A sixty-seven-year-old black woman with a medical history of
hypertension and chronic obstructive pulmonary disease but no history of a
bleeding disorder presented with a two-day history of increasing pain and
swelling in the right forearm. The patient took aspirin (325 mg daily) as well
as medications for the treatment of pulmonary disease and hypertension. There
was no history of trauma. The patient reported a distinct onset of pain after
lifting her coat with the affected arm. The following day, approximately
twenty-four hours after the onset of pain, the patient noted increased pain
and swelling in the right forearm, hand, and fingers. Approximately
forty-eight hours after the onset of symptoms, she presented to the emergency
department.
Physical examination revealed that the patient had mild discomfort
secondary to pain in the right upper extremity. Clinically relevant findings
on examination were limited to the right forearm, hand, and digits. There was
tenderness to palpation extending from the right forearm to the distal aspects
of the digits; the tenderness was greater dorsally than volarly. Radial and
ulnar pulses were palpable at the wrist. The patient had mild forearm swelling
with soft compartments, intact sensation to touch, and warm skin throughout
the extremity. The site of greatest swelling was the mobile wad of Henry.
There was minimal discomfort to passive extension of the digits at the
metacarpophalangeal joints, with the greatest discomfort involving the second
and third digits. There was no tenderness to passive flexion and extension of
the wrist. Radiographs of the right upper extremity were negative for
fracture. The patient was admitted for observation.
Laboratory findings on admission included a hemoglobin level of 9.2 g/dL
(92 g/L) (normal, 11.5 to 16.0 g/dL [115 to 160 g/L]), a hematocrit of 31.8%
(normal, 35% to 47%), an activated partial thromboplastin time of 51.0 seconds
(normal, 27.0 to 38.0 seconds), a prothrombin time of 11.4 seconds (normal,
10.9 to 13.5 seconds), and an international normalizing ratio of 0.9 (normal,
0.9 to 1.1).
The patient continued to report increasing pain in the right forearm.
Specifically, she had increased tenderness to palpation dorsally as well as
increased pain to passive motion of all of the fingers and the wrist.
Compartment pressures were measured with a handheld intracompartmental
pressure monitoring system (Stryker Instruments, Kalamazoo, Michigan). At this
time, the blood pressure was 180/100 mm Hg. The findings included a mobile wad
pressure of 70 mm Hg, a dorsal compartment pressure of 50 mm Hg, and a volar
compartment pressure of 50 mm Hg. Concomitantly, it was noted that the patient
had begun to bleed profusely from the blood-drawing and intravenous line sites
on the contralateral arm. On the basis of the clinical findings, the
laboratory values, and the elevated compartment pressures, the decision was
made to proceed to the operating room for emergent fasciotomy for the
treatment of compartment syndrome secondary to an unknown coagulopathy.
The procedure began with a fasciotomy through Henry's volar
approach2 to the
forearm, with complete release of both the superficial and deep volar
compartments extending from the antecubital fossa through the carpal tunnel.
No hematoma or active bleeding was found. A dorsal fasciotomy was then
performed through Thompson's dorsal
approach3 to the
forearm. A large hematoma was found within the mobile wad; the hematoma
consumed the entire compartment. Evaluation of all of the muscles of the
forearm showed gross viability except for duskiness and decreased
contractility of the extensor carpi radialis brevis muscle. The wounds were
thoroughly irrigated. In order to allow complete decompression of the
compartments, no closure was attempted. No tourniquet was used
intraoperatively.
Postoperatively, the patient complained of incisional pain but had an
immediate decrease in pain on passive motion of the digits and wrist. A
consultation with the hematology service was obtained in the recovery room,
and additional serological testing was performed. Testing revealed a factor
VIII level of 0.14 U/mL (normal, 0.5 to 1.5 U/mL) and an activated partial
thromboplastin time of 49.5 seconds. Factor IX and XI levels were within
normal limits. Testing of the partial thromboplastin time with a mixture of
the patient's plasma and normal plasma with a two-hour incubation period
revealed values of 38.3 seconds immediately after testing, 42.3 seconds at one
hour, and 45.8 seconds at two hours. On the basis of the low factor VIII level
and the worsening partial thromboplastin time with two-hour incubation, the
patient was diagnosed as having an acquired factor VIII inhibitor. After
consultation with the regional hemophilia center, the patient was managed with
a factor VIII concentrate regimen of 5000 U initially, with interval dosing
every eight hours. On the first postoperative day, the patient was able to
actively move the fingers and wrist with minimal discomfort. On the second
postoperative day, the patient was returned to the operating room for wound
irrigation and reevaluation. Examination revealed no gross bleeding or
hematoma. Again, all of the forearm muscles appeared to be viable, except for
the extensor carpi radialis brevis, which continued to appear dusky. The
decision was made not to close the wounds at that time. On the fourth
postoperative day, the patient returned to the operating room for repeat
irrigation and wound closure. The volar incision was closed primarily with use
of 3-0 nylon on the skin only; the fascia was not closed. The dorsal incision
required a split-thickness skin graft.
The patient was followed as an outpatient. She received factor VIII
replacement under the supervision of the hematology service, and the factor
VIII level normalized. At the time of the three-month visit, the wounds were
healing well. The patient had grade-5 (of 5) muscle strength globally in the
hand and wrist, except for grade-4 wrist extension strength.
Production of acquired factor VIII inhibitor is a rare disorder
occurring in one in 1,000,000 to one in 5,000,000 individuals per
year4,5.
Clinical symptoms associated with acquired factor VIII inhibitor usually
include large, spontaneous ecchymoses and/or hematomas of unknown origin.
Acquired factor VIII inhibitor is primarily incurred through an IgG autoimmune
mechanism, similar to autoimmune disorders like systematic lupus erythematosus
and rheumatoid arthritis, in association with drug reactions, in the
postpartum period, and in the
elderly4-6.
Research has shown that unlike bleeding due to inherited hemophilia, which is
X-linked, acquired factor VIII inhibitor has an approximately equal gender
distribution7. Green
and Lechner, in 1981, also reported that almost 60% of their sample of 215
patients with factor VIII inhibitor were more than sixty years of
age7.
Laboratory results consistent with acquired factor VIII inhibitor include a
prolonged activated partial thromboplastin time and a normal prothrombin
time6. In patients
with acquired factor VIII inhibitor, factor VIII levels are well below normal,
while other factors are within normal limits. If the partial thromboplastin
time on testing of a mixture of patient plasma and normal plasma following one
to two hours of incubation is also prolonged, the presence of an inhibitor is
strongly
suggested6.
If acquired factor VIII inhibitor is suspected, prompt consultation with an
experienced hematologist is required to confirm the diagnosis and to
coordinate treatment. Depending on the bleeding status of the patient,
treatment may require transfusion of factor VIII concentrate, factor IX
concentrate, or recombinant factor VIIa. It has been reported that
approximately 38% of patients with acquired factor VIII inhibitor will have
spontaneous disappearance of the
inhibitor8. For
patients with continued factor VIII inhibitor, long-term management consists
of immunosuppression therapy aimed at decreasing autoantibody levels.
The recognition of a bleeding disorder as the cause of compartment syndrome
is important in terms of overall patient care. An increased compartment
pressure of >30 mm Hg does not necessarily warrant a fasciotomy for
patients with bleeding disorders, including those with factor VIII
inhibitor9. The
first-line treatment for patients with bleeding disorders consists of clotting
factor
replacement9,10.
This alone can stabilize compartment pressures. A fasciotomy is recommended if
the compartment pressure is unstable, if the compartment pressure does not
normalize within two hours after the initiation of treatment with clotting
factors, or if clinical symptoms
worsen9. There are
currently no specific perioperative guidelines for factor replacement levels
during fasciotomy in patients with acquired factor VIII inhibitor. However,
for patients with hemophilia A, it is recommended that factor VIII levels be
>50% to 100% of
normal9. On the
basis of criterion, preoperative and perioperative transfusion of factor VIII
concentrate may have an implied benefit for patients with acquired factor VIII
inhibitor as it may decrease perioperative blood loss during fasciotomy.
In the absence of a history of trauma, a clinician may hesitate to
attribute a patient's increasing pain and swelling to a compartment syndrome.
This delay could lead to permanent neurovascular and muscular damage. Although
the suspicion of compartment syndrome was raised immediately in the case of
our patient, diagnosis of the etiology of the compartment syndrome was
delayed. Appropriate clotting factor replacement was begun immediately after
consultation with a hematologist and confirmation of the diagnosis. With
rapidly worsening clinical symptoms at the time of presentation and without
active bleeding during surgery, it is unlikely that immediate factor
replacement would have changed the clinical course, treatment, or outcome.
Perhaps earlier detection and clotting factor therapy may have prevented the
need for surgery. We present this case for the purpose of improving
familiarity with this rare, acquired bleeding disorder as a potential cause of
compartment syndrome. ?
The authors did not receive grants or outside funding in support of their
research or preparation of this manuscript. They did not receive payments or
other benefits or a commitment or agreement to provide such benefits from a
commercial entity. No commercial entity paid or directed, or agreed to pay or
direct, any benefits to any research fund, foundation, educational
institution, or other charitable or nonprofit organization with which the
authors are affiliated or associated.