Severe Complications of Reflex Sympathetic Dystrophy: Infection

Transcription

Severe Complications of Reflex Sympathetic Dystrophy: Infection
424
Severe Complications of Reflex Sympathetic Dystrophy:
Infection, Ulcers, Chronic Edema, Dystonia, and Myoclonus
L~]ckle van der Laan, MD, Peter H.J.M. Veldman, PhD, R. Jan A. Goris, PhD
ABSTRACT. van der Laan L, Veldman PHJM, Goris RJA.
Severe complications of reflex sympathetic dystrophy: infection, ulcers, chronic edema, dystonia, and myoclonus. Arch
Phys Med Rehabil 1998;79:424-9.
Objective: To determine the prevalence, type of complication, predisposing factors, and treatment for severe complications in a population of reflex sympathetic dystrophy (RSD)
patients.
Design: Retrospective analysis of the data from RSD patients
collected over a 12-year period, to investigate the involvement
of predisposing factors in an RSD population without severe
complications compared with an RSD population with severe
complications.
Setting: Outpatient clinic of a department of surgery of a
university hospital.
Patients: A total of 1,006 patients with the diagnosis of RSD
established according to prospectively defined criteria.
Main Outcome Measures: The signs and symptoms of
every RSD patient who visited the department were prospectively documented in the medical history; these data were
retrospectively analyzed with special regard to RSD with severe
complications--infection, ulcers, chronic edema, dystonia,
and/or myoclonus--for prevalence, type of complication, and
treatment.
Results: Seventy-four RSD patients who were mostly young
and female developed severe complications. More than one
complication occurred in 91% of the affected extremities.
Severe complications developed more frequently in the lower
extremity (65%). In patients in whom the acute RSD started
with a decreased skin temperature of the affected extremity,
severe complications developed significantly more often than in
acute RSD patients with a warm skin temperature of the
extremity from the onset of the disease (p < .001).
Conclusions: It is important to recognize "cold" RSD
immediately at the onset of the disease because this group of
RSD patients has a higher risk of developing a severe complication, mostly followed by a severe disability that is resistant to
therapy.
© 1998 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and
Rehabilitation
EFLEX SYMPATHETIC dystrophy (RSD) is a syndrome
generally occurring after trauma or after an operation on an
R
extremity. In some cases, it may appear spontaneously. 1 The
From the Department of Surgery, University Hospital Nijmegen, Nijmegen (Drs.
van der Lann, Goris), and Department of Surgery, St. Joseph Hospital, Veldhoven
(Dr. Veldman), The Netherlands.
Submitted for publication February 10, 1997. Accepted in revised form July 25,
1997.
No commercial party having a direct financial interest in the results of the research
supporting this article has or will confer a benefit upon the authors or upon any
organization with which the authors are associated.
Reprint requests to L. van der Laan, MD, Department of Surgery, PO Box 9101,
6500 HB Nijmegen, The Netherlands.
© 1998 by the American Congress of Rehabilitation Medicine and the American
Academy of Physical Medicine and Rehabilitation
0003-9993/98/7904-436853.00/0
Arch Phys Med Rehabil Vol 79, April 1998
injury initiating RSD may be a contusion, dislocation, or a
fracture of an extremity. In prospective studies, 2,3 the incidence
of RSD after Colles fracture varied from 7% to 37%. Carpal
tunnel release, tumor resection, arthroscopy, or nail extraction
are examples of operations on extremities that can induce
RSD.4,5
At present, there is no consensus about the pathophysiology
of RSD. As its name indicates, one theory is that the illness is
induced by an abnormal reflex of the orthosympathetic system.
However, blockade of the orthosympathetic system is not
always an effective treatment. 6-9 For this reason, RSD was
recently renamed Complex Regional Pain Syndrome (CRPS). 1°
Sudeck in 194211 introduced the hypothesis that RSD is based
on an exaggerated inflammatory response to injury or operation. In a prospective study by Veldman et al, 1 it was shown that
the acute phase of RSD is characterized by classical signs and
symptoms of inflammation (edema, increased skin temperature,
redness of the skin, limited range of motion, and pain).
Histologic and scintigraphic studies support this theory, 12,13 in
which oxygen-derived free radicals and/or neuropeptides may
play a role. 14,~5RSD is difficult to treat, but the best results are
obtained when it is diagnosed early and treated immediately. 14,16,17
Only 20%-30% of RSD patients return full-time to their
previous employment. 17 In our experience, patients with a
severe complication such as infection, ulcers, chronic edema,
dystonia, or myoclonus of the affected extremity are very
difficult to treat. Such complications generally lead to severe
additional disability. For this reason, we retrospectively analyzed the extensively and prospectively documented data of a
population of RSD patients with severe complications for
prevalence, type of complication, and treatment. To investigate
a possible involvement of predisposing factors, we compared
the data (medical history and clinical signs and symptoms) from
the RSD patients without a severe complication within this
population to the data from the RSD patients with severe
complications.
PATIENTS AND METHODS
All new patients presenting at the outpatient clinic of a
university hospital department of surgery were examined for
signs and symptoms of RSD. The following were the criteria for
the diagnosis of RSDI:
1. At least four of the following: unexplained pain within the
affected area; difference in skin color compared with the
other extremity; edema of the affected area; difference in
skin temperature compared with the other extremity;
limited active range of motion.
2. The occurrence of, or increase in, the above signs and
symptoms after the extremity was used.
3. Above signs and symptoms present in an area larger than
the area of primary injury or operation, including the area
distal to the primary injury.
These signs and symptoms present at the time of the first
examination were noted in a fixed patient record file. ~ During
the period January 1, 1984 to January 31, 1996, 1,006 patients
RSD AND SEVERE COMPLICATIONS, van der Laan
met the criteria for RSD. Almost 90% of these patients visited
our department only once for a second opinion or for therapeutic advice. From this selected population, no conclusions could
be drawn as to the prevalence of severe complications in RSD
patients.
RSD patients presenting at our outpatient clinic have been
examined since 1984 according to an extensive standardized
protocol, and the data entered into a computerized patient
record file. One part of this file consists of data obtained by a
questionnaire and includes age at the time of the visit to our
outpatient clinic, age at the onset of the disease, gender, the
initiating cause of the RSD (eg, trauma, surgery, spontaneous,
others), the patient's description of the skin temperature of the
affected extremity at the onset of the RSD, the presence of RSD
in another extremity, and modes of treatment before the patient
visited our outpatient clinic. The file also included data obtained
by physical examination of the patient as described by Veldman
et al. 1 The pain of the affected area was scored by the Visual
Analog Scale, while edema (present or absent) skin color (blue,
red, normal) and skin temperature (cold, warm, normal) of the
affected extremity were assessed clinically and documented in
comparison to the unaffected contralateral limb. 1 Limitation of
the active range of motion was measured with a goniometer.
Infection, ulcers, chronic edema, dystonia, and myoclonus of
the RSD extremity were noted. From this documented population of RSD patients, we retrospectively studied the subgroup of
RSD patients that developed (recurrent) infection, ulcers,
chronic edema, dystonia, and/or myoclonus of the affected
extremity. According to the Dystonia Medical Research Foundation, is dystonia is defined as a syndrome of sustained muscle
contraction, frequently causing twisting and repetitive movements, or abnormal postures. The dystonia seen in our study
consisted of severe focal dystonia with abnormal posture of the
affected extremity such as a clenched fist or an inversion and
equinus position of the foot. Myoclonus was defined as
involuntary, brief, jerklike movements, produced by muscle
contraction. 19Dystonia and myoclonus belong to the movement
disorders of RSD. 2°-22
In this population of RSD patients with severe complications,
we also analyzed cases suspected of auto-mutilation.
Statistical analysis was performed by Fischer's exact test,
two-sided. For the age range only, the Kruskal-Wallis test,
two-sided, was performed. The population of RSD patients
without a severe complication was compared with the population who developed a severe complication in their RSD
extremity. If an RSD patient was found to have complications in
more than one extremity suffering from RSD, the data from the
extremity first affected with RSD (primary RSD extremity)
were used when describing the patient population. When
describing the complications, the extremities affected with RSD
were described. The level of significance was set at p < .05.
RESULTS
Within the documented population of 1,006 RSD patients, 74
patients (10 male and 64 female) developed a severe complication in the primarily affected extremity (table 1). In 5 patients, a
complication developed in a second extremity with RSD, while
in 2 patients complications occurred in all four extremities with
RSD. In the RSD complication population, the age varied
between 15 and 69 years (median 35 years). The severe
complications were localized in the upper extremity in 26
patients and in the lower extremity in 48 patients. The skin
temperature of the affected extremity noted by the patient at the
onset of RSD (primary skin temperature) was cold in 46
patients and warm in 19 patients; in 9 patients no difference in
425
Table 1: Population of RSD Patients Without
and With Severe Complications
Without
Complication
n
Total
Age range (median)
Gender
Female
Male
Extremity
Upper
Lower
Primarily cold skin
temperature
Multiple RSD
Etiology
Trauma
Surgery
Spontaneous
Other
With
Complication
%
932
10-84 (44)
n
%
74
15-69 (35)
P
Value
<.001
699
233
75
25
64
10
87
13
.02
578
354
62
38
26
48
35
65
<.001
280
120
30
13
46
21
62
28
<.001
<,001
621
182
94
35
66
20
10
4
44
20
5
5
59
27
7
7
*
*
*
*
*Not significant.
skin temperature was observed or remembered at the time of
RSD onset.
In 44 patients, RSD developed after injury (ie, contusion,
dislocation or fracture); in 20, it after an operation (ie, carpal
tunnel release, arthroscopy of the knee); in 3, after an inflammatory process (ie, paronychia, infected ulcer); and in 2 patients,
RSD developed after an injection. In 5 patients, no precipitating
factor was found.
RSD developed in a second extremity in 21 patients and was
present in all four extremities in 6 of the 21. In 15 of these
patients, the RSD developed in another limb without any
precipating factor, in 4 after injury, in 1 after an operation, and
in 1 patient after an injection.
Most patients from this RSD complication population were
referred to our outpatient clinic after intensive treatment
elsewhere. The medical treatment of these patients in other
hospitals most frequently consisted of intravenous regional
sympathetic blockade and/or sympathetic blockade. Intravenous regional sympathetic blockades with guanethidine were
performed in 28 of these 74 patients before they visited our
outpatient clinic, with no improvement in 27 patients (96%) and
with some decrease of complaints in 1 patient. In 32 of the 74
RSD patients who developed a severe complication, a sympathetic ganglion block (stellate ganglion blockade for the upper
extremity, lumbar sympathetic block for the lower extremity)
was performed in other hospitals. Only 2 patients showed some
improvement, whereas in 30 cases (94%) the sympathetic block
had no success.
In comparison with the data of our overall population
(n = 932) of RSD patients without severe complications, the
patients (n = 74) with complications in the primary extremity
were younger (p < .001) and more often female (p = .02)
(table 1). Also, the skin temperature at the onset of RSD was
significantly colder in patients with complications (p < .001).
Severe complications developed more frequently in a lower
extremity affected by RSD than in an upper extremity
(p < .001). Development of the RSD in another limb occurred
more frequently in the complication group of patients (p < .001).
The severe complications of RSD were analyzed individually
for each extremity affected (table 2). In total, 154 complications
were seen in 85 extremities in 74 patients. A combination of
Arch Phys Med Rehabil Vol 79, April 1998
426
RSD AND SEVERE COMPLICATIONS, van der Laan
Table 2: Severe Complications in 85 Extremities With RSD
in 74 Patients
Upper
Extremity
Severe
Complication
Infection
Ulcers
Chronic edema
Dystonia
Myoclonus
All complications
Lower
Extremity
Total
n
%
n
%
30
26
27
50
21
9
3
3
19
9
30
11
11
38
43
21
23
24
31
12
70
89
89
62
57
154
43
28
111
72
In 78 (91%) of the limbs suffering from RSD a combination of
complications in one extremity was seen,
complications in one extremity was present in 78 of these 85
limbs (91%). Presentation of a single complication was seen in
7 extremities, including infection (n = 1), ulcers (n = 3), and
chronic edema (n = 3).
Infection as a complication was seen in 30 extremities.
Ulceration of the skin and/or chronic edema was frequently
followed by infection of the affected limb (n = 24). In 13 of
these cases, a subsequent amputation was necessary. The
infection consisted in 9 cases of erysipelas due to Streptoccocus
haemolysis group [3, and in 4 cases the chronic edema was
followed by cellulitis due to Staphylococcus aureus. In two
cases, erysipelas was controlled successfully with intramuscular long-acting penicillin (on our advice, a dosage of 2 million
units), whereas in one case penicillin was ineffective and
amputation was necessary. In the 6 other cases, erysipelas was
treated in our hospital with a combination of intravenous
mannitol (100 grams per 24 hours) infusion 23 and intravenous
penicillin (6 million units per 24 hours), curing the infection in
5 cases, and failing in one case, in which there was a subsequent
amputation.
In 5 patients, RSD developed in an area with a traumatic or
surgical wound, contaminated with anaerobic bacteria. Appropriate antibiotic therapy performed in other hospitals, in
combination with debridement, did not cure the infection. In
one of these patients, who had a chronic ulcer after a superficial
second-degree burn wound complicated by RSD of the hand,
debridement, intravenous antibiotics, intravenous mannitol, and
verapami124 healed the ulcer and reduced the RSD complaints.
In 4 patients, the ulcerating wound infected with anaerobic
micro-organisms was resistant to the above therapy, but was
cured by transplantation of a free revascularized myocutaneous
flap performed in our hospital.
Ulcers were observed in 26 RSD extremities. Ulceration was
followed in 73% by infection of the extremity with RSD.
Chronic edema as a complication of RSD was seen in 27
extremities (fig 1). In 18 RSD extremities (67%), chronic
edema was followed by infection. Chronic edema progressed to
mimic elephantiasis in 5 cases. Because of the coexistent
hyperpathy, compressive stockings were tolerated in only 3
patients, reducing the edema in these cases. Elevation of the
edematous extremity was the only alternative, and 55% of the
patients with this problem in the lower limb used a wheelchair
(mostly on our advice) to avoid a continuously dependent
position of the leg.
Dystonia as a complication of RSD was found in 50
extremities, localized in 19 upper extremities and 31 lower
extremities. Dystonia of the upper extremity resulted in fixed
flexion of the finger flexor muscles, leading to the so called
"clenched-fist" syndrome (fig 2A). In the lower extremity, the
dystonia presented in 30 extremities as an inversion and
Arch Phys Med Rehabil Vol 79, April 1998
equinus position of the foot (fig 2B) and in one case was seen as
a dystonic plantar flexion. This severe complication of RSD
was resistant to any therapy. Mobilization of the affected
extremity under general anesthesia performed in other hospitals
temporarily improved range of motion in some cases, but was
subsequently followed by recurrence of the dystonia and
exacerbation of RSD signs and symptoms. Our auxiliary
therapy consisted of providing an orthesis, which supported the
limb in the presenting position, for comfort and protection.
Tenotomy or lengthening tenoplasty of the finger flexor tendons
was only performed in our hospital when, because of the
"clenched fist," daily hygiene became impossible or pressure
spots developed. Dystonia always persisted after this operation
but was less severe and allowed for better care of the hand.
Myoclonus as a complication of RSD was found in 21 limbs,
localized in 9 upper extremities and in 12 lower extremities.
This complication was mostly present in an extremity that had
RSD with almost no residual function. For these RSD patients,
we provided an orthesis for rest in a comfortable position. In 3
patients, treatment of the affected muscle group with botulin
injections failed to stop the myoclonus. In 9 RSD patients,
dystonia and myoclonus were present together.
In 51 RSD patients, movement disorder (dystonia and/or
myoclonus) developed in 62 extremities. These movement
disorders were complicated by infection, ulcers, and/or chronic
edema in 25 limbs (40%).
Auto-Mutilation
In this population of RSD patients with severe complications,
auto-mutilation was regularly suspected. In these cases, it
Fig 1. Severe chronic edema (elephantiasis) and trophic ulcer of the
foot.
RSD AND SEVERE COMPLICATIONS, van der Laan
!i ~ !
427
il ¸¸
i i i!ili i !;!i !
Fig 2. Typical dystonia in an RSD extremity: (A) "clenched-fist" syndrome; (B) inversion and equinus position of a foot.
proved difficult to establish or exclude auto-mutilation as a
cause of the complication. Nine patients with severe complications were suspected of auto-mutilation by other specialists. We
could confirm auto-mutilation in 5 patients and disclaim this
diagnosis in 3 patients. In 1 patient, the concerned extremity
was amputated before the patient was seen by us.
All 3 RSD patients, inappropriately suspected of automutilation bad a chronic therapy-resistant ulcer with infection.
These ulcers all healed after a split-skin transplantation or
covering with a free revascularized myocutaneous flap.
In 5 RSD patients, we confirmed auto-mutilation. In 4 of
these patients, edema was recognizable in an area distal to a
sharply delineated skin grooving, induced by pinching off a
limb. The extremity affected by auto-mutilation was not the
primary extremity suffering from RSD.
One patient who was cured from RSD in a hand that was
complicated by infection and ulcers had a recurrence of RSD
after a coin-sized burn on the dorsal side of the same hand.
DISCUSSION
In a population of 1,006 RSD patients, 74 patients (7%)
developed one or more severe complications in the affected
extremity because of infection, ulcers, chronic edema, dystonia,
or myoclonus. As far as we could determine, except for case
reports, no single study has addressed the prevalence of such
complications in a large population of RSD patients. Severe
complications occurred more frequently in younger and female
patients. The initiating factor for the development of RSD was
similar in RSD patients with or without complications. RSD
patients with one severe complication are at risk to develop
subsequent complications in the affected extremity. Progression
of RSD to another extremity was seen frequently in patients
who had complications, compared with RSD patients without
complications.
Classically, RSD develops in three phases, based on the
alterations in skin temperature. During the beginning period (2
to 3 months), the skin is warm, followed by a phase of
vasomotor instability lasting several months, and ending with a
cold phase. 25,26 In a previous report, 1 13% of the patients who
had RSD started with a cold skin temperature, while others
remained in the first "warm" stage for more than 10 years.
Forty-six of the 74 RSD patients (62%) who developed a
complication in the affected extremity had a cold skin temperature from the onset. Also, in the population of RSD patients who
finally required an amputation of the affected extremity, a cold
skin temperature was found significantly more frequently at the
onset. 27 In our view, the subdivision of RSD into primarily cold
and primarily warm is clinically relevant because of the worse
functional outcome of the primarily cold RSD, 28 and because of
a higher incidence of recurrence or development of RSD in a
second extremity29 and the development of severe complications.
Arch Phys Med Rehabil Vol 79, April 1998
428
RSD AND SEVERE COMPLICATIONS, van der Laan
Because it has been demonstrated that in "cold" RSD blood
flow to the affected extremity is significantly decreased, 13,3°
while in "warm" RSD it is increased, and because there is
evidence that in both "cold" and "warm" RSD the oxygen
extraction is decreased, 31 we hypothesize that the worse outcome of primarily "cold" RSD results from severe tissue
hypoxia. Accordingly, in treating RSD patients since 1984,
"primarily cold" RSD is treated early and vigorously with
vasodilators such as verapamil retard (240 mg/24 h), ketanserin, 32 or sympathetic blockade to optimize perfusion of the
affected extremity. 28
In RSD extremities complicated by recurrent infections,
treatment with only antibiotics was frequently not effective, and
in 13 of the 30 infected RSD extremities a subsequent
amputation was necessary. In our opinion, infected RSD
extremities are frequently resistant to conventional therapy
because of impaired oxygen consumption in the RSD extremity.31 RSD patients with chronic ulcers were often suspected of
auto-mutilation because of the failure of conventional therapy.
By curing the infected ulcer with a split-skin transplantation or
a free revascularized myocutaneous flap, the suspicion of
auto-mutilation could be disclaimed.
In our population of RSD patients, skin lesions consisted
only of ulcers. We never noticed purpuraiike inflammatory
dermatitis or bullae as described by Webster and colleagues. 33
An RSD extremity with ulcers and or chronic edema is at risk of
developing severe infection. In such cases, amputation eventually may be necessary.
In 1984, the movement disorder described in 4 RSD patients
was interpreted as a distinct clinical syndrome of RSD. 2°
Schott 21 introduced the theory that causalgia induces involuntary movements. Schwartzman and Kerrigan 22 reported 15 RSD
patients with severe dystonia in a population of 43 RSD patients
with movement disorders in a total of 200 RSD patients.
Dystonia associated with RSD was reported in 9 RSD patients
by Jankovic and van der Linden. 33 Bhatia 35 described 18 RSD
patients with dystonia. In this series, 16 patients were female
and the upper extremity was affected in 12 cases. In the present
study, 51 RSD patients were found to have a movement
disorder in 62 extremities. In 40% of these affected extremities,
the movement disorder was complicated by infection, ulcers,
and/or chronic edema. This association with other complications has not been reported previously. It is unknown whether
the central nervous system (central and subcortical reorganization 35,36) or the peripheral nervous system (by action of
neuropeptides 22) generates the movement disorders of RSD.
The disability resulting from severe dystonia and from
chronic edema is immense. In our experience, further efforts at
curative therapy in these cases are ineffective and should be
stopped in favor of an approach that accepts the situation and
looks for solutions for the resulting disability.
Cooperation with a rehabilitation specialist is required for
adaptation of the home situation. In addition, the RSD extremity should be supported with an orthesis; if the lower extremity
is affected, a wheelchair should be provided.
CONCLUSIONS
In a population of 1,006 RSD patients, 74 patients, mostly
young and female, developed a severe complication in 85
extremities affected by RSD. In 91% of these extremities more
than one complication occurred. RSD patients with a severe
complication have a higher risk for the development of RSD in
another limb. Severe complications in an extremity suffering
from RSD most frequently occurred when RSD started with a
cold skin temperature. In the end phase of RSD with severe
complications, these patients have to be supported by providing
Arch Phys Med Rehabil Vol 79, April 1998
an orthesis, a wheelchair, or adaption of their home situation, as
appropriate.
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Arch Phys Med Rehabil Vol 79, April 1998