Functional Disorders and Treatment Modalities in Hemophilic Children

in Hemophilic Children

A. Seuser, G. Schumpe, H.H. Brackmann and T. Wallny

Introduction

Functional defects are identified by motion analysis today. Motion analysis is done in three spatial dimensions.

Functional analysis is the only way to detect subclinical functional defects of the knee joint. Independently of bleeds, x-rays, MRI imaging, and clinical and ortho- pedic procedures, motion analysis is a suitable means of identifying functional defects of coordination or strength in the muscles supporting a joint.

Why is it important to investigate functional defects of this kind?

An excursion into physics is necessary at this point. Knee joint motion is cru- cially determined by what is called the center of motion or center of rotation.

The way we move our knee joint determines the position of the respective cen- ter of rotation, and the position of the center of rotation determines the length of the lever, which, by definition, is at right angles to the forces acting upon the center of rotation.

Displacements of the center of rotation may therefore cause very major strains on the joint in a worst-case scenario. The increased strain goes unnoticed but may cause microtrauma in the joints and significantly impair joint integrity in the long term.

Material and Methods

Functional analysis has been conducted to date in 98 children (196 knees) by tread- mill and knee bend tests. The children’s ages ranged from 3 to 16 years. Analysis was conducted using an ultrasound-based motion analysis system (original ultrasound topometry). Ultrasound transmitters attached above and below the knee joint send ultrasound impulses to ultrasound receivers positioned at fixed points in space.

Run time is taken and, in the presence of a known ultrasound velocity and known distance between the receivers, the three-dimensional coordinates of the individual transmitter can be determined accurate to less than 1 mm. A software algorithm calculates the angle, angular velocity, angular acceleration and roll and glide motion of the knee joint under strain.

The studies were conducted in collaboration with hemophilia treatment centers in Berlin, Bonn, Bremen, Brunswick, Dresden, Erlangen, Erfurt, Frankfurt, Munich, Halle, Hanover, Potsdam and Würzburg.

I. Scharrer/W. Schramm (Ed.)

34

Hemophilia Symposium Hamburg 2003

” Springer Medizin Verlag Heidelberg 2005

Joint angle, angular velocity and angular acceleration curves during locomotion and during knee bends, and the roll and glide pattern of both knee joints during weight-bearing were assessed by qualitative criteria.

Results

Age-dependent typical deviations from healthy adult locomotion were identified.

Four main problems were pinpointed.

Three examples from different age groups are shown.

Figure 1 above shows a normal gait with shaded areas highlighting regular stance phase changes of 12° to 14° in a 16-year-old hemophiliac. The stance phase comprises the following cycles:

Fig. 1. Absent, inadequate or poor-

ly controlled stance phase activity

of the knee joint during locomoti-

on (shadowed: stance phase of

knee jointg).

1. Heel strike: the knee is almost fully extended as it touches down 2. Load transfer: the knee is first flexed, then extended

3. Toe off: the knee enters the swing phase from an almost fully extended position The larger downward excursions designate the swing phase.

Figure 1 in the middle shows a significant difference in a child aged 10.

The stance phase is indicated by a small tick only. Although the knee joint extends almost fully at heel strike, it is only flexed initially and transfers to the swing phase during flexion.

Figure 1 below shows a typical gait of a boy aged 3 at the time of investigation.

Gait analysis detects almost no real knee joint activity during the stance phase.

Full stance phase activity of the knee joint is very important because load sur- face transfer takes place and distributes body weight to the largest possible knee joint surface area. If there is no transfer of load-bearing surfaces, i.e. there is no change of angle during weight-bearing, the cartilage comes under strain at that point and overstrain may result in the long-term, with the possibility of microtrau- mata.

This is a brief illustration of the possible differences in leg axis compliance during gait. Figure 2 above shows fairly regular, rhythmic, sinusoidal lateral devia- tion in a subject walking on a treadmill.

The lateral deflections range from 10° to 25° and are well above normal.

However, because the deviation is regular, rhythmic and sinusoidal, it does not qua- lify as detrimental. However, it significantly increases the energy requirement and results in more rapid exhaustion.

10 – 25 °

4 – 5 °

Fig. 2. Leg axis deviation during gait

Figure 2 below: Another patient displays a significantly smaller degree of lateral deviation (4°-5°) but a more arrhythmic overall pattern with loss of sinusoidal cur- vature. Energy consumption is much lower but muscular control is more difficult as smaller amplitudes need to be controlled. However, the peak velocities and accele- rations are smaller, with the result that, on the whole, the smaller lateral deviations make for better kinematics despite the increased dysrhythmia.

Acceleration Peaks in Motion Transition Phases

This is mainly seen during knee bends (Fig. 3) but is also evident during weight- bearing phases of locomotion.

Figure 3 shows on top the knee bend angle of a 16-year-old male patient per- forming knee bends from 0° to 100°. The corresponding acceleration curve shows the abnormality that is not represented in the angle curvature.

Interim acceleration occurs near full extension in the acceleration curves be- tween ± 20°. This happens during each repetition only when nearing full extension and does not occur in flexion phases. Motion is otherwise regular and rhythmic.

These interim accelerations significantly increase the load on the joint during a very sensitive phase of motion, where forces are in transition from one direction to the other (similar to the baton exchange zone in a 4x100 m relay race, where diffi- culties are most likely to occur).

Fig. 3. Acceleration

peaks in motion

transition phases

(arrows)

This redirection of forces and the associated interim acceleration as shown in our example impose a shock and heavy load on the joint in a closely circumscribed area of cartilage. The cartilage can be assumed to come under heavy strain.

Loss of the Roll Element in the Roll and Glide Pattern during Weight-Bearing (Fig. 4) Figure 4 shows an ideal roll and glide pattern of a weight-bearing knee joint. This 16-year-old boy has a roll element of 25° when performing knee bends in the 20° to 90° range. The curve rises in an almost linear pattern with a high degree of repeat- ability and congruence from the lower left to the upper right. (Fig. 4 top)

In contrast, the curvatures observed in a 10-year-old subject are very different.

He produces the same linear rise when flexing his knees from 20° to 90° consistent with rolling (R). When straightening up from a hunkering position, gliding on its own is seen from 60° onward (G). (Fig. 4 middle)

Our 3-year-old subject produced the bottommost curve (Fig. 4). A roll and glide pattern is barely discernible apart from a negative roll (nR) with linear progression of the angle presented from the upper left to the lower right.

A perfect roll and glide motion is the ideal way of distributing load on the knee joint. Pronounced gliding elements in themselves are associated with oblique forces

Fig. 4. Loss of the roll element in the roll and glide pattern during weight-bearing (R = rolling, G = gliding,

nR = negative rolling)

and increased strain on joint elements. Negative roll can be described by the ana- logy of a car that rolls backwards when attempting to ascend a steep hill.

On the basis of these main functional defects, all of which increase the physical forces acting on the joints and thus may promote bleeding due to the higher load on the joints, an easily implemented physiotherapeutic activity program was deve- loped with the aim of counteracting the functional deficits with just a few exercises.

Care was taken to ensure that all the exercises included in the routine are easy to perform and feasible in the home situation under parental guidance.

1. Stance phase exercises for the knee joint (Fig. 5):

The figure above shows a fully normal process from right to left. Heel strike with the knee joint almost fully extended, transfer of body weight, knee bend, and raising of the leg almost fully extended for the swing phase.

This process should be performed carefully and deliberately in slow motion. The speed of the exercise can be increased once the subject has mastered the technique.

This exercise can be varied through the use of different surfaces (solid to more unstable surfaces) and by the application of resistance over the upper legs as shown in Figure 5 below from left to right.

Fig. 5. Stance phase exercises for the knee joint

Fig. 7. Leg axis exercises

Fig. 6. Transition phase training

2. Leg axis exercises

Lateral deflection of the leg during gait or knee bends can be counteracted by leg axis exercises. The training program should be started in slow motion, the child’s job being to keep his leg compliant to the load axis. An additional force may be applied from outside or inside to strengthen the axis muscles (Fig. 6).

3. Transition phase training (Fig. 7)

Acceleration peaks in the motion transition phase can be counteracted by transition phase exercises. The movement should be repeated in slow motion depending on where the acceleration peaks were identified, for example between 20° flexion and 20° extension.

If the motion transition defects were localized in squatting position, the motion transition exercises should mainly take place during knee bends. The exercise can be varied by starting on stable surfaces and progressing to unstable surfaces, by pro- gressing from slow to rapid motion, and by applying resistance (standing on a Theraband).

4. Training with weights

Loss of the rolling element in the roll and glide pattern during weight-bearing should be treated with suitable weight-lifting exercises (Fig. 8). The program should

Fig. 8. Training

with weights and

coordination

start in glide-only areas, with small excursion movements (escalation through co- ordination-enhanced effects). Weight training should be initiated precisely in the area where gliding takes place, starting with an endurance training. The size of the motions and also the weights should be increased gradually until a submaximal weight has been reached.

Negative roll phases should be treated by physiotherapy to start with. The pri- mary aim is to improve internal knee joint kinematics through manual therapy, including rotation in the knee bend situation.

Conclusion

As presented above, greater or lesser deviations from normal adult motion can be seen in the various age groups. Children with hemophilia should be encouraged at the earliest possible age to promote optimum inner knee kinematics in order to minimize external bleeding risks through overstrain and false motion as well as the internal bleeding risks. The largest existing study on this subject has clearly defined these abnormal movements and established an exercise program for prevention and control of these kinematic abnormalities. Follow-up studies after implementation of the exercise program can help show whether the small effort involved managed to improve the patient’s situation.

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Chairmen:

R. Schneppenheim (Hamburg)

M. von Depka Prondzinski (Hanover)