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'Bailey, Joseph A.: Disproportionate short stature: Diagnosis and management. Philadelphia, W.B. ... BILL SCHILLING. Zimmer-Schilling Assoc. 2435 Kimberly ...

RECURRENT LEGG-CALVE-PERTHES DISEASE Wayne Z. Burkhead, M.D.* Dennis R. Wenger, M.D.*,+

Recurrence of Legg-Perthes disease after complete healing of a previously affected femoral head is a rare entity, with only four cases previously described.3'5'6'7 This case provides another example of the characteristic age at onset and clinical course of recurrent LeggPerthes disease.

Case Report C. W., a six year old white male, first saw an orthopaedic surgeon because he had a painless mass in his left thigh. With the exception of the mass, his physical examination, including hip motion, was normal. Radiographic examination revealed a sessile osteochondroma, and as an additional finding, irregularity of the ipsilateral proximal femoral epiphysis (Fig. 1). An excisional biopsy of the mass established the diagnosis of osteochondroma. Because he had no hip symptoms he received no treatment for his hip at that time.

Later he began to complain of pain in both hips. Examination revealed mild limitation of internal rotation and abduction of his hips. Radiographic examination (Fig. 2) revealed irregular densities of the proximal femoral epiphysis bilaterally, suggesting avascular necrosis with collapse of the anterior segments, but without "at risk" signs. He was diagnosed as having Legg-Perthes disease and was treated with a two week period of bed rest, followed by limitation from

Figure 2.

strenuous activity for several months. His clinical course was benign and radiographs taken three years later revealed complete healing (Fig. 3).

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Figure 1. *

Texas Scottish Rite Hospital 2222 Welborn Street, Dallas, Texas 75219

+ Former Resident, Department of Orthopaedic Surgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242

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Figure 3.

At age ten the child developed a limp and complained of left hip pain. Radiographs revealed a subchondral fracture and increased density in the left

Recurrent Legg-Calve-Perthes Disease

femoral head (Figs. 4a and b), and he was referred to the Texas Scottish Rite Hospital. Physical examination revealed a left Trendelenburg gait and decreased

Figure 4b.

Figure 4a.

abduction and internal rotation of the left hip. Subsequent radiographs taken at the age of ten years and nine months (Fig. 5) revealed further collapse with significant head involvement and lateral subluxation. Radiographs of other epiphyses, as well as his spine, were normal. Bone age films revealed a bone age two standard deviations less than his chronological age. Laboratory exam revealed normal calcium, phosphorus, blood urea nitrogen, creatinine, alkaline phosphatase and normal thyroid function. Because of the significant femoral head involvement with lateral subluxation and inability to comply with brace treatment, we elected to surgically contain the femoral head by proximal femoral varus osteotomy (Fig. 6). He did well postoperatively and two years after surgery has a pain-free hip with a nearly full range of motion. He continues to have a mild Trendelenberg gait secondary to proximal femoral physeal arrest (as a consequence of the Legg-Perthes disease). with resulting relative greater trochanteric overgrowth (Fig. 7).

Discussion Recurrent Legg-Perthes disease is rare. This case is only the fifth reported. In these five cases, the age at onset of the initial episode ranged from two to six years, with an average of 3.9 years. The length of time until recurrence ranged from three to five years, with an average of 4.5 years. All five patients were male. Two patients had unilateral disease, while three were affected bilaterally. Judging from previous reports,3'6'7 all cases had Catterall 2 or 3 involvement at the initial episode. With the repeat episode, all but one had complete head involvement (Catterall 4), and three of five had "at risk" signs, including lateral subluxation, lateral calcification, as well as Gage's sign. Evaluation of this small group of patients suggests that the second episode of avascular necrosis is more severe and usually affects the entire head. Thus, the prognosis is worse. In addition, the child is older at the second episode, and older age is also widely accepted as being associated with a worse prognosis.4

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Figure 5. Figure 7.

Figure 6.

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Any case of bilateral Legg-Perthes disease should raise the suspicion of epiphyseal dysplasia.1 Our patient had a thorough radiographic and laboratory workup which revealed no generalized disorder. This negative survey, coupled with the fact that the initial lesions healed, suggests that this case represents recurrent avascular necrosis (Legg-Perthes disease) rather than an epiphyseal dysplasia. One concept of the etiology of Legg-Perthes disease is that of repeated episodes of infarction.8 Patients with recurrent Legg-Perthes, therefore, may have repeated vascular insults over a several year period. What predisposes this very small group of patients to recurrence, and what protects the majority of patients from recurrence, is unanswered. We selected surgical containment for this patient because of progressive subluxation and head collapse and the patient's refusal of brace containment. He developed one of the known complications following containment by proximal femoral osteotomy, that is, greater trochanter overgrowth. The overgrowth was not caused by the surgical procedure but, instead, was

Recurrent Legg-Calve-Perthes Disease

due to physeal closure which occurs in a significant number of older children with Legg-Perthes disease. However, addition of the varus osteotomy to the already compromised biological situation (physeal closure secondary to severe disease) resulted in an even farther proximally positioned greater trochanter with resulting limp. Evidence of premature growth plate closure in Legg-Perthes disease may be an indication for containment by some method other than proximal femoral varus osteotomy.2

Bibliography 'Bailey, Joseph A.: Disproportionate short stature: Diagnosis and management. Philadelphia, W.B. Saunders and Company, 1971, pp. 380-435. 2Barnes, J.M.: Premature epiphysial closure in Perthes' disease. J. Bone and Joint Surg., 62-B:432-437, 1980.

3Caffey, J.P.: Pediatric x-ray diagnosis, 4th edition. Chicago, Yearbook Medical Publishers, Incorporated, 1961. 4Catterall, A.: The natural history of Perthes disease. J. Bone and Joint Surg., 53-B:37-53, 1971. 5Katz, J.F.: Recurrent avascular necrosis of the proximal femoral epiphysis in the same hip in Gaucher's disease. J. Bone and Joint Surg., 49-A:514-518, 1967. 6Katz, J.F.: Recurrent Legg-Calve-Perthes disease. J. Bone and Joint Surg., 55-A:833-836, 1973. 7Kemp, H.B.S., Cholmeley, J.A., Baijens, J.K.: Recurrent Perthes disease. Brit. J. Radiology, 44:675-681, 1971. 8Sanchis, J., Azhir, A., and Freeman, M.A.R.: Experimental stimulation of Perthes disease by consecutive interruption of the blood supply to the capital femoral epiphysis in the puppy. J. Bone and Joint Surg., 55-A:335-342, 1973.

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