in Hemophiliacs
T. A. Wallny, P. Pennekamp, H.-H. Brackmann, P. Hofmann, C. N. Kraft and A. Seuser
Introduction
In the juvenile and adolescent hemophiliac with no or only inadequate substitution therapy, the calf muscles and the ankle joint remain one of the primary localizations of bleeding episodes [1, 2]. Subsequent fibrous atrophy after recurrent bleeding typically causes an ankle equinus deformity, which may functionally be complicated by an extension-deficit of the knee-joint [3].
In cases where non-surgical management options remain ineffective, moderate- ly simple operative techniques such as Achilles tendon lengthening and simulta- neous dorsal capsulotomy have been propagated [3-5]. The aim of this study is to assess long-term clinical and radiographic results after Achilles tendon lengthening for hemophilic equinus, in an effort to determine whether this procedure is a feasi- ble management option.
Materials and Methods
Between 1975 and 1986, 30 hemophilic patients with secondary equinus deformity of the ankle due to recurrent bleeding episodes underwent Achilles tendon lengthening.
Of these, 23 patients, all with severe hemophilia A (factor VIII less than 1 %), could be followed up. 12 were operated on the right side, 11 on the left side. The average age at the time of operation was 28.7 years (range 14 to 46). Clinical results were assessed by using the section »clinical examination« and »pain« of the Advisory Committee of the World Federation of Hemophilia (WFH)-Score [6], while roentgenographic results were evaluated using the Petterson-Score [7]. The clinical score is still asses- sed prospectively at least twice a year, the radiographic score every two years.
The average preoperative WFH-score for the treated ankle was 4.2 points (min.
1, max. 8) and the average preoperative Petterson-score was 4.4 (range 1 to 10) points (see Table 1).
Preoperatively an equinus position averaging 21° (range 5-55°) was found.
The factors pain (0-3 points), bleeding (0-3 points) and physical examination (0-12 points) make up the clinical score (WFH), whereby a normal joint will have 0 points and a severely afflicted joint a maximum of 18 points. The radiographic score ranges from 0 points for a healthy joint and 13 points for apparent massive radiographic destruction. Average follow-up was 13 years (range 1 to 24).
I. Scharrer/W. Schramm (Ed.)
34thHemophilia Symposium Hamburg 2003
” Springer Medizin Verlag Heidelberg 2005
Operative Technique
The indication for surgical management was a persistent or a progressively deterio- rating equinus position of the ankle. Lengthening was performed by an open Z-inci- sion of the tendon and, if considered necessary, a simultaneous posterior capsule release of the ankle-joint. In case of a tendency to hindfoot valgus the distal lateral limb of the split tendon was detached and in patients with a hindfoot varus the distal medial limb of the incised tendon was detached. Postoperatively the treated lower leg and foot was fixed with a circular cast in a neutral position over a period of 6 weeks to maintain correction and allow tendon healing. After this, intensive physiotherapy was performed and patients were permitted to fully weight bear.
Factor Replacement
Factor substitution to achieve an activity of 30-40% was performed preoperatively.
Directly prior to surgery, factor activity was raised up to 80-100% by giving approx- imately 30-40 units per kg body-weight. A coagulation-test was performed post- operatively and, if required, factor was again given. Factor activity was kept at 60%
up to the fourth postoperative day and subsequently maintained at 50% until day 14 after surgery. In the following period factor activity was held at 20%. After rehabili- tation, a return to normal concentrations was allowed.
Results
The average WFH-clinical score (clinical examination) improved after surgery to 2.7 points (range 1 to 4). 11 (47.8%) ankles showed clinical improvement, 1 (4.4%) ankle showed a postoperative deterioration while 11 (47.8%) further ankles remained unchanged. Taking the postoperative observation period into consideration, patients who were observed over a period of up to 10 years (n=6) showed an average impro- vement of the clinical score by 3 points. Those patients with an observation period of over 10 years after surgery improved clinically by an average 1 point.
The Petterson-Score increased to an average of 6.9 (range 3 to 12) points. Here the radiographs of 18 (78.2%) patients indicated postoperative deterioration, 4 (17.4%) remained unaltered and 1 (4.4%) patient showed improvement. Radio- graphic assessment of patients 10 years or less after surgery (n=12) showed an aver- Table 1. Patients and Results
number average number follow-up clinical radiographic
of age of pro- (average) score score
patients cedures (points) (points)
23 28.7 years 23 11 years 4.2 4.4
at follow-up 2.7 6.9
age drop of the Petterson-Score by 2 points, while those patients radiographically over a period of more than 10 years deteriorated radiographically by an average 4 points.
The average postoperative equinus position was 10° (range –4° to 20°). Of the 20/23 (87%) patients with a measurable improvement of the ankle equinus at follow- up. Only 7 patients still had complete persisting correction of the initial deformity.
The range of motion (ROM) of the ankle for dorsal extension and plantar flexi- on was improved in 8 patients (average 10°) and deteriorated in 15 patients (aver- age 9°), suggesting that an improvement of the plantar flexion was frequently asso- ciated with a loss of dorsal extension.
6 patients had a persistent reduction of pain while 17 patients remained unchanged.
Achieved improvement of the postoperative equinus position did not signifi- cantly alter over the observation period: an average measured postoperative im- provement of 16° of the equinus deformity was measured. Overall these remained unchanged over the subsequent observation period. Even after 15 and 20 years, the increase of ROM compared to preoperative values were much the same as after initial surgery (Fig. 1). In contrast the time-span between surgery and follow-up had an influence on the total ROM of the ankle. Scrutiny of plantar flexion and dorsi- flexion showed values more or less unchanged 1, 5 and 10 years postoperatively (on
--- postoperatively ---
pre- 1 5 10 15 20 25 years
Pes equinus [-°]
-30 -25 -20 -15 -10 -5 0 5 10
(n=23) (n=23)
(n=22)
(n=19)
(n=12)
(n=8)
(n=1)
Fig. 1. Average change of the severity of equinus deformity pre- to postoperatively (95% con- fidence interval; negative values = equinus deformity, pos. values = dorsiflexion possible).
Deformity showed an average improvement of 16° pre- to post-Achilles tendon lengthening, and remained almost unchanged over the subsequent years (n=number of patients)
average 6° higher than preoperatively). After this, average ROM of the ankle joint tended to deteriorate, and at 15 years postoperatively values equal to or worse than the preoperative situation were documented (Fig. 2). In this context it must be pointed out that for the last two observation periods relatively few patients were assessed. Therefore conclusions can only be drawn very tentatively.
Pearson’s coefficient of correlation showed no association between the clinical score (discrepancy pre- to post-operative), equinus deformity and the improvement of ROM (p>0.145-1.0). Furthermore a correlation between the preoperative clinical and radiographic score and the equinus deformity, respectively the ROM was not found. The preoperative situation obviously hat no statistically verifiable influence on the postoperative ROM.
Discussion Clinical Results
Achilles-tendon lengthening is a probate method for the treatment of hemophilic pes equinus. As from an equinus contracture larger than 30° tendinous correction is usually insufficient and additional corrective wedge-osteotomy should be con- --- postoperatively ---
pre- 1 5 10 15 20 25 years
ROM [°]
0 5 10 15 20 25 30 35
(n=23)
(n=23) (n=22) (n=19)
(n=12)
(n=8)
(n=1)
Fig. 2. Mean values of pre-and post-operative total range of motion in plantarflexion and dorsiflexion after Achilles tendon lengthening for hemophilic equinus deformity (95% con- fidence interval; n=number of patients).
templated. Interestingly we found no suture-insufficiencies in our patients, despite the fact that in some cases a marked lengthening of the Achilles tendon was per- formed.
Though no surgery of the ankle joint as such was performed, tendon length- ening in general had a positive effect on the patients post-operative clinical score of the ankle. This substantiates the conclusion that by tendinous correction and the simultaneously achieved indirect slackening of the periarticular soft-tissue struc- tures, the motion pattern of the ankle joint is brought closer to the physiological situation. Whether surgery had an influence on the intraarticular structures of the ankle cannot satisfactorily be answered by this study. The attempt to rectify the motion pattern of the ankle may have a positive effect on intraarticular cartilage.
Through Achilles tendon lengthening the ankle’s overall range of motion is only slightly improved, as a gain of extension is typically associated with loss of flexion.
Johnson and Babbitt (1985) [8] could show a correlation between the loss of ROM and the successive receding of articular cartilage in the hemophiliac. One can the- refore hypothesize that the preoperative documented loss of ROM in our patients is not solely a result of the ankle equinus deformity but probably has multiple factors.
Consequently Achilles tendon lengthening treats only part of the problem.
This study shows that Achilles-tendon lengthening is an acceptable method for treatment of hemophilic ankle equinus deformity. Nonetheless it must be pointed out that these results also suggest that in the long-run there is a tendency of overall loss of ankle ROM. This may be due to the almost always associated arthropathy of the ankle. Most of our patients had some degree of radiographically verified arthro- pathy of the treated ankle prior to surgery. Whether loss of motion is due to a pro- gressive arthropathy or possibly based on an insufficient surgical management can- not conclusively be answered through this study.
Radiographic Results
The long-term postoperative radiographic assessment of our patients showed that, despite surgical management, joint damage in hemophiliacs with an ankle equinus deformity continually progresses. This finding is not new. In previous studies we could demonstrate that operative procedures in the vicinity of afflicted hemophilic joints do not necessarily prevent further destruction [9,10]. Yet untreated or only conservatively managed equinus deformities also show continuous arthropathy [11]. It therefore seems that with or without surgical management subsequent de- terioration of the ankle joint is inevitable and most likely not a direct result of Achilles tendon lengthening.
Conclusion
The majority of patients treated for hemophilic pes equinus by Achilles tendon lengthening showed a temporary or permanent functional improvement. Long term improvement of the equinus deformity is achieved by the procedure, yet this is fre-
quently associated with loss of plantar flexion. Achilles tendon lengthening does not prevent progression of hemophilic ankle arthropathy. In view of the fact that in a significant number of our patients the clinical status was improved, the procedure seems a feasible management option for equinus deformity of the ankle, with accept- able long-term results.
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