The Classic: On Juvenile Arthritis Deformans

Abstract

This Classic Article is a reprint of the original work by G. Perthes, On juvenile arthritis deformans. An accompanying biographical sketch of G. Perthes is available at DOI 10.1007/s11999-012-2432-2. The Classic Article is © 1910 and is reprinted from Über Arthritis deformans juvenilis. Deutsch Zeitschr Chir. 1910;107:111–159.

Appendices

Appendix I – Case Histories

Unilateral Cases

1. Ida A., age 8.⁴ Parents and three siblings live and healthy. From previous diseases, especially symptoms of rickets are not reported. 7 year old child was kicked while throwing a snowball into a ditch, but was not in bed, a doctor who examined the child the day after the fall “found nothing.” Soon after, his parents noticed the child was limping. There was moderate pain in the left hip which disappeared after a few months. The child is brought because of limp. There is no pain even after prolonged walking.

Status April 26, 1909. Child is weak but shows no standing deformity of the spine. While walking and stepping on the left leg the upper body sways to the left every time. In the recumbent position both hip joints normal. Real shortening of the left leg. Anterior superior iliac spine – external malleolus right 61.4 cm, left 60.6 cm. Tip of the right trochanter almost 1 cm, left almost 2 inches above the Roser-Nélentonschen Line. No palpable deformity of region of the hip. No tenderness on pressure over the trochanter or the femoral head. Normal movements on the right. Left extension and flexion free. Adduction to 20° is possible and abduction completely abolished. When attempts at abduction the pelvis moves with it immediately. External rotation in extension is not limited, internal rotation is completely eliminated. All movements, if they are possible, are without pain or crepitus. The radiograph (Fig. 1) shows slight flattening of the femoral head in its upper and medial part. The acetabulum points directly below the center of small irregularities of the boundary line, flat prominences according to the cavity.

Fig. 13

Case 1 on March 17, 1909

Status June 12, 1910 In general similar to 14 months before. The limp has not become greater, no pain, but now leg length from the anterior superior iliac spine – external malleolus right 64.2, left 63.2. On the left diseased hip joint, free flexion, abduction absent, adduction to 10° possible. In flexion, the thigh is in an approximately 30° abducted position, from which he may be abducted or adducted further. Outward rotation in flexion to 60° possible inward absent. When standing on the left leg, the right half of the pelvis drops significantly down to the inclination of the upper body on the left (positive Trendelenburg sign). X-ray image: the flattening of the head has increased in the upper portions. The height of the epiphysis of the head has decreased from 16 mm to 10 mm. In place of the spherical shape of the head, the shape has entered a truncated cone. In the epiphysis in the whole thickness of the epiphysis shows irregularly defined bright spots. This suggests the normal bone part is missing.

2. Eugene H., 11 years. Parents and four siblings healthy. The boy himself was always weak, but never seriously ill, especially not from rickets. At the age of 9 years, he often complained about pain in his right hip, and, “went lame at times.” January 1909 the parents noticed an increase of limping. The pain in the region of the right hip varies in intensity, usually only slight and but for days painfree.

Status February 1, 1909. When standing, slight left convex lumbar scoliosis. Region of the right trochanter more prominent than on the left. Left anterior superior iliac spine is lower than the right. The boy is always bent left and stretches his right knee, with left knee in extension as the right heel rises from the floor. Limping slightly. When stepping on the left leg the trunk seems to sway more each time. Lying down the right leg is adducted by 10° and with similar abduction on the left. Right heel, right malleoli, etc., are about 5 inches higher than the left. Leg length from the anterior superior iliac spine – external malleolus, right 63.5, left 64.2 cm. Right trochanter almost 1 cm above the Roser-Nélentonschen line, left the same. No palpable abnormality of the hip area.

No pain, either by pressure on the joint or on the trochanter with impact against the heel in the longitudinal direction of the leg. Movements: flexion and extension movements in the right hip joint active and passive, totally free and painless, however, abduction and abduction and rotation completely absent. Even with little shaking the pelvis immediately moves. Left has completely free movements.

Fig. 14

Case 2 on June 12, 1910

X-ray (Fig. 3). The femoral head to the right shows the distinct shape of a cone or sugar loaf wide. The rounded tip of the cone is exactly opposite the center of the acetabulum. The conical shape of the head is due to the fact that the proximal epiphyseal line at the location of the head which contrast to the perfectly normal conditions on the other side. The distal location of the epiphyseal portion of the head and the femoral neck and shaft appear left and right, especially with respect to the normal angle of the femoral neck.

Status June 12, 1910. The boy received salt baths, but otherwise remained without treatment and has in spite of intermittent pain regularly attended school. The pain presents itself only after some time, several hours of walking, but also after prolonged sitting at school, but appears to have eased in recent months. The limp is especially noticeable when the child is tired. The status differs from the previous years only in that when lying there is adduction of the right hip to compensate for the tension on the right leg. It is now up to 20° abduction, adduction to 30°, rotation to 15° in either direction possible, flexion is still completely free. Limb length from the anterior superior iliac spine- external malleolus, right 66.0 cm, left 66.9 cm. No tenderness, no pain during motion. Pelvic brim to the prominence of the trochanter measured on both sides is the same (31 cm). When standing on the right leg, the left half of the pelvis is lower by 2–3 cm below the horizontal (positive Trendelenburg sign). Nevertheless, despite freer movements in the past few years, X-ray image (see Figs. 4 and 14) now shows severe changes in the right hip: The flattening of the femoral head in its upper sections has increased, the proximal to the epiphyseal location of the head, the epiphysis has not increased, but rather in the last few years its former height of 12 decreased to 8 mm! The epiphyseal line now shows significant irregularities with several large bright regions, suggesting bone loss. Regions of the epiphyseal plate extend into the femoral epiphysis. The femoral neck and trochanter are unchanged. Normal structural drawing clearly visible. Left hip still shows normal radiographic findings.

3. F. Ernst, 13 years. Healthy parents. Of eleven children he suffered more neck gland swelling. According to precise information from the parents, at the age of about 10 weeks, he fell ill suddenly with heavy sweating and fever. The right leg was held in the hip joint rigid. “Any attempt to stretch the legs, caused the child severe pain.” The child was born on 6 August 1898 at the Leipzig University Clinic of Surgery. In the outpatient clinic of the journal entry is found: right femoral osteomyelitis. Therapy: lead lotion. According to the parents and a splint was made. The illness lasted about 5 months after complete healing was observed in the clinic. The child, “learned to walk at the age of 2 years.” There were no noted subsequent consequences of the disease. At the age of 10 years, the boy began to limp, however, and later to complain about pain after prolonged walking in the right leg. The right-sided inguinal glands were swollen considerably. Nevertheless, the boy went to school and after the first years of life was never been treated medically. There was no history of injury.

Status June 5. 1909. Well-developed boy looking healthy. At present no subjective complaints. He occasionally complains of pain in his right leg that causes him to stop but not to participate in the running games of his friends. When standing, there is a low convex right lumbar scoliosis, lumbar lordosis very slightly greater than normal. The pelvis is tilted with the right anterior superior iliac spine lower than the left. While walking, the torso tends to sway when the right leg to the right. In a reclining position of the pelvis and both hip joints normal. Real shortening of the right leg. Leg length from anterior superior iliac spine-external malleolus, right 67.4 left 69.4 cm.

Corresponding elevation of the right trochanter. No palpable deformity of the hip joints. The right hip is a bit sensitive to pressure. Pressure on the trochanter and slight impact in the longitudinal direction of the leg are sensitive. In the right groin are fairly hard, painful lymph glands, some the size of beans. Movements: in the right hip abduction by only 25° possible, adduction, flexion from 25° through a right angle of about 100° with internal rotation in the extended position 15° and 15° external, making a total of 30°. The movements are carried out without pain and without crepitation. Left hip is all normal movements. The radiograph (Fig. 7) shows that the right femoral head has largely disappeared. As is clear from the position of the epiphyseal line on initial impression, there is an impressively flattened head, perpendicular to the larger structure to wider part of the femoral neck, just in its uppermost sections of the small remains of the head cap. The height of the head and neck of the femoral intertrochanteric line on the left side is 5 cm, it measures only 3 inches to the right. Therefore the greater trochanter and the femoral axis have shifted by 2 cm from the inside to the acetabulum. The normal upper femoral neck-shaft angle has been lost. The rest of the head is nearly in the extension of the axis of the femur. On the head can be seen lateral bone “osteophytes.” The acetabulum is flattened and widened and above (with incipient enlargement of the acetabulum). The left hip is completely normal.

Fig. 15

Case 3 on March 16, 1910

Status June 12, 1910. The treatment has consisted of saline baths and massage of the gluteal muscles. At times, the boy had no particular cause of pain in either hip, despite the disease, but the pain in recent months had rather increased. The boy has not missed a day of school in the past year. Very slight limp. Upper body leans when stepping on the right leg to the right. When standing on two legs, the right spine is lower than the left, the upper body tilted slightly to the right (shortening of the right leg). When standing on the right leg, the left half of the pelvis drops slightly below the horizontal, while standing on the left (healthy) leg, the right half of the pelvis in the normal way, rises above the horizontal (positive Trendelenburg sign). Periphery of the right buttock reveals prominence of the trochanter, measured 34 cm, 37 cm to the left. The right gluteus region is flattened and the muscle feels flaccid compared to the left. Leg length anterior superior iliac spine – external malleolus, 71 right, left 74 cm. Right trochanter almost 5 cm, left 1½ inches above the Roser-Nélentonschen line. With heavy pressure from the front there is little tenderness in either hip.

Movements: flexion to 100° possible. With strong flexion there is about 20° from abducted position, abduction to 30°, 30° adduction possible. All movements are without pain and without crepitation. Rotation in a flexed position not possible.

One the left few restrictions of motion: abduction to 50°, abduction to 25° are possible, with free flexion. Radiograph of the right hip 15 (taken on March 16, 1910.): The deformity of the right hip joint has increased. The osteophytes on the head are larger. Between the upper osteophytes of the head and the greater trochanter seems to be developing a connection. Those, “migration of the acetabulum” has progressed by ½ inch. The left shows some irregularities in the acetabulum base which may or may not be normal.

4. Alfred R., 13 years. Other than measles, the boy has had no serious no illnesses, and in particular not rickets. Neither the mother nor the boy are aware of any injury. Three fourths years ago it was noticed that the boy, “became lame.” There is no pain. The boy takes part in all running sports, but tires quickly. He apparently felt fatigue only in the left leg.

Status March 14, 1910. Normally developed boy. When standing, when both legs are kept straight and the spine, the left heel very slightly raised so that the body burden rests largely on his right leg.

While walking on the left leg, the upper body leans slightly to the left. Supine the left hip is adducted 7°. Real shortening of the left leg by 1 cm. Distance from the anterior superior iliac spine-external malleolus, right 75.5, left 74.5 cm. Apex of the left trochanter 1 cm above the R. N. line, while that of the right is at most 0.5 cm above this line. Pressure on the trochanter, and strong impact against the heel does not hurt, the boy jumps off a table without pain. Only strong pressure on the head from the front left leg is sensitive. Movements in the right hip joint: totally free in the left hip with complete flexion and extension, but at the beginning of each active and passive flexion there is some resistance, which is then overcome. Abduction and adduction active and passive completely absent. Rotation in extension only in very small extent around 20° is possible, in a rectangular flexion rotation absent.

Fig. 16

Case 4 on June 7, 1910

The x-ray shows the left femoral neck, trochanter, and shaft and is unchanged. Femoral neck angle is normal. Significant deformity of the femoral head. Flattening of the upper and the medial portion, which abut one another both obtuse at an approximately corresponding to the center of the head surface location at an angle. Very irregular epiphyseal line. The distance from the epiphyseal to the surface of the head measures only 8 mm. (The same measure on the healthy side of 20 mm.)

Acetabulum face flat and not very regular, small extension of the acetabulum to the top. The radiograph of the right hip joint is completely normal.

Status June 7, 1910. Findings generally unchanged. Absolutely no pain when walking, running and jumping – despite considerable deformation of the femoral head. On the left side the pronounced limping gait is very similar to that of a congenital hip dislocation. Each occurrence standing on the left foot causes the upper body to lean to the left. When standing on the left foot with the right leg raised the right hemipelvis drops down so that the anterior superior iliac spine is at least two finger-breadths lower than on the left (positive Trendelenburg sign). When standing on the right foot to the left buttock lifts in the normal way, so that the left spine is at least two fingers’ breadth higher than the right. Movements same as on March 14th. Flexion-free. Abduction absent. But compared to the previous low (7°) adduction, the leg is now adducted in adducted position by another 20°. Measurement of the circumference of both halves of the pelvis at the greatest prominence of the trochanter major results are left 37.7, right 38 cm. The right gluteal muscles appear slightly atrophic.

5. Minna G., 13 years. Parents and six younger sisters are alive and healthy. The girl has for a long time, suffered with “neck glands,” but apparently not the English disease, has learned to run over the past 1½ years. At the age of 12 years, the child began to limp with no known cause. She had suffered no injury and never missed school. Now pain is only intermittent – when the child has walked for more than half an hour – but usually the child is completely painless.

February 10, 1909. Strong, well developed child with scars on the right side of the neck arising from tuberculous lymphatic glands.

Standing: slight normal lordosis of the lumbar spine. Both spines equal. Left foot is held slightly inwardly rotated. In the lower third of both lower legs varus bowing. The gait is similar to that of a left-sided congenital hip dislocation. With each step on the left leg, the upper body leans to the left side. Lying down: Position of both legs at the hip in flexion and abduction is normal, however, the left leg at 45° flexion is rotated inward. Slight shortening of the left leg compared to the right. Anterior superior iliac spine – external malleolus, left 66 cm, 68 cm right. Left trochanter at 5.5, and right 3 cm above the R. N. line. Both trochanters are massive by palpation. No tenderness of the hip joints, no apparent atrophy of the thigh muscles. Movements: Right hip joint. Flexion and extension completely normal. Abduction to 30°, adduction to 40° possible. Rotation in extension is free but in right angle flexion only 45° is possible. The magnitude of the side opposite to the abducted leg in the air can be obtained without difficulty.

Left hip: flexion possible only to 80°, abduction absent, Adduction to 40°. Rotation (of the limb) causes rotation of the pelvis. Upon flexion of the hip, the leg is held in marked adduction. Sometimes hip movements cause loud left hip joint crepitation. The left leg is in the right lateral position, insofar as permitted by the absent abduction, are kept under misalignment of the pelvis raised against the affected side.

Radiograph of the right hip joint (Fig. 12): Head and acetabulum normal. Femoral shaft very thick. Femoral neck angle of 115°. The trochanter is markedly developed, surpassing in size and scope of the normal by 3 to 4 times and stands about ½ inch higher than the highest level of the head. Above the acetabulum directly over the trochanter appears a pit, into which the tip of the trochanter would fit with abduction of the leg.

Radiograph of the left hip joint (Fig. 11). The head shows significant flattening in its upper sections, and irregularity of its surface. The epiphyseal line can be seen, but the portion of the epiphyseal line of the head is minimal. At the lower portions of the head bone apposition has taken place. The acetabulum is completely congruent with the abnormal shape of the head so that it fits precisely. Femoral shaft is very thick and bulky. Femoral neck angle approximately 110°, but is hard to determine because of the deformity of the head. The trochanter is like one the right an abnormally bulky structure, which rises high above the head of the femur and the hip.

Status on March 21. 1910. Right hip similar to that three months previously, but on the left the following changes occurred: a little more flexion possible, up to 110°, internal rotation in extension now up to 90°. Shortening of the left leg has increased slightly. Anterior superior iliac spine-external malleolus, right now 71 cm, left 68.5 cm. Gait similar to three months earlier. Pain has not increased. Radiograph shows slight increase in irregularity in the surface of the left femoral head.

Bilateral case

6. Wilhelm P., age 9. Nine brothers and sisters live and be healthy; only a younger brother suffers from chronic otitis. The boy himself has had no illnesses except measles and suffered no injury. At the age of 8 years – about 2 months before the first visit to at the clinic, “he began to limp on his right leg.’ The pain was only intermittent after strenuous activity. Now, complaining of pain in his right knee. In his free time outside the home he plays with no complaints with his comrades.

Status October 8. 1909: Normally developed boy. Standing: left convex lumbar scoliosis Low. Right anterior superior iliac spine is significantly higher than the left. The right heel is not touching the floor when the left knee is in extension. Right gluteal fold compared to the left is clearly flattened. Slight limping gait. The upper body leans very slightly to the right when stepping on the right leg. Lying down: Right hip joint is at an angle of 14°, adduction, abduction left similar. The level of spina right anterior superior iliac spine and knee and malleoli, etc, are high compared to the left. Anterior superior iliac spine-external malleolus, right and left 64 cm. Greater trochanter on both sides ½ inch above the R. N. line. No palpable deformity of the hip joints. No tenderness on either side. Circumference of the thigh 18 cm above the patella on the right ½ inches less than the left. In both groins there are enlarged, indurated, painless lymph nodes about the size of beans. On the left, they are larger and more numerous than on the right. Movements: Right hip joint. Flexion and extension almost completely free and painless, but lacks 10° full flexion. Abduction and adduction completely absent, only a few degrees of rotational movements are possible. Left hip joint. Flexion and extension, completely free. Abduction, up to 40 degrees, adduction to 30° rotations in extension by approximately 30°. All movements painless. In abduction loud crepitation.

X-ray image from the right hip joint (Fig. 10): flattening of the femoral head and irregularity of its surface at the top and medial portions. Moderate irregularity of the inner surface of acetabulum (pathological). Femoral neck angle normal. Trochanter very small.

X-ray image of the left hip joint (Fig. 9): Very significant deformity of the upper femur.

The location of the epiphyseal line, so the actual femoral head corresponding part is almost completely gone, however, appears at the lower and medial portions of bone apposition to have taken place. This places the head in coxa vara rolled inferiorly. If one, however, draws the femoral neck and shaft axes, it is a narrow angle.

Status June 12, 1910. Pain has not worsened, the boy still runs and plays. The boy can jump without pain from a table to the floor. He has complained only occasionally, especially at night. Still limping slightly, upper body leans on each occurrence the right foot hitting the floor. The Trochanter height on both sides is increased to 2 cm above the R. N. line. Anterior superior iliac spine-external malleolus, right 65.3, left 65. 9 cm. Adduction of the right hip is now absent. The joint remains normal, except as before the loss of abduction. Adduction to 20°. Virtually free flexion. Mobility of the left hip joint still similar to that in previous years. In abduction at any given moment a sudden loud crack. The Trendelenburg sign is very positive right. When standing on the right leg the left spine anterior spine drops significantly below the horizontal, while standing on the left leg the right spine does not drop, however, is does not rise in the normal way above the horizontal. The X-ray image of deformity has increased especially in the right femur, so that above the right femoral head there is now a pit several millimeters deep.

Appendix II – Review of the cases observed up to now, sorted by the occurrence of the first symptoms

Unilateral Cases

Nr.	Case	Age at onset of symptoms	Etiology
1	Zesas, 11 year-old girl	6 years	After inflammatory disease of the right hip
2	Eigner, Case 1, 8 year-old girl	7 years	Minor contusion of the left hip
3	Matsuoka, 18 year-old girl	8 years	–
4	Eigner, Case 2, 10 year-old boy	9 years	–
5	Negroni, 9 year-old girl	9 years	Joint arthritis
6	Eigner, Case 3, 13 year-old boy	10 years	Acute hip joint inflammation (osteomyelitis of the femur?) at age 10 weeks
7	Hofmeister, 12 year-old boy	11 years	–
8	Eigner, Case 4, 13 year-old boy	12 years	–
9	Eigner, Case 5, 13 year-old girl	12 years	–
10	Gruer-Wollenberg, 17 year-old girl	13 years	Congenital hip dislocation
11	Fridberg, 14 year-old boy	13 years	–
12	Maydl, 15 year-old girl	14 years	Minor hip contusion (?)
13	Hofmeister, 15 year-old girl	14 years	Apparently acute onset
14	G. Schmidt, 43 year-old man	14 years	Trauma as a seven-year-old (?)
15	Immelman, 33 year-old woman	End of second decade	Trauma as a 14-year-old (?)
16	Küster-Zesas, 16 year-old boy	15 years	Subluxation of left hip, but repositioned
17	Riedel-Cornils, 17 year-old girl	15 years	–
18	Fridberg, 22 year-old man	16 years	–
19	G. Schmidt, 32 year-old man	16 years	Subluxation of left hip, but repositioned
20	Riedel-Cornils, 18 year-old man	17 years	Minor trauma (?)
21	Negroni, 18 year-old man	17 years	–
22	Maydl, 18 year-old man	18 years	–
23	Immelmann, 18 year-old woman	26 years	Minor trauma (?)
24	Hoffa and Wollenberg, 30 year-old man	17 years	Tuberculous coxitis age 5–7 years
25	Frangenheim, 39 year-old man	32 years	Purulent inflammation of the left gluteal region (?)
26	Preisser, 35 year-old man	34 years or more	Congenital joint deformity (?)

Bilateral Cases

Nr.	Case	Age at onset of symptoms	Etiology
1	Eigner, case 6, 8 year-old boy	7 years	–
2	Okada, 17 year-old man	7 years	“Infantalism” (?)
3	Guhl, 36 year-old woman	7 years	–
4	v. Brunn, 12 year-old girl	9 years	Minor trauma (?)
5	Hesse, 32 year-old woman	10 years	–
6	Bibergeil, 14 year-old girl	11 years	Minor trauma
7	Negroni, 13 year-old girl	13 years	–
8	v. Brunn, 23 year-old woman	16 years	–
9	Frangenheim, 17 year-old man	17 years	Trauma (?)
10	Axhausen, 35 year-old man	20 years	Scarlet fever and diphtheria at age 6 with painful swelling both hips
11	Kuliga, 41 year-old woman	28 years	–
12	Wollenberg, 35 year-old woman	30 years	Arthritis associated with abdominal typhus at age 12

Note. In the above chart are not listed a case of Oberst in which the findings at the hip joint were not indicated more precisely, two cases of Okada in which the diagnosis was not proven, three cases of Borchardt in which the author himself recognized the disease as arthritis deformans but as osteomyelitis.