Femoral Head Deformity Associated With Hip Displacement in Nonambulatory Cerebral Palsy: Results at Skeletal Maturity.

BACKGROUND: Maintaining femoral head shape (FHS) and acetabular sphericity are important goals in preventing long-term osteoarthritis in hips in children with cerebral palsy (CP). As acetabular morphology has been widely studied, our objective was to determine FHS in CP after triradiate cartilage (TRC) closure, a proxy for skeletal maturity, and the risk factors associated with residual deformity and osteoarthritis. METHODS: In this retrospective cohort study, patients with CP [Gross Motor Function Classification System (GMFCS) IV to V], minimum 4 yearly hip radiographs after age 10 years, and at least 1 radiograph after age 16 years, were included. Primary outcome was FHS (Rutz), stratified as "less severe" (Rutz A to B) and "more severe" (Rutz C to D). Secondary outcomes included migration percentage (MP), age at TRC closure, previous reconstructive (femoral with/without pelvic osteotomies) surgery, previous intrathecal baclofen, Tönnis osteoarthritis grade, and GMFCS level. Statistical analyses included χ 2 analysis and multiple logistic regression. RESULTS: One hundred sixty-three patients (326 hips) met the inclusion criteria, with TRC closure at age 14.0 (SD: 1.8) years. At final follow-up of 4.4 (SD: 2.4) years after TRC closure, 17% (55 hips), had a "more severe" FHS. From TRC closure to final follow-up, the frequencies of "less severe" hips decreased (-10%, P <0.001), while "more severe" increased (+115%, P <0.001). In multiple regression analysis, MP at TRC closure was the only significant risk factor associated with a "more severe" FHS at final follow-up ( P =0.03). Receiver operating characteristic curve analysis determined MP≥30.5% to be associated with a "more severe" FHS at final follow-up ( P <0.009). The FHS was not affected by reconstructive surgery, sex, GMFCS level, or intrathecal baclofen use. "Less severe" hips had lower Tönnis grades (0 to 1) compared with "more severe" hips (Tönnis grades 2 to 3) at final follow-up ( P <0.001). CONCLUSIONS: FHS at skeletal maturity was not influenced by prior reconstructive surgery but was negatively affected when MP≥30.5% at the time of TRC closure. The extent of residual femoral head deformity correlated with the severity of osteoarthritis at final follow-up. LEVEL OF EVIDENCE: Level III.

Does Flexion Varus Osteotomy Improve Radiographic Findings Compared With Patients Treated in a Brace for Late-onset Legg-Calvé-Perthes Disease?

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) is a childhood hip disease characterized by osteonecrosis of the femoral head. Because severe deformity of the femoral head can cause secondary osteoarthritis in adulthood, progressive collapse should be prevented in children with a necrotic epiphysis. The prognosis of patients with LCPD generally worsens as the age at disease onset increases, and the appropriate treatment for late-onset LCPD remains unclear. Based on the limited effect of nonoperative treatment using a nonweightbearing brace, flexion varus osteotomy (FVO) was introduced in 2010 as an initial treatment for late-onset LCPD in place of brace treatment, which we used in our institution before that time. QUESTIONS/PURPOSES: We asked, (1) Which treatment, FVO or a nonweightbearing brace, is associated with a lower likelihood of progressive femoral head collapse in children whose diagnosis of LCPD was made at the age of ≥ 8 years and who were followed for a minimum of 3 years after their intervention? (2) What proportion of patients in the brace group had surgery despite the treatment, and what percentage of children in the FVO group had a second operation to remove hardware and/or additional operations? METHODS: The initial treatment was applied in 181 patients with LCPD between 1995 and 2018 in our institution. Patients whose disease onset was at ≥ 8 years old (late-onset LCPD) with complete clinical and radiologic data were considered potentially eligible. In 2010, treatment for these patients changed from brace treatment to FVO for all patients. A total of 35% (42 of 121) of patients who were treated with a nonweightbearing brace between 1995 and 2009 and 40% (24 of 60) of patients who were treated with FVO between 2010 and 2018 were eligible. Among patients treated with a brace, 21% (nine of 42 patients) were excluded because of hospital transfer (three patients), short-term follow-up (three), the period from onset to the first visit was ≥ 7 months (two), and inability to use the brace because of mental incapacity (one patient). In patients treated with FVO, 12% (three of 24 patients) were excluded (two patients with a period from onset to the first visit ≥ 7 months and one with a comorbidity and multiple-epiphyseal dysplasia). Among the remaining patients, 79% (33 of 42 patients) were classified into the brace group and 88% (21 of 24 patients) were classified into the FVO group for analyses. There were no overlapping patients at the timepoint when the treatment strategy for late-onset LCPD changed. In the FVO group, subtrochanteric osteotomy with 35° to 40° of flexion and 15° to 20° of varus was performed using a locking compression plate for pediatric use. Patient demographics, radiographic parameters, and the assessment of femoral head deformity using the Stulberg classification were compared between the two groups. There was a greater proportion of boys than girls in both groups (brace: 88% and FVO: 86%), and there were no differences in the distribution of genders between the groups (p = 0.82). The right side was more frequently treated in the brace group, but there was no difference in laterality between the groups (brace: 58% right and FVO: 62% left; p = 0.16). There was no difference between groups in the median age at disease onset (9.0 years [range 8.0 to 12.5 years] in the brace group and 9.6 years [range 8.0 to 12.4 years] in the FVO group; p = 0.26). There was no difference between the groups in the period of treatment from onset (1.7 ± 1.9 months in the brace group and 1.5 ± 1.5 months in the FVO group; p = 0.73) or the follow-up period (6.7 ± 2.1 years in the brace group and 6.2 ± 2.1 years in the FVO group; p = 0.41). The LCPD stage at the first visit was assessed using the modified Waldenström classification. The intraobserver and interobserver values of the modified Waldenström classification, evaluated using kappa statistics, were excellent (kappa value 0.89 [95% CI 0.75 to 0.97]; p < 0.01) and good (kappa value 0.65 [95% CI 0.43 to 0.87]; p < 0.01). The radiographic degree of collapse at the maximum fragmentation stage was assessed using the lateral pillar classification. The intraobserver and interobserver reliabilities of the lateral pillar classification were excellent (kappa value 0.84 [95% CI 0.73 to 0.94]; p < 0.01) and excellent (kappa value 0.83 [95% CI 0.71 to 0.94]; p < 0.01). The degree of femoral head deformity at the most recent follow-up examination was compared between the groups in terms of the Stulberg classification, in which Classes I and II were classified as good and Classes III through V were classified as poor. The intraobserver and interobserver reliabilities of the Stulberg classification were good (kappa value 0.74 [95% CI 0.55 to 0.92]; p < 0.01) and good (kappa value 0.69 [95% CI 0.50 to 0.89]; p < 0.01). The evaluators were involved in the patients' clinical care as part of the treating team. RESULTS: Good radiographic results (Stulberg Class I or II) were obtained more frequently in the FVO group (76% [16 of 21 patients]) than in the brace group (36% [12 of 33 patients]), with an odds ratio of 5.6 (95% CI 1.7 to 18.5; p < 0.01). In the brace group, a subsequent femoral varus osteotomy was performed in 18% (six of 33) of patients with progressive collapse and hinge abduction, and implant removal surgery was performed approximately 1 year after the first procedure. This traditional varus osteotomy was occasionally performed in patients who were considered for conversion from nonoperative treatment before 2009 because FVO had not yet been introduced. In the FVO group, all patients (n = 21) had a second procedure to remove the implant at a mean of 10.5 ± 1.2 months postoperatively. Additional procedures were performed in 24% (five of 21) of patients, including a second FVO for progressive collapse (one patient), guided growth for a limb length discrepancy (one patient), and flexion valgus osteotomy for coxa vara in patients with a limb length discrepancy (three patients). CONCLUSION: Our historical control study found that FVO may increase the possibility of obtaining good radiographic results (Stulberg Class I or II) compared with brace treatment for patients with late-onset LCPD, although surgical interventions after the first and second implant removal procedures may be indicated. Surgeons can consider FVO if they encounter patients with late-onset LCPD, which is a challenging condition. A larger study with long-term follow-up is needed to confirm the efficacy of FVO. LEVEL OF EVIDENCE: Level III, therapeutic study.

Prolonged non-weightbearing treatment decreases femoral head deformity compared to symptomatic treatment in the initial stage of Legg-Calvé-Perthes disease.

The purpose of this study is to investigate the potential benefit of using prolonged non-weightbearing (PNWB) as a treatment option for early-stage Legg-Calvé-Perthes disease (LCPD). An Institutional Review Board (IRB) approved this retrospective study of patients with LCPD and ≥2-year follow-up. Patients 6-12 years of age were included if treatment began in Waldenstrom stage 1 or 2A. PNWB consisted of ≥6 months of non- or toe-touch weightbearing. PNWB was recommended if perfusion MRI demonstrated ≥40% hypoperfusion of the femoral head and parents decided against operative treatment. The control group consisted of symptomatically treated patients. Deformity index and epiphyseal quotient were measured at 2-year follow-up. Stulberg classification and sphericity deviation score (SDS) were determined at skeletal maturity or at a minimum of 5-year follow-up. When treatment was initiated in Waldenstrom stage 1, the PNWB group had significantly less femoral head deformity, including deformity index (0.21 vs. 0.52; P < 0.001), epiphyseal quotient (69% vs. 43%; P < 0.001), SDS (18 vs. 52; P = 0.004), and Stulberg (50% good vs. 0% good; P = 0.044). The PNWB group mean hypoperfusion was 68%, indicating severe hypoperfusion. Duration of recommended non-weight bearing in the PNWB group was 11.5 months (range 7-17 months). Despite severe femoral head hypoperfusion, PNWB begun during the initial stage of LCPD decreased femoral head deformity. PNWB should be considered a treatment option for patients/parents who do not wish to pursue operative intervention in early-stage LCPD with substantial hypoperfusion. Level of Evidence III - retrospective comparative study.

Treatment for Post-Slipped Capital Femoral Epiphysis Deformity.

In situ pinning of slipped capital femoral epiphysis (SCFE) is a safe and effective treatment modality, but often results in residual deformity leading to femoroacetabular impingement, which may limit patient activities and predispose to early onset arthritis. Enhanced understanding of the implications of femoroacetabular impingement and new surgical techniques have prompted interest in treating post-slipped capital femoral epiphysis deformity to both improve current symptoms and delay or prevent hip arthrosis.

Preoperative planning of femoral head reduction osteotomy using 3D printing model: A report of two cases.

The deformed and enlarged femoral head secondary to hip diseases such as Legg Calve Perthes and Developmental Dysplasia usually causes impingement between the aspherical head and the acetabulum. To restore and reduce the size of enlarged femoral head, a femoral head reduction technique has been described previously. The goal is to obtain a spherical femoral head and to cover the gliding surface with best available cartilage. Planning of osteotomy to achieve spherical head is the crucial point of surgery. It is usually done intra-operatively and dependent on experience of surgeon. Preoperative 3- Dimension (D) modeling of femoral head is commonly preferred to minimize this risk. In this technical note, preoperative planning with 3-D printing was demonstrated in two separate patients with Legg-Calve-Perthes Disease and developmental hip dysplasia. Surgical time was approximately 150 and 120 min, respectively. Blood loss was 230 and 300 cc, respectively. Patients were followed up 9 months and 12 months, respectively. None of the patients in this study developed avascular necrosis; however, the follow-up period is very limited. Moreover, none of the patients developed post-operative complications or required additional surgery. With a more detailed preoperative planning done on computer model and printed in 3-D, one can mimic the surgical procedure before the procedure. Finally, this technique is advantageous both for the patient and surgeon.

Morphofunctional features of articular cartilage structure.

BACKGROUND: Articular cartilage is highly-organized nonvascularized tissue which is responsible in humans for pressure absorption under load, as well as for the smoothness of the opposite tangential bone surfaces. The purpose of our research is to study structural and functional features of articular cartilage at light- optical level by using state-of-the-art research methods of bone-cartilage tissue. MATERIAL AND METHODS: the study was conducted on samples of femoral heads. Hyper fine sections were subject to hematoxylin and eosin, Van Gieson's and PAS staining. In order to identify the receptor profile of chondrocytes and the features of protein arrangement in extracellular matrix we undertook an immunohistochemical study. RESULTS: An articular cartilage is quite organized tissue. As any other organ, it has parenchyma and stroma. Parenchyma is represented by one type of cells - chondrocytes, which, depending on how deep they are located in cartilage, have a different shape, size and functional features. The chondrocytes and extracellular matrix have different degrees of receptors expression. CONCLUSIONS: the cartilage is being constantly self-renewed, what is manifested by means of a rather slow division of the surface-located chondrocytes and programmed death of dystrophic-modi ed cells. The features of extracellular matrix structure determine the originality of cell location in different areas of cartilage tissue. Due to synthesis of specific proteins, chondrocytes self-regulate properties of cartilage tissue.

The disappearance of femoral head and neck resulting from extensive bone defect caused by secondary syphilis: a case report and literature review.

BACKGROUND: Treponema Pallidum (TP), the pathogen of syphilis, commonly infects bones in cases of congenital and tertiary syphilis, but it is rare in the primary and secondary stages. With its mild symptoms and rare clinical findings, it might be easy to dismiss the diagnosis of early syphilis. Usually, effective results can be achieved after the conventional strategy of antibiotic treatments, mainly penicillin. To our knowledge, our case is so far the most serious reported case of destructive bone lesion in secondary syphilis, and our treatment for the case is the first strategy using total hip arthroplasty in secondary syphilis. CASE PRESENTATION: We present the case of a 71-year-old man with local repeated pain and dysfunction in the right hip. Radiologic examinations showed the disappearance of the ipsilateral femoral head and neck. After excluding the aetiologies of cancer metastasis and tuberculosis, we confirmed the diagnosis of syphilitic arthritis. The patient received the medical treatment of antibiotics and the surgical treatment of total hip arthroplasty. At the follow-up of 1, 3, and 5.5 years after the operation, the patient presented with a pain-free and functional hip prosthesis without local signs of infection and loosening. CONCLUSIONS: This report highlights the difficulties of early diagnosis of secondary syphilis with bone involvement. Bone defect of the femur with secondary syphilis, especially at the proximal femur, was an extremely rare complication in the previous reports. Our case was the first case of a patient who experienced the disappearance of femoral head and neck caused by secondary syphilis. Follow-up after the operation proved the successful treatment of the extensive bone defect of femur by total hip arthroplasty.

A long term follow-up study of the development of hip disease in Mucopolysaccharidosis type VI.

Hip problems in Mucopolysaccharidosis type VI (MPS VI) lead to severe disability. Lack of data on the course of hip disease in MPS VI make decisions regarding necessity, timing and type of surgical intervention difficult. We therefore studied the development of hip pathology in MPS VI patients over time. Data were collected as part of a prospective follow-up study. Standardized supine AP pelvis and frog leg lateral radiographs of both hips were performed yearly or every 2years. Image assessment was performed quantitatively (angle measurements) and qualitatively (hip morphology). Clinical burden of hip disease was evaluated by physical examination, six minute walking test (6MWT) and a questionnaire assessing pain, wheelchair-dependency and walking distance. A total of 157 pelvic radiographs of 14 ERT treated MPS VI patients were evaluated. Age at first image ranged from 2.0 to 21.1years. Median follow up duration was 6.8years. In all patients, even in the youngest, the acetabulum and os ilium were dysplastic. Coverage of the femoral head by the acetabulum improved over time, but remained insufficient. While the femoral head appeared normal in the radiographs at young age, the ossification pattern became abnormal in all patients over time. In all patients the distance covered in the 6MWT was reduced (median Z scores -3.3). Twelve patients had a waddling gait. Four patients were partially wheelchair-dependent and ten patients had limitations in their maximum walking distance. In conclusion, clinically significant hip abnormalities develop in all MPS VI patients from very early in life, starting with deformities of the os ilium and acetabulum. Femoral head abnormalities occur later, most likely due to altered mechanical forces in combination with epiphyseal abnormalities due to glycosaminoglycan storage. The final shape and angle of the femoral head differs significantly between individual MPS VI patients and is difficult to predict.

3D MRI Quantification of Femoral Head Deformity in Legg-Calvé-Perthes Disease.

The purpose of this study was to quantify femoral head deformity in patients with Legg-Calvé-Perthes disease (LCPD) using a novel three dimensional (3D) magnetic resonance imaging (MRI) reconstruction and volume based analysis. Bilateral femoral heads of 17 patients (mean age 9.9 ± 2.0 years; 12 boys, 5 girls) with LCPD were scanned 1-2 times (n = 33 LCPD heads, 20 normal heads) using a 1.5T MRI scanner. Fourteen patients had unilateral and three had bilateral LCPD with five hips in the Waldenström initial stage, 9 in the fragmentation stage, 14 in the reossification stage, and 5 in the healed stage. 3D digital reconstructions of femoral heads were created using MIMICS software. Deformity was quantified using a 3D volume ratio method based on reference hemisphere volume as well as two surface geometry methods. Intra-observer analysis showed that 97% of the LCPD femoral heads were within 10% of the original value and test shapes had 99.6% accuracy. For normal femoral heads, the volume ratios of all except one were between 95 and 98% (n = 20) of a perfect hemisphere volume. For femoral heads affected with LCPD, the volume ratios ranged from 43% to 96% of a perfect hemisphere (n = 33). The volume ratio method and the two surface geometry comparison methods had high correlation (r = 0.89 and 0.96). In summary, the 3D MRI volume ratio method allowed accurate quantification and demonstrated small changes (<10%) of the femoral head deformity in LCPD. This method may serve as a useful tool to evaluate the effects of treatment on femoral head shape. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:2051-2058, 2017.