segments 8 centimetres long the left side.
BONE
was
AND
and
treated
JOINT
after
by
SURGERY
SURGICAL
TREATMENT
Radiograph
Radiograph VOL.
38 B,
NO.
2,
MAY
1956
OF
THE
of the upper
of the upper
thorax
KLIPPEL-FEIL
thorax
after
before
bilateral
SYNDROME
operation.
partial
thoracoplasty.
447
448
A.
At
follow-up
condition
was
respiration.
examination good.
Spirometry
Rotation,
She
four
BONOLA
months
after
the
had gained weight, and was 1,060 cubic centimetres,
flexion-extension,
and
lateral
flexion
second
did
at the
operation
the
not complain scarcely lower
patient’s
general
of any difficulty with than before operation.
cervico-thoracic
junction
allowed
good
L 9
FIG.
The
mobility, that with
a little less than the normal. the lowering of the pulmonary
the
uppermost
and
function did not
right
patient
three
of cervical affect the
thoracic of
and
The scoliosis apices and
vertebrae,
vertebrae shape
before
(Figs. the new
freed
after
operation.
was also improved. Radiographs the disappearance of the” cervical
from
7 and 8). neck (Figs.
the
ribs,
Traces 9 and THE
had
acquired
of periosteal 10). JOURNAL
OF
the
showed thorax” appearance
ossification
BONE
AND
JOINT
on
the
SURGERY
SURGICAL
TREATMENT
OF
THE
KLIPPEL-FEIL
10
FIG. Back
Comment-I performed
believe that with a careful
deformity
which
though optimistic
the
of
the method technique.
hitherto
was
of the method, bear out these
view
not
patient
before
of treatment It provides
considered
and
after
operation.
described appreciable
unsuitable
easily obtained conclusions.
449
SYNDROME
for
because
here is free from serious risk correction of an ugly congenital
surgical
of the
A larger
treatment.
rarity
of these
if
experience
cases,
will
Organi
di
probably
REFERENCES
U.
ALDROVANDI,
(1642):
L. (1950):
BELLONI,
M.
BERTOLOTTI,
Monstrorum
11 Mito (1920):
Historia,
Degli
Le
Acefali.
anomalie
p. 401.
Rivista
congenite
Bonon:
Ciba, del
N. Tebaldini.
4, 761.
rachide
cervicale.
Chirurgia
degli
Movimento,
4, 395. M.
BERTOLOTTI,
Minerva
GIl
(1950):
CHAPELLE,
Medica, 41, 481. E. H. La (1934):
Nederlandsch
Tijdschrift F.,
DELITALA,
37,
and
collo
(Aplasia
C.
Medi;che en Geneeskunde,
sociale 78,
indicaties 4,638.
(1952):
Osteotomia
e fusione bij
Iombare
dcl
de
rachide
tuberculose
posteriore.
cervicale van
Chirurgia
con
beenderen
degli
Organi
spina
bifida).
en
gewrichten.
di
Movimento,
148. A.
HALLER,
Surgery,
von
30-A,
Icones
W.
e Arebon
A.
vol. FElL,
38 B,
A. Vandenhoeck. Indications,
and
Results.
Journal
of Bone
and
Joint
Tip.
propos
d’une
observation
d’ost#{233}otomie
cervicale.
M#{233}moires de
191. (1912):
Osteotomia
Un de
cas
Ia columna
Delle
sedi
di Sanson
Coen
1847
M.
N.,
Deformity
NO.
IV, A.
A
412.
B. (1746):
SMITH-PETERSEN,
of Flexion
Gottingae:
Technique,
d’absence
des
vert#{232}bres cervicales.
Nouvelle
Iconographie
de
223.
(1951): G.
J. (1951):
77,
and
Salp#{233}tri#{232}re, 25, MORGAGNI,
Osteotomy.
PAILLOT,
Stone,
M.,
KLIPPEL,
and
de Chirurgie,
HERODOTUS:
Anatomicae.
Vertebral
680.
J. J.,
HERBERT,
l’Acad#{233}mie
LAW,
(1743):
J. J. (1948):
HERBERT,
VOL.
voor
PAls,
senza
uomini
2,
LARSON,
in Rheumatoid
MAY
1956
C.
vertebral.
e cause
di malattie.
lettera
48 a, pp.
B.,
and
Arthritis.
AUFRANC,
Journal
CirugIa Vol.
del
3,48.
Aparato
(Tradotto
8, 113.
Locomotor,
in italiano
con
note
di f. Chussier
50. 0.
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of Bone
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Osteotomy Joint
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the 1.
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for
Correction
Ia
