THE
MANAGEMENT
OF
AND
H. S. From
the
Nuffield
The
Centre
and
is uncommon
case
reports
and
that
clinical problems haemophiliacs and
that
collaboration
the
follow
were
patients
with
and
LONDON,
Orthopaedic
Haemophilia met.
KEMP,
J. M.
the
Oxford
at
the
the
had,
however,
reduced
protective
bled
he fell from elsewhere.
there
no
was
sensory
each
swelling
fractured
of the
first
of the
elbow.
olecranon
in a plaster post-operative employing then daily removed VOL.
back
days On
process it
a potent
concentrate;
nine
days.
cover
with
50 B, NO. 2,
MAY
1968
bruising radial
frozen
was
process patient
haemostasis and
Research
in
Council.
fixed
AHG
was
finger
was
of flexion
which
and
the
was
forearm
were
restricted
by simple
of the ulna and a comminuted was given a litre of fresh frozen
fracture plasma
to
given
He
working
showed
to encourage of
the
by a screw.
given
bleeding.
While
Radiographs
considerable was
cent of AHG stoker, which
operations.
movements
head
uneventful.
with 9 per as a naval
FiG. 2 1-At the time of injury. and screw fixation.
and
justifiable
plasma
where
conditions
of the left elbow,
present.
fragmented
involved
considered porcine
and
pulse
the
reduced
often of the
Oxford,
spontaneous
and
in 90 degrees
held
of the left elbow. Figure later, showing the reduction
Convalescence fresh
injury
was
day
Medical
in preventing
after
a fracture-dislocation
elbow
operation was
AHG
sustained
third
was
The
for and
of
occasions
to establish
the
of the
not
aspects
orthopaedic
affected haemophiliac despite heavy work
and of the coronoid (Figs. 1 and 2). The
two
splint. bleeding
level
the
for
O.ford
are
various Centre,
treated
Unit
Hospital,
patients
illustrate
the
and
Churchill
REPORTS
considerable
loss
fractures of the olecranon of the head of the radius on
and
The
Orthopaedic are
Research
low
admission
There was
the
ENGLAND
in haemophilic fractures
disease
on several
FIG. I fracture-dislocation 2-Two months
1-The Figure
mid-pronation.
pain,
of
a roof
On
Case
in
effect
excessively
Centre,
Nuffield
Christmas
man aged fifty years was a mildly not previously suffered haemarthroses
shows
as a slater
Haemophilia
with
Coagulation
HAEMOPHILIA
OXFORD,
fractures
CASE
Case 1-A and he had
MATTHEWS,
therefore
seen
IN
DISEASE
of patients
with
Blood
FRACTURES
CHRISTMAS
some radius
The
limb
soft-tissue provide
After for the
was
two
weeks days
the
of the
immobilised and
AHG
before
eight first
excised;
damage
maximal
immediately
reduction
was
to avoid cover by
operation
the
plaster
and
was
of mobilisation. 351
352
H.
FIG.
S. KEMP
AND
J.
M.
MATTHEWS
3
FIG.
4
-ii 7 FIG. 8 Case 2. Figure 3-The fracture of the left ankle on admission. Figure 4-Three months later. Figure 5-The supracondylar fracture of the right femur at the time of admission. Figure 6-Three months later. Figure 7-The fracture of the left tibial plateau on admission. Figure 8-Three months later. FIG.
THE
JOURNAL
OF
BONE
AND
JOINT
SURGERY
THE
Case
2-A
MANAGEMENT
man
globulin
of the
with
impaction
three
fractures
injuries
right
of plasma.
The
superficial
injuries
later
femur,
and
fractures
and
cuts
all the
four
steady
in
large
daily
clinical
split
that
were
united.
neck
of the
had left
sustained fibula
and
a transverse
casts.
CHRISTMAS
In
the
absence
view
of
the
with
from
been
no
fracture
of the of the
left
left
the
patella
AHG,
Case 3-The
9 to 11).
was
active
he
extensive
movement
had
been
stopped, level
given
was
on each immobilised fracture
Skin
the foot.
The
one
4-A
repeated knee
joints
days
before
not
50 B,
He
delayed
given
plaster sensory
forty-nine, affecting he
had
lost
distal
were
Clinical
must
have
two
days’
red
cells.
flexion injuring
was the
fractured. year after
mid-calf
and
Four
days
indicated a litre
that
of
fresh
no further ultimately
bleeding. returned
with no circulatory most of the larger to On
15 degrees examination
of the thigh. on movement.
9-
was
no
admission bleeding
the
in good
Figure injury.
there
before
because
were
restricted right hip.
distending the anterior aspect lateral rotation, with severe pain
1968
with
fractures
splint. There was and motor function
active
fallen,
the
11
was also lI-One
considerable
treatment
The
to the
present.
assessment
been
a severe haemophiliac at one time or another
in which
admission
2, MAY
was
was
pulses
plasma. loss
of packed and
in particular
NO.
of fresh
posterior
man aged haemarthroses
sensibility
fall
of
On admission
FIG.
and left fibula. The patella weeks after injury. Figure
peripheral
blood
pints
large diffuse haematoma flexion, abduction and VOL.
litre
cent.
four
in a long was
Case
and
Figure
previous
of 57 per
day
plateau 10-Twelve
bruising. of
although
left tibial
suffered
_____
10
FIG.
fractures of the weeks after injury.
Three
there
9
had
a fracture
U FIG.
other months
a characteristic a stumbling
plateau, (Figs.
and
Three
circulating
his
infusions
dental
obtained.
tibial
of
daily
of the left knee, which showed He was admitted eight days after fracture
3 to 8), and all
multiplicity
by three
of bleeding
haemophiliac
antihaemophilic a supracondylar
the left ankle (Figs. was manipulated
followed
had
353
DISEASE
no circulating and sustained
AHG
haemostasis
a comminuted
and
with accident
of human
adequate
3-A man aged forty-two, a severe many previous haemarthroses, particularly arthropathy and limitation of movement. he
plaster
infusions
Case
in which
AND
left tibial plateau and The ankle fracture
bruises.
recovery
indicated
fractures
HAEMOPHILIA
ofthe and
immobilised
given
IN
a severe haemophiliac was involved in a road
of a tooth were
he was
FRACTURES
aged forty-four, or Factor VIII),
(AHG
fracture
OF
had
haemoglobin frozen
position
plasma
and
were
Union of the to normal. AHG, joints, in each. there
The hip was Radiographs
had and
had the
Two was a held in showed
354
H.
S. KEMP
AND
J. M.
MATTHEWS
intertrochanteric fracture of the right femur. The patient’s haemoglobin was 62 per cent and for this reason packed cells were transfused. Soft-tissue damage was assumed to have occurred at the time of fracture, so initially human AHG was given. The hip was supported in a padded plaster spica which was split. Daily infusions of plasma were given for a further
an
days.
four
Twelve
without
weeks
later
the
fracture
was
consolidated
Case
5-A completely. haemarthroses,
man,
aged
eighteen,
Other males especially before
had
very
in his family in the right
Four
days
a considerable distance home. He attended hip. On examination the hip was observed passive flexion limited to about 15 degrees
soft-tissue femur.
swelling. Because
treatment
was
the
patient
was
mobilised
he
had
fallen
Radiographs there
given
was
and
no
heavily
showed reason
a well
haemophilia
an
to suspect
padded
on
to his
his
blood
lacked
He had suffered be flexed above
right
side.
He
AHG
many previous a right angle.
rose
unassisted
and
hospital because of increasing pain in the to be flexed, laterally rotated and abducted, by
pain
and
undisplaced spica
muscle
linear
excessive
plaster
and
afflicted. could not
bleeding was
spasm.
fracture into
applied.
the The
There
was
of the
neck
tissues,
no
subsequent
no
of the infusion progress
uneventful.
Case 6-A He
admission
severe
were similarly knee, which
drove right with
was
and
complications.
had
boy had
of eleven numerous
had
been
diagnosed
haemarthroses
and
as a haemophiliac the left knee was
at the age severely
of eighteen affected.
months. Nine
days
I..
1
iii FIG.
Case 6-The Figure
12-On
*
tJi 12
spiral
FIG. fracture
admission.
of the shaft
Figure
of the
13-Twelve
13 right
femur.
weeks later.
before admission he fell and sustained a spiral fracture of the right femur He had been transfused with a pint of fresh plasma and a pint of packed cells
(Figs. before
Despite this, on admission of packed cells and a single
so a further pint proved refractory
his haemoglobin dose of human
level was only AHG were given. THE
53 per cent, The anaemia JOURNAL
OF
BONE
AND
12 and 13). admission.
JOINT
SURGERY
THE
and
two
was
needed.
further
later
it was on
uniting
transfusions was
with
was
of the right of these episodes
on
each
Case 7-A months.
of the
first
many
and that concentrate on the
the
had
been
day.
Five
weeks
later
boy
of nine
factor
had
severe
(Factor
IX).
was
to the
Centre
bleeding surgical the VOL.
50 B,
being
actively
four
days
later
the
soft
tissues.
Christmas
of
was NO.
He
In 1957
had
evidence
and
many
his
he tripped
2,
to
discharged MAY
1968
he
had The
factor
were
extend
the
with
a flexion
deformity
fractures
were
given knee
a residual
on
Union
flexion
deformity
age
and
was
been
traction bleeding
At ten swimming
all
the
weeks pool.
deficient
commonly
15
fell,
knee
occasions of
five
admitted
completely
of
Figure
only
the
upper
end
a litre of fresh plasma level. When transferred of 90 degrees because
to prevent fracture
14-
fracturing
undisplaced.
of the
of
bleeding
bleed into the left knee which treated elsewhere by traction.
of the
several
six a
While on traction Correction of the
in the
blood
relatively
joint.
by
sufficient only to give a frozen plasma was given
haemarthroses
he had a severe was successfully
when
he had
of union.
FIG.
mobilised
the
excessive
was in good alignment, heal well without undue
gentle mobilisation weeks.
disease
had
of
transverse fracture ofthe upper end ofthe left tibia and fibula. At the time of admission. Figure 15-Three months later.
manipulations
patient
Christmas
in a plaster
of human AHG. cover of plasma,
at
history
and fibula (Figs. 14 and 15). He was transfused with and a pint of packed cells to restore the haemoglobin
into
concentrate
cover under
haemophiliac
The
14
FIG.
left tibia bleeding
a severe
radiological
Three months before admission contracture of9O degrees. This
Case 8--The
patient
was
six weeks
home
deformities of the knees. fracture of the right femur.
to permit at thirteen
or AHG
and
uneventful.
variousjoints.
flexion
there
Case 8-A in Christmas
joints. a flexion
sent
damage was relatively slight, it was thought AHG on the first day for haemostasis and fresh
sufficiently consolidated able to walk in a caliper
of the control
as
plasma
splint
When he was readmitted was twice complicated
was
been obtained by this time and, as the fracture On the assumption that the fracture would
second
with
subsequently
left shoe. mobilisation
in AHG.
affecting
of bilateral supracondylar
was was
The
treatment
355
DISEASE
in a Thomas’s
recovery
diagnosed
ofhaemarthrosis
soft-tissue of human
CHRISTMAS
on each occasion under was restarted cautiously
deficient
the fracture The patient
affected caused
but
Subsequent
completely
to the Centre for the correction he had sustained a spontaneous deformity had was continued.
united
days.
no further
the
under
soundly
AND
traction
he was
a patten
three
episodes
but
balanced
knee, aspirated physiotherapy
boy of fourteen His blood was
included
given
by
malalignment;
with
fracture
IN HAEMOPHILIA
were treated
some
walking
the
haemarthrosis After each
OF FRACTURES
fracture
crutches,
later
given
blood The
spica
weeks
MANAGEMENT
was 10 degrees.
Infusions bleeding not
delayed
to of
of a during and
356
H. S. PRINCIPLES
Transfusion
therapy-The
replacing used
the deficient
are
and
fresh
concentrates
activity of
which
involves
plasma,
infused
blood
concentrations,
infusing
prepared
and,
it
TREATMENT
by
may
be
injured from
human give
The
materials plasma,
duration
on
infusions
at
of
or animal
The
is dependent
to
is that
factor.
human
plasma.
haemostasis
necessary
haemophiliac
or Christmas
prepared
from
because
the
AHG
of AHG
factor is short
MATTHEWS
presented
concentrates
factor
J. M.
ANTIHAEMOPHILIC
always
of Christmas
of the
high
OF
AND
problem factor
frozen
KEMP
the
of the
maintenance
least
once
a
day.
Treatment is limited by the volume of fluid which can be given intravenously, by the shortness of supply of materials derived from human plasma, or by the risk of the development of inhibitors to AHG following the administration of animal concentrates. In general, there are certain principles in treatment. Fresh human plasma has the easiest availability
because
kilogram
it can
of body
cent.
Plasma
during
the
be
weight.
is used later
stages
stored
in the
frozen
state
Within
an hour
to prevent
bleeding
from
of more
severe
of healing
it produces minor
Concentrates
of AHG,
are several
of producing this material
high may
prepared
injuries,
injuries,
wound dressing or the removal of sutures. When be given twice daily in order to obtain high AHG to prevent fluid retention. plasma,
ether
for volume,
or cold
more
levels render
of AHG. The large number its use in adults impracticable of children, in whom high of the blood volume. commercially from pig and
ten
human
plasma.
Suitably
or twice
a day.
However,
days
of treatment.
level
more
can
than
be achieved
to its use, the
potent
usually
alternate
once after
animal
again
develops.
such
reactions
a week
source
Severe
may
to ten
may
then
reactions
are
necessitate
produce now
a change
and
to cover
continuous The presence
surgery
in the
uncommon
severely
materials
used
in the
treatment
Christmas
as
This tends
is in the adult
inhibitor.
a normal
to become until though
occasionally
potentially
same
animal
management
are
antigenic concentrate
of very who
with human treatment If blood
from
sensitisation
are
haemophiliacs
disease
AHG
refractory
of concentrate
materials
ofthe
to produce Its main
is approximately
of response
materials
human
are capable
be economically
to adults
these
courses
fresh
and
may
Administration
affected
of
from
plasma,
plasma.
period The
of the cells.
procedures
donors required for short courses.
patient
with
the titre packed
treatment
such
of AHG
high level protection, which cannot be achieved ofcirculating inhibitor to AHG contra-indicates
containing AHG as this may increase case it is administered as saline-washed The
ox
a further
of treatment.
more
levels
the
they contain protein ofanimal origin. Repeated should be administered with the greatest caution. The main indications for the use of animal AHG and
fresh
administered
because
injuries
cover
precipitation
than of blood except
application is in the treatment obtained with little disturbance AHG is also prepared times
to
is the only available treatment it can in the blood, but care must be taken
volume
times
as a continuation
and
plasma levels
by alcohol,
potent,
1 5 to 20 millilitres are given per in AHG from nil to about 20 per
and
a rise
extracts. with any is needed
plasma
and
severe
need
long
material in such
Factor
a IX
concentrate. Plasma, which need not be freshly collected, may be used for the same indications as in haemophilia but it is less effective in the treatment of Christmas disease. The Factor IX concentrate of Christmas factor is prepared in the M.R.C. Research Unit laboratory. It is a potent material which is in short supply and is not presently available for use outside the controlled General of
any
during
supervision
of the
management-Three injury
Unit.
in haemophilia:
healing, and First, appropriate
principles
important
production
immobilisation materials
until the next transfusion lowest level to which the
of
are initial
of the injury. should be infused
is given. patient’s
It is important clotting factor
involved
in the
haemostasis,
in a dose
sufficient
when planning will fall, because THE
haemostatic
management
maintenance
JOURNAL
of
to maintain
haemostasis
haemostasis
treatment to consider the a recurrence of bleeding OF
BONE
AND
JOINT
SURGERY
THE
will
MANAGEMENT
be largely
tissues
OF FRACTURES
determined
need
higher
extensive the Secondly,
by this
clotting
injury daily
of the
injured
transfusions
to
This
often
blood
to delay
from
or
past
any and
of the
the
importance
that
that,
of
the
time
usually
necessary
ofrecurrent
absolute
of this and
bleeding
and
and soft-tissue
The anaemia associated be necessary to administer
fractures,
cases
more
immobilisation
haemarthroses
undisplaced
damaged
general,
risk
of bleeding. and it may
therapy
some
in
shortest
to fractures,
In
infusion
and
357
DISEASE
observation
is a considerable
equally
cells.
CHRISTMAS
others
causes a recurrence may be considerable, packed
healing,
there the
applies
bleeding is usually small. Infection did not complicate
be expected
AND
than
because
emphasise
part.
of whole
It is known
concentrations
injury,
injuries because movement with fractures in haemophiliacs of
HAEMOPHILIA
the concentration required. should be continued over
for the healing ofthe particular during this period. Finally it is necessary protection
level.
factor
the higher infusions
IN
series.
however,
Infection
the
would,
immobilisation
in such
amount
however, cases
would
need to be extended. Manoeuvres such as changing a plaster should be covered by infusion of the appropriate factor. When wounds are sutured, the sutures are left for a longer period than is usual, and if they are removed carefully bleeding is usually insignificant. Occasionally it is considered justifiable to give the appropriate factor before the removal of sutures. To avoid this problem it is good practice to approximate the edges of the incision with interrupted subcutaneous catgut and to close the skin edges with Steri-strip.” “
and
Soft-tissue bleeding in the limb vessels with consequent neurapraxia
ischaemia
been
have
been
described
observed
of a haemophiliac and ischaemia.
complicating
( 1 957)
by Fraenkel
may produce compression Peripheral gangrene and
untreated
and
by
soft-tissue
others.
injuries.
Because
of
these
of nerves Volkmann’s
Such
hazards
effects no
have
fracture
or
other lesion in a haemophilic limb should be immobilised in a complete plaster unless haemostasis is absolute and the swelling is diminishing. Consequently, all plasters should be adequately padded and completely split. During the period of immobilisation it is essential to exercise the
unaffected
limbs,
haemarthroses. Fracture
because
healing
disuse
in haemophilia
treatment radiographs
with coagulation factors show little periosteal
periosteal
reaction
(Fig.
but
may
render
is not
the
delayed,
calcification
more
even
during the whole callus formation.
widespread
joints
susceptible
in patients
who
to have
period of fracture repair. In one of our cases there
of
the
surrounding
subsequent not
received
However, the was a limited
periosteal
haematoma
13). DISCUSSION
The literature on fractures in haemophiliacs the association of fractures with haemophilic (1948) and Egeberg, Borchgrevink and Hjort suggested
that
following
a comprehensive in
haemophilia
unite
he postulated
well
that
hyperaemia,
the
Five
of
relatively
slight
callus
susceptible
to fracture
associated
osteoporosis.
VOL.
50 B,
NC).
2,
MAY
growth
six
formation,
reported
cases
because
bony
cases, the are of the
Consequently
union,
usually
in
the
for
which
exception
epiphyses
in both
the
in numbers
limitation the
of joint fractures
were that
movement, to
and
be
Kemp
our
stress
muscle lesions
same
(1966)
response
to
haemophilic
published,
haemophiliacs poor
time:
to the
the
with
in
fractures
average due
and
identical
believe
tend
the
(1958)
that
is a local
normal
radiographs virtually we
than
Boldero
affected
the
he said
is probably
However,
being
Jordan
and
in less
in haemophilia
to fractures
few
in twelve,
epiphyses.”
occurs
is inevitable.
fractures
formation
is common
Jordan’s
1968
solid
of affected that
which
patient.
the
with
formation
reported
ofcallus
overgrowth
a factor
Although
and speed
“
suggested
pseudotumour
of 1 10 patients
that the stimulates
that
influence
non-union
review
is sparse. Several authors have overemphasised cysts or pseudotumours. Ghormley and Clegg (1960) described such cases. Harrison (1964)
showed Case
6. are
function near
more and joints
358
H.
showing evidence by severe trauma
S. KEMP
of haemophilic and these were
AND
J. M.
arthropathy. in apparently
MATTHEWS
In only normal
two of our bone.
As Jordan pointed out, the most surprising feature is that union is not delayed, despite the osteoporosis and Basing his hypothesis on the work of Rizza (1961), who is raised in both normal persons and in haemophiliacs (1966) postulated that AHG may possibly be fixed in may therefore neighbouring
take place soft tissues.
locally at the An associated
This
that
healing
suggests
fracture
fracture site observation
in these
cases
the contention of Urist and McLean response to trauma, which initiates
(1941) fracture
I
in seven
cases
were
fractures
caused
of fractures in haemophilic patients the absence of plasma clotting factors. showed that the plasma level of AHG as a result of intensive exercise, Trueta the medullary tissue and that clotting
even though is the relative
is largely
endosteal.
that it is the healing.
haematoma,
bleeding continues lack of periosteal This
in the callus.
observation
rather
than
supports the periosteal
SUMMARY
The
.
management
disease 2.
fractures
haemophiliacs
and
one
patient
with
Christmas
is described.
The
soft
of
problems
of
management
are
essentially
those
fractures,
which
associated
with
haemorrhage
into
the
tissues.
3.
There
is no
periosteal
delay
in the
healing
of
usually
occurs
with
a relative
lack
of
callus.
4.
The
principles
We
thank
of transfusion
therapy
are
discussed.
Professor J. Trueta, Dr Rosemary Biggs and Professor R. G. MacFarlane for permission to publish and for their help and useful criticism during the preparation of the paper. The prints of radiographs were prepared by Mr D. Drury, formerly Photographer of the Nufiueld Orthopaedic Centre. Human AHG concentrate was processed and supplied by the Blood Products Laboratory ofthe Lister Institute, animal AHG
the cases
by Maws
of Barnet,
Coagulation
Research
and Christmas Unit.
factor
concentrate
by Dr E. Bidwell
of the Medical
Research
Council
Blood
Similar
Blood
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