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Synovectomy and total joint arthroplasty for recurrent hemarthroses in the arthropathic joint in hemophilia.

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Severe hemophiliacs with intractable bleeding into
one or more joints despite adequate clotting factor replacement therapy are difficult management problems.
Synovectomy has controlled bleeding only in joints without significant arthritic destruction. Total joint replacements have been performed in arthropathic joints, but not
when uncontrolled bleeding was a concurrent problem.
This report describes a hemophiliac with uncontrolled
bleeding into an arthritic knee who was successfully managed by combining synovectomy with total knee replacement.
Severe hemophiliacs with clotting factor levels
less than 2% of normal have frequent, seemingly spontaneous hemorrhages into multiple joints (I ). Some severe
hemophiliacs have been successfully managed with clotting factor replacement therapy either on a prophylactic
basis or self-administered at the first sign of hemorrhage
(2). Despite adequate replacement therapy some severe
hemophiliacs continue to have frequent hemorrhages
From the Divisions of Orthopedic Surgery and Rheumatology. University of California at Los Angeles, Torrance, California.
James T. London, M . D.: Division of Orthopedic Surgery.
University of California at Los Angeles/Harbor General Hospital,
Torrance, California: James S. Louie, M.D.: Division of Rheumatology, University of California at Los Angeles/Harbor General Hospital, Torrance. California.
Address reprint requests to James T. London, M.D., Division of Orthopedic Surgery. University of California at Los Angeles/Harbor General Hospital. 1000 West Carson Street, Torrance,
California 90590.
Submitted for publication April 14. 1977; accepted May 27.
Arthritis and Rheumatism, Vol. 20, No. 8 (November-December 1977)
into one or several joints (2,3-5). I n these patients hernarthroses result in a friable, hypervascular synovium
which is prone to bleed even with normal or restricted
use of the joint. Excision of this abnormal synovium has
been successful in eliminating recurrent hemarthroses in
some cases (4-7). Synovectomy had not decreased the
incidence or severity of hemarthroses, however, when
the preoperative x-rays revealed moderate to far-advanced arthropathic joint destruction (5). Presumably
the arthritic joint is prone to recurrent synovitis and
hence to recurrent hemarthroses.
Total joint replacements have been performed in
hemophiliacs with arthropathic joints (8.9). There are,
however, no reports to o u r knowledge of total replacements in joints where frequent, uncontrolled hemarthroses were a concurrent problem. We would like to
report on a severe hemophiliac with intractable bleeding
into a severely arthropathic knee. In this case neither
synovectomy nor total knee arthroplasty alone would be
expected to eliminate the hemarthroses. Synovectomy
was therefore combined with total knee arthroplasty to
eliminate hemarthroses and to resurface the destroyed
joint surfaces.
A 30-year-old black male with hemophilia A was first
seen in November 1973. He had more than 200 prior hospitalizations for treatment of hemarthroses. In the previous 2
years he had noted a marked increase in hemorrhages into the
right knee and left ankle. By November 1973 he was hernorrhaging at least once each week into his right knee or his left
ankle. These acute hemarthroses were managed with rest, immobilization, and Factor VllI (Abbott) replacement therapy.
When bleeding subsided, isometric exercises and protected
ambulation were resumed, and Factor VIll therapy was continued a t 20 units/kg each 48 hours.
Despite this management he continued to hemorrhage
into the right knee and/or left ankle on a weekly basis. In the
90 days prior to his orthopedic evaluation he had been hospitalized for hemarthroses eight times (42 days) and had been
infused as an outpatient on four additional occasions. He was
receiving an average of 4,200 units of Factor V l l l each week. In
addition to bleeding the patient complained of increasing pain,
decreasing motion, and crepitation in both the right knee and
left ankle. The patient required crutches t o ambulate even
short distances within his home.
On initial examination the right knee showed marked
inflammation and synovial hypertrophy with active motion
Fig. 1. Preoperative weight-bearing x-ray of the right knee.
from 45 to 85 degrees. The quadriceps muscle was atrophic.
Examination of the left ankle revealed marked synovial hypertrophy with active motion from 5 degrees dorsiflexion to 30
degrees plantar flexion. Roentgenograms of the right knee indicated severe arthropathic changes (Figure 1).
After 8 months on the previously described management. the patient had not improved and was, therefore, considered for surgery. Because the recurrent hemorrhages into
the right knee were associated with far-advanced arthritic
changes. a synovectomy and total knee arthroplasty were
planned. The left ankle was to be arthrodesed at the same time
because of the expense of Factor V l l l coverage. Hematologic
testing preoperatively revealed 0% Factor VIII leuel; screening
for inhibitors to Factor V l l l was negative. On the morning of
surgery the Factor V l l l level was brought to 100% with the
administration of 2,800 units of Factor VIII.
Two surgical teams simultaneously performed a right
Fig. 2. Postoperative x-ray oJ the right knee.
knee synovectomy and geometric total knee arthroplasty and a
left ankle arthrodesis (Campbell) (Figure 2) (10). Total estimated blood loss was 1,500 cc. At surgery, the capsule and
synovium of the knee were noted to be markedly thickened,
acutely inflamed, and hypervascular. Pannus extended across
most of the articular surface with only a few remaining islands
of degenerated cartilage. Similar findings were present in the
left ankle.
Postoperatively the patient was infused with sufficient
Factor V l l l to keep his level at 50% of normal for 14 days.
Additionally. he was given 1.300 mg/kg/day of epsilon amino
caproic acid (EACA) ( I I ). Isometric quadriceps exercises were
resumed on the first postoperative day and active ranging was
started on the fifth day. Two weeks after surgery Factor VlII
therapy was reduced to a single dose just before physical therapy to transiently raise the level to 30% and EACA was continued as before. This regimen was continued until 5 weeks
after surgery when the Factor V l l l and the EACA were discontinued. The patient was discharged 6 weeks after surgery.
After discharge, the patient received no Factor V l l l
or EACA. Fourteen weeks after surgery the left ankle arthrodesis was considered to be consolidated and immobilization
was discontinued. Six months after discharge the patient was
fully ambulatory and asymptomatic. The right knee moved
actively from 5 to 85 degrees and the quadriceps muscle was
strong. I n the first 6 months after surgery the patient sustained
only one hemarthrosis in the right knee. This hemorrhage
occurred during physical therapy and was less severe than the
preoperative hemorrhages. Subsequently, the patient has received 500 units of Factor V l l I three times each week just
before physical therapy sessions. On this program he sustained
only two additional hemarthroses in the last 28 months-one
in the right knee and one in the left elbow. Thirty-four months
after surgery he is fully ambulatory without crutches and has
recently returned to college.
The outlook for a severe hemophiliac prior to
availability o f specific clotting factor concentrates was
dismal. Kerr reported 75% of severe hemophiliacs were
disabled by age 16 (12). The availability of clotting
factor concentrates for the last 10 years has resulted in
more aggressive treatment o f acute hemarthroses. This
may lead to a decrease in the incidence o f arthropathic
joints ( 2 ) . The recent introduction of home therapy with
self-administration of clotting factor concentrates either
at t h e first sign of joint hemorrhage or on a prophylactic
basis has further improved the outlook for the severe
hemophiliac ( 2 ) . Despite aggressive replacement programs severe hemophiliacs may continue to hemorrhage
into one or more joints as in the case reported here.
S y n ovect om y can eliminate hem art hroses when
arthropathic destruction is not already present. Synovectomy alone has not been successful in eliminating
recurrent hemarthroses in joints with far-advanced arthropathic changes (5,7). In our case, synovectomy was
therefore combined with total knee arthroplasty. Arthritic changes of moderate severity in the ipsilateral
ankle and contralateral knee were considered to be contraindications to arthrodesis of the right knee. The combined procedures in this case dramatically reduced the
incidence of hemarthroses from one a week to less than
one a year. The patient's requirement for Factor VIII
decreased from 4,200 units per week to 1,500 units per
week. The patient has been hospitalized only four times
in the last 34 months and his activity level has increased
to a point where he is currently attending college.
I . Briggs R. McFarlane RG: Treatment of Haemophilia and
Other Coagulation Disorders. Philadelphia, FA Davis.
2. Telfer MC: Home transfusion programs in hemophilia.
Springfield, 111, Charles C Thomas Co. 1972
3. Pool JG: Recent chapters in the Factor V l l l saga-perils
of a protein. West J Med 122:406, 1975
4. Storti E. Traldi A. Tosatti E, Dovali PG: Synovectomy, a
new approach to haemophilic arthropathy. Acta Haemat
41:193. 1969
5. Storti E. Ascari E: Surgical and chemical synovectomy.
Ann N Y Acad Sci 240:316, 1975
6. Ewald BE. Myers M. Crooks J: Synovectomy in hemophilia-a preliminary report. J Bone Joint Surg 57A:139,
7. Pietrogrande V, Dioguardi N , Mannucci PM: Short-term
evaluation of synovectomy in haemophilia. Brit Med J
2:378, 1972
8. D'Ambrosia RD. Niemann KMW, O'Grady L. Scott
CW: Total hip replacement for patients with hemophilia
and hemorrhagic diathesis. Surg Gynecol Obstet I39:381,
9. Post M. Telfer M: Surgery in hemophilic patients. J Bone
Joint Surg 57A:I 136, 1975
10. Campbell CJ. Rinehart WT, Kalenak A: Arthrodesis of
the ankle. J Bone Joint Surg 56A:63. 1974
1 1 . Storti E, Ascari E, Turpini R, et al: Epsilon-amino-caproic
acid for synovectomy in haemophilia patients. Acta Haemat 47: 146, I972
12. Kerr CB: The Management of Hemophilia. Sydney. Australian Medical Pub1 Co, 1963
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hemophilia, arthropathy, tota, recurrent, joint, hemarthrosis, synovectomy, arthroplasty
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