Minimally Displaced Lateral Humeral Condyle Fractures: Optimizing Follow-up and Minimizing Cost.

BACKGROUND: Minimally displaced fractures of the lateral humeral condyle (LHC) may be treated nonoperatively in a long arm cast, but there is not a standardized evidence-based protocol. The aim of this study was to evaluate our nonoperatively treated LHC fractures, determine the risk of subsequent displacement, analyze our practice variability, and develop an evidence-based protocol to safely manage children with LHC fractures and to evaluate potential savings related to this analysis. METHODS: We retrospectively reviewed clinical and radiographic parameters of all patients with LHC fractures at our institution from 2009 to 2015. All patients treated nonoperatively initially were included. We recorded demographic data and calculated the number of visits, casts applied, and radiographs within the first 4 weeks. We also analyzed practice variation among 27 treating providers. The number of children with subsequent displacement needing operative fixation was determined. We also looked at the average duration for each follow-up visit and the charges/costs associated with casting and radiographs. RESULTS: There were 271 children with LHC fractures treated nonoperatively. Twenty-one were excluded because of the lack of adequate radiographs. There were 157 boys and 93 girls (average age 6.7 y). According to the Jakob classification, fracture types were as follows: 1 (230), 2 (20), and 3 (0). Within the first 4 weeks, the average number of visits was 2.6 (range: 1 to 5), average number of casts was 2.4 (range: 1 to 4), and the average number of radiographs was 9.4 (range: 2 to 31). Only 3 patients with LHC fracture type Jakob 1 were taken to the operating room post injury (9, 12, and 15 d, respectively) for subsequent displacement. CONCLUSION: Displacement in appropriately selected LHC fractures treated nonoperatively was rare (3/250, 1.2%) in this cohort, and the data question the need for multiple visits and radiographs in the first 4 weeks. Optimal follow-up (proposed follow-up at 10 to 15 d after injury and then 4 to 6 wk with radiographs, including an internal oblique view) would be safe, minimize waste, and result in better value-based care. LEVEL OF EVIDENCE: Level IV.

The Fate of the Bent Rod in Children With Osteogenesis Imperfecta.

BACKGROUND: Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones and long bone deformity. Realignment and intramedullary rodding with telescopic rods are indicated for progressive deformity and can help prevent fractures. Rod bending is a reported complication of telescopic rods and a common indication for revision; however, the fate of bent lower extremity telescopic rods in the setting of OI has not been reported. METHODS: Patients with OI at a single institution who underwent lower extremity telescopic rod placement with at least 1-year follow-up were identified. Bent rods were identified, and for these bone segments, we collected the location and angle of bend, subsequent telescoping, refracture, increasing angulation of bend, and date of revision. RESULTS: One hundred sixty-eight telescopic rods in 43 patients were identified. Forty-six rods (27.4%) bent during follow-up, with an average angulation of 7.3 (range: 1 to 24) degrees. In patients with severe OI, 15.7% of rods bent compared with 35.7% in nonsevere OI ( P =0.003). The proportion of bent rods was different between independent and nonindependent ambulators (34.1% and 20.5%; P =0.035). Twenty-seven bent rods (58.7%) were revised, with 12 rods (26.0%) revised early (within 90 d). The angulation of rods that were revised early was significantly higher than rods not (14.6 and 4.3 degrees, P <0.001). Of the 34 bent rods not revised early, the average time to revision or final follow-up was 29.1 months. Twenty-five rods (73.5%) continued to telescope, 14 (41.2%) increased in angulation (average 3.2 degrees), and 10 bones (29.4%) refractured. None of the refractures required immediate rod revision. Two bones had multiple refractures. CONCLUSIONS: Bending is a common complication of telescopic rods in the lower extremities of patients with OI. It is more common in independent ambulators and patients with nonsevere OI, possibly because of the increased demand placed on the rods. Rods with a small bend and maintained fixation can telescope and need not be an indication for immediate revision. LEVEL OF EVIDENCE: Level III-Retrospective review.

Neuromonitoring for Proximal Fibular Osteochondroma Excision.

BACKGROUND: The peroneal nerve is at risk when excising tumors in the proximal fibula. The rate of nerve injuries during proximal fibular tumor resection varies from 3% to 20%. Our goal was to report our experience with resection of osteochondromas in the proximal fibula and describe the technique and utility of neuromonitoring during excision of proximal fibular osteochondromas (PFO). METHODS: Patients with a diagnosis of symptomatic PFO who had undergone excision at one institution from 1994 to 2018 were included. An institutional review board-approved retrospective review was performed. Intraoperative neuromonitoring was provided from 2006 on by a single group utilizing a multimodality protocol. RESULTS: This study contains 29 patients who had excision of osteochondromas in the proximal fibula. Of these 29 consecutively monitored patients, there were 34 involved extremities. Intraoperative neuromonitoring alerts occurred in 10/29 (34.5%) procedures, which included 3 electromyography (EMG) (30%), 2 motor-evoked potential (20%), 1 somatosensory-evoked potential (10%), and 4 alerts with a combination of EMG/motor-evoked potential/somatosensory-evoked potential changes (40%). The interventions that were taken resulted in resolution of the neuromonitoring changes in all procedures. Postoperatively, we noted 2 (6.9%) new mild sensory deficits, which resolved during follow up. There were 3 patients in whom pre-existing sensory-motor deficits improved but not completely after surgery, 1 motor weakness, and 2 with residual paresthesia. In those initially presenting with paresis, there was improvement in 8 of the 8 extremities by the last follow-up visit. Pain as a symptom was resolved in all cases. There were no iatrogenic foot drop injuries. The average follow up was 32.2 months. CONCLUSIONS: Neuromonitoring during PFO excision demonstrated a high number of alerts, all of which resolved following timely corrective action. The use of neuromonitoring may help decrease the risk of iatrogenic postoperative neurological deficits following fibular osteochondroma surgery. LEVEL OF EVIDENCE: Level IV.

Risk Factors for Rebound After Correction of Genu Valgum in Skeletal Dysplasia Patients Treated by Tension Band Plates.

BACKGROUND: Growth modulation using tension band plates (TBPs) is increasingly important for lower limb deformity correction in patients with skeletal dysplasia (SKD). Development of rebound deformity is a concern after TBP removal. Data regarding this complication are rare; therefore, we evaluated the prevalence and risk factors for rebound deformity in children with SKD undergoing correction of genu valgum using TBP. METHODS: All patients with SKD with genu valgum treated by TBP at the distal femur or/and proximal tibia at a single center were reviewed. Inclusion criteria were: (1) minimum 2-year follow-up after TBP removal or having revision surgery for rebound deformity and (2) implant removal age for girls 14 years and below and boys 16 years and below. Exclusion criteria were any femoral/tibial osteotomies during TBP treatment or follow-up. A change of ≥3 degrees of mechanical lateral distal femoral and/or medial proximal tibial angle was accepted as rebound deformity and analyzed statistically. RESULTS: Thirty-three patients (59 limbs; 52 femur and 29 tibia physes) met our criteria. Mean follow-up after implant removal was 43.7 months. Rebound deformities were seen in 43 limbs (39 femurs and 13 tibias). Boys had more rebound than girls; however, this was not influenced by body mass index. Femurs had more rebound than tibias. Patients in the rebound group were younger than the nonrebound group. Time from application to removal of TBP was shorter in the rebound versus nonrebound group. Overcorrected limbs had more rebound deformity than not overcorrected. The difference in growth velocity of lower limbs in the rebound versus nonrebound group was statistically significant. Patients with epiphyseal dysplasia had more rebound than metaphyseal dysplasia, but this was not statistically significant. CONCLUSION: Risk factors for developing a rebound deformity after correction of genu valgum using TBP in SKD included male sex, TBP surgery at a young age, short duration of TBP implantation, overcorrected extremity (mechanical axis deviation ≤1), and high percent growth velocity after TBP removal. LEVEL OF EVIDENCE: Level IV-retrospective study.

Pediatric Tumor Management: Current and Controversial.

Bone and soft-tissue tumors are common in the pediatric population. It is important to be familiar with the appropriate workup, principles of biopsy, differences between unicameral and aneurysmal bone cysts, and principles of managing pathologic fractures in children. The management approach to pediatric soft-tissue masses and some of the recent advances in the field warrant discussion.

Risk Factors for Failure of Pavlik Harness Treatment in Infants With Dislocated Hips That Are Evaluated by Dynamic Sonography.

BACKGROUND: Frankly dislocated hips occur in ∼1% to 3% of infants with developmental dysplasia of the hip and are often difficult to treat. In the most severely dislocated hips, the femoral head is positioned outside the posterior/lateral rim of the acetabulum and is irreducible, that is, the femoral head will not reduce by positioning the leg. The purpose of this study was to determine risk factors, using univariate and multivariate analyses, for Pavlik harness failure in infants who initially presented with irreducible/dislocated hips (confirmed by dynamic sonography). METHODS: Following institutional review board approval, 124 infants (170 hips) with frankly dislocated hips treated using a Pavlik harness between 2000 and 2018 were evaluated. Patients' demographic characteristics, clinical findings, dynamic sonographic findings (dislocated-fixed vs. dislocated-mobile), age at onset of Pavlik harness treatment, duration of harness usage, and follow-up treatments were recorded. Univariate analyses were used to determine risk factors for treatment failure. RESULTS: In frankly dislocated hips (confirmed by dynamic sonography to be positioned outside the posterior/lateral rim of the acetabulum), Pavlik harness treatment was successful in 104 of 170 hips (61%) while it failed in 66 hips. Mean follow-up was 4.86±4.20 years. Univariate analysis determined the risk factors to be onset of treatment after the seventh week of age (P=0.049) and initial mobility (dislocated-fixed group) (P<0.001) by dynamic sonography. In addition, multivariate analysis (P=0.007) showed infants of multigravida mothers (non-firstborn) to be another risk factor for failure. Six percent of hips with no risk factors failed Pavlik harness treatment, those with 1 risk factor had 42% failure, 2 risk factors had 69% failure, and all 3 risk factors had 100% failure. CONCLUSIONS: In our patients with frankly dislocated irreducible hips, 39% of hip failed Pavlik harness treatment. Independent multivariate, logistic regression analysis, and multivariate analysis determining the risk factors for failure of Pavlik harness treatment were onset of treatment after the seventh week of age, infants of multigravida mothers, and initial hip mobility (fixed-dislocated hips) by dynamic sonography. LEVEL OF EVIDENCE: Level III.

Pediatric Chondroblastoma and the Need for Lung Staging at Presentation.

PURPOSE: Chondroblastoma is a benign, but potentially locally aggressive, bone tumor with predilection for the epiphysis of long bones in growing children. Historically, there is a reported 2% risk of lung metastasis, however these cases are mostly in the form of isolated single reports and the vast majority in adults. The purpose of this study was to identify the "true" risk of lung metastases at presentation in skeletally immature patients with a benign chondroblastoma, and therefore revisit the need for routine chest staging. METHODS: This was a multi-institution, international retrospective study of children and adolescents diagnosed and treated for a benign chondroblastoma. We focused on the screening and diagnosis of lung metastasis, type of staging utilized and the incidence of local recurrence. Detailed review of the available literature was also performed for comparison. RESULTS: The final studied cohort included 130 children with an average age of 14.5 years (range: 6 to 18 y). There were 94 boys and 36 girls. Lesions more often involved the proximal humerus (32/130), proximal tibia (30/130), and proximal femur (28/130). At an average follow-up of 50 months, there were 15 local recurrences (11% rate) and no cases of lung metastasis. All patients underwent chest imaging at presentation. The overall reported lung metastases rate in the pulled literature review (larger series only) was 0.4% (7/1625), all patients were skeletally mature. CONCLUSIONS: This is the largest cohort of pediatric-exclusive chondroblastoma in the literature. Despite minor differences in management between the centers included, the recurrence rate was similar and there was no evidence of lung metastasis (0 in 130). The incidence of distant involvement in a true benign chondroblastoma in children is much lower than the 2% previously reported in the literature, and the need for routine chest staging should be revisited. LEVEL OF EVIDENCE: Level III.

Validation of Pediatric Self-Report Patient-Reported Outcomes Measurement Information System (PROMIS) Measures in Different Stages of Legg-Calvé-Perthes Disease.

OBJECTIVES: Patient-reported outcomes (PRO) assessing health-related quality of life (HRQoL) are important outcome measures, especially in Legg-Calvé-Perthes disease (LCPD) where symptoms (pain and limping), activity restrictions, and treatments vary depending on the stage of the disease. The purpose of this study was to investigate the validity of the Patient-reported Outcomes Measurement Information System (PROMIS) for measuring HRQoL of patients with LCPD in various stages of the disease. METHODS: This is a multicenter validity study. Patients with LCPD between 4 and 18 years old were included and classified into modified Waldenström stages of disease: Early (1 or 2A), Late (2B or 3), or Healed (4). Seven PROMIS domains were collected, including Pain Interference, Fatigue, Mobility, Depression, Anger, Anxiety, and Peer Relationships. Convergent, discriminant, and known group validity was determined. RESULTS: A total of 190 patients were included (mean age: 10.4±3.1 y). All 7 domains showed the worst scores in patients in the Early stage (known group validity). Within each domain, all domains positively correlated to each other (convergent validity). Patients who reported more anxiety, depression, and anger were associated with decreased mobility and increased fatigue and pain. Peer relationships had no to weak associations with other domains (discriminant validity). CONCLUSIONS: PROMIS has construct validity in measuring the HRQoL of patients in different stages of LCPD, suggesting that PROMIS has potential to serve as a patient-reported outcome tool for this population. LEVEL OF EVIDENCE: Diagnostic level III study.

Infantile Tibia Vara (Blount Disease) with Iatrogenic Changes Causing Residual Tibial Deformities.

Treatment of infantile tibia vara or Blount disease (ITV/BD) in patients < 3 years old and Langenskiold stages I-III consists of orthosis and, in relapsing cases, proximal tibial osteotomy and/or proximal tibial guided growth laterally with a tension band plate. Our aim was to evaluate the results of treatments in a consecutive group. After Institutional Review Board approval, data from 2002 to 2018 were collected. Thirty-nine knees (average age 22.4 months) with ITV/BD were treated with orthoses, and 10 knees failed. Six knees showed hyperintense T2-weighted signal in the medial proximal tibial epiphyseal cartilage on magnetic resonance imaging. Three of six knees with tibial osteotomy failed and underwent guided growth. Tibial plateau slopes were abnormal medially from the ITV/BD and laterally from the guided growth (triangular physis and depressed plateau deformities) because of factors such as orthotic treatment, tibial osteotomy, magnetic resonance imaging "physis severity score," and guided growth. (Journal of Surgical Orthopaedic Advances 29(3):141-148, 2020).

Costello syndrome: Clinical phenotype, genotype, and management guidelines.

Costello syndrome (CS) is a RASopathy caused by activating germline mutations in HRAS. Due to ubiquitous HRAS gene expression, CS affects multiple organ systems and individuals are predisposed to cancer. Individuals with CS may have distinctive craniofacial features, cardiac anomalies, growth and developmental delays, as well as dermatological, orthopedic, ocular, and neurological issues; however, considerable overlap with other RASopathies exists. Medical evaluation requires an understanding of the multifaceted phenotype. Subspecialists may have limited experience in caring for these individuals because of the rarity of CS. Furthermore, the phenotypic presentation may vary with the underlying genotype. These guidelines were developed by an interdisciplinary team of experts in order to encourage timely health care practices and provide medical management guidelines for the primary and specialty care provider, as well as for the families and affected individuals across their lifespan. These guidelines are based on expert opinion and do not represent evidence-based guidelines due to the lack of data for this rare condition.

Magnetic Resonance Imaging in Symptomatic Children With Hereditary Multiple Exostoses of the Hip.

BACKGROUND: Magnetic resonance imaging (MRI) is useful in evaluating nontraumatic hip pain. It provides information about associated injuries like labral/chondral tears or ischiofemoral impingement (IFI). However, in hereditary multiple exostoses (HME) there has been no report about MRI findings in symptomatic children with hip involvement. METHODS: Records of children with HME and hip osteochondromas, who had hip MRI/magnetic resonance arthrography, were reviewed. The presence of chondral lesions and labral tears, as well the presence of IFI, was recorded. IFI was defined as edema or fatty replacement/atrophy in the quadratus femoris muscle or decrease of the space for this muscle between the ischium and the proximal femur. The measurements used to determine the space included the ischiofemoral space, the quadratus femoris space, and the minimum ischiofemoral space (MIFS). All measurements were performed on axial T1-weighted images. RESULTS: Ten children were included (4 males, 6 females). In 2 patients, MRI was unilateral, therefore a total of 18 hips were analyzed. The indication for MRI was hip pain. Mean age, when MRI was performed, was 11.7 years. Labral tears were found in 44% (8/18) and chondral lesions in 33% (6/18) of the hips. The mean ischiofemoral space was 17.2 mm (SD, 7.3), the mean quadratus femoris space was 14.9 mm (SD, 5.3), and the mean MIFS was of 12.8 mm (SD, 5.9). IFI was seen in 44% (8/18) of hips. Two patients had bilateral IFI. MIFS was <10 mm in all hips with IFI (8/8). Of these hips, 88% (7/8) had edema of the quadratus femoris muscle and 38% (3/8) had fatty replacement/atrophy in the muscle. Osteochondromas were seen in the lesser trochanter in all hips with IFI (8/8) and in the ischium in 50% of them (4/8). CONCLUSIONS: In symptomatic children with HME of the hip, MRI is helpful in detecting the source of pain. A high percentage of these children have IFI and intra-articular lesions. These findings can play an important role in the indication and planning of the surgical approach. LEVEL OF EVIDENCE: Level IV-diagnostic study.

Radiographic Analysis of the Pediatric Hip Patients With Hereditary Multiple Exostoses (HME).

BACKGROUND: This study aimed to report the radiographic presentation of involved hips in children with hereditary multiple exostoses (HME). This included radiographic hip measurements, osteochondromas location, and relationship with hip subluxation. METHODS: Anteroposterior pelvis radiographs of children with HME, seen between 2003 and 2014, were retrospectively reviewed. Only patients who were skeletally immature at the first visit were included. One radiograph per patient per year was reviewed. Radiographs were examined for the presence of osteochondromas and their locations. Different parameters were evaluated: femoral neck-shaft angle, Reimer migration percentage (MP), Sharp acetabular angle, Wiberg angle, femoral head-neck ratio (coronal plane), and Shenton line. All measured radiographs were divided into 3 age groups:≤8,>8 and <13, and ≥13 years. Differences of the measured parameters with age were evaluated. Children with hip subluxation were identified and any relationship with osteochondromas locations, as well as MP changes over time, was recorded. Radiographs of children with a minimum 2-year follow-up were identified and changes of their hip measurements and osteochondromas' presence over time were recorded. RESULTS: A total of 51 children (102 hips) with HME were identified. In most locations, there was an overall increase of the occurrence of osteochondromas in the older age groups. However, in the medial femoral neck, a significantly less numbers of osteochondromas were found after 13 years of age (P=0.018). There was a decrease in MP with age (P<0.05). There was also an increase in Sharp and Wiberg angles in the older patients (P<0.05). Hips with broken Shenton line decreased in number with age (P 0.028). Hip subluxation was encountered in 23 hips. No specific location of osteochondromas was found to have a relationship with subluxation. Thirty-six children had a minimum follow-up of 2 years (mean age at first visit 8.5 y and at last visit 13.1 y). In these children, an increased occurrence of lesions was found in medial femoral neck and ischium (P<0.05) between the first and the last visit. CONCLUSIONS: In children with HME, radiographic evaluation of the hip is necessary based on the high percentage of hip involvement. When hip osteochondromas are found, radiographic surveillance is recommended to detect hip subluxation. Surgery may certainly be necessary for symptomatic osteochondromas. However, given the possibility of improvement in hip parameters with age, early surgical treatment to improve hip longevity does not seem to be warranted. LEVEL OF EVIDENCE: Level IV-prognostic study.

Aneurysmal bone cysts and pathologic fracture associated with supernumerary ring chromosome 6 in two unrelated patients.

Small supernumerary ring chromosome 6 (sSRC[6]) is a rare chromosomal abnormality characterized by a broad clinical phenotype. The spectrum of this disorder can range from phenotypically normal to severe developmental delay and congenital anomalies. We describe two unrelated patients with small SRCs derived from chromosome 6 with a novel bone phenotype. Both patients presented with a complex bone disorder characterized by severe osteopenia, pathologic fractures, and cyst-like lesions within the bone. Imaging revealed decreased bone mineral density, mutiple multiloculated cysts and cortical thinning. Lesion pathology in both patients demonstrated a bland cyst wall with woven dysplastic appearing bone entrapped within it. In patient 1, array comparative genomic hybridization (CGH) detected a tandem duplication of region 6p12.3 to 6q12 per marker chromosome. Cytogenetic analysis further revealed a complex patient of mosaicism with some cell lines displaying either one or two copies of the marker indicative of both tetrasomy and hexasomy of this region. Patient 2 was mosaic for a sSRC that encompassed a 26.8 Mb gain from 6p21.2 to 6q12. We performed an in-depth clinical analysis of a phenotype not previously observed in sSRC(6) patients and discuss the potential influence of genes located within this region on the skeletal presentation observed.

Comparing Percutaneous Physeal Epiphysiodesis and Eight-Plate Epiphysiodesis for the Treatment of Limb Length Discrepancy.

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.

Unicameral Bone Cysts in the Humerus: Treatment Outcomes.

BACKGROUND: Several treatment modalities have been described for the treatment of unicameral bone cysts (UBC). The aim of this study was to examine the outcome of various treatment modalities of UBC in a specific anatomic location, the humerus. METHODS: This study is a retrospective case-only study of patients with humeral UBC with minimum follow-up of 1 year. Medical records and radiographs were assessed and UBC healing status was determined based on most recent follow-up radiographs and divided into 3 groups (healed, partially healed, and not healed). Descriptive statistics were utilized to summarize study outcome. RESULTS: Sixty-eight patients (54 boys and 14 girls) with humeral UBC comprised the study population. Sixty-four cases (94.1%) presented with a pathologic fracture. Fifty-one cases were in the proximal metaphysis and 17 were in the diaphysis. Mean age at diagnosis was 9.2±3.7 years, and mean follow-up was 4.0±2.6 years. Twenty-five patients were treated with observation, 38 by injection (27 with steroids and 11 with bone marrow), and 5 by open surgery. Patients who underwent open surgery had relatively larger cyst length, width, and cyst index, and all healed or partially healed at last follow-up. UBC persistence was observed in 29% of diaphyseal and 27.5% of metaphyseal cysts. Of the 19 patients with persistence, 8 were treated with observation, 9 with steroid injection, and 2 with bone marrow injection. Inner wall disruption before injection was performed in 17 patients (24% did not heal), whereas 21 patients did not have inner wall disruption (33% did not heal). Twenty patients received >1 injection. Eleven patients complained of pain at the last visit (8 had a persistent cyst, 2 were partially healed, and 1 had a healed UBC). CONCLUSIONS: Complete healing of humeral UBC is challenging to achieve irrespective of treatment modality. UBCs treated with open surgery tended to heal better. Unhealed cysts were more likely to be associated with pain. LEVEL OF EVIDENCE: Level III-a retrospective comparative study.

Inter-observer reliability of the ankle-brachial index in a pediatric setting.

OBJECTIVE: The aim of our study was to determine the inter-observer reliability of the ankle-brachial index (ABI) in the pediatric population. This was done to determine if this simple diagnostic test could be utilized in lieu of CT angiograms to reduce radiation exposure to children sustaining trauma, particularly physeal fractures about the knee. DESIGN: Diagnostic study of consecutive patients with no applied "gold-standard" test. SETTING: Level I pediatric hospital. PATIENTS/PARTICIPANTS: Thirty consecutive patients from a busy fracture clinic were recruited. Patients ranged in age from 7-17 years. INTERVENTION: Manual systolic blood pressure measurements from uninjured limbs were taken by two independent attending orthopaedic surgeons from the ipsilateral brachial, dorsalis pedis, and posterior tibial arteries of each patient. MAIN OUTCOME MEASUREMENTS: The intraclass correlation coefficients from each anatomic area were calculated. RESULTS: Intraclass correlation of measurements from all three anatomic locations showed good inter-observer reliability. The intraclass correlation coefficients from the brachial, dorsalis pedis, and posterior tibial arteries were 0.699, 0.815, and 0.740, respectively. Therefore, the calculation of the ABI was consistent between the two observers despite any variability in individual pressure measurements. CONCLUSIONS: The ABI is a fast, non-invasive test that can be applied to the pediatric population in the evaluation of lower extremity arterial injury associated with orthopaedic trauma. It can obviate the need for tests such as CT angiograms that have inherent risks, particularly those associated with radiation exposure that are of concern in a young child.

Correction of lower extremity angular deformities in skeletal dysplasia with hemiepiphysiodesis: a preliminary report.

BACKGROUND: Lower extremity angular deformities are common in children with skeletal dysplasia and can be treated with various surgical options. Both acute correction by osteotomy with internal fixation and gradual correction by external fixation have been used with acceptable results. Recently, the Guided Growth concept using temporary hemiepiphysiodesis for correction of angular deformities in the growing child has been proposed. This study presents the results of temporary hemiepiphysiodesis using eight-Plates and medial malleolus transphyseal screws in children with skeletal dysplasia with lower extremity angular deformities. METHODS: Twenty-nine patients (50 lower extremities) with skeletal dysplasia of different types were treated for varus or valgus deformities at 2 centers. The mean age at the time of hemiepiphysiodesis was 10±2.9 years. A total of 66 eight-Plates and 12 medial malleolus screws were used. The average follow-up time between the index surgery and the latest follow-up with the eight-Plate in was 25±13.4 months. Erect long-standing anteroposterior and lateral view radiographs were obtained for deformity planning before the procedure. Angular deformities on radiograph were evaluated by mechanical axis deviation, mechanical lateral distal femoral angle, medial proximal tibial angle, and lateral distal tibial angle. Mechanical axis deviation was also expressed as a percentage to one half of the width of the tibial plateau, and the magnitude of the deformity was classified by determining the zones through which the mechanical axis of the lower extremity passed. Four zones were determined on both the medial and lateral side of the knee and the zones were labeled 1, 2, 3, and 4, corresponding to the severity of the deformity. A positive value was assigned for valgus alignment and a negative for varus alignment. RESULTS: Patients were analyzed in valgus and varus groups. There was correction in 34 of 38 valgus legs and 7 of 12 varus legs. In the valgus group, the mean preoperative and postoperative mechanical lateral distal femoral angles were 82.1±3.7 and 91.1±4.9 degrees, respectively (P<0.001). The mean preoperative and postoperative medial proximal tibial angles were 98.5±8 and 87.8±7.1 degrees, respectively (P<0.001). Six patients with bilateral ankle valgus deformities (12 ankles) underwent single-screw medial malleolus hemiepiphysiodesis. The mean preoperative and postoperative lateral distal tibial angles were 73.9±8.7 and 86.1±6.8 degrees, respectively (P<0.001). The numbers of plates in each anatomic location were not enough to make statistical conclusions in varus legs. Four patients in the valgus group and 3 patients in the varus group did not benefit from the procedure. Mechanical axes were in zone 2 or over in 94% of the legs preoperatively, whereas postoperatively, only 23% of the legs had mechanical axes in zone 2 or over in varus and valgus groups. CONCLUSIONS: Growth modulation with an eight-Plate is a relatively simple surgery and has low risk of mechanical failure or physeal damage. It can be performed in very young patients, which is an important advantage in skeletal dysplasia. Screw purchase is reliable even in the abnormal epiphysis and metaphysis. Our results show that Guided Growth using eight-Plates in skeletal dysplasia is safe and effective. LEVEL OF EVIDENCE: Level IV.

Cervicothoracic myelopathy in children with Morquio syndrome A: a report of 4 cases.

BACKGROUND: Craniovertebral junction anomalies and C1-C2 instability resulting in myelopathy have been well described in the literature on mucopolysaccharidosis IV (MPS-IV). Spinal involvement in MPS-IV patients, with neurological impairment, other than atlanto-axial instability and thoracolumbar kyphosis, has been scarcely mentioned in the literature. METHODS: Retrospective clinical and radiologic review of the medical records and imaging studies of 4 individuals with Morquio A syndrome, who had undergone decompression and fusion of the cervicothoracic spine for myelopathy secondary to cervicothoracic stenosis between 1990 and 2009. Data regarding the presence of kyphosis at the cervicothoracic and upper thoracic spine, and neurological symptoms and signs were obtained. RESULTS: There were 3 girls and 1 boy with an average age of 5 years and 11 months at presentation with neurological symptoms. Half of the patients had previously undergone occipitocervical fusion for atlanto-axial instability, whereas the other half were noted to have spinal cord compression at both the upper cervical and cervicothoracic regions, and underwent decompression and fusion at both levels concomitantly. All patients showed postoperative neurological improvement. All patients presented with the classical Morquio syndrome vertebral morphology. Cervicothoracic kyphosis was found in all of our patients in a varying severity (10 to 35 degrees). Levels of stenosis were similar in 3 patients, C7-T2; and occurred at a lower spinal level, T1-T4, in the remaining patient. Posterior disk bulging and thecal sac indentation were found in all 4 patients. CONCLUSIONS: Neurological problems secondary to progressive kyphosis and stenosis at the cervicothoracic and upper thoracic spine are seen in children with Morquio syndrome. Early detection with a careful neurological assessment, whole spine MR imaging, and appropriate surgical treatment can prevent permanent neurological sequelae.

Morquio A syndrome: diagnosis and current and future therapies.

Morquio A syndrome is an autosomal recessive disorder, one of 50 lysosomal storage diseases (LSDs), and is caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Deficiency of this enzyme causes specific glycosaminoglycan (GAG) accumulation: keratan sulfate (KS) and chondroitin-6-sulfate (C6S). The majority of KS is produced in the cartilage, therefore, the undegraded substrates accumulate mainly in cartilage and in its extracelluar matrix (ECM), causing direct leads to direct impact on cartilage and bone development and leading to the resultant systemic skeletal spondyloepiphyseal dysplasia. Chondrogenesis ,the earliest phase of skeletal formation that leads to cartilage and bone formation is controlled by cellular interactions with the ECM, growth and differentiation factors and other molecules that affect signaling pathways and transcription factors in a temporal-spatial manner. In Morquio A patients, in early childhood or even at birth, the cartilage is disrupted presumably as a result of abnormal chondrogenesis and/ or endochondral ossification. The unique clinical features are characterized by a marked short stature, odontoid hypoplasia, protrusion of the chest, kyphoscoliosis, platyspondyly, coxa valga, abnormal gait, and laxity of joints. In spite of many descriptions of the unique clinical manifestations, diagnosis delay still occurs. The pathogenesis of systemic skeletal dysplasia in Morquio A syndrome remains an enigmatic challenge. In this review article, screening, diagnosis, pathogenesis and current and future therapies of Morquio A are discussed.

Failure of Pavlik Harness Treatment in Infants Under 6 Months Old with Dislocated Hips: Short- and Intermediate-Term Results of Subsequent Treatment Modalities.

Objective: This study aimed to determine the short- and medium-term outcomes of hip dislocation in infants who failed Pavlik harness therapy and were subsequently treated with brace, closed reduction (CR) or open reduction (OR) before 6 months of age. Methods: Fifty infants (66 hip dislocations) who failed Pavlik harness therapy between 2000 and 2018 and were treated with a rigid abduction brace or undergoing a CR or OR/cast were evaluated. All demographic data obtained from the medical system, developments and complications during the follow-up and treatment process were recorded and evaluated. Results: Fifty infants (66 hips) with dislocated hips failed Pavlik harness therapy. Of these, 9 infants (12 hips) underwent rigid abduction splint therapy: 9 hips were successful, 2 hips had CR and 1 had OR. Thirty-eight infants (51 hips) had index CR, of which 3 (3 hips) failed and had OR. Radiographs of 49 hips (44 patients) were normal at the final evaluation. Pavlik harness therapy starting after 3 weeks (P = 0.028) and unilateral dislocations (P = 0.028) increased the risk of needing operating room. There was an association between OR and avascular necrosis (P = 0.025), but not between OR and other complications-dysplasia and re-dislocation/subluxation (P = 0.257 and P = 0.508, respectively). Conclusion: Closed treatment of hip dislocation is possible in most babies who fail Pavlik treatment. Babies who are started on Pavlik therapy after 3 weeks of age may be at increased risk of needing an operating room. Level of Evidence: IV.