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THE CLASSIC: On a Particular Form of Pseudo-Coxalgia Associated with a Characteristic Deformity of the Upper End of the Femur

2006; Lippincott Williams & Wilkins; Volume: 451; Linguagem: Inglês

10.1097/01.blo.0000238799.05338.5a

1528-1132

Jacques Calv,

Orthopedic Infections and Treatments

Jacques Calvé was born in Paris in 1875. After finishing his education and medical training in Paris, he obtained the position as assistant surgeon at the Maritime Hospital in Berck-sur-mer. This was a major hospital for the treatment of patients with tuberculosis and was directed by V. Menard. There were large numbers of children with tuberculosis of the hip and a newly installed X-ray apparatus. The combination of clinical acumen and the new technology resulted in the definition and characterization of a new, hitherto unrecognized, disease of the hip. Calvé remained active in his work until he became absorbed in the treatment of casualties from World War I. Following the war, he and his wife, the daughter of an American army officer, headed a Franco-American foundation that established the great orthopedic hospital at Berck-sur-mer. Calvé served as chief surgeon of this hospital until his retirement in 1945. During his tenure he made substantial contributions to the treatment of tuberculosis of the bones and joints. After a long sojourn in the United States Calvé returned to his old hospital to die in 1954. L.F.P. In the course of the last three years, I have had the occasion to observe 10 cases of chronic arthritis of the hip of a very special type, defined distinctly clinically and radiographically. They did not fit any previously reported description, and differ from the known types in several ways. One can be convinced by reading the observations in this article and examining the radiographs that the principal features of this arthritis are: Signs of a joint reaction, a subacute or chronic course, short duration, and healing with preservation of movement. Bony deformation which precedes the symptoms and persists. These changes are: coxa vara, hypertrophy of the head of the femur, atrophy and fragmentation of the bony center of the epiphysis, and absence of total destruction of the bone. ETIOLOGY Incidence: This arthritis occurs infrequently. I have observed 10 cases in more than 500 cases of hip disease. [Hip disease or coxalgia as used in this paper refers to tuberculosis of the hip. Ed.] Age: These cases have occurred in young patients between 3 1/2 and ten years of age. History: All except one were children of the working class. I have not been able to obtain any exact information regarding any hereditary tendencies. Of the 10 children, 2 were brother and sister. The number of our cases does not permit us to draw any conclusions regarding a familial tendency. The general health of the patients, without exception, was excellent. Their external habitus represents a type which may remotely have a certain importance in considering the pathogenesis. They were all, to some degree, rachitic. Each of them carried stigmata more or less associated with rickets. Two of them were severely bow-legged. The signs in the others were less obvious, showing a disproportion between the length of the trunk and the lower extremities, a rachitic rosary, and a cranial asymmetry with prominent frontal or parietal bossing. There were no signs of congenital syphilis. Onset: Of these 10 cases, I do not have precise information in 7. Three cases have just recently been discharged from the hospital. The diagnosis was established retrospectively by examining the records and radiographs of the hospital. In 7 of the cases, the onset was very variable. In 3 cases, it was abrupt, as with an acute arthritis, with pain, fever, and flexion of the hip. In one case, the symptoms followed a violent fall on the greater trochanter. The onset was slow and progressive in 3 cases; in one, it closely resembled the classic coxalgia: the child “dragged his leg at the end of the day,” limping and complaining of pain in the knee for several days before being examined for the first time. CLINICAL STUDIES All of the patients presented with very clear signs of arthritis of the hip, i.e., limitation of motion, spontaneous and provoked pain at the joint, thickening about the joint, muscular atrophy and limping. An examination for inguinal adenopathy was not made regularly and I cannot give any useful information on this point. The clinical examination revealed 2 constant findings: a certain degree of coxa vara and bony hypertrophy of the head of the femur. Coxa vara: In 2 of the small patients, the coxa showed itself at first glance by the exaggerated prominence of the greater trochanter. In the others, it had to be looked for; in all of the cases, there was an elevation of the greater tro-chanter in relation to the line of Nelaton-Roser. This was confirmed on the radiographs. Bony hypertrophy of the head of the femur: This was obvious in most cases. One could plainly see a bulging prominence in Scarpa's triangle. On palpation, this prominence was hard and situated just underneath the femoral vessels. It was easily distinguished from the soft, thick tumefactions or fungous periarticular masses seen in other forms of coxalgia. On grasping the area between the thumb and fingers, and moving the hip, one received a clear sensation of the presence of an enlargement of the femoral head. RADIOGRAPHIC FINDINGS The radiographic examination confirmed the 2 findings discussed previously and revealed other characteristics which could not be determined by the clinical examination and which give this condition its particular aspect. Coxa vara: An angle of 90-120°. Hypertrophy of the head and neck: The neck appears to be shorter than on the normal side and thicker in its vertical diameter. The head is large, formed almost entirely by the diaphyseal portion of the neck, which has grown more than normally. Atrophy and deformation of the bony center of the epiphysis: This deformation is constant and as we have seen constitutes the most important characteristic of this condition. Normally in children, the epiphysis makes up the greater portion of the head of the femur. It forms a hemisphere, separated from the head and neck of the femur by a clear straight band of epiphyseal cartilage. This lies almost horizontally from medial to lateral, slightly inclined from above downward, not as indicated in some classic books, at the inferior border of the head, but at the junction of the superior three quarters and the inferior quarter of the head. In all of our cases, this normal disposition of the epiphysis was greatly altered. The epiphyseal bone was flattened, fragmented, atrophied, and the superior surface of the head was incompletely covered with bone. The epiphyseal line was sinuous, irregular, and curved. The diaphyseal bone of the neck which had hypertrophied did not supply as much of the head portion as before. In three cases in which I have been able to obtain radiographs at different stages in the evolution of this lesion, I have noted three very interesting changes in the development of the epiphysis. In the young patients, the first radiographic examination shows that the bony center is not single, but is divided into two or more fragments or secondary centers in the midst of the cartilaginous mass. Little by little, these tiny bony islands grow and approach one another. Later they unite and form a single mass. It will be interesting to see after several years if these patients will have abnormal epiphyses. Undeniably, there is a disturbance in the ossification of the femoral epiphysis. We will come back to this in discussing the pathogenesis of this disease. In addition to these primary characteristics, the radio-graph demonstrates other valuable findings. There is always a total absence of loss of bone or cartilage. The joint space is always the same thickness as on the normal side. Shenton's line is normal. One never sees the local dystrophic changes that one sees in tuberculous arthritis of the hip, i.e., decalcification, thinning of the diaphyseal cortex, etc. PROGNOSIS This articular syndrome has a very short duration. In all of the patients, the recovery of hip motion has been very rapid. Only abduction remains more or less limited depending upon the amount of coxa vara. The pain, spontaneous or provoked, disappears very quickly. This is also true of any periarticular thickening; this should not be confused with hypertrophy of the head of the femur, which is not ephemeral. The absence of inguinal adenopathy is constant for several months after onset. If, after a long period of time some small patients present with a diminution of the size of the buttock due to immobilization because of an error in diagnosis, the muscles are not hypotonic. We have shown the value of these negative findings in the diagnosis of joint disease. Inguinal adenopathy and hypotonia always indicate involvement, even minimal, of the joint (tuberculosis). There has never been any trace or sign of an abscess in any of our cases. In all of these patients whom I have examined and followed and in whom I have eliminated the diagnosis of coxalgia, I have gotten rid of immobilizing apparatus and allowed the patients to walk. I have never seen a relapse or recurrent irritation of the joint. The children, after an initial awkwardness, have very rapidly regained their normal gait, for the most part without limping. Two of them have had a slight waddling gait, a slight swaying. This was noted in one case, alternating to both sides. I believe this to be due to the presence of the coxa vara; the waddling is caused by the functional insufficiency of the gluteal muscles (Trendelenburg's sign). If the signs of joint irritability recede quickly, the bony deformity persists without much change. Nevertheless, the radiographs as we have seen show the regeneration of the epiphyseal center. The prognosis has been very favorable in all of the cases we have observed. Examinations repeated year after year will allow us to be certain on this point. PATHOGENESIS In resumé, all of these cases showed a transitory arthritis of short duration, developing in subjects having a deformity of the upper end of the femur: coxa vara, hypertrophy of the head of the femur, fragmentation of the bony center of the epiphysis, and preservation of the normal cartilaginous surfaces. How can these bony lesions be interpreted? What is the exact nature of the arthritis? What is the cause-and-effect relationship between them? It is difficult, given the limited number of cases and the lack of postmortem material, to give a definite answer to these questions. It is natural when presented with a chronic arthritis of the hip to think of tuberculosis, and in all our cases, this was the first diagnosis which was made. The special predilection of Koch's bacillus for the hip, the relative frequency of a slight degree of coxa vara in the course of coxalgia, the similarity of the premonitory signs, warrant this supposition. As we have seen, this diagnosis is erroneous and we reject it for many reasons. In the first place, the bony deformities clearly antedate the arthritis; it is an epiphenomenon arising on a preexisting lesion. One patient had a definite coxa vara and had a gait associated with this, a swaying, for a long time before the arthritis. The radiograph taken only a few days after the onset of the arthritis showed the presence of considerable bony deformity. We have done the skin test for tuberculosis (Mantoux) in 5 cases. In 3 the result was positive, in 2, clearly negative. In those with a positive reaction, we have been unable to establish the diagnosis of tuberculosis of the hip and believe the reaction to be due to tuberculosis elsewhere in the body. A negative reaction rules out tuberculosis anywhere in the body. Comparing the similarity and evolution of the lesions in these 5 patients, we can logically infer that they were not due to tuberculosis. Another argument is the absence of the characteristic changes in a tuberculous lesion, i.e., spreading, ulceration, and dystrophic changes. A bacterial arthritis lasts 2 or 3 years, on the average, if not longer. In all of my cases, the duration of the arthritis has been very short and in spite of the resumption of walking, there has been no recurrence. This would have occurred without fail if Koch's bacillus were the cause. [After an extensive discussion of tuberculosis, the author then touches upon congenital syphilis, congenital coxa vara, arthritis deformans, rickets, mechanical disorders, subluxations, static defects, and locus minoris resistentiae, and concludes: “It is impossible to answer the question regarding etiology.” Ed.] CONCLUSIONS We have described a type of arthritis of the hip which does not correspond to any previously described and which until now has been considered to be coxotuberculosis.