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The Limping Child
The Limping Child G. WILBUR WESTIN, M.D.
TOXIC SYNOVITIS OF THE HIP
Toxic or acute synovitis of the hip joint is probably the most common orthopedic condition causing a limp in the otherwise normal child. This condition, usually short in duration, is thought to be either an inflammatory involvement of the synovial membrane of the hip, or possibly the result of minor trauma to the joint. It is always considered a precursor, or a possible initial symptom, of the more serious LeggPerthes disease, but fortunately in a large percentage of the patients it lasts for three to 10 days and subsides without sequelae. Etiology Frequently there may be a history of an upper respiratory tract infection or streptococcal throat a week to 10 days prior to symptoms in the hip joint. In a few patie\ts there may be a definite history of trauma which may be incidentally remembered and may not be an important factor in the causation. Approximately 25 consecutive cases of toxic synovitis were aspirated at the Children's Hospital in Boston, and no organism was grown from the small amount of fluid that was present in these cases, which subsequently subsided in the typical manner. There may be some causal relation between the streptococcal infection and subsequent inflammatory changes in the hip joints similar to that of rheumatic fever. Some feel that this may be an allergic manifestation. No specific cause has been adequately documented for this condition. Symptoms The patient is typically a young boy, age five to 10, but the disease may occur at any age in childhood or in the adult. Typically, in the child, the parent may be the first to notice a mild limp, or the child may complain of a mild discomfort in the knee or hip joint which may gradually increase in severity over a period of 24 to 48 hours. He may or may not have a mild elevation of temperature, and symptoms are actually relieved by bed rest. On physical examination, one will find limitation of motion of the involved hip joint and, commonly, a mild hip flexion contracture. Pediatric Clinics of North America-Vol. 14, No.3, August, 1967
601
602
G.
WILBUR WESTIN
Depending on the severity of the involvement, the patient may complain when examiners attempt to test any motion of the hip joint. The ranges of motion most commonly limited are extension, rotation and abduction, and the child usually does not appear acutely ill, in fact, usually presents as a healthy person. Laboratory Work The laboratory findings are usually benign with a normal differential count and a normal or slightly elevated sedimentation rate during the acute phase of the disease. X-ray Findings Two findings may be present by x-ray: a bulge of the capsule des-cribed as the "obturator sign" seen on the lateral aspect of the involved hip joint, and an associated minimal widening of the joint as measured from the femoral head to the acetabulum. These are findings which the practiced eye of a radiologist may interpret, but frequently the x-ray findings are "negative." There usually are no findings typical of the early findings in Legg-Perthes disease. The provisional diagnosis involves all conditions in which the hip joint may be involved in an inflammatory type of affliction. The rapid subsidence of symptoms and absence of positive findings by x-ray may rule out the active stages of such conditions as Legg-Perthes disease, tuberculosis, rheumatic fever and rheumatoid arthritis, all of which have other findings more typical of these diseases. Probably the most important differential is from a septic hip joint. If there is any question, aspiration of the hip joint is indicated, and in the absence of findings of any purulent material in the joint, further radical treatment is not indicated at the time of admission. Perhaps this disease is best defined by Finder, 2 who states that "in transitory synovitis of the hip joint, the synovitis constitutes the entire disease but in other hip joint lesions, the synovitis may merely represent the point of departure into any one of the several ,disorders of the hip." Treatment Treatment for acute synovitis of the hip joint is conservative. The patient will get considerable relief of symptoms by placement in Russell's traction in bed, pulling the hip joint in the line of deformity at the outset and gradually extending it as the patient tolerates it. Progress of the disease will be indicated by a prompt return of the temperature to normal, a constant finding of essentially normal differential and white cell count, and, in particular, a rapid decrease in pain in the hip joint with recovery to a normal range of motion. It is our practice to keep these children in traction until they have a full range of normal motion and then to keep them on crutches for an additional three weeks. At the end of that time new x-ray films will confirm the negative findings. Most
THE LIMPING
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important, any child with transient synovitis of the hip joint should be followed at three- and six-month intervals to be assured that there has not been, in reality, a precursor to a more serious hip disease. I do not recommend the use of antibiotics in such a condition, since they may cloud the real issue should other conditions develop, and the antibiotic is of no specific benefit in the transient synovitis case. LEGG-CALW-PERTHES DISEASE
In 1909 Legg, Calve and Perthes separately published independent observations on degeneration in the hip joint. This was specifically differentiated from the then common entity of tuberculosis of the hip. This condition has since been called by many names, the most accepted of which include Legg-Calve-Perthes disease, coxa plana and osteochondritis deformans juvenilis (see also p. 551). Seventy-five to 85 per cent of the cases occur in males, and approximately 20 per cent are bilateral. The disease is characterized by aseptic necrosis of the femoral head and sometimes of the adjacent proximal femoral metaphysis. This initial process is followed by revascularization and regeneration of the epiphyseal bone. This sequence takes an average of four to five years for completion. Etiology There is no generally accepted causative agent. Some authors have felt that trauma was an inciting event, but it is difficult to accept this explanation in the bilateral cases. Others have explained the disease on an infectious basis, but no evidence of a bacterial process has been documented. Still others have felt that there is a metabolic deficiency, but most of the cases worked up in metabolic clinics have yielded little evidence to substantiate this. The lesion in the early stage of the disease (the degenerative stage) is reported by Haythom. 3 In examiners' curettements from the heads of femurs of 36 patients in early disease stages, he noted: "In every case, more or less degeneration, necrobiosis or necrosis was present without any local evidence of the cause.... The absence of infection was supported by many negative cultures. . . . In more advanced lesions, necrosis was extensive and complete involving not only marrow, but cartilage and bony spicules from broken trabeculae." Trueta of Oxford, England, has made a detailed study of the blood supply to the capital femoral epiphysis and from his work points out that the blood supply between the ages of four and seven years (corresponding with the most common age at onset of this ,disease) is undergoing extensive reorganization and is thus vulnerable to the secondary changes described above. It is my belief that the aseptic necrosis is primarily due to inter-
G.
604
Wn.BUR WESTIN
ruption of the blood supply to the femoral head and adjacent metaphyseal regions due to unknown cause. In a review of patients admitted to the Los Angeles Unit of the Shriners Hospitals between 1952 and 1957, the 42 cases that had reached maturity had the following mean time of various phases in this disease: MEAN TIME
Degenerative phase .................. Regenerative phase . . . . . . . . . . . . . . . . . . . Total duration . . . . . . . . . . . . . . . . . . . . . . Onset to weight-bearing ..............
1 8 4 2
year, years, years, years,
7 1 9 6
months month months months
The determination of the different phases as listed above is made primarily by x-ray evaluation; however, the onset of illness can be obtained only from an accurate history. Of these 42 cases completely evaluated, the average age at onset was six years and six months, with a range from three years and five months to nine years and 11 months. Clinical Manifestations and Treatment The onset of the disease is usually insidious. The child complains of mild discomfort in the knee or hip and ·manifests a mild limp. On physical examination one finds evidence of an irritable hip joint, with limited motion in all directions and commonly a mild flexion deformity. This corresponds to the initial synovitis. There is no single way to treat every case of Legg-Calve-Perthes disease. The extremes are from "do nothing" and allow full activity of the child with this condition, to complete bed rest through the reparative phase. Decisions about the course of treatment should be guided by a careful clinical evaluation of the hip with lesser emphasis placed on the x-ray changes that are characteristic in the natural course of this disease. Our program of treatment consists of admission of a child to the hospital upon making the initial diagnosis of an irritable hip joint. He is then placed in bilateral leg traction to keep him in bed and put the hip at rest. The period of traction necessary to obtain a full free range of motion of the hip without evidence of clinical irritability in the typical early case of this disease varies from three to 10 weeks. During this early phase the patient may be ambulatory in a cart in the sitting position with portable traction applied, or he may be allowed in the swimming pool under supervision for range of motion, but no weight-bearing. During this time other conditions of the hip joint such as tuberculosis, infection, complications of a congenitally dislocated hip, rheumatic fever or rheumatoid arthritis must be ruled out. This is not difficult as soon as the typical x-ray changes become apparent as the disease progresses. When the child has regained a full range of normal motion, when the hip is no longer irritable and he is free of pain, he is ambulated. The
THE LIMPING CHILD
605
method we prefer is the use of an ischial weight-bearing brace. The extremity is suspended free in a double upright brace with a Pattonbottom splint so that the toe cannot touch the ground. A build up of the opposite shoe of approximately 4 inches is usually supplied to faciliate ambulation. This is started with the usa of crutches until the child is used to wearing the brace. He then may discard crutches, and it is surprising how he will do on his own. It is granted that there is no single type of apparatus that will guarantee full protection of the hip joint, but the advantages of the ischial weight-bearing brace with the loose thigh and calf cuffs are many. The child has both hands free. The brace has no knee joint, so that the child cannot bend the knee and bear weight in a kneeling position, and it can accommodate readily to both standing and sitting. The parents and the child are both instructed to lengthen the brace as growth occurs so that the toe never touches the ground. A program of exercises to maintain a full range of extension and abduction of the hip is outlined and is to be performed on a regular basis to maintain the integrity of the musculature of the involved extremity. After a trial of approximately one month, the child is seen again, and if the hip is still irritable, the ambulatory treatment must cease. The child is returned to bed until the synovitis has again disappeared, and only then is ambulation resumed. The use of the brace for protecting weight-bearing through the hip joint is maintained until the reparative or regenerative phase of the disease has been well established, so that the soft femoral head is protected from the crushing and flattening that commonly occur in the untreated patient. The child in such a brace should be seen on regular visits with a maximum of three months between visits, and x-ray films and clinical evaluation of the hip should be made at each visit to check the progress of the disease. When the repair process has progressed to the point at which there is a spherical outline of the femoral head visible by x-ray, it is our practice to allow the patient to begin full weight-bearing without the brace. It is assumed at this time that the areas of necrosis have been replaced with ossifying cartilage, and it is rare that progressive changes will occur when the reparative process has reached this stage. The completion of the disease, however, is not indicated until complete reossification of the femoral head has occurred. In all cases there is a varying amount of residual flattening of the femoral head ("coxa plana") and widening of the epiphyseal line and the adjacent metaphysis, giving some degree of coxa magna. The only constant determinant of extent of radiographic residual and clinical loss of motion is the age at onset of the disease. The younger the age of onset, the better the prognosis for complete recovery. Experience with a number of patients sent to our hospital after having been allowed intermittent weight-bearing over a period of months
606
G.
Wn.BUR WESTIN
or even one or two years since the onset of disease has taught us that this type of management may result in changes by x-ray with subluxation of the femoral head and fragmentation. This may necessitate, in some cases, surgical reconstructive procedures. In the late stage of inadequately treated cases with gross deformity, and persistent limited motion with pain, one finds grossly the picture of an osteoarthritis of the joint which presents a serious problem to the attending orthopedic surgeon. In a few instances, in the early phases of this disease in a younger child, there may be a massive early destruction of the femoral head. Even after prolonged treatment with bed rest and traction, there may be a failure of irritability and limited range of motion to subside. In spite of prolonged traction, some of these femoral heads may sublux out of the joint. In the last six years we have had six such children on whom a Pemberton or Salter type of iliac osteotomy has been pedormed to provide better coverage for the femoral head. This operation has been limited only to those cases resistant to conservative therapy as outlined above. In the continued experience of treating 140 cases of Legg-Perthes disease, it has become evident that the hip must be treated clinically, as well as by x-ray. Specific cases with severe complications require specific procedures. The child with onset at early age, and with early treatment, rarely requires anything other than a conservative program of protecting the hip and treating it as outlined above. ·Characteristic x-ray findings are seen according to the stage of this disease. Initially, with the onset of synovitis, there may be a widening of the joint space and some demineralization of the femoral metaphysis with no apparent changes in the femoral head. In the degenerative phase (or aseptic necrosis phase) there is an increase in density of the femoral head which may involve a portion of, or the entire femoral head. There may also be changes on the metaphyseal side of varying degree. When the aseptic changes are manifested by the increased density of the head, one can expect, and should inform the parents, that as progression of disease occurs, the x-ray views will look much worse to the unpracticed eye before they begin to fill in and have a definite improvement. This stage of degeneration is the most important phase for protection of the hip joint from weight-bearing as well as prognosticating the result. If apparent aseptic necrosis is confined within the acetabulum and treatment is instituted, the prognosis is much better than when a fragmented epiphysis subluxes out of the confines of the acetabulum. The regenerative phase occurs after the absorption of the densities in the femoral head with revascularization and the conversion of invading cartilage into new epiphyseal bone proximal to, and new metaphyseal bone distal to, the epiphyseal line. The persistent radiolucent areas are considered to be composed of cartilage, but are not as subject to progressive flattening. It is felt by some that, once the regenerative phase
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has been established, weight-bearing is safe. It is our practice, however, to wait until that stage of the regenerative phase when a spherical outline of the femoral head is present before full weight-bearing is allowed.
SLIPPED CAPITAL FEMORAL EPIPHYSIS
This is a condition in which the femoral head is displaced from its normal relation to the femoral neck with disturbance of the mechanical alignment of the proximal femur. It may occur insidiously or acutely, and is primarily found in the adolescent between the ages of 10 and 15. Approximately 20 per cent of the cases are bilateral. Etiology The cause of this condition is not known, but many contributing factors have been considered. In approximately 50 per cent of the male patients one will note the Frohlich type of female distribution of fat and immature secondary sexual characteristics. It has been my experience and that of others that this condition may occur in otherwise normalappearing and normal-functioning children. With rapid growth, the cartilaginous epiphyseal plate is widened as the increased activity of the maturation of the cartilage cells into bone takes place. With this widening, some decrease in mechanical stability may occur. This happens in all the epiphyses; however, the proximal femur has a configuration which leads to the production of considerable shearing stress at the epiphyseal line, and this may well render this area particularly vulnerable to disruption. Some authors have felt that there is a definite endocrine disturbance in these children which produces abnormal weakness of the epiphyses. The factor of trauma is probably incidental to the underlying pathology. Sudden symptoms following an injury are most accurately interpreted as resulting from the addition of stress to an already unstable system with the production of epiphyseal disruption and slippage. Symptoms In the majority of cases the symptoms comprise the insidious onset of pain and a limp. The symptoms progress as the femoral head slips posteriorly and inferiorly. The typical patient will have noted pain in the hip, thigh or knee for months, or perhaps for several weeks in mild involvement. Diagnosis The diagnosis is made by physical and x-ray examination. On physical examination, a hip that externally rotates as one attempts to Hex it should alert one's suspicions. Depending on the degree of irritation
608
G.
WILBUR WESTIN
in the hip joint and the amount of slippage, one will find varying degrees of limitation of motion. Of particular note is the loss of internal rotation and of full flexion. Most of the patients will have an external rotation deformity of the hip. The x-ray evaluation depends on satisfactory anteroposterior and lateral views of the hip; however, the lateral view is of prime importance, since it is here that one can obtain the clearest picture of the amount of slippage that has occurred. It is this view that is often used as the basis of the treatment which the orthopedic surgeon will initiate. Treatment The treatment of this condition has been and still is somewhat controversial. It is our practice, once the diagnosis has been established, to put the patient in bed in traction until the acute symptoms have subsided. If the displacement of the femoral epiphysis is not more than one third the diameter of the femoral neck, we surgically place two or three threaded %6 -inch pins across the epiphysis from the lateral approach into the femoral head. Such fixation is usually adequate to prohibit further slipping. If the child is ske~etally immature, with one or more years of growth, it is common for the deformity to diminish considerably as growth proceeds. The range of motion will also return toward normal. Cases in which the epiphysis has slipped more than 50 per cent of the width of the femoral neck often warrant surgical correction. This, however, carries with it the too frequent severe complication of disruption of the blood supply to the femoral head with ensuing aseptic necrosis. At the Massachusetts General Hospital, Klein and his associates report a most remarkable series of open reductions in such cases without serious complications. This operation was performed by a group of men familiar with this condition. Because of the danger of compromising the nutrient supply to the femoral head, a longitudinal incision was made through the joint capsule, paralleling the vessels. This was then made into a "T," with the top parallel to the acetabulum above the vessels. Through such an exposure the lesion was well visualized without undue danger. The retinaculum carrying the blood vessels up the neck of the femur may be jeopardized by placing the slipped femoral head on the neck of the femur, particularly if the slip has been present for a long time. It behooves the surgeon to carefully separate the femoral head in the line of the cartilaginous plate and with a skid to slowly, gradually and carefully replace the femoral neck under the femoral head and fix this position with threaded pins. If the patient has a long history and no obvious epiphyseal plate is visible by x-ray film, and in particular if there is no plate visualized at the time of operation, no separation or osteotomy is indicated. Instead, an osteotomy in the subtrochanteric or intertrochanteric area is recommended to regain a better relationship between the femoral head and
609
THE LIMPING CHIT..D
acetabulum. Major reconstruction in the intra-articular portion of the joint is fraught with serious complications. Some authors consider that patients with a "preslipped" or minimally slipped epiphysis should be placed in bed with traction, or in plaster, until the epiphyseal plate closes. We have seen the slip progress despite this "conservative" treatment, and it is my contention that the most conservative treatment for this condition is to insert threaded Steinmann pins across the epiphyseal plate. This is much preferred to the "watch and wait" attitude. I would warn against the use of the larger nails for internal fixation, since their insertion through the dense bone of the femoral head may further separate the head at the epiphyseal line and thus increase the pathology. In my postoperative care of the patients with mild slips, I place them in bed in traction until the synovitis of the hip joint has subsided. Thereafter, if adequate fixation with three pins is present, I do not hesitate to allow full activity. Removal of the pins, if they produce symptoms, may be done after the epiphysis has closed. The results following pinning "in situ" of the minimal or "preslipped" hips are excellent.
REFERENCES 1. Ferguson, A. B., Jr.: Orthopedic Surgery in Infancy and Childhood. Baltimore,
Williams & Wilkins Company, 1957. 2. Finder, J, G.: Transitory Synovitis of the Hip Joint in Childhood. ].A.M.A., 107:3, 1936. 3. Haythorn, S. R.: Pathological Changes Found in Material Removed at Operation in Legg-Calve-Perthes Disease. ]. Bone & ]oint Surg., 31-A:599, 1949. 4. Lucas, L. S.: Painful Hips in Children. AAOS Instructional Course Lectures V-1948, pp. 144-52. 2300 S. Hope St. Los Angeles, Calif. 90007
TOXIC SYNOVITIS OF THE HIP
Toxic or acute synovitis of the hip joint is probably the most common orthopedic condition causing a limp in the otherwise normal child. This condition, usually short in duration, is thought to be either an inflammatory involvement of the synovial membrane of the hip, or possibly the result of minor trauma to the joint. It is always considered a precursor, or a possible initial symptom, of the more serious LeggPerthes disease, but fortunately in a large percentage of the patients it lasts for three to 10 days and subsides without sequelae. Etiology Frequently there may be a history of an upper respiratory tract infection or streptococcal throat a week to 10 days prior to symptoms in the hip joint. In a few patie\ts there may be a definite history of trauma which may be incidentally remembered and may not be an important factor in the causation. Approximately 25 consecutive cases of toxic synovitis were aspirated at the Children's Hospital in Boston, and no organism was grown from the small amount of fluid that was present in these cases, which subsequently subsided in the typical manner. There may be some causal relation between the streptococcal infection and subsequent inflammatory changes in the hip joints similar to that of rheumatic fever. Some feel that this may be an allergic manifestation. No specific cause has been adequately documented for this condition. Symptoms The patient is typically a young boy, age five to 10, but the disease may occur at any age in childhood or in the adult. Typically, in the child, the parent may be the first to notice a mild limp, or the child may complain of a mild discomfort in the knee or hip joint which may gradually increase in severity over a period of 24 to 48 hours. He may or may not have a mild elevation of temperature, and symptoms are actually relieved by bed rest. On physical examination, one will find limitation of motion of the involved hip joint and, commonly, a mild hip flexion contracture. Pediatric Clinics of North America-Vol. 14, No.3, August, 1967
601
602
G.
WILBUR WESTIN
Depending on the severity of the involvement, the patient may complain when examiners attempt to test any motion of the hip joint. The ranges of motion most commonly limited are extension, rotation and abduction, and the child usually does not appear acutely ill, in fact, usually presents as a healthy person. Laboratory Work The laboratory findings are usually benign with a normal differential count and a normal or slightly elevated sedimentation rate during the acute phase of the disease. X-ray Findings Two findings may be present by x-ray: a bulge of the capsule des-cribed as the "obturator sign" seen on the lateral aspect of the involved hip joint, and an associated minimal widening of the joint as measured from the femoral head to the acetabulum. These are findings which the practiced eye of a radiologist may interpret, but frequently the x-ray findings are "negative." There usually are no findings typical of the early findings in Legg-Perthes disease. The provisional diagnosis involves all conditions in which the hip joint may be involved in an inflammatory type of affliction. The rapid subsidence of symptoms and absence of positive findings by x-ray may rule out the active stages of such conditions as Legg-Perthes disease, tuberculosis, rheumatic fever and rheumatoid arthritis, all of which have other findings more typical of these diseases. Probably the most important differential is from a septic hip joint. If there is any question, aspiration of the hip joint is indicated, and in the absence of findings of any purulent material in the joint, further radical treatment is not indicated at the time of admission. Perhaps this disease is best defined by Finder, 2 who states that "in transitory synovitis of the hip joint, the synovitis constitutes the entire disease but in other hip joint lesions, the synovitis may merely represent the point of departure into any one of the several ,disorders of the hip." Treatment Treatment for acute synovitis of the hip joint is conservative. The patient will get considerable relief of symptoms by placement in Russell's traction in bed, pulling the hip joint in the line of deformity at the outset and gradually extending it as the patient tolerates it. Progress of the disease will be indicated by a prompt return of the temperature to normal, a constant finding of essentially normal differential and white cell count, and, in particular, a rapid decrease in pain in the hip joint with recovery to a normal range of motion. It is our practice to keep these children in traction until they have a full range of normal motion and then to keep them on crutches for an additional three weeks. At the end of that time new x-ray films will confirm the negative findings. Most
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important, any child with transient synovitis of the hip joint should be followed at three- and six-month intervals to be assured that there has not been, in reality, a precursor to a more serious hip disease. I do not recommend the use of antibiotics in such a condition, since they may cloud the real issue should other conditions develop, and the antibiotic is of no specific benefit in the transient synovitis case. LEGG-CALW-PERTHES DISEASE
In 1909 Legg, Calve and Perthes separately published independent observations on degeneration in the hip joint. This was specifically differentiated from the then common entity of tuberculosis of the hip. This condition has since been called by many names, the most accepted of which include Legg-Calve-Perthes disease, coxa plana and osteochondritis deformans juvenilis (see also p. 551). Seventy-five to 85 per cent of the cases occur in males, and approximately 20 per cent are bilateral. The disease is characterized by aseptic necrosis of the femoral head and sometimes of the adjacent proximal femoral metaphysis. This initial process is followed by revascularization and regeneration of the epiphyseal bone. This sequence takes an average of four to five years for completion. Etiology There is no generally accepted causative agent. Some authors have felt that trauma was an inciting event, but it is difficult to accept this explanation in the bilateral cases. Others have explained the disease on an infectious basis, but no evidence of a bacterial process has been documented. Still others have felt that there is a metabolic deficiency, but most of the cases worked up in metabolic clinics have yielded little evidence to substantiate this. The lesion in the early stage of the disease (the degenerative stage) is reported by Haythom. 3 In examiners' curettements from the heads of femurs of 36 patients in early disease stages, he noted: "In every case, more or less degeneration, necrobiosis or necrosis was present without any local evidence of the cause.... The absence of infection was supported by many negative cultures. . . . In more advanced lesions, necrosis was extensive and complete involving not only marrow, but cartilage and bony spicules from broken trabeculae." Trueta of Oxford, England, has made a detailed study of the blood supply to the capital femoral epiphysis and from his work points out that the blood supply between the ages of four and seven years (corresponding with the most common age at onset of this ,disease) is undergoing extensive reorganization and is thus vulnerable to the secondary changes described above. It is my belief that the aseptic necrosis is primarily due to inter-
G.
604
Wn.BUR WESTIN
ruption of the blood supply to the femoral head and adjacent metaphyseal regions due to unknown cause. In a review of patients admitted to the Los Angeles Unit of the Shriners Hospitals between 1952 and 1957, the 42 cases that had reached maturity had the following mean time of various phases in this disease: MEAN TIME
Degenerative phase .................. Regenerative phase . . . . . . . . . . . . . . . . . . . Total duration . . . . . . . . . . . . . . . . . . . . . . Onset to weight-bearing ..............
1 8 4 2
year, years, years, years,
7 1 9 6
months month months months
The determination of the different phases as listed above is made primarily by x-ray evaluation; however, the onset of illness can be obtained only from an accurate history. Of these 42 cases completely evaluated, the average age at onset was six years and six months, with a range from three years and five months to nine years and 11 months. Clinical Manifestations and Treatment The onset of the disease is usually insidious. The child complains of mild discomfort in the knee or hip and ·manifests a mild limp. On physical examination one finds evidence of an irritable hip joint, with limited motion in all directions and commonly a mild flexion deformity. This corresponds to the initial synovitis. There is no single way to treat every case of Legg-Calve-Perthes disease. The extremes are from "do nothing" and allow full activity of the child with this condition, to complete bed rest through the reparative phase. Decisions about the course of treatment should be guided by a careful clinical evaluation of the hip with lesser emphasis placed on the x-ray changes that are characteristic in the natural course of this disease. Our program of treatment consists of admission of a child to the hospital upon making the initial diagnosis of an irritable hip joint. He is then placed in bilateral leg traction to keep him in bed and put the hip at rest. The period of traction necessary to obtain a full free range of motion of the hip without evidence of clinical irritability in the typical early case of this disease varies from three to 10 weeks. During this early phase the patient may be ambulatory in a cart in the sitting position with portable traction applied, or he may be allowed in the swimming pool under supervision for range of motion, but no weight-bearing. During this time other conditions of the hip joint such as tuberculosis, infection, complications of a congenitally dislocated hip, rheumatic fever or rheumatoid arthritis must be ruled out. This is not difficult as soon as the typical x-ray changes become apparent as the disease progresses. When the child has regained a full range of normal motion, when the hip is no longer irritable and he is free of pain, he is ambulated. The
THE LIMPING CHILD
605
method we prefer is the use of an ischial weight-bearing brace. The extremity is suspended free in a double upright brace with a Pattonbottom splint so that the toe cannot touch the ground. A build up of the opposite shoe of approximately 4 inches is usually supplied to faciliate ambulation. This is started with the usa of crutches until the child is used to wearing the brace. He then may discard crutches, and it is surprising how he will do on his own. It is granted that there is no single type of apparatus that will guarantee full protection of the hip joint, but the advantages of the ischial weight-bearing brace with the loose thigh and calf cuffs are many. The child has both hands free. The brace has no knee joint, so that the child cannot bend the knee and bear weight in a kneeling position, and it can accommodate readily to both standing and sitting. The parents and the child are both instructed to lengthen the brace as growth occurs so that the toe never touches the ground. A program of exercises to maintain a full range of extension and abduction of the hip is outlined and is to be performed on a regular basis to maintain the integrity of the musculature of the involved extremity. After a trial of approximately one month, the child is seen again, and if the hip is still irritable, the ambulatory treatment must cease. The child is returned to bed until the synovitis has again disappeared, and only then is ambulation resumed. The use of the brace for protecting weight-bearing through the hip joint is maintained until the reparative or regenerative phase of the disease has been well established, so that the soft femoral head is protected from the crushing and flattening that commonly occur in the untreated patient. The child in such a brace should be seen on regular visits with a maximum of three months between visits, and x-ray films and clinical evaluation of the hip should be made at each visit to check the progress of the disease. When the repair process has progressed to the point at which there is a spherical outline of the femoral head visible by x-ray, it is our practice to allow the patient to begin full weight-bearing without the brace. It is assumed at this time that the areas of necrosis have been replaced with ossifying cartilage, and it is rare that progressive changes will occur when the reparative process has reached this stage. The completion of the disease, however, is not indicated until complete reossification of the femoral head has occurred. In all cases there is a varying amount of residual flattening of the femoral head ("coxa plana") and widening of the epiphyseal line and the adjacent metaphysis, giving some degree of coxa magna. The only constant determinant of extent of radiographic residual and clinical loss of motion is the age at onset of the disease. The younger the age of onset, the better the prognosis for complete recovery. Experience with a number of patients sent to our hospital after having been allowed intermittent weight-bearing over a period of months
606
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Wn.BUR WESTIN
or even one or two years since the onset of disease has taught us that this type of management may result in changes by x-ray with subluxation of the femoral head and fragmentation. This may necessitate, in some cases, surgical reconstructive procedures. In the late stage of inadequately treated cases with gross deformity, and persistent limited motion with pain, one finds grossly the picture of an osteoarthritis of the joint which presents a serious problem to the attending orthopedic surgeon. In a few instances, in the early phases of this disease in a younger child, there may be a massive early destruction of the femoral head. Even after prolonged treatment with bed rest and traction, there may be a failure of irritability and limited range of motion to subside. In spite of prolonged traction, some of these femoral heads may sublux out of the joint. In the last six years we have had six such children on whom a Pemberton or Salter type of iliac osteotomy has been pedormed to provide better coverage for the femoral head. This operation has been limited only to those cases resistant to conservative therapy as outlined above. In the continued experience of treating 140 cases of Legg-Perthes disease, it has become evident that the hip must be treated clinically, as well as by x-ray. Specific cases with severe complications require specific procedures. The child with onset at early age, and with early treatment, rarely requires anything other than a conservative program of protecting the hip and treating it as outlined above. ·Characteristic x-ray findings are seen according to the stage of this disease. Initially, with the onset of synovitis, there may be a widening of the joint space and some demineralization of the femoral metaphysis with no apparent changes in the femoral head. In the degenerative phase (or aseptic necrosis phase) there is an increase in density of the femoral head which may involve a portion of, or the entire femoral head. There may also be changes on the metaphyseal side of varying degree. When the aseptic changes are manifested by the increased density of the head, one can expect, and should inform the parents, that as progression of disease occurs, the x-ray views will look much worse to the unpracticed eye before they begin to fill in and have a definite improvement. This stage of degeneration is the most important phase for protection of the hip joint from weight-bearing as well as prognosticating the result. If apparent aseptic necrosis is confined within the acetabulum and treatment is instituted, the prognosis is much better than when a fragmented epiphysis subluxes out of the confines of the acetabulum. The regenerative phase occurs after the absorption of the densities in the femoral head with revascularization and the conversion of invading cartilage into new epiphyseal bone proximal to, and new metaphyseal bone distal to, the epiphyseal line. The persistent radiolucent areas are considered to be composed of cartilage, but are not as subject to progressive flattening. It is felt by some that, once the regenerative phase
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has been established, weight-bearing is safe. It is our practice, however, to wait until that stage of the regenerative phase when a spherical outline of the femoral head is present before full weight-bearing is allowed.
SLIPPED CAPITAL FEMORAL EPIPHYSIS
This is a condition in which the femoral head is displaced from its normal relation to the femoral neck with disturbance of the mechanical alignment of the proximal femur. It may occur insidiously or acutely, and is primarily found in the adolescent between the ages of 10 and 15. Approximately 20 per cent of the cases are bilateral. Etiology The cause of this condition is not known, but many contributing factors have been considered. In approximately 50 per cent of the male patients one will note the Frohlich type of female distribution of fat and immature secondary sexual characteristics. It has been my experience and that of others that this condition may occur in otherwise normalappearing and normal-functioning children. With rapid growth, the cartilaginous epiphyseal plate is widened as the increased activity of the maturation of the cartilage cells into bone takes place. With this widening, some decrease in mechanical stability may occur. This happens in all the epiphyses; however, the proximal femur has a configuration which leads to the production of considerable shearing stress at the epiphyseal line, and this may well render this area particularly vulnerable to disruption. Some authors have felt that there is a definite endocrine disturbance in these children which produces abnormal weakness of the epiphyses. The factor of trauma is probably incidental to the underlying pathology. Sudden symptoms following an injury are most accurately interpreted as resulting from the addition of stress to an already unstable system with the production of epiphyseal disruption and slippage. Symptoms In the majority of cases the symptoms comprise the insidious onset of pain and a limp. The symptoms progress as the femoral head slips posteriorly and inferiorly. The typical patient will have noted pain in the hip, thigh or knee for months, or perhaps for several weeks in mild involvement. Diagnosis The diagnosis is made by physical and x-ray examination. On physical examination, a hip that externally rotates as one attempts to Hex it should alert one's suspicions. Depending on the degree of irritation
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WILBUR WESTIN
in the hip joint and the amount of slippage, one will find varying degrees of limitation of motion. Of particular note is the loss of internal rotation and of full flexion. Most of the patients will have an external rotation deformity of the hip. The x-ray evaluation depends on satisfactory anteroposterior and lateral views of the hip; however, the lateral view is of prime importance, since it is here that one can obtain the clearest picture of the amount of slippage that has occurred. It is this view that is often used as the basis of the treatment which the orthopedic surgeon will initiate. Treatment The treatment of this condition has been and still is somewhat controversial. It is our practice, once the diagnosis has been established, to put the patient in bed in traction until the acute symptoms have subsided. If the displacement of the femoral epiphysis is not more than one third the diameter of the femoral neck, we surgically place two or three threaded %6 -inch pins across the epiphysis from the lateral approach into the femoral head. Such fixation is usually adequate to prohibit further slipping. If the child is ske~etally immature, with one or more years of growth, it is common for the deformity to diminish considerably as growth proceeds. The range of motion will also return toward normal. Cases in which the epiphysis has slipped more than 50 per cent of the width of the femoral neck often warrant surgical correction. This, however, carries with it the too frequent severe complication of disruption of the blood supply to the femoral head with ensuing aseptic necrosis. At the Massachusetts General Hospital, Klein and his associates report a most remarkable series of open reductions in such cases without serious complications. This operation was performed by a group of men familiar with this condition. Because of the danger of compromising the nutrient supply to the femoral head, a longitudinal incision was made through the joint capsule, paralleling the vessels. This was then made into a "T," with the top parallel to the acetabulum above the vessels. Through such an exposure the lesion was well visualized without undue danger. The retinaculum carrying the blood vessels up the neck of the femur may be jeopardized by placing the slipped femoral head on the neck of the femur, particularly if the slip has been present for a long time. It behooves the surgeon to carefully separate the femoral head in the line of the cartilaginous plate and with a skid to slowly, gradually and carefully replace the femoral neck under the femoral head and fix this position with threaded pins. If the patient has a long history and no obvious epiphyseal plate is visible by x-ray film, and in particular if there is no plate visualized at the time of operation, no separation or osteotomy is indicated. Instead, an osteotomy in the subtrochanteric or intertrochanteric area is recommended to regain a better relationship between the femoral head and
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acetabulum. Major reconstruction in the intra-articular portion of the joint is fraught with serious complications. Some authors consider that patients with a "preslipped" or minimally slipped epiphysis should be placed in bed with traction, or in plaster, until the epiphyseal plate closes. We have seen the slip progress despite this "conservative" treatment, and it is my contention that the most conservative treatment for this condition is to insert threaded Steinmann pins across the epiphyseal plate. This is much preferred to the "watch and wait" attitude. I would warn against the use of the larger nails for internal fixation, since their insertion through the dense bone of the femoral head may further separate the head at the epiphyseal line and thus increase the pathology. In my postoperative care of the patients with mild slips, I place them in bed in traction until the synovitis of the hip joint has subsided. Thereafter, if adequate fixation with three pins is present, I do not hesitate to allow full activity. Removal of the pins, if they produce symptoms, may be done after the epiphysis has closed. The results following pinning "in situ" of the minimal or "preslipped" hips are excellent.
REFERENCES 1. Ferguson, A. B., Jr.: Orthopedic Surgery in Infancy and Childhood. Baltimore,
Williams & Wilkins Company, 1957. 2. Finder, J, G.: Transitory Synovitis of the Hip Joint in Childhood. ].A.M.A., 107:3, 1936. 3. Haythorn, S. R.: Pathological Changes Found in Material Removed at Operation in Legg-Calve-Perthes Disease. ]. Bone & ]oint Surg., 31-A:599, 1949. 4. Lucas, L. S.: Painful Hips in Children. AAOS Instructional Course Lectures V-1948, pp. 144-52. 2300 S. Hope St. Los Angeles, Calif. 90007