A Novel Method for Assessing the 3-dimensional Morphology of Cartilaginous Acetabulum Via Childhood Magnetic Resonance Imaging.

BACKGROUND: Periarticular cartilage is abundant in children, making evaluations of 3-dimensional (D) cartilaginous acetabular morphology using x-ray or computed tomography (CT) difficult. The study aimed to visualize the 3D cartilaginous acetabular morphology in normal children and patients with pediatric developmental dysplasia of the hip (DDH). METHODS: Magnetic resonance imaging (MRI) of 17 female children without acetabular dysplasia at 7.5 years and CT of 33 normal female adolescents with mature bones at 14.6 years were used as controls. Subjects were 26 female patients with unilateral DDH who underwent angulated Salter innominate osteotomy (A-SIO) at 5.5 years. Preoperative and postoperative MRIs were performed at 5.2 and 7.0 years, respectively. The MRI sequence was 3D-MEDIC. The medial intersection (point A) of the line connecting the centers of the bilateral femoral head and the femoral head were defined as point zero. The 3D coordinates (X, Y, Z) of the cartilaginous acetabular edge (point C) from anterior to posterior were calculated. Subsequently, a 3D scatter plot was created using 3D graph software. The subjects were divided into 6 groups, including control MRI, control CT, unaffected DDH before and after A-SIO, and affected DDH before and after A-SIO. The femoral head coverage ratio (FHCR: AC/AB) was used to quantify coverage and was compared in each group. RESULTS: In the control MRI group, the acetabular coverage was small anteriorly, largest anterolaterally, and gradually decreased posteriorly, similar to the bony acetabulum in adolescents. In the affected DDH before A-SIO group, the coverage was significantly lower than that of the control MRI and unaffected DDH groups. After A-SIO, the morphology improved beyond the unaffected DDH and the control MRI group. CONCLUSIONS: The global defect of the cartilaginous acetabulum in the affected DDH group was significantly improved to normal morphology after A-SIO. Evaluating the cartilaginous acetabulum using MRI was useful for assessing hip morphology in childhood. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Outcomes of Linear Circumferential Skin Closure for Congenital Constriction Ring Syndrome.

Background: Congenital constriction ring syndrome (CCRS) is a rare condition diagnosed at birth characterised by deformation due to a constriction ring. The usual treatment for CCRS involves excision of the constriction ring and suture of the skin incorporating a Z-plasty to prevent scar contracture. A Z-plasty often results in an unsightly scar. In order to avoid this, we performed linear circumferential skin closure (LCSC). The aim of this paper is to report the outcomes of LCSC for CCRS. Methods: We retrospectively investigated all patients with CCRS who underwent LCSC between 2002 and 2020. Two linear incisions were placed proximal and distal to the constriction ring in parallel, and the constriction ring was excised carefully so as not to damage nerves or vessels. The deep subcutaneous and dermis layers were sutured. The skin was closed using adhesive tape. Two-stage surgery was performed in two patients with severe CCRS of the lower leg to avoid problems with distal circulation. Patients were followed up for at least 1 year and assessed for complications and quality of scar. Results: We performed LCSC for 31 sites in 19 patients, including one forearm, 14 fingers, 10 lower legs and six toes. The median age at the operation was 16 months (range: 4-175). The median follow-up period after surgery was 5.8 years (range: 1.9-16.0). The linear surgical scar had healed well in all patients and there were no complications. There was no recurrence of the constriction ring and no scar hypertrophy, though we did not perform fat mobilisation in all cases. None of the patients required additional surgery and the aesthetic outcome of the linear circumferential surgical scar was maintained at the final observation. Conclusions: Treatment for CCRS using LCSC resulted in no complications, no recurrence of constriction and an excellent aesthetic outcome. Level of Evidence: Level IV (Therapeutic).

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.

The recent bacterial etiology of childhood osteoarticular infections focusing on the vaccine initiation for Streptococcus pneumoniae and Haemophilus influenzae: A single-center retrospective analysis in Japan.

Background: Pediatric acute osteomyelitis and septic arthritis can destroy growth plate cartilage and joint cartilage, causing permanent deformities and growth disorders. Preventing the contraction of osteoarticular infections is important. Various types of bacteria cause osteoarticular infections in children. Since 2013, when routine vaccination against Streptococcus pneumoniae and Haemophilus influenzae was initiated in Japan, diseases caused by these bacteria (other than osteoarticular infection) are reported to decrease. In this study, we aimed to re-confirm the actual situation including the presence of pathogenic bacteria of pediatric bone and joint infections. Methods: The subjects were patients of 15 years old or younger who had been diagnosed with acute osteomyelitis or septic arthritis and received initial treatment in our hospital from April 1995 to March 2019. We obtained information from the medical records and analyzed them statistically. Results: There were 65 patients with 65 bones with acute osteomyelitis, and 120 patients with 124 joints with septic arthritis. The pathogenic bacteria were identified in 26 (40.0%) osteomyelitis patients and 59 (49.2%) septic arthritis patients. Staphylococcus aureus was the most common pathogenic bacterium, and S. pneumoniae and H. influenzae were identified in four and seven patients respectively, frequently in younger patients. After routine vaccination against S. pneumoniae and H. influenzae, these bacteria were no longer detected in patients under five years old. Conclusions: The efficacy of the S. pneumoniae and H. influenzae vaccine against orthopedic infectious diseases in Japan was indicated.

Hip Prognosis in Patients With Congenital Diastasis of the Pubic Symphysis.

BACKGROUND: Congenital diastasis of the pubic symphysis (CDPS) is a rare musculoskeletal malformation in the exstrophy-epispadias complex that is potentially associated with the development of hip dysplasia. The purpose of this study was to investigate the incidence and prognostic factors of hip dysplasia in patients with CDPS. METHODS: Fifty-four hips in 27 patients with CDPS initially evaluated between 1983 and 2016 were retrospectively reviewed. The mean age at the first visit was 2.3 (0 to 8) years. The mean follow-up duration was 10.5 (1 to 36) years. Patient characteristics at the first visit, the clinical course at the most recent follow-up, and radiologic parameters on pubic malformation and hip dysplasia during at least 2 time points (first visit or age 1, and either the most recent visit or before hip surgery) were evaluated. Prognostic factors associated with the development of hip dysplasia were analyzed using univariate/multivariate analysis. The Kaplan-Meier survival curves were generated and compared based on these factors. RESULTS: Nine of 27 patients (33%) and 13 of 54 hip joints (24%) with CDPS developed hip dysplasia. Paraplegia (odds ratio, 10.0; 95% confidence interval, 1.7-76.6) and center-edge angle of <5 degrees at the first visit or age 1 (P<0.001) were independent predictors of the development of hip dysplasia. Patients with CDPS and either paraplegia or center-edge angle <5 degrees at the first visit or age 1 were significantly more likely to develop hip dysplasia than other patients (hazard ratio, 29.3; 95% confidence interval, 3.4-250). CONCLUSIONS: Approximately one third of patients with CDPS develop hip dysplasia. Paraplegia and center-edge angle of <5 degrees at the first visit are independent risk factors. LEVEL OF EVIDENCE: Level III.

Treatment approaches for congenital transverse limb deficiency: Data analysis from an epidemiological national survey in Japan.

BACKGROUND: Congenital limb deficiency is a rare anomaly that impairs limb function. Transverse deficiency accounts for approximately half of congenital limb deficiency cases. In Japan, there have been no detailed data of clinical features, especially treatment approaches, of this disorder. The present study aimed to investigate the status of treatment approaches of congenital transverse limb deficiency in Japan. METHODS: From the national epidemiological survey of congenital limb deficiency undertaken in Japan in 2016, all the data of 200 patients with congenital transverse limb deficiencies were extracted. These data were analysed to reveal the treatment approaches of congenital transverse limb deficiency and its basic clinical features. RESULTS: Surgical treatments and prosthetic or orthotic intervention had been implemented or planned for about one-quarter of patients, respectively. In the upper limb deficiencies, prosthetic or orthotic intervention was likely chosen in cases of deficiency at the metacarpal or proximal to metacarpal level. Surgical treatment was chosen only in cases of deficiency at the carpal or distal to carpal level. Although the number of patients with transverse lower limb deficiencies was small, prosthetic or orthotic intervention was likely chosen in proximal deficiencies, and surgical treatment was likely chosen in distal deficiencies. CONCLUSIONS: Herein, we revealed the status of treatment approaches for congenital transverse limb deficiency in Japan. Approximately half of the patients had no history of-and no plans for-surgical, prosthetic, or orthotic interventions. Further treatment advances may enable patients with congenital limb deficiencies to increase their participation in daily activities. STUDY DESIGN: Cross-sectional survey.

A longitudinal study for the prediction of the mature acetabular morphology using childhood magnetic resonance imaging.

BACKGROUND: Although acetabular dysplasia is a common etiology of osteoarthritis of the hip regardless of the history of developmental dysplasia of the hip (DDH), whether or not corrective surgeries are beneficial for the childhood asymptomatic acetabular dysplasia remains controversial due to a lack of evidence. We conducted a longitudinal study to compare the cartilaginous morphology on childhood magnetic resonance imaging (MRI) and the mature hip morphology of the same patient and to assess the predictive indicators for future acetabular dysplasia. METHODS: A total of 92 unaffected hips (47 unilateral DDH and 45 unilateral Legg-Calvé-Perthes disease) were reviewed for X-ray and MRI findings on childhood (mean age: 6.0 years) and X-ray findings from a skeletally mature age with a mean follow-up period of 15.1 years. The following parameters were measured and compared: the immature-acetabular index (AI) and center edge angle (CE) on immature X-ray; the cartilage- and bone- AI, CE, Sharp and acetabular head index (AHI) on childhood MRI; and the mature-acetabular roof obliquity (ARO), CE, Sharp and AHI on skeletally mature X-ray. The prognostic factors on childhood MRI for acetabular dysplasia, defined by a CE of <20° on skeletally mature X-ray were also assessed. RESULTS: Positive correlations were shown between the cartilage-AI and mature-ARO (7.6°/6.3°; r = 0.44), the cartilage-CE and mature-CE (27.8°/28.0°; r = 0.62), the cartilage-Sharp and mature-Sharp (44.4°/41.8°; r = 0.52) and the cartilage-AHI and mature-AHI (78.7%/80.3%; r = 0.46). A multivariate analysis indicated cartilage-CE to be an independent predictor for acetabular dysplasia with a cut-off value of 22°. Children with a cartilage-CE <22° developed more frequently acetabular dysplasia compared to the others (52.4% vs. 1.4%). CONCLUSIONS: Childhood MRI findings are useful for the prediction of acetabular dysplasia without a DDH history. Children with a cartilage-CE ≥23° are likely to achieve a non-dysplastic hip without the need for surgical intervention.

Subchondral fracture of the femoral head in children: Differential diagnosis of pediatric hip pain.

This report describes clinical and radiographic characteristics of two pediatric patients with a presumptive diagnosis of subchondral fracture of the femoral head made based on their clinical course and imaging findings. An 8-year-old boy and an 8-year-old girl had subchondral fracture in the femoral head without osteonecrosis, which was verified by contrast-enhanced magnetic resonance imaging. Although complete disappearance of the fracture line was confirmed in the boy, the girl had a residual femoral head deformity after conservative treatment. Subchondral fracture of the femoral head should be included in the differential diagnosis of pediatric hip pain.

Congenital limb deficiency in Japan: a cross-sectional nationwide survey on its epidemiology.

BACKGROUND: Congenital limb deficiency is a rare and intractable disease, which impairs both function and appearance of the limbs. To establish adequate medical care, it is necessary to reveal the actual conditions and problems associated with this disease. However, there have been no extensive epidemiological surveys in Japan addressing this disease. This is the first nationwide epidemiological survey of congenital limb deficiency in this country. METHODS: With the cooperation of epidemiology experts, we performed a two-stage nationwide survey to estimate the number of patients with congenital limb deficiency and reveal basic patient features. We targeted orthopaedic surgery, paediatric, and plastic surgery departments. Hospitals were categorized according to the institution type and the number of hospital beds; hospitals were randomly selected from these categories. We selected 2283 departments from a total 7825 departments throughout Japan. In this study, we defined congenital limb deficiency as partial or total absence of the limbs, proximal to the proximal interphalangeal joint of the fingers/lesser toes or interphalangeal joint of the thumb/great toe. We distributed the first survey querying the number of initial patient visits from January 2014 to December 2015. Targets of the second survey were departments that reported one or more initial patient visits in the first survey. RESULTS: In the first survey, 1767 departments responded (response rate: 77.4%). Among them, 161 departments reported one or more initial patient visits. We conducted the second survey among these 161 departments, of which 96 departments responded (response rate: 59.6%). The estimated number of initial visits by patients with congenital limb deficiency was 417 (95% confidence interval: 339-495) per year in 2014 and 2015. The estimated prevalence of congenital limb deficiency in Japan was 4.15 (95% confidence interval: 3.37-4.93) per 10,000 live births. The sex ratio was 1.40. Upper limbs were more affected than lower limbs. CONCLUSIONS: We revealed the estimated number of initial patient visits per year and birth prevalence of congenital limb deficiency in Japan. Our results will contribute to establishing the disease concept and grades of severity of congenital limb deficiency.

Clinical outcomes of conservative treatment with a non-weight-bearing abduction brace for Legg-Calvé-Perthes disease.

BACKGROUND: Treatment with a brace is the first choice as conservative treatment via the containment method for Legg-Calvé-Perthes disease (LCPD). The purpose of this study is to evaluate clinical outcomes and influential factors of conservative treatment with the non-weight-bearing abduction brace for LCPD. METHODS: One hundred thirty hips in 130 patients were examined in this study. The mean age at onset was 7.0 years (3.3-12.4 years) and the mean follow-up period was 8.4 years (4.1-17.6 years). The extent of necrosis and lateral collapse of the femoral head were evaluated using the Catterall classification and the lateral pillar classification, respectively. Radiological outcome was assessed as good (classes I and II), fair (III), and poor (IV), according to the modified Stulberg classification. RESULTS: Radiographic outcome at final follow-up was good in 82 hips (63%), fair in 40 hips (31%), and poor in 8 hips (6%). Multinomial logistic regression analysis showed that major influential factors for good outcomes were as follows: age at onset, lateral pillar classification, and Catterall classification. From the receiver operating characteristic curve, the cut-off value for age at onset was 8.4 years old to obtain good outcomes. Hips with Catterall group I and II and lateral pillar group A and B had significantly better results. CONCLUSION: Patients younger than 8.4 years old at onset with lateral pillar group A or B or Catterall group I or II showed good outcomes with a non-weight-bearing abduction brace for LCPD. These results show that alternative treatment, such as surgery, may be another option for patients who are not included in the above groups.

Subclinical bilateral involvement of the hip in patients with slipped capital femoral epiphysis: a multicentre study.

PURPOSE: This study was conducted to assess the posterior inclination of the contralateral femoral epiphysis in patients with unilateral slipped capital femoral epiphysis (SCFE). METHODS: The posterior sloping angle (PSA) was measured using lateral radiographs in 67 patients with a unilateral SCFE and in 41 age-matched normal controls. A symptomatic epiphyseal slip was defined as the development of SCFE. RESULTS: The contralateral PSA in SCFE patients was more widely distributed and significantly larger compared to controls (15.0° vs. 9.0°, p < 0.0001). Forty-seven hips (70.1%) had a PSA of greater than 12.8°, which was +2SD of the control hips. Of the 65 hips excluding the two cases with prophylactic pinning, 11 hips (16.9%) eventually developed a contralateral SCFE during adolescence and their PSA at the initial visit was significantly larger compared to patients without a contralateral SCFE (18.0° vs. 14.3°, p < 0.005) with a cutoff value of 19°. CONCLUSIONS: These findings suggested the possibility of bilateral hip involvement in SCFE patients. Hips with greater degrees of PSA (> 19°) are likely to become symptomatic.

Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure.

BACKGROUND: We evaluated the pathologic anatomy of the Sprengel deformity using radiographs, 3-dimensional computed tomography (3D-CT), and magnetic resonance imaging (MRI), and reviewed our results of the modified Green procedure. METHODS: Between 2002 and 2009, 23 scapulae in 22 patients were treated. The average age at the time of surgery was 3.4 years (range, 1.9 to 7.1 y). The outcome of surgery was assessed on the basis of changes in shoulder abduction and the radiographic findings using Cavendish classification, Rigault classification, scapular elevation, and medialization. Preoperative appearance was classified as grade III in 13 cases and grade IV in 10 cases according to the Cavendish classification, and as grade II in 4 cases and grade III in 19 cases according to the Rigault classification. Using 3D-CT, we assessed the height to width ratio on the scapular posterior view, the superior displacement ratio and rotational difference on the trunk posterior view, and the anterior curvature of the supraspinous portion on the scapular medial view. RESULTS: The average follow-up postoperative period was 4.4 years (range, 2.2 to 8.7 y). Postoperatively, the shoulder abduction improved well, with a mean improvement of 63 degrees. Improvement of at least 1 Cavendish grade and 1 Rigault grade was attained in all cases. On preoperative 3D-CT, the mean height to width ratio of the affected scapula was significantly smaller than that of the contralateral scapula. There was an inverse relationship between the superior displacement ratio and the rotational difference. Anterior curvature of the supraspinous portion was seen in all cases. Omovertebral bone was found in 20 cases. MRI identified unossified omovertebral cartilage that was not evident by radiographs or CT. Transient brachial plexus palsy occurred in 2 patients, both of whom recovered within 4 months. Postoperative scapula winging and unsightly scarring were seen in 3 and 8 cases, respectively. CONCLUSIONS: 3D-CT and MRI were helpful in evaluating the pathologic anatomy of the deformity and in planning the surgery. The modified Green procedure provided successful functional and cosmetic results. LEVEL OF EVIDENCE: Level IV.

Multicenter study of Blount disease in Japan by the Japanese Pediatric Orthopaedic Association.

BACKGROUND: In order to investigate the epidemiology and features of Blount disease in Japan, the Japanese Pediatric Orthopaedic Association conducted a multicenter study on Blount disease in 2003. METHODS: Questionnaires were sent to 1,350 training hospitals of the Japanese Orthopaedic Association. This study included those with stage I or II diagnosed between 1990 and 2002 and those with stage III or higher diagnosed between 1980 and 2002 based on the Langenskiöld classification. The questionnaire items included age at diagnosis, sex, presence or absence of a family history of bowlegs, past history of trauma, birthplace, age when starting to walk, height and weight at the initial presentation, laterality of the affected knee, disease types (infantile or adolescent), treatments, and plain radiographic findings. RESULTS: The results for 212 patients with 296 affected knees were obtained. The disease types were infantile in 190 patients with 270 affected knees and adolescent in 22 patients with 26 affected knees. There were more girls among cases with both types. Among the infantile-type cases, there were more patients with bilaterally affected knees; however, in the adolescent-type cases, there were more patients with unilaterally affected knees. Patients with either type tended to be obese at diagnosis. There was little difference in age at which patients with either type started to walk, compared with the mean age for the general population. Conservative treatment was applied to most infantile-type cases in stage I or II, whereas surgery was performed in all but one of those with stage III or IV of the disease. Most adolescent-type cases underwent surgery. CONCLUSIONS: This was the first multicenter study on Blount disease in Japan. Among 296 knees, the majority of these knees had stage I or II Blount disease. Very few cases had stage III or higher disease, and there were 35 knees with the infantile type and 19 with the adolescent type in the 23 years from 1980 through 2002.

Remodelling of femoral head-neck junction in slipped capital femoral epiphysis: a multicentre study.

PURPOSE: We examined the remodelling of the femoral head-neck junction in patients with slipped capital femoral epiphysis (SCFE) and the frequency of residual cam deformities. METHODS: We reviewed 69 hips in 56 patients with stable SCFE who had undergone in situ pinning. Mean age at slip was 11.7 years and the follow-up period 63.4 months. Cam deformity was evaluated using the anterior offset alpha (α) angle and head-neck offset ratio (HNOR). RESULTS: The average α angle and HNOR significantly improved from 76.2° to 51.3° and 0.086 to 0.135, respectively; 25 hips (36.2%) still had an α angle greater than 50°, and 32 hips (46.4%) had an HNOR of under 0.145. A multivariate analysis selected age at onset and slip angle as risk factors for cam deformity, with cutoff values 11.1 years and 21.0°, respectively. CONCLUSIONS: Although most hips had remodelling of the head-neck junction, 29.4 % had residual cam deformities that may be susceptible to femoroacetabular impingement.

Limb salvage treatment for Gollop-Wolfgang complex (femoral bifurcation, complete tibial hemimelia, and hand ectrodactyly).

We reported the findings from three patients with Gollop-Wolfgang complex and demonstrated the results of five limb salvage treatments for this condition. All three femoral bifurcations were accompanied by ipsilateral complete tibial hemimelia. Two patients showed contralateral complete or partial tibial hemimelia, and one patient had hand ectrodactyly. The five limb salvage treatments included resection of the anteromedial bifurcated femur in three limbs, foot centralization in five limbs, tibiofibular fusion in one limb with partial tibial hemimelia, fibular transfer (Brown's procedure) in three limbs with complete tibial hemimelia, and callus distraction lengthening in one limb. The duration from the first operation to the final follow-up ranged from 3.5 to 5.4 years. None of the three knees treated by fibular transfer achieved a successful functional result, but all of the knees were ultimately able to withstand weight bearing. Early knee disarticulation and resection of the protruded bifurcated femur, followed by fitting of a modern prosthesis is likely to be the best treatment for patients with Gollop-Wolfgang syndrome. We note that limb salvage treatment is an alternative in patients who opt to retain their feet and refuse amputation.

PAPSS2 mutations cause autosomal recessive brachyolmia.

BACKGROUND: Brachyolmia is a heterogeneous group of skeletal dysplasias that primarily affects the spine. Clinical and genetic heterogeneity have been reported; at least three types of brachyolmia are known. TRPV4 mutations have been identified in an autosomal dominant form of brachyolmia; however, disease genes for autosomal recessive (AR) forms remain totally unknown. We conducted a study on a Turkish family with an AR brachyolmia, with the aim of identifying a disease gene for AR brachyolmia. METHODS AND RESULTS: We examined three affected individuals of the family using exon capture followed by next generation sequencing and identified its disease gene, PAPSS2 (phosphoadenosine-phosphosulfate synthetase 2). The patients had a homozygous loss of function mutation, c.337_338insG (p.A113GfsX18). We further examined three patients with similar brachyolmia phenotypes (two Japanese and a Korean) and also identified loss of function mutations in PAPSS2; one patient was homozygous for IVS3+2delT, and the other two were compound heterozygotes for c.616-634del19 (p.V206SfsX9) and c.1309-1310delAG (p.R437GfsX19), and c.480_481insCGTA (p.K161RfsX6) and c.661delA (p.I221SfsX40), respectively. The six patients had short-trunk short stature that became conspicuous during childhood with normal intelligence and facies. Their radiographic features included rectangular vertebral bodies with irregular endplates and narrow intervertebral discs, precocious calcification of rib cartilages, short femoral neck, and mildly shortened metacarpals. Spinal changes were very similar among the six patients; however, epiphyseal and metaphyseal changes of the tubular bones were variable. CONCLUSIONS: We identified PAPSS2 as the disease gene for an AR brachyolmia. PAPSS2 mutations have produced a skeletal dysplasia family, with a gradation of phenotypes ranging from brachyolmia to spondylo-epi-metaphyseal dysplasia.

Surgical treatment of hip dislocation in amyoplasia-type arthrogryposis.

We reviewed our clinical and radiographic results in the operative treatment of five unilateral and six bilateral hip dislocations in 11 patients with amyoplasia-type arthrogryposis. The mean age at surgery was 1.8 (range, 1.1-3.2) years. Femoral and/or pelvic osteotomies were combined with an open reduction. The mean follow-up period was 12.7 (range, 5.2-23.2) years. Nine patients were community walkers and two were household walkers. The range of motion and the St Clair hip score of the bilateral hips were not significantly different from those of the unilateral hips. Successful reduction increases the ambulation potential in both unilateral and bilateral hip dislocations.

High prevalence of acetabular retroversion in both affected and unaffected hips after Legg-Calvé-Perthes disease.

BACKGROUND: Acetabular retroversion is observed in hips after various pediatric hip diseases. This study sought to examine the frequency of acetabular retroversion in both affected and unaffected hips after Legg-Calvé-Perthes disease and its correlation with the prominence of the ischial spine. METHODS: We retrospectively investigated the version and morphological features of the acetabulum using pelvic radiographs after Legg-Calvé-Perthes disease (107 affected hips treated non-operatively and 72 unaffected hips from the contralateral side). The diagnosis of acetabular retroversion was made based on the presence of a positive cross-over sign on anteroposterior pelvic radiographs. The correlation between the presence of a positive cross-over sign and modified Stulberg classes, the onset age of Legg-Calvé-Perthes disease, radiographic parameters for acetabular dysplasia and the prominence of the ischial spine were examined. RESULTS: The prevalence of a positive cross-over sign was 49.5 % (45 of 91 hips) in affected hips and 45.8 % (33 of 72 hips) in unaffected hips. Hips with a positive cross-over sign were significantly coexistent bilaterally. The prevalence of prominence of the ischial spine in the positive cross-over sign group was 71.4 % in the affected side and 81.8 % in the unaffected side, indicating a significant correlation between the cross-over sign and the prominence of the ischial spine in both affected and unaffected hips. The positive cross-over sign did not have any correlation with the parameters for acetabular dysplasia and the onset age, however, had a significant correlation with the severity of the femoral head deformity. CONCLUSIONS: High prevalence of acetabular retroversion in both affected and unaffected hips after Legg-Calvé-Perthes disease was demonstrated. Symmetric acetabular deformity and the coexistence of prominence of the ischial spine suggested the effects of the Legg-Calvé-Perthes disease lesions on the skeletal development of the whole pelvis. Further follow-up is needed to clarify the pathological significance of acetabular retroversion after Legg-Calvé-Perthes disease.

Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency.

PURPOSE: Kabuki syndrome is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, postnatal growth deficiency and mental retardation. This report reviews our results in the operative treatment of hip dislocations in patients with Kabuki syndrome. METHODS: Between 2001 and 2009, seven dislocated hips (three unilateral and two bilateral hips) in five patients (all girls) were operatively treated at our institution. The operative treatment consists of open reduction, femoral derotation varus osteotomy, pelvic osteotomy (Salter in one and incomplete periacetabular osteotomy in six hips) and capsular plication. The age of the patients at the time of surgery ranged from 2.4 to 5.7 years, with an average of 3.6 years. RESULTS: The follow-up postoperative period ranged from 3.2 to 6.3 years, with an average of 5.0 years. At the final follow-up, all patients reported no click and no pain, and showed well-contained hips by radiographs. All seven hips were graded as Severin class I-II. One patient presented as having habitual dislocation of the hip 4.4 years after surgery. Computed tomographic (CT) scans revealed posterior acetabular wall deficiency, which was not corrected by the anterolaterally directed Salter osteotomy. The incomplete periacetabular osteotomy provided sufficient posterolateral coverage of the acetabulum. CONCLUSION: Operative treatment combining open reduction, femoral derotation varus and incomplete periacetabular osteotomies, and capsular plication provided successful results in patients with Kabuki syndrome who had the characteristics of hip instability such as ligamentous laxity, muscular hypotonia and posterior acetabular wall deficiency.

Septic sacroiliitis in children.

We retrospectively reviewed the clinical, laboratory, and magnetic resonance (MR) imaging features of 8 patients with septic sacroiliitis. The age of the patients ranged from 4.3 to 15.2 years. None of the patients were accurately diagnosed before presentation. Misdiagnosis was largely caused by the wide variety of clinical symptoms including hip, back, knee, and abdominal pain, by low suspicion of the disease, and by negative radiographic findings. Magnetic resonance examination was performed in all patients and confirmed the diagnosis. All patients were treated with bed rest and intravenous antibiotics. Clinical and laboratory findings improved in all patients during antibiotic treatment, but MR findings showed a mixed response with resolution of effusions in the sacroiliac joint, muscular infiltration, and abscess formation, but progressive changes in signal intensity in the adjacent bone marrow of the sacrum and ilium. After treatment, the signal changes in the bone marrow persisted for 2 to 3 months.