Legg-Calvé-Perthes (LCP) disease is a puzzling childhood hip disease, in which the femoral head unexpectedly loses its blood supply resulting in aseptic necrosis of the femoral head (Fig. 1A, B).1 Subsequent revascularization leads to femoral head softening, femoral head shape change, and eventual flattening and subluxation if treatment is not initiated. The goal of intervention has been to prevent femoral head deformation by containing the femoral head within the acetabulum during Waldenstrom fragmentation and reossification phases, using the acetabulum as a mold for femoral head development with the ultimate goal of avoiding premature arthritis.2,3
Initial treatment to achieve this goal centered on abduction casts and brace, which were not only tedious and psychologically difficult for the child but were also shown to be ineffective.4–10 Surgical containment methods were then developed, and now provide the mainstay of treatment.11–16
SURGICAL CONTAINMENT METHODS
Initial surgical containment methods concentrated on containing the femoral head within the acetabulum by proximal varus osteotomy (VO) as described by Soeur,11 Axer,12 and Lloyd-Roberts et al.13 Although proximal femoral osteotomy can effectively contain the femoral head within the acetabulum, and has substantial benefits in that the procedure naturally slightly decompresses the hip joint due to the modest femoral shortening effect that angle change/wedge excision provides, VO sometimes resulted in a prolonged limp and limb shortening.17–19
Salter20 introduced the concept of innominate osteotomy as a method of containment to avoid the consequences of femoral osteotomy. Salter felt that acetabular rotation would also provide better containment than VO of the femur; however, studies have shown little difference in the radiographic or functional outcomes with either of these 2 methods.21,22 Furthermore, usage of a Salter osteotomy to achieve containment may not allow for adequate acetabular rotation to cover the femoral head in severe cases, potentially leading to iatrogenic hinge abduction.23 These problems of limp, limb shortening, and iatrogenic impingement are common in older patients with severe disease, and have led to the development and study of advanced containment methods (Fig. 2).
ADVANCED CONTAINMENT METHODS: SALTER PROCEDURE PLUS PROXIMAL FEMORAL VO
Rab23demonstrated that the Salter osteotomy provides only modest additional coverage, which was often inadequate in many cases of Perthes disease where the femoral head enlarges. Thus, the first effort to provide advanced containment was to combine the Salter procedure with VO of the proximal femur (Figs. 3, 4). This combination of procedures was introduced in North America by Craig and Kramer24 (Los Angeles), Crutcher and Staheli25 (Seattle), and Olney and Asher26 (KS).
The benefits of a combined procedure include that one can gain full coverage of the femoral head without the risk for iatrogenic impingement of the enlarged head, which can occur with the Salter procedure alone. Furthermore, the femoral osteotomy decompresses the joint (effectively a slight shortening) while at the same time not requiring as great a degree of varus correction of the femur (and its associated prolonged limp) because containment is assured by the concomitant Salter procedure. The early reports by Craig and Kramer,24 Crutcher and Staheli,25 and Olney and Asher26 were followed by a series presented by Vukasinovic et al27 who described the benefit of the combined procedure, noting that the combined procedure was effective in severe cases of Perthes disease.
Several considerations should be given when considering the combined osteotomy method for Perthes disease. The sequence of performing concomitant osteotomy is not extremely important; however, many surgeons would consider doing the femoral osteotomy first, as the internal fixation is more stable. This would be followed by the innominate osteotomy, which is secured with 2 threaded pins.
Performing a proper femoral osteotomy requires achieving only a modest degree of varus. Most authors state that this should be a maximum of 20 degrees, and perhaps even 15 degrees in an older child. As in any femoral osteotomy, the femoral cut should also be as proximal as possible, usually at the intertrochanteric level, so that one does not lateralize the distal shaft, which may insure a prolonged limp. One should also select a fixation device with appropriate offset, which medializes the femoral shaft. The traditional Synthes (West Chester, PA) blade plate has been a good choice because one can select a variety of fixed offsets to match the degree of varus performed. In addition, Weiner et al18 have emphasized the importance of performing a simultaneous proximal femoral greater trochanteric epiphysiodesis to minimize the chances of subsequent trochanteric overgrowth.28,29 This is particularly important when an isolated femoral osteotomy is performed, and likely should be done when a femoral osteotomy is performed in a combination with a Salter osteotomy. Subsequent overgrowth of the greater trochanter remains a problem with progressive functional varus, even when combined procedures are performed (Fig. 3).18,28,29
As for the Salter osteotomy, the traditional methods of Salter can be used, and the approach is well described.20 Most surgeons use a Gigli saw to make the osteotomy cut, which allows one to advance the acetabulum slightly anteriorly. This unfortunately increases the instability of the osteotomy, and some have elected to use a modified method where the posterior cortex of the ilium at the sciatic notch remains intact. This method includes using a saw for the anterior two thirds of the osteotomy, and then an osteotome to complete the cut back to cortical bone in the sciatic notch. An appropriate triangular bone graft is then placed in position. Threaded pins are used with care taken to direct the distal tips medially and posteriorly to avoid entering the acetabulum. In addition, one must avoid creating iatrogenic retroversion. Retroversion is avoided by pulling the distal acetabular fragment forward rather than lateral. As Salter20 said in his original description, the anterior inferior spine should remain in line with the anterior superior spine when positioning a Salter osteotomy for final fixation.
Combining a proximal femoral varus with a Salter osteotomy for more severe Perthes disease remains a good choice with no particular disadvantages, other than that one can still provide too much femoral varus with a prolonged limp. Some cases will require a late valgus osteotomy or greater trochanter transfer to correct the limp (Fig. 3C–D).30,31
ADVANCED CONTAINMENT METHODS: TRIPLE PELVIC OSTEOTOMY
With a goal of providing full containment in severe cases while avoiding a femoral osteotomy, the triple pelvic osteotomy has been used in our institution to treat Perthes disease.32 The philosophy was developed after extensive experience with femoral osteotomy, Salter osteotomy, and combined osteotomies (Table 1).
Dr Christoph Meyer, a Swiss and then South African orthopaedic surgeon, introduced us to the concept of using triple pelvic osteotomy for treatment of Perthes disease in the mid 1990s. This advanced osteotomy allows more complete coverage of an enlarged femoral head with less risk for the iatrogenic impingement that can occur with a Salter procedure alone. Meyer emphasized that one should not select a VO to treat a disorder (Perthes) that spontaneously grows into varus over time (patients with Perthes disease have delayed growth or sometimes complete closure of the proximal femoral physis) while the greater trochanter continues to grow (Fig. 3C–D). Most patients with Perthes disease have relative coxa vara when healed—even when no osteotomy has been performed. Meyer, therefore, theorized that one should avoid proximal femoral osteotomy in treating Perthes disease.
Triple pelvic osteotomy in LCP disease has been studied throughout Europe. Poul and Vejrostova33 in 2001 reported on 12 patients with LCP disease between the age of 9 to 12 years who underwent triple osteotomy, and they noted successful containment and remodeling in 11 of 12 cases (even in deformed femoral heads). Kumar et al34 in 2002 reported on 21 cases that underwent the procedure with severe disease with an 18% gain in the acetabular index, and a 22% gain in the center edge angle. Vukasinovic et al35 in 2009 reported their results in 30 patients with marked improvements in the center edge angle and femoral head containment. As a result, as discussed at the 2010 European Pediatric Orthopaedic Society meeting, triple innominate osteotomy has become a common surgical method for the treatment of severe Perthes disease in older children in European centers.36
In our center, we have performed the procedure in 75 hips. We recently reviewed the results of 40 hips in 39 children with a minimal follow-up of 3 years.32 All were lateral pillar B or C. At final follow-up, 42% had a good outcome (Stulberg I or II), 47% had a fair outcome (Stulberg III), and 11% had a poor outcome (Stulberg IV or V). Lateral pillar B hips were more likely to have a good outcome (65%) compared with lateral pillar C hips (12.5%) with no lateral pillar B hips having a poor outcome. Seventeen percent of lateral pillar C hips <8 years had a poor outcome compared with 50% in children over age 8 years. Four of these children required further surgery, which included femoral head and neck recontouring or valgus osteotomy.
We now recommend triple pelvic osteotomy for older children with more severe symptomatic Perthes disease. We still use the Catterall groupings and at-risk factors as our primary radiographic determinant but also consider the Herring classification, which is now more commonly used but may be less useful, in that the head changes can be visualized in only 1 plane.
SURGICAL METHOD FOR TRIPLE PELVIC OSTEOTOMY
Our surgical technique focuses on making the 3 bony cuts near the acetabulum to allow free rotation while avoiding the separate posterior skin incision for the ischial cut as described by Tonnis (requiring separate prepping and draping).37,38 Our procedure is performed in 3 steps with a Salter-type iliac cut made through an anterolateral incision above the acetabulum, a superior pubic ramus cut made through a transverse medial groin incision, and an ischial osteotomy just below the acetabulum (through the same skin incision as the pubic ramus cut) (Fig. 5A–F).32
Late impingement in Perthes disease can occur with large femoral heads being prominent anteriorly and laterally, causing labral damage, which can lead to premature hip joint arthritis. Accordingly, when one performs triple innominate osteotomy, care is taken to avoid errors in positioning the acetabular segment. Acetabular rotation needs to be done with great care, now that we are aware that a femoral head can be overcontained and that some patients may have late impingement problems. Owing to the fact that 3 cuts are made, which greatly frees the acetabular segment, it is easy to create iatrogenic acetabular retroversion by rotating the acetabulum externally in the transverse plane.39 This should be avoided by bringing the anterior superior spine forward but not laterally. We try to rotate the segment in the frontal plane with lateralization of the upper segment of the acetabulum, and medialization and slight upward positioning of the ischial and pubic cuts, respectively.
A relatively small triangular bone graft is used, as the graft does not need to be as large as when a Salter osteotomy alone is performed. We have noted the difference in graft size required when a specific acetabular rotation procedure is selected, noting that a Salter procedure requires a relatively large triangular graft,20 a triple procedure requires a graft that is only half the size of that for a typical Salter procedure,32,37,38 with the Ganz acetabular osteotomy requiring no graft at all.40 The principle is that with rotation at 3 sites, iliac rotation is limited.
Once the graft has been placed, temporary Kirschner wires are used to position the acetabular fragment followed by image intensification analysis of acetabular position. The sourcil should be horizontal, and one can image the opposite hip to compare the position. The ischial spine should not be unusually prominent; as such prominence suggests iatrogenic acetabular retroversion. The femoral head should be well covered with a center-edge angle of approximately 30 degrees, but one should avoid excessive acetabular rotation, which might lead to protrusio acetabuli with a risk for late impingement.
The osteotomy is then permanently fixed with threaded Kirschner wires or 4.5 mm diameter fully threaded A-O screws. Using fully threaded screws prevents excessive compression at the osteotomy site, which may cause you to lose coverage by pulling the distal fragment upward. In older children, one can use a screw to fix the pubic osteotomy or A-O reconstruction plate to avoid hip spica use. In younger children, we put the patient in a hip spica for 6 weeks to avoid the risk for pseudarthrosis.
Advanced containment methods provide a good option for treatment of Perthes disease in an older child with severe femoral head involvement. Combining proximal femoral osteotomy with Salter osteotomy seems a good choice for most centers, as children's orthopaedic surgeons are generally familiar with the procedures and can easily combine them.
Triple innominate osteotomy provides a progressively more attractive option, as it allows relatively complete coverage of the femoral head (unlike the Salter osteotomy) while avoiding the risks of VO of the femur. Other methods for older children with more severe head involvement include shelf acetabuloplasty,41 joint distraction,42 and femoral head and neck osteochondroplasty.43 Advanced containment methods for older LCP patients with severe involvement will continue to evolve, as we gain a further understanding of this fascinating orthopaedic disease.
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