An Analysis of Relative Gait Impairment in Commonly Diagnosed Pediatric Conditions.

BACKGROUND: Gait indices were developed to represent the magnitude of impairment extracted from a gait analysis with a single value. The Gillette Gait Index (GGI), and the Gait Deviation Index (GDI) are 2 widely used indices that represent gait impairment differently based on their statistical properties. Our purpose was to (1) report on the results of gait analysis for a broad spectrum of pediatric conditions using the GGI and GDI, and (2) identify the parameters that dominate impairment. METHODS: A total of 1439 children with 13 different diagnoses with a complete, baseline gait analysis were identified. The GGI and its 16 parameters were calculated in all cases, and the GDI was calculated from a smaller subset. T tests, and z-scores were used to compare each of these values to typically developing children for each diagnosis. A separate linear regression controlling for age, sex, and use of an orthosis, or assistive device was performed for the GGI. RESULTS: In our series, there were 71 typically developing children with a GGI of 31. We qualify relative gait impairment as severe, mild, or moderate as based on the GGI, and propose that values <100 represent mild, 100 to 200 represent moderate, and >200 represents severe impairment. On the basis of strong correlation between the GGI and GDI, we suggest that GDI values >80 represent mild, and values <70 represent severe impairment. T tests and z-scores demonstrated that both the number and magnitude of abnormal parameters increase the GGI. These tests also identified the most clinically relevant parameters contributing to functional impairment for each diagnosis. Multivariate linear regression showed that all diagnoses except flatfoot and scoliosis demonstrated statistically significant differences in GGI scores. CONCLUSIONS: This is the first study to apply these gait indices to a large population of diverse pediatric conditions. We propose GGI and GDI values to qualify gait impairment among these conditions as severe, moderate, or mild. Furthermore, impairment in gait reflects both the number and magnitude of abnormal parameters within each condition. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Two cases of Legg-Perthes and intellectual disability in Tricho-Rhino-Phalangeal syndrome type 1 associated with novel TRPS1 mutations.

Tricho-Rhino-Phalangeal syndrome is a rare autosomal dominant genetic disorder caused by mutations in the TRPS1 gene. This malformation syndrome is characterized by distinctive craniofacial features including sparse scalp hair, bulbous tip of the nose, long flat philtrum, thin upper vermilion border, and protruding ears. Skeletal abnormalities include cone-shaped epiphyses at the phalanges, hip malformations, and short stature. In this report, we describe two patients with the physical manifestations and genotype of TRPS type I but with learning/intellectual disability not typically described as part of the syndrome. The first patient has a novel heterozygous two-base-pair deletion of nucleotides at 3198-3199 (c.3198-3199delAT) in the TRPS1 gene causing a translational frameshift and subsequent alternate stop codon. The second patient has a 3.08 million base-pair interstitial deletion at 8q23.3 (113,735,487-116,818,578), which includes the TRPS1 gene and CSMD3. Our patients have characteristic craniofacial features, Legg-Perthes syndrome, various skeletal abnormalities including cone shaped epiphyses, anxiety (first patient), and intellectual disability, presenting unusual phenotypes that add to the clinical spectrum of the disease.

Head reduction osteotomy with additional containment surgery improves sphericity and containment and reduces pain in Legg-Calvé-Perthes disease.

BACKGROUND: Severe femoral head deformities in the frontal plane such as hips with Legg-Calvé-Perthes disease (LCPD) are not contained by the acetabulum and result in hinged abduction and impingement. These rare deformities cannot be addressed by resection, which would endanger head vascularity. Femoral head reduction osteotomy allows for reshaping of the femoral head with the goal of improving head sphericity, containment, and hip function. QUESTIONS/PURPOSES: Among hips with severe asphericity of the femoral head, does femoral head reduction osteotomy result in (1) improved head sphericity and containment; (2) pain relief and improved hip function; and (3) subsequent reoperations or complications? METHODS: Over a 10-year period, we performed femoral head reduction osteotomies in 11 patients (11 hips) with severe head asphericities resulting from LCPD (10 hips) or disturbance of epiphyseal perfusion after conservative treatment of developmental dysplasia (one hip). Five of 11 hips had concomitant acetabular containment surgery including two triple osteotomies, two periacetabular osteotomies (PAOs), and one Colonna procedure. Patients were reviewed at a mean of 5 years (range, 1-10 years), and none was lost to followup. Mean patient age at the time of head reduction osteotomy was 13 years (range, 7-23 years). We obtained the sphericity index (defined as the ratio of the minor to the major axis of the ellipse drawn to best fit the femoral head articular surface on conventional anteroposterior pelvic radiographs) to assess head sphericity. Containment was assessed evaluating the proportion of patients with an intact Shenton's line, the extrusion index, and the lateral center-edge (LCE) angle. Merle d'Aubigné-Postel score and range of motion (flexion, internal/external rotation in 90° of flexion) were assessed to measure pain and function. Complications and reoperations were identified by chart review. RESULTS: At latest followup, femoral head sphericity (72%; range, 64%-81% preoperatively versus 85%; range, 73%-96% postoperatively; p = 0.004), extrusion index (47%; range, 25%-60% versus 20%; range, 3%-58%; p = 0.006), and LCE angle (1°; range, -10° to 16° versus 26°; range, 4°-40°; p = 0.0064) were improved compared with preoperatively. With the limited number of hips available, the proportion of an intact Shenton's line (64% versus 100%; p = 0.087) and the overall Merle d'Aubigné-Postel score (14.5; range, 12-16 versus 15.7; range, 12-18; p = 0.072) remained unchanged at latest followup. The Merle d'Aubigné-Postel pain subscore improved (3.5; range, 1-5 versus 5.0; range, 3-6; p = 0.026). Range of motion was not observed to have improved with the numbers available (p ranging from 0.513 to 0.778). In addition to hardware removal in two hips, subsequent surgery was performed in five of 11 hips to improve containment after a mean interval of 2.3 years (range, 0.2-7.5 years). Of those, two hips had triple osteotomy, one hip a combined triple and valgus intertrochanteric osteotomy, one hip an intertrochanteric varus osteotomy, and one hip a PAO with a separate valgus intertrochanteric osteotomy. No avascular necrosis of the femoral head occurred. CONCLUSIONS: Femoral head reduction osteotomy can improve femoral head sphericity. Improved head containment in these hips with an often dysplastic acetabulum requires additional acetabular containment surgery, ideally performed concomitantly. This can result in reduced pain and avascular necrosis seems to be rare. With the number of patients available, function did not improve. Therefore, future studies should use more precise instruments to evaluate clinical outcome and include longer followup to confirm joint preservation.

The sphericity deviation score: a quantitative radiologic outcome measure of Legg-Calvé Perthes disease applicable at the stage of healing and at skeletal maturity.

AIM: This study aimed to determine if a new measure of sphericity of the femoral head, the Sphericity Deviation Score (SDS), could be estimated reliably at the stage of healing of Legg-Calvé-Perthes disease (LCPD) and to determine if the SDS at the healing stage reflects the outcome at skeletal maturity. METHODS: The SDS was measured with image analysis software on radiographs of 120 children with unilateral LCPD at healing of the disease and at skeletal maturity. The reproducibility of measurement was assessed. SDS values for hips in different Stulberg classes were calculated. On the basis of SDS values at healing of 82 children, a logistic regression model was developed to determine the probability of a good outcome at skeletal maturity. The validity of the model was tested on another 38 children. RESULTS: The SDS values at the stage of healing and at skeletal maturity were comparable. The SDS values were lowest for Stulberg Class I hips and highest for Class IV and V hips. The validated regression model showed a very high probability of a good outcome (Stulberg Class I or II) at skeletal maturity if the SDS at the stage of healing was below 10. CONCLUSIONS: A reliable estimate of the outcome of LCPD at skeletal maturity can be made by computing the SDS as soon as the disease heals.

Timing of premature physeal closure in Legg-Calve-Perthes Disease.

PURPOSE: Premature physeal closure of the proximal femoral physis has been reported in Legg-Calve-Perthes Disease (LCPD). However, the timing of its occurrence had not yet been reported. We proposed (1) to determine the timing of premature physeal closure in unilateral LCPD with serial radiographic evaluation, and (2) to evaluate the relationship between the premature physeal closure and Herring classification, leg-length discrepancy (LLD), Stulberg classification, and trochanteric overgrowth. METHODS: We performed a retrospective study with serial radiographs of 27 patients diagnosed with LCPD. The difference in the timing of physeal closure between the hips was calculated. The involved hip was classified according to Herring classification. The LLD and ATD index at latest follow up was measured. The mean values were calculated and statistical comparison of variables was done using the Fisher's exact test. RESULTS: The mean difference of physeal closure at the involved hip compared to the uninvolved side was 3.5 years (range, two to five years). Hips demonstrating premature physeal closure were associated with Herring B/C and C (p = 0.01) and LLD >1 cm (p = 0.02). There is no correlation between Stulberg classification, trochanteric overgrowth and premature physeal closure (p = 0.06 and p = 0.19). CONCLUSIONS: We may expect premature physeal closure of the proximal femoral physis in patients with LCPD to occur 3.5 years earlier than normal hips. Presence of premature physeal closure can be an adjunct diagnostic tool in the prognostication of LCPD outcomes. Future studies directed toward premature physeal closure in LCPD and associated growth disturbances are necessary.

Investigating pelvic drop gait abnormality in adolescent hip pathology patients.

BACKGROUND: Trendelenburg gait describes contralateral pelvic drop during single leg stance (SLS) with occasional lateral trunk lean compensation over the stance limb. However, quantitative research on 'uncompensated Trendelenburg' gait (pelvic drop independent of lateral trunk lean) remains sparse among populations that commonly utilize this gait pattern, such as adolescent hip pathology patients. RESEARCH QUESTION: How prevalent is uncompensated Trendelenburg among various adolescent hip pathologies and how is it related to hip load, hip abduction strength, and self-reported hip pain? METHODS: Gait, strength, and pain data were collected among 152 pre-operative patients clinically diagnosed with acetabular hip dysplasia, femoroacetabular impingement, Legg-Calvé-Perthes, or slipped capital femoral epiphysis (SCFE). Patients with ≥ 5.4° of dynamic pelvic drop in SLS were divided into a 'pelvic drop' group and screened to exclude those with excessive ipsilateral trunk lean. They were then compared to the 'stable pelvis' patients using a Mann-Whitney test. RESULTS: Dysplasia patients represented the highest proportion of the pelvic drop group (46%). The pelvic drop group showed a significant increase in self-reported hip pain (p = 0.011), maximum hip abductor moment (p = 0.002), and peak coronal power absorption at the affected hip during SLS loading response, (p < 0.001) while showing no difference in abduction strength (p = 0.381). SIGNIFICANCE: Uncompensated Trendelenburg gait may lead to increased loading of the affected hip in adolescent hip pathology patients. Disadvantageous hip biomechanics can create increased abductor muscle demand among these pathological populations, with dysplasia patients showing the highest prevalence. Maximal abduction strength did not correlate with pelvic drop. Future work should aim to identify and quantify causal factors. Increased coronal hip power absorption during weight acceptance warrants clinical attention, as there may be a detrimental, over-reliance on passive hip structures to support load among a population that that is already predisposed to hip osteoarthritis.

Does valgus femoral osteotomy improve femoral head roundness in severe Legg-Calvé-Perthes disease?

BACKGROUND: Many surgeons perform a varus femoral or Salter pelvic osteotomy in patients with Legg-Calvé-Perthes (LCP) disease. However, more severely deformed femoral heads show greater congruency in adduction rather than in abduction. Therefore, a valgus-(flexion) femoral osteotomy (VFO) seems preferable rather than a varus femoral or Salter pelvic osteotomy. QUESTIONS/PURPOSES: We evaluated whether the VFO improves (1) femoral head roundness, (2) radiographic parameters reflecting hip subluxation, and (3) function. METHODS: We treated 25 patients (25 hips; 18 lateral pillar C and seven B) in the late fragmentation stage by VFO. Seven patients had additional pelvic procedures. VFO was performed at a mean age of 9.8 years. Three hips were Stulberg Class II, 20 were Class III, and two were Class IV. The following components of femoral head roundness were calculated from preoperative MRI and final radiographs: lateral and medial head roundness (LHR and MHR); anterior and posterior head roundness (AHR and PHR); central head height; and the ratios MHR/LHR and PHR/AHR. Continuity of Shenton's line, medial gap ratio were evaluated. Function was determined with the Iowa hip score. Minimum followup was 3.1 years (mean, 6.3 years; range, 3.1-11.2 years). RESULTS: All femoral head roundness measurements improved, with greatest improvement in the lateral and anterior head. Pillar C hips showed greater relative improvement than pillar B hips. The continuity of Shenton's line improved and the mean medial gap ratio decreased. Mean Iowa hip score improved from 71 before surgery to 90 at the last followup. CONCLUSIONS: VFO appears to help the deformed femoral head in the fragmentation stage to remodel to fit the acetabulum. LEVEL OF EVIDENCE: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Management of Legg-Calvé-Perthes disease using an A-frame orthosis and hip range of motion: a 25-year experience.

BACKGROUND: Containment treatment is widely accepted in the management of Legg-Calvé-Perthes disease. Many reports indicate the need to regain hip motion before pelvic or femoral osteotomy, but have not indicated how osteotomy affected motion. Recent studies have suggested that osteotomy treatment of lateral pillar B hips may result in a higher proportion of spherical hips than those managed nonoperatively; however, outcomes for children older than 8 years of age or with pillar C involvement remain unsatisfactory. METHODS: The records of all patients with a diagnosis of Legg-Calvé-Perthes disease seen at our facility from 1985 through 2001 were reviewed. Two hundred and thirteen patients (175 males, 38 females), average age 6.4 years (range, 2.6 to 11.3 y), with 240 involved hips in the necrotic or the fragmentation stage were managed under a protocol to restore and maintain satisfactory hip abduction with an adductor tenotomy and abduction cast, followed by daily hip range-of-motion exercises and an A-frame orthosis to facilitate the concentric position of the epiphysis within the acetabulum. Assessment included measurement of hip abduction, femoral head sphericity and congruence, presence of femoral neck deformity, limb-length inequality, and later reconstructive surgical procedures. Hips were grouped by lateral pillar class (12A, 113B, 115C) and evaluated at maturity using a modified Stulberg grade. RESULTS: All pillar A hips were spherically congruent. Of pillar B hips, 101 were spherically congruent, 8 were aspherical but congruent, and 4 were aspherical and incongruent. Of pillar C hips, 77 were spherically congruent, 26 were aspherical but congruent, and 12 were aspherical and incongruent. Age did not correlate with outcome. Hip abduction improved and was maintained in all groups. CONCLUSIONS: Treatment that restored and maintained hip range of motion along with the use of an A-frame orthosis resulted in a high proportion of spherically congruent hips for patients of all ages irrespective of the extent of disease. Seventy-eight percent of pillar B and C hips were spherically congruent hips at maturity; overall, 93% of hips were congruent. This regimen has supplanted all other methods of treatment at our institution. LEVEL OF EVIDENCE: Level IV-case series.

Gait findings in the adolescent subject with a stiff hip.

BACKGROUND: Adolescent subjects with severe unilateral hip disease are often stiff and painful yet have limited surgical options. Although hip fusion has been used successfully to minimize pain, acquired gait compensations after arthrodesis are factors felt to lead to knee and back pain over time. However, these gait compensations may already be present in a person with a stiff hip. The purpose of this study was to describe the quantitative gait findings of the adolescent subject with a unilateral stiff hip and to determine whether these findings are similar to those of subjects presenting after arthrodesis. METHODS: This study was a retrospective review of 6 subjects seen in a motion analysis laboratory between 2005 and 2009 (age 13 to 17 y). All adolescents had been referred to the motion analysis laboratory for a routine clinical gait study. Subjects were selected for this study based on kinematic sagittal plane hip motion found to be < 25 degrees (mean 16.2 degrees). Diagnoses included: Legg-Calvé-Perthes (3) and hip avascular necrosis (3). RESULTS: Compared with laboratory-based normative data, the following findings were significant: increased arc of trunk and pelvic motion (sagittal, coronal); involved side--decreased arc of hip and knee motion (sagittal), decreased peak hip abduction in swing; contralateral side--increased arc of hip and knee motion (sagittal); and increased peak hip abduction in swing. CONCLUSIONS: Gait compensations in multiple planes and joints were identified in adolescent subjects with a unilateral stiff hip. These compensations are necessary for these subjects to generate forward progression in gait and are similar to deviations found after hip arthrodesis. Subjects with a stiff hip may already be at risk to develop pain and/or arthrosis in adjacent motion segments due to these obligatory gait characteristics. Hip fusion may not increase these risks (in this patient population) since the compensations are already present and requisite, but may provide an opportunity to decrease pain and improve function. LEVEL OF EVIDENCE: Level IV, Case Series.

Hyperactivity and the psychological burden of Perthes disease: a case-control study.

BACKGROUND: Legg-Calve-Perthes Disease (LCPD) is a childhood precursor to hip osteoarthritis, for which the etiology is unknown. There is a widespread belief that affected individuals are "hyperactive," propagating a theory that such children may have sustained an epiphyseal injury that precipitated the onset of LCPD. This study seeks to quantify the association with hyperactivity, and the wider psychological burden of the disease. METHODS: A case-control study was conducted among 146 cases of LCPD and 142 hospital controls, frequency matched by age and sex. Psychological domains were measured using the Strength and Difficulties Questionnaire. Adjustment was made for age, sex, and socioeconomic deprivation. Results were stratified by the time elapsed since LCPD was diagnosed. RESULTS: Significant associations (P<0.05) existed with the majority of the psychological domains captured by the Strength and Difficulties Questionnaire [odds ratio (OR) for "high" level of difficulties-Emotion OR 3.2, Conduct OR 2.1, Inattention-Hyperactivity OR 2.7, Prosocial Behavior OR 1.9]. Hyperactivity was especially marked among individuals within 2 years of diagnosis (OR 8.6; P<0.001), but not so among individuals over 4 years from diagnosis. Emotional symptoms persisted long after resolution of the active phase of disease. CONCLUSIONS: There was a marked psychological burden among individuals with LCPD, which was most marked amongst individuals with a recent diagnosis. The breadth and inferred temporality of these disturbances may be a function of the disease process, through restriction of activities and disability, or may be a fundamental disease characteristic related directly to disease or to its etiological determinant.

Valgus femoral osteotomy for noncontainable Perthes hips: prognostic factors of remodeling.

BACKGROUND: The authors have performed valgus femoral osteotomy (VFO) with rotational and sagittal components for Legg-Calvé-Perthes disease hips with hinge abduction. We analyzed skeletally mature patients to determine: (1) whether VFO improved hip function; (2) whether favorable radiographic remodeling of the hip occurred; and (3) whether any clinical or radiographic factors were associated with remodeling of femoral head deformity. METHODS: Thirty-one patients (31 hips, 25 boys and 6 girls) who underwent VFO between 1986 and 2007, and subsequently followed until skeletal maturity constituted the study cohort. The mean age at surgery was 9.4 years (range, 3.5 to 15 y) and the mean age at the most recent follow-up was 20.2 years (range, 14.6 to 28.3 y). Clinical outcomes were evaluated using Iowa Hip Scores and ranges of hip motion. Radiographic outcomes were assessed with respect to the radiographic indices for femoral head deformity and subluxation. Clinical and radiographic parameters were analyzed to find correlations with the femoral head remodeling (preoperative to final follow-up changes in deformity index). RESULTS: Iowa Hip Score improved from 71 (30 to 91) to 92 (76 to 100). Ranges of hip abduction, internal rotation, and external rotation increased. At last follow-up evaluations, mean Mose sphericity index of the femoral head was 4.2 mm (range, 0 to 13 mm) and femoral heads had 4 Stulberg type II, 21 type III, and 6 type IV deformity. Overall radiographic indices for femoral head deformity and subluxation did not change during follow-up period except decreased medial joint space, but greater amount of preoperative to final follow-up changes in deformity index was associated with younger age (<10 y) and earlier disease stages (fragmentation and early reossification stage) at time of surgery. CONCLUSIONS: VFO modified to accommodate the various hinging patterns of Legg-Calvé-Perthes disease hips was found to beneficially improve hip function at skeletal maturity. Although overall radiographic remodeling was not definite, favorable remodeling of the femoral head can be expected when younger patients undergo this procedure at the fragmentation or early reossification stage.

Clinical and radiographic analysis of Perthes deformity in the adolescent and young adult.

The residual deformity of Perthes disease can present with a wide variety of proximal femoral pathomorphology with associated intra- and extra-articular sources of pain. A systematic clinical and radiographic analysis is critical to understanding the deformity and identifying the source of pain. Groin pain and lateral hip pain are the most common complaints but patients can present with buttock, thigh, knee or even low back pain. Each location can suggest a different source of pain. Understanding the potential associated deformities can also help one identify the source of the clinical complaints as intra- or extra-articular. A thorough physical examination of the hip and lower extremities is critical and should include evaluation of the patient standing, supine, prone and in the lateral position. Each examination element can help link the clinical complaint to the underlying proximal femoral deformity. Radiographic evaluation should utilize a standard technique and the initial imaging should include an anteroposterior pelvis, lateral of the proximal femur and false-profile of the hip. Advanced imaging, including computed tomographic scans and gadolinium enhanced arthro-magnetic resonance imaging, can be helpful in delineating the three dimensional deformity and labral and articular cartilage injury. A thorough understanding of all three elements (clinical complaints, physical examination, radiographs) is critical to tailoring a treatment plan for the symptomatic patient with residual Perthes disease.

Application of the surgical dislocation approach to residual hip deformity secondary to Legg-Calvé-Perthes disease.

Long-term prognosis after Legg-Calvé-Perthes disease (LCPD) depends on the severity of residual hip deformities including a nonspherical femoral head, a short and wide femoral neck, a high riding greater trochanter, and secondary changes in the acetabulum. Hip deformity after healed LCPD may lead to abnormal hip mechanics including femoroacetabular impingement and hip instability secondary to acetabular dysplasia. The surgical dislocation approach has recently been applied to LCPD hip deformity with encouraging short-term results. This approach allows complete dynamic evaluation of the hip joint, identification, and correction of the most common residual deformities. The purpose of this paper is to describe the application of the surgical hip dislocation approach to LCPD deformity and to evaluate the current available literature on the results of this approach to the healed LCPD hip.

Role of femoral head surgery in skeletally mature Perthes disease.

Legg-Calve-Perthes disease (LCPD) nearly always results in femoral deformity at skeletal maturity that may lead to symptoms due principally to femoroacetabular impingement. Treatment for the skeletally mature patient with LCPD varies from observation with activity modifications to surgical procedures which range from isolated femoral or acetabular-sided surgery to the more common combined surgery. On the femoral side, the traditional procedures have been proximal varus and valgus osteotomies (with some component of flexion) to reorient the femoral head and allow better femoral head weight-bearing cartilage across the hip joint. Acetabular procedures have been used less frequently including reorientation or shelf procedures to provide improved femoral head coverage. The challenge in the skeletally mature hip patient is to determine which of these components require addressing at the time of surgical treatment. More recently, procedures to the femoral head itself have been developed to reshape the femoral head and assist in matching the size of the femoral head to the acetabulum, prevent impingement and restore more normal articular cartilage in the weightbearing zone. This review will focus on these surgical treatments specific to the femoral head in skeletally mature LCPD.

Legg-Calvé-Perthes disease: role of isolated adductor tenotomy?

BACKGROUND: The aim of the study was to compare the radiological results at maturity of patients with Legg-Calve-Perthes disease, treated either by a uniform conservative treatment or by an adductor longus tenotomy. METHODS: The study cohort comprised 349 hips, mean age 4.4 years. Patients were classified in two different groups depending on the treatment performed. The conservative group (Group I) consisted of 318 hips that had been treated by physical therapy and abduction cast/brace, with a mean age 4.3 years (range 1-10). The tenotomy group (Group II) consisted of 31 hips (treated conservatively but developed an adduction contracture limited to 30°), which had been treated by adductor longus tenotomy with a mean age of 6.2 years (range 2-9). Hip range of motion and radiographic studies were performed at the time of admission. The extent of femoral epiphyseal involvement was assessed at each follow-up by the method of Herring. The final outcomes were assessed at skeletal maturity according to the Stulberg classification system. RESULTS: Only one patient (two hips) from Group II experienced an improvement in abduction, which was maintained throughout the follow-up until complete the healing of Legg-Calvé-Perthes disease was achieved. In the remaining 29 hips, we observed a progressive loss of ROM previous to 4.3 months from the tenotomy. According to the Stulberg classification, we did not find differences between both groups at final follow-up (p > 0.05). CONCLUSIONS: These preliminary data suggest that the isolated tenotomy of the adductor longus tendon does not modify the natural evolution of Legg-Calvé-Perthes disease.

Evidence for using bisphosphonate to treat Legg-Calvé-Perthes disease.

BACKGROUND: The rationale for using bisphosphonate (BP) therapy for Legg-Calvé-Perthes disease (LCPD) is the potential to prevent substantial femoral head deformity during the fragmentation phase by inhibiting osteoclastic bone resorption. However, it is unclear whether BP therapy decreases femoral head deformity. QUESTIONS/PURPOSES: In this systematic review, we answered the following questions: (1) Does bisphosphonate (BP) therapy decrease femoral head deformity and improve pain and function in LCPD or other juvenile osteonecrotic conditions? And (2) does BP therapy decrease femoral head deformity in experimental studies of juvenile femoral head osteonecrosis? METHODS: We searched the literature from 1966 to 2011 for clinical and experimental studies on BP therapy for juvenile femoral head osteonecrosis. Studies specifically addressing clinical and/or radiographic/histologic outcomes pertaining to pain and function and femoral head morphology were analyzed. RESULTS: Three Level IV clinical studies met our inclusion criteria. Only one study initiated BP therapy during the precollapsed stage of osteonecrosis and reported prevention of femoral head deformity in nine of 17 patients. All studies noted subjective improvements of pain and gait in patients treated with intravenous BPs. Of the eight experimental studies reviewed, seven reported reduced femoral head deformity and six found better preservation of trabecular framework in animals treated with BPs. CONCLUSIONS: Clinical evidence lacks consistent patient groups and drug protocols to draw definitive conclusions that BP therapy can decrease femoral head deformity in juvenile osteonecrotic conditions. Experimental studies suggest BP therapy protects the infarcted femoral head from deformity, but it lacks bone anabolic effect. Further basic and clinical research are required to determine the potential role of BPs as a medical treatment for LCPD.

LCPD: reduced range of motion resulting from extra- and intraarticular impingement.

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) often results in a deformity that can be considered as a complex form of femoroacetabular impingement (FAI). Improved preoperative characterization of the FAI problem based on a noninvasive three-dimensional computer analysis may help to plan the appropriate operative treatment. QUESTIONS/PURPOSES: We asked whether the location of impingement zones, the presence of additional extraarticular impingement, and the resulting ROM differ between hips with LCPD and normal hips or hips with FAI. METHODS: We used a CT-based virtual dynamic motion analysis based on a motion algorithm to simulate the individual motion for 13 hips with LCPD, 22 hips with FAI, and 27 normal hips. We then determined the motion and impingement pattern of each hip for the anterior (flexion, adduction, internal rotation) and the posterior impingement tests (extension, adduction, external rotation). RESULTS: The location of impingement zones in hips with LCPD differed compared with the FAI/normal groups. Intra- and extraarticular impingement was more frequent in LCPD (79% and 86%, respectively) compared with normal (15%, 15%) and FAI hips (36%, 14%). Hips with LCPD had decreased amplitude for all hip motions (flexion, extension, abduction, adduction, internal and external rotation) compared with FAI or normal. CONCLUSIONS: Hips with LCPD show a decreased ROM as a result of a higher prevalence of intra- and extraarticular FAI. Noninvasive assessment of impingement characteristics in hips with LCPD may be helpful in the future for establishment of a surgical plan.

Low early failure rates using a surgical dislocation approach in healed Legg-Calvé-Perthes disease.

BACKGROUND: Hip deformity secondary to Legg-Calvé Perthes disease (LCPD) may result in femoroacetabular impingement (FAI) and ultimately osteoarthritis. Observations made with the surgical hip dislocation approach have improved our understanding of the pathologic mechanics of FAI. However, owing to concerns about complications related to the vascularity, the role of surgical hip dislocation in the treatment of healed LCPD remains controversial. QUESTIONS/PURPOSES: We present an algorithm to treat deformities associated with healed LCPD and asked (1) whether femoral head-neck osteochondroplasty and other procedures performed with the surgical hip dislocation approach provide short-term clinical improvement; and (2) is the complication rate low enough to be acceptable. METHODS: We retrospectively reviewed 29 patients (19 males, 10 females; mean age, 17 years; range, 9-35 years) with symptomatic LCPD between 2001 and 2009. All patients underwent a surgical hip dislocation approach and femoral head-neck osteochondroplasty and 26 patients had 37 additional procedures performed. Clinical improvement was assessed using the WOMAC index. The minimum followup was 12 months (mean, 3 years; range, 12-70 months). RESULTS: WOMAC scores improved at final followup (8 to 4 for pain, 21 to 13 for function, and 4 to 2 for the stiffness subscales). No patients had osteonecrosis, implant failure, deep infection, or nonunion. Three patients underwent THA at 1, 3, and 6 years after their index procedure. CONCLUSIONS: Using the surgical hip dislocation approach as a tool to dynamically inspect the hip for causes of FAI, we were able to perform a variety of procedures to treat the complex deformities of healed LCPD. In the short term, we found improvement in WOMAC scores with a low complication rate.

Does shelf acetabuloplasty influence acetabular growth and remodeling?

BACKGROUND: Shelf acetabuloplasty has the potential to cause iatrogenic acetabular growth arrest, although accelerated acetabular growth has been reported based on plain radiographic evaluations in patients with Legg-Calvé-Perthes disease. Because plain radiographs may be limited in depicting actual acetabular morphology, it is unclear whether there are growth disturbances. QUESTIONS/PURPOSES: We therefore determined (1) whether focal or generalized acetabular growth stimulation or retardation occurred based on CT analysis in combination with plain radiographic assessment; and (2) whether radiographically assessed femoral head deformity, subluxation, and acetabular dysplasia were associated with residual zonal rim dysplasia of the acetabulum. METHODS: We examined 14 patients who had undergone shelf acetabuloplasty for "reducible subluxation" and underwent CT scans at a mean 7 years after surgery (range, 3-11 years). We measured radiographic indices reflecting acetabular depth and rim dysplasia on multiplanar reformatted images in 10 radial planes and on plain radiographs and calculated their operation-to-control ratios to assess growth changes. The mean age at surgery was 9.3 years (range, 7-12 years). RESULTS: We observed generalized accelerated growth in 11 hips and equivocal growth in three. None of the 14 hips showed an abrupt change in acetabular geometry. Despite improved acetabular depth in all hips, eight hips had focal rim dysplasia in the superior zone on CT examinations, and this was associated with a smaller center-edge angle, a greater deformity index, and preexisting acetabular dysplasia. CONCLUSIONS: Our observations support the notion that shelf acetabuloplasty has a favorable, stimulatory effect on acetabular growth. However, the possible persistence of preexisting zonal rim dysplasia should be considered.

What is the evidence supporting the prevention of osteoarthritis and improved femoral coverage after shelf procedure for Legg-Calvé-Perthes disease?

BACKGROUND: The evidence supporting continued use of shelf acetabuloplasty in Legg-Calvé-Perthes disease (LCPD) is not well-defined, and there is controversy regarding the long-term benefits related to clinical and functional improvement. QUESTIONS/PURPOSES: Our goals were to determine whether shelf arthroplasty for LCPD (1) prevents the onset of early osteoarthritis; (2) improves pain, ROM, activity, and functional outcomes; (3) maintains or improves femoral head containment, sphericity, and congruency; (4) changes the acetabular index; and (5) is associated with a low rate of complications. METHODS: We performed a systematic review of the medical literature from 1966 to 2009 using the search terms Perthes, shelf procedure, and acetabuloplasty. We excluded reports using multiple/combined treatment methods and those not clearly stratifying outcomes. Thirteen studies met the criteria. There were no Level I studies, one Level II prognostic study, five Level III therapeutic studies, and seven Level IV studies. Mean followup ranged from 2.6 to 17.9 years. RESULTS: Only one study reported progression to early osteoarthritis in one patient. We found no evidence for improvement in ROM and continued pain relief at long-term followup. Mean decrease in lateral subluxation ratio was 13% to 30%, demonstrating an improvement in femoral head containment. Mean acetabular cover percentage improved 16% to 38%, and mean acetabular and center-edge angles improved 4° to 14° and 8° to 33°, respectively. There were no reports of any major complications after the procedure. CONCLUSIONS: While radiographic measurements indicate improved coverage of the femoral head after shelf acetabuloplasty for LCPD, available evidence does not document the procedure prevents early onset of osteoarthritis or improves long-term function.