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Background: This study evaluates the radiological parameters of developing subsequent contralateral slips in unilateral slipped capital femoral epiphysis (SCFE) patients at the time of initial presentation., Methods: The study group included the review of unilateral SCFE patients treated between June 2007 and August 2018. Age, gen-der, side, stability, posterior slope angle, grade of slip, modified Oxford bone age score (mOBAS), the Risser classification, and the ap-pearance of the triradiate cartilage were evaluated retrospectively. Data were analyzed between two groups: subsequent contralateral SCFE (SCFESC) patients that developed contralateral slip during follow-up and unilateral SCFE (SCFEU) patients that did not develop contralateral slip up to skeletal maturity. Descriptive statistics were used to compare risk factors between groups., Results: This study included 48 patients and 6 patients (12.5%) developed a SCFESC. Only mOBAS was significantly different be-tween groups. The mOBAS scores in SCFESC were 18 in 2 patients (33.3%), 19 in 4 patients (66.7%). The mOBAS scores in SCFEU were 18 in 1 patient (2.4%), 19 in 24 patients (57.1%), and >20 in 17 patients (40.5%). In the SCFESC group, all patients had a Risser score of 0 and all had open triradiate cartilage., Conclusion: Patients with unilateral SCFE are at risk for SCFESC, and the mOBAS is the best predictor of risk assessment. We agree that mOBAS score of 16,17 or 18 patients' contralateral hips can be prophylactically pinned. We also suggest pinning or close screening of mOBAS 19 patients that some carry relatively high risk of subsequent contralateral slip.

The Beckwith-Wiedemann spectrum (BWSp) ranges from isolated lateralized overgrowth (ILO) to classic phenotypes. In this broad clinical spectrum, an epigenetic alteration on chromosome 11p15.5 can be detected. The risk for embryonal tumors is high, especially in patients with lateralized overgrowth (LO). The aim of this study is to investigate epigenetic alterations in 11p15.5 and tumor risk in 87 children with LO. The methylation level of 11p15.5 was examined in the blood of all patients and in skin samples or buccal swabs from 40 patients with negative blood tests; 63.2% of patients were compatible with the ILO phenotype, 18.4% were atypical, and 18.4% were classic. The molecular diagnosis rate was 81.2% for the atypical and classic phenotypes, and 10.9% for the ILO phenotype. In patients with epigenetic alterations, LO was statistically significantly more severe than in test negatives. Tumors developed in six (6.9%) of the total 87 patients with LO; four belonged to the atypical or classical phenotype (12.5%) and two to ILO (3.5%). Three of the four patients with atypical/classical phenotypes had pUPD11, one had IC1-GOM alteration, and two ILO patients were negative. We conclude that LO patients should be monitored for tumor risk even if their epigenetic tests are negative.

Purpose: To compare the critical shoulder angle (CSA), acromion index (AI), acromion angulation (AA) and glenoid version angle (GVA) between patients with full-thickness rotator cuff tears (RCTs) and patients with intact rotator cuffs., Methods: Between 2014 and 2018, the CSA, AI, AA and GVA were measured in consecutively included patients aged > 40 years who underwent shoulder arthroscopy for full-thickness RCTs. A total of 437 patients with RCTs and a mean age of 51.2 years (± 5.8) were included, 35.7% of whom were male. In the control group, there were n = 433 patients (36.3% male) with an intact rotator cuff, and the mean age was 50.7 years (± 5.3)., Results: The mean AI for the RCT group was 0.7 ± 0.1, which was significantly higher than the mean AI for the control group (0.6 ± 0.1, p < 0.001). The mean CSA for the RCT group was 33.6° ± 3.9°, which was significantly higher than the mean CSA for the control group (31.5° ± 4°, p < 0.001). The mean AA for the RCT group was 13.9° ± 9°, which was significantly higher than the mean AA for the control group (12.4 ± 8.6, p = 0.012). The mean GVA for the RCT group was - 3.5° ± 4.6° and significantly retroverted compared with the mean GVA for the control group (- 2.2° ± 4.6°, p < 0.001). The cutoff values determined by the ROC curve analyses were as follows: 0.6 for AI, 31.4° for CSA, 9.6° for AA and - 2.6° for GVA., Conclusion: The CSA, AI, GVA and AA values measured by MRI were determined to be significantly related to full-thickness rotator cuff ruptures. The AI, CSA, AA and GVA may be considered risk factors for degenerative rotator cuff tears. Assessing the CSA, AI, GVA and AA can be helpful for diagnostic evaluation of patients with full-thickness RCTs., Level of Evidence: III.

Background: There are anatomical differences in the femur and acetabulum on the dysplastic hips. Yet, although there are detailed researches on bony structure differences, studies regarding anatomical differences for the vascular structure are insufficient. The study aimed to determine the relationship between the femoral artery and vein with acetabulum in computed tomography evaluation of Crowe type 4 hips and to compare with normal hip femoral vein and artery anatomic position., Methods: Forty patients with one side hip Crowe type 4 deformity and opposite side normal hip were found suitable for the study. Pelvis CT was previously applied to all patients for the planning of total hip arthroplasty surgery. Normal hip acetabulum and the true acetabulum of the dysplastic hip were marked with the Ranawat's triangle and were then divided into quarters with four axial slices of the acetabulum; as dome, proximal one-fourth, center, and distal three-fourths. The shortest distance from the femoral vessels to the pelvis was measured at these 4 axial slices at the dysplastic side and compared with the healthy side., Results: The distance of the vein to the acetabulum is closer to the dysplastic side. At distal three-fourths of Ranawat's triangle axial images, both femoral artery and femoral vein are closer to acetabulum at the dysplastic side., Conclusion: The analysis of preoperative images in cases of Crowe type 4 hip shows that the femoral vessels are closer to the anterior wall of the dysplastic true acetabulum at the level of the center of the hip (vein, mean: 2.7 mm closer) and below the center of the hip (vein, mean: 3 mm; and artery, mean: 3.3 mm closer) when compared with the normal acetabulum. Anatomic relationships knowledge can be used intraoperatively to avoid iatrogenic vascular injury during Crowe type 4 dysplastic hip arthroplasty surgery., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Arthrogryposis is a clinical finding that is present either as a feature of a neuromuscular condition or as part of a systemic disease in over 400 Mendelian conditions. The underlying molecular etiology remains largely unknown because of genetic and phenotypic heterogeneity. We applied exome sequencing (ES) in a cohort of 89 families with the clinical sign of arthrogryposis. Additional molecular techniques including array comparative genomic hybridization (aCGH) and Droplet Digital PCR (ddPCR) were performed on individuals who were found to have pathogenic copy number variants (CNVs) and mosaicism, respectively. A molecular diagnosis was established in 65.2% (58/89) of families. Eleven out of 58 families (19.0%) showed evidence for potential involvement of pathogenic variation at more than one locus, probably driven by absence of heterozygosity (AOH) burden due to identity-by-descent (IBD). RYR3, MYOM2, ERGIC1, SPTBN4, and ABCA7 represent genes, identified in two or more families, for which mutations are probably causative for arthrogryposis. We also provide evidence for the involvement of CNVs in the etiology of arthrogryposis and for the idea that both mono-allelic and bi-allelic variants in the same gene cause either similar or distinct syndromes. We were able to identify the molecular etiology in nine out of 20 families who underwent reanalysis. In summary, our data from family-based ES further delineate the molecular etiology of arthrogryposis, yielded several candidate disease-associated genes, and provide evidence for mutational burden in a biological pathway or network. Our study also highlights the importance of reanalysis of individuals with unsolved diagnoses in conjunction with sequencing extended family members., (Copyright © 2019 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Background: Coxa vara has been frequently reported in spondyloepiphyseal dysplasia congenita (SEDC), and proximal femoral osteotomy has been described as a useful treatment. The aim of this study was to discuss the clinical, radiographic, and gait outcomes after valgus extension osteotomy of the proximal femur. Changes of lumbar lordosis, associated with coxa vara correction, are reported as well as the outcome differences between different ages., Methods: Records of children with SEDC, who were followed at our institution between 2004 and 2014, were reviewed; and children had hip surgery were identified. Hip pain and passive range of motion, radiographic neck shaft angle (NSA), and Hilgenreiner trochanteric (H-T) angle, sagittal spinopelvic parameters, and gait data were recorded. Preoperative and last follow-up data were compared. Outcomes were also compared between 3 age groups., Results: Of the 79 children with SEDC, 26 children (12 boys and 14 girls) had hip osteotomy in 48 hips. Mean age at surgery was 9.6 years and the mean follow-up was 5 years. Preoperative hip pain was noted in 30 hips. At the last follow-up, 3 hips were painful at the extreme range of motion. Passive range of motion, NSA, and H-T improved postoperatively. Although NSA was maintained over the follow-up, H-T deterioration was noted. Spinopelvic measurements changed significantly and gait data remained stable except pelvic tilt that reduced significantly after surgery. The changes of radiographic measurements in each age group were similar to the total group of patients., Conclusions: In children with SEDC, surgical correction of coxa vara, by proximal femoral valgus osteotomy, is an effective treatment that improves hip pain and range of motion in addition to the radiographic alignment of the proximal femur and the sagittal spinopelvic alignment. Children are expected to maintain their level of function after surgery and to have good results over the midterm regardless of their age at surgery., Level of Evidence: Level IV-therapeutic study.

Mucopolysaccharidosis IVA (MPS IVA) is a lysosomal storage disease caused by biallelic mutations in GALNS gene and characterized by progressive skeletal deformities with short stature. The aim of this study was to evaluate the genotype, longitudinal height measurement and clinical features of MPS IVA patients. Thirty-two patients from 22 families were enrolled. The ages of patients at diagnosis ranged from two months to 18 years of age, and followed up for three to twenty years. They were classified as severe and attenuated form (intermediate and mild) according to their height measurements. The mean height standard deviation scores (SDS) for Turkish standards at 0-3, 5 and 10 years of ages were found to be -1.1, -4.2 and -7.3 respectively in patients with severe phenotype, while they were +0.4, -1.5 and -3 for intermediate phenotype. Patients with severe form reached a mean final height of -8.5 SDS, and mild phenotype -3.6 SDS. The most common initial and current symptoms in the patients with the severe phenotype were pectus carinatus and/or kyphosis deformities which occurred between 5 months and 3 years of age, and genu valgum deformity which developed after 3 years of age. However, kyphoscoliosis was the most common initial and current findings in the attenuated phenotype. Although, initial symptoms appeared in early childhood in the intermediate phenotype, similar to the severe phenotype, the clinical findings progressed slowly and genu valgum deformity did not develop. In patients with mild phenotype, the onset of symptoms was after 5 years of age. In conclusion, this study provides significant insights into the initial and follow-up clinical features and height values that contribute to the differential diagnosis of the severe and intermediate phenotypes in early childhood. Eleven mutations in GALNS gene in which one of them is novel (c.416G>A) were associated with the severe phenotype and three mutations (c.1038C>A, c.850T>G, c.752G>A) lead to the attenuated phenotype., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: Greater trochanter apophysiodesis (GTA) is relatively minimal invasive technique for the treatment of trochanteric overgrowth. Various types of implants can be used in each procedure. The purpose of this study was to compare outcomes of three different types of implants that were used in treatment of trochanteric overgrowth in Legg-Calve-Perthes disease., Materials and Methods: We retrospectively studied radiological results of three implants (screw, screw washer, and EP) on inhibiting trochanteric growth in 32 patients. Articulo-trochanteric and trochanter-trochanter distances (TTDs) were measured on radiographs. Embedding of implant evaluated on final radiographs., Results: The mean of age at the surgery was 10 ± 2.3 years, and the mean of follow up period was 50.0 ± 16.7 months. In all groups, articulo-trochanteric distance was decreased on final radiographs. In screw and screw washer group, increase of TTD was not statistically significant ( P < 0.05). Twelve, one, and two implants were embedded, respectively, in screw, screw washer, and EP groups. Two patients in EP group had revision surgery due to loosening., Conclusions: In this study group, GTA using screw and screw washer methods could slow down but did not restore trochanteric overgrowth. We suggest using washer to reduce embedding of the screw., Competing Interests: There are no conflicts of interest.

Aim: To compare the postoperative changes of both sagittal spinal and spinopelvic parameters in patients with Lenke Type 1 adolescent idiopathic scoliosis (AIS) who underwent selective and non-selective fusion surgery., Material and Methods: We conducted a retrospective study among 53 Lenke Type 1 AIS patients who underwent corrective surgery at our centre between 2006 and 2012. Patients were classified as group 1 if they underwent selective surgery and as group 2 if they underwent non-selective surgery. Surgical results of preoperative and postoperative sagittal and spinopelvic measurements, pelvic tilt (PT), pelvic incidence (PI), sacral slope (SS), lumbar lordosis (LL) and thoracic kyphosis (TK) values were analysed using the SURGIMAP© Software (Nemaris İnc. USA) measurement system., Results: In both groups, a comparison of pre- and postoperative sagittal spinal parameters did not show a statistically significant difference. In both groups, pre- and postoperative measurements of LL and TK did not show a statistically significant difference., Conclusion: After selective and non-selective surgery, sagittal spinal and spinopelvic parameters are not affected in the middle term. We think that the long-term studies to be done in this regard will increasingly require the necessity of keeping the pelvis in mind while evaluating the sagittal plan in AIS surgery.

Purpose: The purpose of this study was to assess the variations in tibial tubercle-trochlear groove distance and angle as a function of age and gender in a population of children without patellar instability (PI) compared with those with PI., Methods: A retrospective review of 869 children's knee MRIs, ages 5 to 15 years, were evaluated using a control group (792 children) without evidence of PI and a group with PI (77 children). Tibial tubercle-trochlear groove distance (TT-TGd) and angle (TT-TGa) were measured twice by two readers to assess intra- and inter-observer reliability and compared between PI and control groups. In both groups, functions of age and gender on TT-TGd and TT-TGa values were evaluated., Results: Both TT-TGd and TT-TGa measurements showed excellent intra- and inter-observer reliability. The mean TT-TGd for the PI group was 17.2 mm (SD 6.6) and significantly higher than the mean TT-TGd for the control group (10.4 SD 3.8 mm, P = 0.001). The mean TT-TGa for the PI was 20.8° (SD 8.3°), which was also significantly higher than the mean TT-TGa for the control group (12.5° SD 4.6°, P < 0.001). Control group revealed a positive correlation between age and TT-TGd measurements (r = 0.243, P < 0.001). The mean TT-TGa for girls (13.3° SD 4.7°) was higher than the mean TT-TGa for boys (11.9° SD 4.4°) in the control group (P < 0.001)., Conclusion: TT-TGa and TT-TGd are reliable and can be used for the evaluation of the extansor mechanism alignment in children with and without PI. However, it must be considered that TT-TGd is increasing in growing patients. Soft-tissue procedures may be prone to failure, since bony procedures for patellar alignment cannot be done until skeletal maturity., Level of Evidence: III.

Background: The incidence of total hip arthroplasty (THA) is increasing in all age groups, especially in young patients. The outcome of THA is believed to be affected by morphological deformities created by previous pelvic or femoral procedures performed in childhood. The aim of the present study was to assess whether previous hip surgery impaired the functional outcome in young patients who underwent THA., Methods: Data were collected from the records of patients aged less than 30 years who had undergone THA between 2002 and 2011. Thirty-five patients (44 hips) were included. The Western Ontario and McMaster Universities Osteoarthritis Index and Harris Hip Scores were collected as primary functional outcome measures. The secondary outcome measures were the major and minor complications. Patients were divided into 2 groups: those who had not undergone hip surgery before THA (group I, 17 patients, 24 hips) and those with a history of prior hip surgery (group II, 18 patients, 20 hips)., Results: The mean age at the time of surgery was 25 ± 4 years in group I and 23 ± 4 years in group II. The mean Harris Hip Score was slightly higher in group I (90 ± 7) than in group II (87 ± 7) (P = .2). The mean Western Ontario and McMaster Universities Osteoarthritis Index scores were similar in both groups (in group I 14 ± 7 and in group II 14 ± 6, P = .9). Complication rates were also similar in both groups (P = .7)., Conclusions: It is often difficult to decide whether to perform THA in young patients. One potential risk factor for complications is hip surgery before THA. Our study revealed that young patients with or without a history of previous hip surgery achieved satisfactory functional outcomes with similar complication rates after THA., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Internal tibial torsion (ITT) is a common boney deformity in children with cerebral palsy (CP). The current recommended treatment is tibial derotation osteotomy (TDO) to improve gait biomechanics. Satisfactory short-term results after TDO have been reported but long-term results have not been studied. The purpose of this study was to evaluate the long-term outcome after external TDO performed to correct ITT in ambulatory children with CP., Methods: Following IRB approval, gait kinematics and passive range of motion measurements were retrospectively evaluated in children with spastic CP who underwent TDO due to ITT comparing preoperative (E0), short-term postoperative (E1; 1 to 3 y post), and long-term postoperative (E2; >5 y post) results. Limbs were categorized as corrected, undercorrected, or overcorrected at both E1 and E2, by comparing the subjects mean tibial rotation (MTR) in gait to a group of typically developing children. Age at surgery, preop MTR (at E0), preop gait velocity (at E0), gross motor function classification system score, and foot deformity were evaluated to determine their influence on long-term results., Results: The study sample consisted of 36 legs (with E0 and E2) and 17 legs (with E0, E1, and E2). The mean age at surgery was 7.4±2.8 (range, 4 to 16.6) years. Comparing the changes over time, kinematic MTR improved from 17±11 degrees initially (E0) to -10±14 degrees short term (E1) and progressed to -23±13 degrees long term (E2) (P<0.05 E0/E1/E2; internal rotation is positive). At E2, 16 legs (44%) were found to be in the kinematic corrected group and 20 legs (56%) in the kinematic overcorrected group. There were no significant differences between the corrected and overcorrected groups of children in respect to age of surgery, gross motor function classification system, E0 MTR, gait velocity, or foot deformity., Conclusions: Although external TDO is an accepted form of treatment in children with CP, in the long term a tendency to move into external tibial torsion is common. Therefore, caution is warranted with children who initially present with ITT to avoid overcorrection., Level of Evidence: Level IV-therapeutic study.

Background: External tibial torsion (ETT) is a common bony deformity in children with cerebral palsy (CP). The current recommended treatment is tibial derotation osteotomy (TDO) to improve gait biomechanics. Satisfactory short-term results after TDO have been reported but long-term results have not been studied. The purpose of this study was to evaluate the long-term outcome following TDO to correct ETT in ambulatory children with CP., Methods: Following IRB approval, gait kinematics and passive range of motion measurements were retrospectively evaluated in children with spastic CP who underwent TDO due to ETT comparing preoperative (E0), short-term postoperative (E1; 1 to 3 y post), and long-term postoperative (E2; >5 y post) results. Limbs were categorized as corrected, undercorrected, or overcorrected at both E1 and E2, by comparing mean tibial rotation (MTR) in gait to a group of typically developing children. Age at surgery, E0 MTR, E0 gait velocity, gross motor function classification system (GMFCS) score, and foot deformity were evaluated to determine their influence on long-term results., Results: The study sample consisted of 43 legs (with E0 and E2) and 22 legs (with E0, E1, and E2). The mean age at surgery was 10.3±3.4 years (range, 6 to 19.2 y). In the group MTR trended toward improvement moving from -26±17 degrees (E0, external negative) to -16±16 degrees (E1) and relapsed to -23±17 degrees at the long term (P=0.071, E0/E1; P=0.589, E0/E2). Improvement was also seen in the transmalleolar axis (P=0.074), mean ankle rotation, and mean foot orientation (P<0.05, E0/E2). At the long-term evaluation, 16 legs (37%) were found to be in the kinematic corrected group, 25 legs (58%) in the kinematic undercorrected group, and 2 legs (5%) in the kinematic overcorrected group. There were no significant differences between the corrected and undercorrected groups of children with respect to age at surgery, GMFCS, E0 MTR, gait velocity, or foot deformity., Conclusions: Although internal TDO improves ETT in the short term, recurrence is frequent with an apparent developmental trend toward external rotation of the tibia., Levels of Evidence: Level IV-therapeutic study.

Background: Either percutaneous or eight-plate epiphysiodesis have been shown to be effective growth modulation techniques for the treatment of limb length discrepancies (LLD). However, few studies compared the outcomes of both techniques with some confounding results. The aim of this study was to evaluate the outcomes of the both techniques in the treatment of the LLD., Methods: Between 2004 and 2012, medical records of all cases that underwent either eight-plate or percutaneous epiphysiodesis (PE) were reviewed. Age at surgery, sex, diagnosis, surgical site (proximal tibia/distal femur), time of follow-up, complications, and additional procedures were noted. Correction of the LLD was evaluated to calculate the rate of correction and the percentage of improvement from the long-leg standing x-rays or scanograms, collected at each visit., Results: There were 24 patients in the eight-plate (10 girls and 14 boys) and 48 patients in the PE group (28 girls and 20 boys). No statistically significant difference was found in the age, sex, preoperative LLD, or follow-up between groups. Both groups reached to an average LLD below 2 cm. The percentage of improvement was significantly higher in the PE group (P=0.031). The rate of individual femoral and tibial correction did not differ between the groups., Conclusions: Both methods are shown to be effective for LLD correction. However, PE led to greater improvement during the same follow-up time with fewer complications and less need for additional surgical procedures.

This study aimed to evaluate the outcomes of nonarthrodesis surgical treatment of hallux valgus (HV) deformity in children with cerebral palsy using radiographic and gait analysis parameters. There were 25 patients who had hallux valgus correction in 39 feet. The mean age at surgery was 15±2.8 years and the mean follow-up duration was 14.6 months. The first metatarsal osteotomy was performed in nine feet, bunionectomy in 25 feet, and Aiken osteotomy in 32 feet. None had metatarsophalangeal joint fusion. We observed a significant correlation between HV correction and other foot and ankle gait parameters. Our study showed correction of HV deformity at short-term follow-up without fusion of the metatarsophalangeal joint., Level of Evidence: Level IV Therapeutic Studies.

Objective: The aim of this study was to compare the treatment outcomes of mid-diaphyseal clavicular fractures between an external fixator and more widely selected treatment options such as plate osteosynthesis or conservative methods., Materials and Methods: The medical records of 64 patients who were treated for mid-diaphyseal clavicular fracture in our clinic from 2009 to 2013 were reviewed. The inclusion criterion was mid-diaphyseal closed clavicular fractures with shortening of more than 2 cm. The Constant and DASH (disabilities of the arm, shoulder, and hand) scores at the final follow-up, initial displacement, nonunion, and complication rates were compared. Statistical differences between groups were assessed with the Kruskal-Wallis test, and pairwise comparison analysis was used to evaluate the differences within the groups., Results: Of the 64 patients, 24 (37.5%) were treated with plate osteosynthesis (group 1), 11 (17.2%) with an external fixator (group 2), and 29 (45.3%) conservatively with a figure-of-eight bandage (group 3). The mean follow-up periods were as follows: group 1: 37 ± 10.4 months, group 2: 33 ± 7 months, and group 3: 35 ± 9.4 months. The initial amount of displacement and DASH score were: group 1: 89.1 ± 7.8, group 2: 89.1 ± 7.8, and group 3: 6.1 ± 6.6) (p = 0.079). The Constant score of group 2 (93 ± 6.1) was significantly higher than that of group 3 (85 ± 8.4) (p = 0.013). No statistical difference was found in the distribution of nonunions (p = 0.387). However, in group 3, the number of malunions (11/29, 37.9%) was significantly higher compared to the other 2 groups (group 1: 1/24, 4.2%; group 2: 2/11, 18.2%) (p = 0.006)., Conclusions: This study revealed that the treatment of acute clavicular fractures with an external fixator was a good alternative to plate osteosynthesis or conservative treatment., (© 2017 The Author(s) Published by S. Karger AG, Basel.)

Several clinical and radiological factors can be prognostic in the development of angular deformity following physeal injuries of the distal tibia. One of the radiological parameters, premature physeal closure (PPC), can be detected during postoperative follow-ups. Aim of our study was to identify the prognostic factors in development of angular deformity and its relationship with PPC. One hundred and four patients treated due to physeal injuries of the distal tibia were included in our study. Patients were divided into three groups based on Salter-Harris (SH) classification. The intergroup relationships between sex, age, the amount of energy sustained during injury, premature physeal closure, the amount of residual gap, and deformity were analyzed. Angular deformity developed in 25% (3/12) of SH Type 2, in 60% (9/15) of Type 3 and 30% (3/10) of Type 4 patients with PPC. A residual displacement of more than 2 mm, age and premature physeal closure were specified as significant risk factors for development of angular deformity. 2 mm limit for residual displacement and findings of premature physeal closure in the radiological evaluations during follow-ups are prognostic factors in avoiding malalignment of the distal tibia., Level of Evidence: Level 3.

This retrospective study compared the results of one-stage surgery of unilateral and bilateral Tönnis grade 4 hips (high dislocation in developmental hip dysplasia, DDH) : 41 hips, 30 patients. Mean follow-up was 56 months. Pelvic radiographs were evaluated at three time points. Acetabular inclination angle improved significantly in both groups. Results were satisfactory (Severin classes I-II) in 13 hips (68.5%) (unilateral group) and 21 hips (89.5%) (bilateral group). Functional evaluation (McKay classification) at final follow-up revealed satisfactory results in 94.8% (unilateral group) and 63.6% (bilateral group). One-stage surgery is a reliable method for treating children with unilateral or bilateral DDH.

The aim of this study was to compare the midterm clinical and radiological outcomes of the Salter osteotomy (SO) and Tonnis lateral acetabuloplasty (TLA) with concomitant open reduction for the treatment of developmental dysplasia of the hip. Twenty-five hips of 20 patients who underwent SO with open reduction and 26 hips of 23 patients who underwent TLA with open reduction were evaluated retrospectively. The average age of the patients at the time of the operation was 35.6 months in the SO and 36.6 months in the TLA group, without a statistically significant difference (P=0.836). The average follow-up times in the SO and TLA groups were 59.9 and 54.8 months, respectively (P=0.397). Preoperative (40.6° in the SO vs. 42.2° in the TLA, P=0.451) and last follow-up acetabular index (12° in the SO vs. 14° in the TLA, P=0.227) and center-edge angle measurements (30° in the SO vs. 26° in the TLA, P=0.069) did not show a statistically significant difference between the SO and TLA groups; however, early postoperative acetabular index improvement was better in the TLA group than in the SO group (21.2° in the SO vs. 17.2° in the TLA, P=0.014). According to the Severin grading system, both groups showed a similar number of good outcomes without a statistically significant difference (P=0.936). Clinical assessment on the basis of McKay's criteria showed similar good and excellent outcomes (P=0.936). Both osteotomy techniques showed similar satisfactory outcomes for the treatment of DDH in patients older than 18 months of age.

Background: The aim of this study was to describe the dynamic lower extremity alignment in children with diastrophic dysplasia (DD) by 3-dimensional gait analyses. Our main hypothesis was that gait kinematics and kinetics are different than the age-normalized population and patellar dislocation can alter the gait in patients with DD., Methods: A retrospective review of clinical data and radiographs was conducted for patients with DD who had gait analysis before lower extremity skeletal surgery excluding foot procedures. Lower extremity range of motion was measured. The Pediatric Outcomes Data Collection Instrument (PODCI) was administered to parents to evaluate their children's functional status. Gait laboratory data were collected to compare the hip and knee kinematics in cases with and without patellar dislocation. Anteroposterior standing radiographs were taken for all patients to assess the correlation between measurements (clinical, radiologic, and gait) for coronal knee alignment., Results: Thirty lower extremities of 15 children (7 females and 8 males) were evaluated. The mean age was 7.4±3 years, the mean height was 97.7±15 cm (z=-5.1), and the mean weight was 20.6±6.2 kg (z=-0.8). The DD PODCI subscores were statistically significantly lower (P<0.05) than the average stature for developing children, except for the happiness score. Gait analysis, compared between all DD and an age-normalized average stature group, showed decreased forward velocity, step length, and stride length with an increased average forward tilt of the trunk and pelvis, hip flexion, hip adduction, and internal rotation (P<0.001). Delta hip and knee motion were also decreased (P<0.001). The patella was dislocated in 19 (63.3%) and central in 11 (36.6%) knees. Comparison of the minimum knee and hip flexion at the stance phase demonstrated increased crouch gait in the patellar dislocation group (P<0.001). Knee alignment measurements between clinical examination and gait analysis showed moderate correlation (r, 0.476; P=0.008)., Conclusions: Children with DD demonstrated lower PODCI subscores except for happiness. Gait analysis showed limited lower extremity function of the children with DD in our study group. Patella dislocation group had increased crouch gait., Levels of Evidence: Level III-diagnostic study.

Background: The aim of this study is to compare acetabular anteversion between children with Down syndrome with or without hip instability. The second aim is to report the surgical experience at our institution in treating unstable hips for this population., Methods: All children with Down syndrome who were seen at our institution between 2004 and 2014 were reviewed, and those who had pelvic axial computed tomographic or magnetic resonance imaging scans were identified. Acetabular anteversion was compared between 2 groups: those with hip instability as a single hip pathology and those without hip instability. For patients who had surgery for their unstable hips, demographic, clinical, and surgical data were recorded. Preoperative and last visit radiographs were reviewed. Extrusion index and Tonnis, Sharp, lateral center edge, and neck shaft angles were measured. Presence of Shenton's line disruption, crossover, and posterior wall signs were recorded. Independent and paired t test, Wilcoxon signed-rank test, and χ test were used with a significance level at 0.05., Results: Out of 308 children with Down syndrome, there were 10 patients with 13 unstable hips and 13 patients with 26 stable hips who had computed tomographic or magnetic resonance imaging scans. Age and sex distributions were similar (P>0.3) with no difference in acetabular anteversion (P=0.926) between them. Twelve patients (6 boys and 6 girls) had reconstruction for 17 hips. The mean age was 9 years (3 to 15 y) and the mean follow-up was 7 years (1.2 to 17.6 y). Five hips were painful preoperatively and 1 hip was painful at last visit. Radiographic measurements improved significantly (P<0.05). Shenton's line disruption was found in fewer hips (P=0.001) at last visit with no difference in the crossover and posterior wall signs (P=0.177)., Conclusions: This report suggests that a wide range of acetabular anteversion measurements exist in children with Down syndrome. After detailed anatomic study of the hip, good results with a low complication rate can be expected over the intermediate term after hip reconstruction., Level of Evidence: Level IV-prognostic and therapeutic study.

Study Design: Single institution cohort data were collected prospectively and reviewed retrospectively., Objectives: This study aims to compare outcomes among three different instrumentation types: unit rod, iliac screws, and sacral alar iliac (SAI) screws in terms of pelvic obliquity correction in children with cerebral palsy (CP)., Summary of Background Data: The optimal choice for spinopelvic fixation in CP scoliosis with pelvic obliquity is controversial., Methods: Patients with minimum 2 years' follow-up were divided into three groups according to instrumentation type and matched based on preoperative pelvic obliquity and coronal major curve magnitude. Radiographic measurements included horizontal pelvic obliquity angle (PO), spinopelvic angle (SPA), coronal and sagittal Cobb angles, and T1 pelvic angle. Procedures were performed in one pediatric institution between 2004 and 2012. All measurements were performed by a single independent reviewer who was not involved in the procedures., Results: Seventy-seven patients (42 unit rod, 14 iliac screw, and 21 SAI screw) were included. Gender and age distribution was similar across all groups (56% males, 44% females, mean age 13.5 years). Mean follow-up was 3.6 years. Comparing pre- and postoperative measurements, there was a significant decrease (p < .05) in PO, SPA, and coronal major cob angle in all groups. No significant loss of correction occurred during follow-up. Postoperatively, TPA improved in all groups. Nonsymptomatic loosening was noted in 59% of unit rods, 57% of iliac screws, and 52% of SAI screws. One prominent iliac screw needed removal. One nonsymptomatic rod fracture, one infected pseudarthrosis, and one rod malposition occurred in unit rod group., Conclusions: This study suggests that for correction of pelvic obliquity in cerebral palsy scoliosis, iliac and SAI screws were similar to the unit rod in comparative effectiveness and implant safety profile., Level of Evidence: Therapeutic study, Level III., (Copyright © 2016 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: Children with cerebral palsy (CP) and spasticity are often managed with intrathecal baclofen treatment (ITB). Complications of ITB include infection at the pump or catheter site and late complications as well as revisions of the pump and catheter because of events such as battery expiration or implant malfunction. The goal of this study is to report the short-term and long-term incidence, risk factors, and treatment outcomes of ITB infections in children., Methods: This was a retrospective review of 294 children with CP. The number of ITB surgeries per patient, risk of infection for primary and secondary ITB-related procedures, microorganisms responsible, and associated factors, such as concurrent orthopaedic interventions, medical comorbidities, and subsequent management of ITB-related infections, were evaluated., Results: Infection occurred in 28/294 patients (9.5%) with a 4.9% rate per procedure. There were 14 acute (within 90 d of surgery) and 14 late infections. The infection risk per ITB procedure was 2.4%. Risk of late infection over 5-year mean follow-up was 0.95% per year. Pump removal with acute contralateral implantation was the most successful treatment of infections. Gross Motor Function Classification System level V and G-tube were the main risk factors for infection. A total of 133 concurrent orthopaedic procedures were performed during 277 ITB procedures with no increased risk of infection., Conclusions: ITB in children with CP has a relatively low and manageable risk of infection. It is important to always consider infection as a complication with ITB because with prompt treatment the positive impact of ITB is still possible. It is safe to perform concurrent orthopaedic procedures with ITB procedures., Levels of Evidence: Level III-therapeutic study.

Purpose: The purpose of this study was to review the postoperative complications after posterior spinal fusion (PSF) in cerebral palsy (CP) scoliosis and identify the predictive preoperative risk factors., Methods: All PSFs consecutively performed for CP scoliosis between 2004 and 2013 were reviewed. Preoperative risk score (ORS) and postoperative complications score (POCS) were used as measures of all recorded preoperative risk factors and postoperative complications, respectively., Results: The review included 303 children with a mean age of 14.6 ± 3.0 years. Mean hospitalization was 16 days. Dependence on G-tube feeding was associated with higher POCS (P = 0.027). Postoperative fever, seizures, and septicemia were associated with higher ORS (P < 0.01). Specifically, postoperative pancreatitis and deep wound infections were more common in children with G-tube., Conclusion: This study suggests that G-tube dependence is a predictive risk factor of complications after PSF in CP scoliosis. Children with G-tube need special perioperative care. No other specific preoperative risk factor predicted postoperative complications.

Background: Arthrogryposis, defined as congenital joint contractures in 2 or more body areas, is a clinical sign rather than a specific disease diagnosis. To date, more than 400 different disorders have been described that present with arthrogryposis, and variants of more than 220 genes have been associated with these disorders; however, the underlying molecular etiology remains unknown in the considerable majority of these cases., Methods: We performed whole exome sequencing (WES) of 52 patients with clinical presentation of arthrogryposis from 48 different families., Results: Affected individuals from 17 families (35.4%) had variants in known arthrogryposis-associated genes, including homozygous variants of cholinergic γ nicotinic receptor (CHRNG, 6 subjects) and endothelin converting enzyme-like 1 (ECEL1, 4 subjects). Deleterious variants in candidate arthrogryposis-causing genes (fibrillin 3 [FBN3], myosin IXA [MYO9A], and pleckstrin and Sec7 domain containing 3 [PSD3]) were identified in 3 families (6.2%). Moreover, in 8 families with a homozygous mutation in an arthrogryposis-associated gene, we identified a second locus with either a homozygous or compound heterozygous variant in a candidate gene (myosin binding protein C, fast type [MYBPC2] and vacuolar protein sorting 8 [VPS8], 2 families, 4.2%) or in another disease-associated genes (6 families, 12.5%), indicating a potential mutational burden contributing to disease expression., Conclusion: In 58.3% of families, the arthrogryposis manifestation could be explained by a molecular diagnosis; however, the molecular etiology in subjects from 20 families remained unsolved by WES. Only 5 of these 20 unrelated subjects had a clinical presentation consistent with amyoplasia; a phenotype not thought to be of genetic origin. Our results indicate that increased use of genome-wide technologies will provide opportunities to better understand genetic models for diseases and molecular mechanisms of genetically heterogeneous disorders, such as arthrogryposis., Funding: This work was supported in part by US National Human Genome Research Institute (NHGRI)/National Heart, Lung, and Blood Institute (NHLBI) grant U54HG006542 to the Baylor-Hopkins Center for Mendelian Genomics, and US National Institute of Neurological Disorders and Stroke (NINDS) grant R01NS058529 to J.R. Lupski.

Background: Treatment of hip instability in patients with lower lumbar level myelomeningocele is clinically challenging. Muscle transfer procedures, release of contractures, and intertrochanteric varus-rotation osteotomies have been described to restore weak or absent abductor strength as well as relocation of the hip. However, controlled trials evaluating hip instability in lower lumbar myelomeningocele are limited in the current literature., Questions/purposes: The purposes of this study were to compare the (1) radiographic evidence for joint stability; (2) clinical outcomes (including abductor strength, ambulatory ability, and residual use of orthoses); and (3) complications between patients undergoing combined periarticular contracture releases and bony procedures with and without external oblique abdominal muscle transfers., Methods: Between 2004 and 2013, 14 pediatric patients (16 hips) were treated for hip instability secondary to myelomeningocele using releases with or without muscle transfer. From those, 13 patients (15 hips) with a mean age of 6 years who had L3 to L5 level involvement were evaluated retrospectively. The patients were separated into two groups. Nine hips (in eight patients) were treated by performing a combination of external oblique abdominal muscle transfer to the greater trochanter, periarticular release of contractures, and bony procedures. These were compared with six hips (five patients) treated by performing a combination of periarticular release of contractures and bony procedures without external oblique abdominal muscle transfer. This study compared the results between two surgeons, one of whom always performed these muscle transfers in this setting and the other who never performed muscle transfer during the study period. The patients were clinically followed up at a mean of 41 months (range, 14-122 months); none of the patients was lost to followup. Radiographic evaluation criteria included Reimer's migration index, acetabular index, femoral neck-shaft angle, pelvic obliquity, and the presence of scoliosis. Clinical evaluation included muscle strength examination, periarticular contractures, necessity for using orthoses as walking aids, and Hoffer criteria of mobility. All complications were also noted from a chart review., Results: There were no differences between the two groups regarding postoperative femoral head localization, reflecting the presence or absence of residual subluxation, according to Reimer's index (Reimer's index = 32%; range, 10%-40%, in the muscle transfer group compared with 27%; range, 15%-43%, in the no-transfer group at latest followup; p = 0.723). Postoperatively, abductor strength improved in the group treated with external oblique transfer compared with the group treated without muscle transfer (median improvement of 2 versus 0, p = 0.02), but this improvement neither resulted in a clinically important difference in Hoffer criteria of mobility (no change was detected in either group) nor decreased the need for use of an orthosis (no change was detected in either group). With the numbers available, there was no difference with respect to complications between the two groups (two complications versus one)., Conclusions: External oblique abdominal muscle transfer did not provide a clinically important improvement in functional recovery when compared with patients with L3 to L5 myelomeningocele and hip instability who were treated without it. We therefore do not recommend a routine muscle transfer procedure during the operative management of hip instability in patients with lower lumbar level myelomeningocele., Level of Evidence: Level III, therapeutic study.

Medial malleolar screw hemiepiphysiodesis has been shown to be an effective procedure for the treatment of ankle valgus in children with spina bifida. The purpose of this study was to highlight the guided growth technique and discuss its efficacy. We retrospectively reviewed 10 spina bifida patients with 18 ankle valgus deformities treated with medial malleolar screw hemiepiphysiodesis. Medical reports were obtained to document age at surgery, gender, concurrent operative procedures and postoperative early and late complications. Anteroposterior and lateral weight bearing radiographs of both ankles were evaluated using the tibiotalar angle, pre and postoperatively. The average age at operation was 10.05 years. Average follow up was 15.33 months (11-21 months). During follow-up, the average preoperative distal tibiotalar angle of 16.27 degrees improved to an average of 2.88 degrees with statistical significance (p < 0.05). No major complications were detected. In conclusion, hemiepiphysiodesis is a safe and sufficient method to correct ankle valgus deformity in children with spina bifida, with a low incidence.

The principles of treatment of developmental dislocation of the hip (DDH) in an older child are different from those in a newborn. In the older child retraction of the muscles around the hip, associated with a marked acetabular dysplasia and elongation of the joint capsule explain the difficulty and instability of reduction and the frequency of complications. The aim of this study is to evaluate the effect of age on the results of a one-stage open reduction in developmental dislocation of the hip. We present the results of 21 children (27 hips) with a mean age of 6 years and 10 months at time of operation, consisting of one- stage open reduction, femoral shortening and Salter or triple pelvic osteotomy. Two groups were formed : one older and one younger than 8 years of age. We evaluated the patients according to the Severin and Mc Kay classifications. After an average follow-up of 49 months (24-84 months) 74% of patients showed a very good or good result according to Severin's radiological classification and 63% according to Mc Kay's clinical classification. When analyzed with regard to age, we found important differences in the clinical and radiological results between the children younger and older than 8 years.