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Reading List: Perthes' disease by Mr A Catterall
Constitutional and aetiological aspects of Perthes' disease Guerado E, Garcès. Perthes' disease: a study of constitutional aspects in adulthood. J Bone Joint Surg [Br] 2001;83-B:569-71. Kealey WDC, Cosgrove AP, Moore AJ, Cook S. Deprivation, urbanisation and Perthes' disease in Northern Ireland. J Bone Joint Surg [Br] 2000;82-B:167-71. Kitoh H, Kitakoji T, Katoh M, Takamine Y. Delayed ossification of the proximal capital femoral epiphysis in Legg-Calvè-Perthes' disease. J Bone Joint Surg [Br] 2003;85-B:121-4. Comment
The three papers discuss the aetiology of Perthes' Disease which remains unknown. There are three important groups of factors in this process: the susceptible child, recurrent epiphyseal infaction, and the subchrondral fracture. Hall et al1 were among the first to identify the susceptible child as a boy between the ages of 4 and 9 years, who shows delayed bone age, short stature of dispropionate type, and is born in a family of social class 4 and 5. There is a 9 fold increase in the incidence of inquinal hernia and an unexpectedly high incidence of obstructive renal disease. These papers are strongly in support of this concept with evidence of constitutional factors (Guerido et al), delayed bone age (Kitoh et al), and social deprivation (Kealy et al)
Additional Reading
1. Hall AJ, Barker DJ, Dangerfield PH, Taylor JF. Perthes' disease of the hip in Liverpool. Br Med J 1983;287:1747-9. Szepesi K, Pósán E, Hársfalvi, Ajzner É, Szücs G, Csernátony Z, Udvardy M. The most severe forms of Perthes' disease associated with the homozygous Factor V Leiden mutation. J Bone Joint Surg [Br] 2004;86-B:426-9.
Kealey WDC, Cosgrove AP, Mayne EE, McDonald W, Murray P. The role of coagulation abnormalities i nthe development of Perthes' disease. J Bone Joint Surg [Br] 2000;82-B:744-6.
Hayek S, Wientroub S, Kenet G, Lubetsky A, Rosenberg N, Gitel S. Does throbophilia play an aetiological role in Legg-Calvé-Perthes disease? J Bone Joint Surg [Br] 1999;81-B:686-90.
Comment
These papers report an investigation of a possible increased incidence of abnormal clotting factors in Perthes' disease. This concept was first introduced by Glueck et al1 suggesting thromophilia as an aetiological factor. The debate has continued, but is well reviewed in the editorial by Liesner2. The problem relates to the controls used in these studies. The papers by Kealy et al and Hayek et al support this review, while the paper by Szepesi et al finds a possible association with the severest forms of this condition. Thrombophilia does not explain the episodes of recurrent epiphyseal infarction which are a feature of the morbid anatomy of this condition, and in the many cases does not involve the whole epiphysis. It is suggested therefore, that it could be part of the evidence for the susceptible child.
Additional Reading
1. Glueck CJ, Crawford A, Roy D, et al. Association of antithrombotic factor deficiencies and hypofibrinolysis with Legg-Perthes disease. J Bone Joint Surg [Am] 1996;78-A:3-13. Management of problems in the older child with Perthes' disease presenting with early or late deformity
Maxwell SL, Lappin KJ, Kealey WD, McDowell BC, Cosgrove AP. Arthrodiastasis in Perthes' disease: preliminary results. J Bone Joint Surg [Br] 2004;86-B:244-50.
Comment
It has always been accepted that the prognosis for the older child without treatment is poor, in particular when the child presents over the age of eight years. The problem at this age is that femoral osteotomy leaves residual shortening and a high trochanter because the femoral neck cannot remodel with the growth remaining. In addition innominate osteotomy will not always control the subluxation at this age. Femoral head flattening and stiffness is an early feature. Arthrodiastasis offers a method of decompressing the joint at an early stage to prevent this happening. It is, however, a technically demanding procedure. Lateral shelf acetabuloplasty has been shown to produce good results in this older age group. It is a simpler procedure to perform but does require the patient to be in a hip spica. Additional Reading
1. Daly K, Bruce C, Catterall A. Lateral shelf acetabuloplasty in Perthes' disease: a review at the end of growth. J Bone Joint Surg [Br] 1999;81-B:380-4.
Bankes MJK, Catterall A, Hashemi-Nejad A. Valgus extension osteotomy for 'hinge abduction' in Perthes' disease: results at maturity and factors influencing the radiological outcome. J Bone Joint Surg [Br] 2000;82-B:548-54.
Yoo WJ, Choi IH, Chung CY, Cho TJ, Kim HY. Valgus femoral osteotomy for hinge abduction in Perthes' disease: decision-making and outcomes. J Bone Joint Surg [Br] 2004;86-B:726=30.
Comment
These two papers are a timely reminder of the value of valgus upper femoral osteotomy in the management of late cases of Perthes' disease with pain shortening and fixed deformity. The object of the operation is to produce stable hip movement as the child stands and walks. It has the advantage of improving leg length, restoring useful abduction, and also a better abduction lever arm. Additional Reading
1. Quain S, Catterall A. Hinge abduction of the hip: diagnosis and treatment. J Bone Joint Surg [Br] 1986;68-B:61-4. Sugimoto Y, Akazawa H, Mitani S, Asaumi K, Aoki K, Inoue H, Miyake Y. A new scoring system for Perthes' disease based on combined lateral and posterior pillar classifications. J Bone Joint Surg [Br] 2004;86-B:887-91.
Comment
This paper is one of the first to use anteroposterior and lateral radiogrphs to classify the severity of Perthes' disease. The classification is based on the lateral pillar concept developed by Dr Herring from Dallas, Texas. Papers report difficulty using this classification although it is proving more reliable than other methods in general use. With the current long-term results now being published from Dr Herring and colleagues from North America using the lateral, pillar grading, this paper may help the practicing Orthopaedic surgeon in caing for these children.
Additional Reading 1. Herring JA, Kim HT, Browne R. Legg-Calvé-Perthes disease part I: classification of radiographs with use of the modified lateral Pillar and Stulberg classifications. J Bone Joint Surg [Am] 2004;86-B:2103-20. Kamegaya M, Saisu T, Ochiai N, Moriya H, Hisamitsu J. A paired study of Perthes' disease comparing conservative and surgical treatment. J Bone Joint Surg [Br] 2004;86-B:1176-81.
Comment
This paper comes to similar conclusions to the large long-term study reported by Herring and colleagues. Like the Herring report it emphasises that treatment with a brace is not as satisfactory as surgical treatment. Remodelling of the neck shaft angle can be anticipated following femoral osteotomy, particularly where the child is under the age of eight years at the time of operation. Mr Anthony Catterall, Consultant Orthopaedic Surgeon
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