Diagnostic Criteria for the Painful Swollen Pediatric Knee: Distinguishing Septic Arthritis From Aseptic Effusion in a Non-Lyme Endemic Area.

Purpose: The child with a painful swollen knee must be worked-up for possible septic arthritis; the classic clinical prediction algorithms for septic arthritis of the hip may not be the best models to apply to the knee. Materials and methods: This was a retrospective case-control study of 17 years of children presenting to one hospital with a chief complaint of a painful swollen knee, to evaluate the appropriateness of applying a previously described clinical practice algorithm for the hip in differentiating between the septic and aseptic causes of the painful knee effusions. The diagnoses of true septic arthritis, presumed septic arthritis, and aseptic effusion were established, based upon the cultures of synovial fluid, blood cultures, synovial cell counts, and clinical course. Using a logistic regression model, the disease status was regressed on both the demographic and clinical variables. Results: In the study, 122 patients were included: 51 with true septic arthritis, 37 with presumed septic arthritis, and 34 with aseptic knee effusion. After applying a backward elimination, age <5 years and C-reactive protein (CRP) >2.0 mg/dl remained in the model, and predicted probabilities of having septic knee arthritis ranged from 15% for the lowest risk to 95% for the highest risk. Adding a knee aspiration including percent polymorphonucleocytes (%PMN) substantially improved the overall model performance, lowering the lowest risk to 11% while raising the highest risk to 96%. Conclusions: This predictive model suggests that the likelihood of pediatric septic arthritis of the knee is >90% when both "age <5 years" and "CRP > 2.0 mg/dl" are present in a child with a painful swollen knee, though, in the absence of these factors, the risk of septic arthritis remains over 15%. Aspiration of the knee for those patients would be the best next step.

Prevalence and Severity of Juvenile Osteochondritis Dissecans in Patients With Juvenile Idiopathic Arthritis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of chronic arthritides presenting in patients aged ≤16 years, with a prevalence of 16 to 150 per 100,000. Juvenile osteochondritis dissecans (OCD) is an idiopathic disease of articular cartilage and subchondral bone, has an onset age of 10 to 16 years, and often affects the knee, with a prevalence of 2 to 18 per 100,000. Currently, there are few studies that have evaluated the relationship between JIA and OCD. HYPOTHESIS: OCD is more prevalent in children with JIA, and when diagnosed in such patients, OCD often presents at an advanced state. STUDY DESIGN: Case series; Level of evidence, 4. METHODS: The medical records of patients with diagnoses of both JIA and OCD treated between January 2008 and March 2019 at a single children's hospital were retrospectively reviewed. Associations between timing of diagnoses, number and types of corticosteroid treatments, category of arthritis, timing of diagnoses, and lesion stability were examined with Spearman correlation coefficients. RESULTS: A total of 2021 patients with JIA were identified, 20 of whom (19 female, 1 male) had OCD of the knee and/or talus for a prevalence of 1 in 100 or 1000 in 100,000, or approximately 50 to 500 times that of the general population. These 20 patients had a total of 28 OCD lesions: 43% (9 femur, 3 talus) were radiographically stable over time, 50% (10 femur, 2 patella, 2 talus) were unstable at initial diagnosis, and 7% (2 femur) were initially stable but progressed to unstable lesions despite drilling. Twelve patients (60%) underwent surgery: 4 (20%) with stable femoral lesions for persistent symptoms despite prolonged nonoperative treatment and 8 (40%) for treatment of their unstable lesions (femoral and patellar). Within our study design, we could identify no significant associations between lesion stability and timing of diagnoses, number of joint injections, or limb deformities, nor were there associations between timing of JIA and OCD diagnoses and category of arthritis. CONCLUSION: In our population of patients with JIA, OCD lesions were found to be 50 to 500 times more prevalent when compared with published rates in the general population and often presented at an advanced state, with instability or delayed healing requiring surgery for stabilization or resolution of symptoms.

Low Preoperative Lung Functions in Children With Early Onset Scoliosis Predict Postoperative Length of Stay.

INTRODUCTION: Children with early onset scoliosis (EOS) undergoing spine surgery often have significant respiratory disease. Preoperative risk assessments that predict an increased length of hospital stay (LOS) for this group have not been previously evaluated. METHODS: A voluntary protocol using preoperative lung function studies began among participants of a multicenter registry in 2016. Preoperative assessments were standardized to include spirometry, blood hemoglobin levels, serum bicarbonate, albumin and prealbumin; radiographic parameters of the spine, C-EOS classification and need for preoperative pulmonary assistance before initial growth friendly device insertion or "definitive" spine fusion. Primary outcome was LOS postoperatively. Data, including age, diagnosis, and type of surgery, was collected prospectively. Secondary outcomes measured included intensive care unit LOS, requirement for new pulmonary assistance on discharge, and pulmonary complications. Groups were compared using the Fisher exact tests. RESULTS: Of 525 children enrolled, 101 (20%) had preoperative spirometry. Median age was 8.9 years [interquartile range (IQR): 4.27]. Etiologies for EOS included 29 neuromuscular (28%), 33 idiopathic (32%), 19 syndromic (19%), and 22 congenital (21%) scoliosis. Eighty (78%) had growing rod (GR) insertions; 23 (22%) had spine fusion SF. Eighteen subjects (17%) were hospitalized ≥7 days (median=9 d); 83 had a LOS <7 days (median=3 d). Percentage of forced vital capacity (FVC%) predicted was inversely associated with LOS ≥7 days with a median of 75.3% (IQR: 41.7) for LOS <7 days and 51.7% (IQR: 41.6) (P=0.02). There were no detectable differences in LOS for other preoperative values. CONCLUSION: FVC predicted ≤50% preoperatively in children undergoing initial growth friendly rod insertion or definitive fusion after growth friendly treatment is associated with an increased risk of postoperative hospital stays ≥7 days. As demonstrated in previous studies, severe restrictive lung disease (FVC% predicted at or below 50%) is associated with increased risk of poorer outcomes for EOS patients.

Best practice guidelines in managing the craniofacial aspects of skeletal dysplasia.

BACKGROUND: Recognition and appropriate management of the craniofacial manifestations of patients with skeletal dysplasia are challenging, due to the rarity of these conditions, and dearth of literature to support evidence-based clinical decision making. METHODS: Using the Delphi method, an international, multi-disciplinary group of individuals, with significant experience in the care of patients with skeletal dysplasia, convened to develop multi-disciplinary, best practice guidelines in the management of craniofacial aspects of these patients. RESULTS: After a comprehensive literature review, 23 initial statements were generated and critically discussed, with subsequent development of a list of 22 best practice guidelines after a second round voting. CONCLUSIONS: The guidelines are presented and discussed to provide context and assistance for clinicians in their decision making in this important and challenging component of care for patients with skeletal dysplasia, in order standardize care and improve outcomes.

Seasonal Variation in Parental Satisfaction With Pediatric Orthopaedics.

BACKGROUND: The degree of parental satisfaction with health care is determined by the family's characteristics and expectations. Many aspects of human physiology and behavior have seasonal rhythms. The purpose of the present study was to determine whether parental satisfaction scores vary across the year in a pediatric orthopaedic outpatient population. METHODS: We retrospectively reviewed a total of 22,951 parental satisfaction scores related to outpatient pediatric orthopaedic encounters between October 2015 and April 2019. Parental satisfaction was measured using the provider subdomain of the shortened version of the Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS). Families were stratified according to various pediatric orthopaedic subspecialty services. RESULTS: The satisfaction scores of all pediatric orthopaedic encounters combined did not vary significantly across the year (P=0.8745). When the families were stratified into groups by pediatric orthopaedic subspecialty clinic, variation in seasons was not associated with statistically significant variation in satisfaction scores for the elective surgery, sports medicine, trauma/fracture care, and hand/upper extremity services. However, satisfaction with the spine service was significantly lower in the winter compared with the summer and fall (73.9±3.8 vs. 83.5±5.3 and 82.6±3.6, respectively; P=0.0147). CONCLUSIONS: The provision of pediatric spine care in a region with 4 distinct seasons received lower parental satisfaction scores during the winter than during the summer and fall. This seasonal variation is an additional source of bias in the measurement of satisfaction with health care. LEVEL OF EVIDENCE: Level II-retrospective study.

Classifying vertebral artery anatomy abnormality in children with skeletal dysplasia.

PURPOSE: Skeletal dysplasia (SKD) have predictably abnormal occipitocervical skeletal anatomy, but a similar understanding of their vertebral artery anatomy is not known. Knowledge and classification of vertebral artery anatomy in SKD patients is important for safe surgical planning. We aimed to determine if predictably abnormal vertebral artery anatomy exists in pediatric SKD. METHODS: We performed a retrospective review of CTAs of the neck for pediatric patients at a single institution from 2006 to 2018. CTAs in SKD and controls were reviewed independently in blinded fashion by two radiologists who classified dominance, vessel curvature at C2, direction at C3, and presence of fenestration and intersegmental artery. RESULTS: 14 skeletal dysplasia patients were compared to 32 controls. The path of the vertebral artery at C2 foramen was no different between the cohorts or by side, right (p = 0.43) or left (p = 0.13), nor for medial or lateral exiting direction from C3 foramen on right (p = 0.82) or left (p = 0.60). Dominance was most commonly neutral in both groups (71% in SKD and 63% in controls). There were no fenestrated nor first intersegmental arteries in our cohort. CONCLUSION: No systematic differences were detected between SKD and control patients with respect to vertebral artery anatomy. Nonetheless, surgically relevant variability was observed in both groups. Paying particular attention to the direction of exit at C3 and curvature at C2 with respect to the foramen and vessel dominance are important and easily classifiable abnormalities that both surgeons and radiologists can use to communicate and employ in pre-operative planning. LEVEL OF EVIDENCE: III.

Best practice guidelines for management of spinal disorders in skeletal dysplasia.

BACKGROUND: Disorders of the spine present a common and difficult management concern in patients with skeletal dysplasia. Due to the rarity of these conditions however, the literature, largely consisting of small, single institution case series, is sparse in regard to well-designed studies to support clinical decision making in these situations. METHODS: Using the Delphi method, an international, multi-disciplinary group of individuals, with significant experience in the care of patients with skeletal dysplasia, convened to develop multi-disciplinary, "best practice" guidelines in the care of spinal disorders in patients with skeletal dysplasia. RESULTS: Starting with 33 statements, the group a developed a list of 31 "best practice" guidelines. CONCLUSIONS: The guidelines are presented and discussed to provide context for clinicians in their decision making in this often-challenging realm of care.

Comparison of Continuous Adductor Canal and Femoral Nerve Blocks for Analgesia and Return of Quadriceps Function After Anterior Cruciate Ligament Reconstruction in Adolescent Patients.

PURPOSE: To compare early pain relief and late quadriceps function after anterior cruciate ligament reconstruction (ACLR) with hamstring autograft in adolescent patients treated with either a continuous femoral nerve block (cFNB) or continuous adductor canal block (cACB). METHODS: We retrospectively reviewed a consecutive series of adolescent patients who underwent ACLR and received either a cACB or cFNB for postoperative pain management. Over a 1-year period, all patients underwent ACLR with cFNBs. Over the subsequent 9 months, all patients underwent their ACLR with cACBs. Patient demographics, postoperative pain scores, opioid consumption, satisfaction and complications, and dates and results of quadriceps function derived at the Return to Sports evaluation were compared. RESULTS: Ninety-one patients (53 cFNB, 38 cACB) were reviewed. There were no differences in the demographics of the 2 groups. There were no statistically significant differences between groups in variations in postoperative pain scores (P = .21), or satisfaction with the blocks (P = .93). Patients in the cFNB group consumed a greater number of opioid doses on postoperative day 3 (2.2 ± 2.1 doses cFNB, 1.1 ± 1.6 doses cACB, P = .03) and a greater number of opioid doses overall for postoperative days 1 to 3 (mean 6.8 ± 5.3 doses cFNB, 3.8 ± 2.1 doses cACB, P = .03). There was no difference in time to return of acceptable quadriceps strength and function when comparing the 2 groups (30.9 ± 7.7 weeks cFNB, 28.9 ± 6.6 weeks cACB, P = .087). CONCLUSIONS: We found few differences in postoperative analgesic requirements when comparing patients who underwent ACLR with hamstring autograft with a cACB to those who underwent a similar procedure with a cFNB. Return of quadriceps strength and function by six months did not appear to vary with regional technique, either cACB or cFNB, employed at surgery. LEVEL OF EVIDENCE: III, Retrospective comparative study.

Prospective spine at risk program for prevalence of intracanal spine lesions in pediatric hereditary multiple osteochondromas.

STUDY DESIGN: Retrospective cohort study OBJECTIVES: To determine prevalence of hereditary multiple osteochondromas (HMO) and utility of MRI surveillance in a prospective Spine at Risk (SAR) program. Unidentified intraspinal exostoses in HMO can lead to neurologic injury in children during sedated procedures but no MRI guidelines exist. We sought to determine the prevalence and age of intraspinal exostoses from MRIs, and indications for MRI surveillance. METHODS: Retrospective review was performed of pediatric HMO patients who underwent total spine MRIs at a single institution after a prospective SAR program was instituted. Charts were reviewed for MRI indication and findings, symptoms, surgery, and location of other exostoses. Fisher's exact test was used to compare categorical variables and T test to compare continuous variables. Predictive value of pelvic/rib exostoses was calculated for intraspinal lesions. RESULTS: Forty-three patients with HMO underwent total spine MRIs with average age of 11.5 years. Fifteen (35%) patients had exostoses on vertebral column, eight (19%) had intra-canal spinal exostoses. Higher prevalence of spine lesions occurred in symptomatic patients than asymptomatic (any spinal lesion: 73% prevalence in symptomatic vs 22% in asymptomatic, p < 0.005; intra-canal spinal lesion: 46% vs 9%, p < 0.05). Only two of the 11 'symptomatic presentations' could be attributable to intracanal spinal exostoses. Only one intra-canal exostosis found on asymptomatic surveillance was treated surgically. Presence of pelvic or rib exostoses were not strongly predictive of intra-canal lesions (23% PPV, 85% NPV, 63% sensitivity, 51% specificity). CONCLUSIONS: Even with the presence of intra-canal exostoses, true symptomatic lesions are rare. Rib and pelvic lesions were not predictive of intra-canal lesions in our population. We recommend obtaining MRIs at time of preoperative evaluation in asymptomatic children old enough to not need sedation, or in patients with true neurologic symptoms to prevent unnecessary sedation of younger children for surveillance MRI. LEVEL OF EVIDENCE: III.

The Utility of the Early Postoperative Follow-up and Radiographs After Operative Treatment of Supracondylar Humerus Fractures in Children.

BACKGROUND: Supracondylar humerus (SCH) fractures are common elbow injuries in pediatric patients. The American Academy of Orthopedic Surgeons published guidelines for the standard of care in the treatment of displaced SCH fractures, however, no recommendations for follow-up care were made. With the recent push to eliminate unnecessary radiographs and decrease health care costs, many are questioning postoperative protocols. The purpose of our study was to evaluate the utility of the 1-week follow-up appointment after closed reduction and percutaneous pinning (CRPP) of displaced SCH fractures. METHODS: A retrospective review performed at a single institution from 2014 to 2016 included patients under 14 years of age with a closed, displaced SCH fracture treated with CRPP. Exclusion criteria included patients without complete clinical or radiographic follow-up. Variables examined included time to initial follow-up, change in treatment plan after 1-week x-rays, complications, demographics, fracture type, pin number and configuration, reduction parameters, immobilization, time to pin removal, duration of casting, and clinical outcome. RESULTS: A total of 412 patients were divided into 2 groups based on time to initial follow-up. Overall, 368 had an initial follow-up at 7 to 10 days (group 1) and 44 at 21 to 28 days (group 2). There was no difference in age, sex, fracture type, pin configuration, or a number of pins between groups. Statistically significant findings included time to initial follow-up and days to pin removal (group 1 at 26.2 d vs. group 2 at 23.8 d), type of immobilization (group 1 with 5% circumferential casts and group 2 with 70%), and time to surgery (26.2 vs. 62.9 h, respectively). There was no significant difference in complication rates and only a 0.5% rate of change in management in group 1. CONCLUSIONS: Early postoperative follow-up and radiographs did not change the patient outcome and might be eliminated in children with displaced SCH fractures treated with CRPP. Given the current focus of on efficiency and cost-effective care, eliminating the 1-week postoperative appointment would improve appointment availability and decrease medical cost. LEVEL OF EVIDENCE: Level III-Therapeutic.

C-Reactive Protein Level at Time of Discharge Is Not Predictive of Risk of Reoperation or Readmission in Children With Septic Arthritis.

Purpose: C-reactive protein (CRP) level is used at our tertiary pediatric hospital in the diagnosis, management, and discharge evaluation of patients with septic arthritis. The purpose of this study was to evaluate the efficacy of a discharge criterion of CRP < 2.0 mg/dL for patients with septic arthritis in preventing reoperation and readmission. We also aimed to identify other risk factors of treatment failure. Methods: Patients diagnosed with septic arthritis between January 1, 2007 and December 31, 2017 were identified with ICD 9/10 and related CPT codes. Systematic chart reviews were performed to obtain demographic data, infection characteristics, and treatment details. Bivariate tests of associations between potential risk factors and readmission and reoperation were performed. Quantitative variables were analyzed using Mann-Whitney tests and categorical variables were analyzed using Chi-square tests. Results: One hundred and eighty-three children with septic arthritis were included in the study. Seven (3.8%) were readmitted after hospital discharge for further management, including six who required reoperation. Mean CRP at discharge for single-admission patients was 1.71 mg/dL (± 1.07) and 1.96 mg/dL (± 1.19) for the readmission group (p = 0.664). Forty-eight children (25.9%) had CRP values greater than the recommended 2.0 mg/dL at discharge. Only three of these patients (6.2%) were later readmitted. The only common variable of the readmitted children was an antibiotic-resistant or atypical causative bacteria. Conclusions: CRP levels are useful in monitoring treatment efficacy of septic arthritis in children but are not reliable as a discharge criterion to prevent readmission or reoperation. We recommend determining discharge readiness on the basis of clinical improvement and down-trending CRP values. There was a higher risk of readmission in children with an antibiotic-resistant or atypical causative bacteria. Close monitoring of these patients and those with negative cultures at time of discharge is suggested to identify signs of persistent infection. Level of evidence: III, retrospective cohort study.

An Updated Algorithm for Radiographic Screening of Upper Cervical Instability in Patients With Down Syndrome.

STUDY DESIGN: Retrospective evaluation of cervical spine images from 2006-2012 for the purposes of "screening" children with Down syndrome for instability. OBJECTIVE: To determine whether a full series of cervical spine images including flexion/extension lateral (FEL) radiographs was needed to avoid missing upper cervical instability. SUMMARY OF BACKGROUND DATA: The best algorithm, measurements, and criteria for screening children with Down syndrome for upper cervical instability are controversial. Many authors have recommended obtaining flexion and extension views. We noted that patients who require surgical stabilization due to myelopathy or cord compression typically have grossly abnormal radiographic measurements on the neutral upright lateral (NUL) cervical spine radiograph. METHODS: The atlanto-dental interval, space available for cord, and basion axial interval were measured on all films. The Weisel-Rothman measurement was made in the FEL series. Clinical outcome of those with abnormal measurements were reviewed. Sensitivity, specificity, and positive and negative predictive values of NUL and FEL radiographs for identifying clinically significant cervical spine instability were calculated. RESULTS: A total of 240 cervical spine series in 213 patients with Down syndrome between the ages of 4 months and 25 years were reviewed. One hundred seventy-two children had an NUL view, and 88 of these patients also had FEL views. Only one of 88 patients was found to have an abnormal atlanto-dental interval (≥6 mm), space available for cord at C1 (≤14 mm), or basion axial interval (>12 mm) on an FEL series that did not have an abnormal measurement on the NUL radiograph. This patient had no evidence of cord compression or myelopathy. CONCLUSIONS: Obtaining a single NUL radiograph is an efficient method for radiographic screening of cervical spine instability. Further evaluation may be required if abnormal measurements are identified on the NUL radiograph. We also propose new "normal" values for the common radiographic measurements used in assessing risk of cervical spine instability in patients with Down syndrome. LEVEL OF EVIDENCE: Level IV.

Return-to-Sport Testing After Medial Patellofemoral Ligament Reconstruction in Adolescent Athletes.

BACKGROUND: Return to sport (RTS) after patellar stabilization surgery involves the return of strength and dynamic knee stability, which can be assessed using isometric strength and functional performance testing. PURPOSE: To investigate the results of isometric strength and functional RTS testing between the surgical and uninvolved limbs in adolescent patients who underwent medial patellofemoral ligament (MPFL) reconstruction for patellar instability. STUDY DESIGN: Case series; Level of evidence, 4. METHODS: A retrospective review of adolescent patients who underwent MPFL reconstruction identified 28 patients (20 female, 8 male) who also underwent isometric and functional RTS testing. Data were compared with the uninvolved limb. The recovery of muscle strength was defined by a limb symmetry index (LSI) ≥90%. Differences in peak torque were compared using the Wilcoxon signed-rank test. Correlations were examined between dependent and independent variables using the Spearman correlation. RESULTS: The mean age of the patients was 14.9 years (range, 12-16 years). Reconstruction was performed with a hamstring autograft in 17 (60.7%) patients. Concomitant tibial tubercle osteotomy was performed in 10 (35.7%) patients. Testing was performed at a mean 7.4 months (range, 5.5-11.9 months) postoperatively. The mean LSIs for quadriceps and hamstring strength were 85.3% and 95.1%, respectively. For knee extension, there was a statistically significant difference between isometric peak torque measured in the surgical and uninvolved limbs (P = .001). Only 32.0% of patients passed all 4 hop tests. Also, 63.0% of patients achieved an anterior reach asymmetry of <4 cm on the Lower Quarter Y-Balance Test (YBT-LQ). There were no statistically significant differences in isometric strength testing, hop tests, or the YBT-LQ based on graft type or concomitant procedures. There was no correlation between isometric strength and performance on the YBT-LQ or hop tests. CONCLUSION: Adolescent athletes undergoing MPFL reconstruction may need prolonged rehabilitation programs beyond 8 months to allow the adequate recovery of muscle strength for safe RTS. There is a significant deficit in isometric quadriceps strength in the surgical limb after surgery. Further investigation is needed to determine safe RTS criteria after MPFL reconstruction in the pediatric and adolescent population.

The Impact of Transphyseal Anterior Cruciate Ligament Reconstruction on Lower Extremity Growth and Alignment.

PURPOSE: To evaluate the effect of transphyseal anterior cruciate ligament (ACL) reconstruction on lower extremity radiographic growth and alignment. METHODS: We retrospectively reviewed patients who underwent transphyseal ACL reconstruction and were followed to skeletal maturity or at least 2 years, with the nonoperative limb used as an internal control. Changes in coronal plane alignments and tibial slope of the operative limb were compared with a Wilcoxon test. Associations among sex, tunnel, and graft characteristics and failure; changes in coronal plane measures and tunnel size; and tunnel angles and the development of deformity were examined by χ-square and correlation coefficients. RESULTS: Fifty-nine patients (41 boys and 18 girls) underwent surgery at a mean age of 12.5 years (range, 6.8-16.0 years). There were differences in changes in the mechanical lateral distal femoral angle comparing operative and nonoperative limbs (decreased 1.1° in girls and 1.9° in boys ≤13 years of age, P = .0008 and .025, respectively) and in changes in tibial slope of the operative limb (decreased 2.1° in male patients >13 years, P = .012). No patient developed a new limb length difference >1 cm. Two boys were treated for deformities. Eight additional patients developed >5° difference in alignment for a rate of radiologic deformity of 10 of 59 or 17%. Neither graft failure nor the presence of deformity was associated with sex, tunnel size, mode of femoral tunnel positioning, inclination of tunnels, or the use of allograft. CONCLUSIONS: Radiographically evident limb deformities following transphyseal ACL reconstruction occurred at a rate of 17%, although these deformities were clinically evident in only 5% of patients. Tunnels intersecting physes near cortical margins may increase the risk of developing deformity. Regular follow-up should include alignment radiographs to detect deformities despite the clinical appearance of neutral limb alignment. LEVEL OF EVIDENCE: Level III, case-control study.

Best practice guidelines regarding diagnosis and management of patients with type II collagen disorders.

PURPOSE: Skeletal dysplasias comprise a heterogeneous group of inherited disorders of development, growth, and maintenance of the human skeleton. Because of their relative rarity and wide phenotypic variability, patients should be accurately identified, uniformly assessed, and managed by clinicians who are aware of their potential complications and possess the knowledge and resources to treat them effectively. This study presents expert guidelines developed to improve the diagnosis and management of patients with type II collagen skeletal disorders to optimize clinical outcomes. METHODS: A panel of 11 multidisciplinary international experts in the field of skeletal dysplasia participated in a Delphi process, which comprised analysis of a thorough literature review with subsequent generation of 26 diagnosis and care recommendations, followed by two rounds of anonymous voting with an intervening face-to-face meeting. Those recommendations with more than 80% agreement were considered as consensual. RESULTS: After the first voting round, consensus was reached to support 12 of 26 (46%) statements. After the panel discussion, the group reached consensus on 22 of 24 revised statements (92%). CONCLUSIONS: Consensus-based, expert best practice guidelines developed as a standard of care to assist accurate diagnosis, minimize associated health risks, and improve clinical outcomes for patients with type II collagen skeletal dysplasias.

Concomitant Anterior Cruciate Ligament Reconstruction and Temporary Hemiepiphysiodesis in the Skeletally Immature: A Combined Technique.

BACKGROUND: Young athletes with an anterior cruciate ligament (ACL) disruption and limb malalignment pose a treatment dilemma. Little has been published regarding limb malalignment in this population. Our aim is to review the results of combined treatment of an ACL deficient knee and genu valgum in skeletally immature patients. METHODS: A retrospective review of skeletally immature patients who underwent transphyseal ACL reconstruction and concomitant hemiepiphysiodesis between 2004 and 2015 by 1 surgeon at a single institution was performed. Included patients had at least a year of growth remaining and were followed to skeletal maturity. Patients with a diagnosis of a connective tissue disorder were excluded. Knee stability, rate of retear, the rate of mechanical axis correction, and time to full correction were determined. RESULTS: Ninety skeletally immature patients underwent transphyseal ACL reconstruction, 8 of which met inclusion criteria. Mean time to correction of the valgus deformity was 13 months (0.4 degree/mo). No patient required additional surgeries for malalignment. All patients had improvement in knee stability. One patient had a retear of their ACL reconstruction, for a failure rate of 13%. Preoperative mechanical lateral distal femoral angle and mechanical axis deviation corrected to near-neutral alignment for all treated limbs and were significantly different (P=0.001) than those measured preoperatively. CONCLUSIONS: Promising results were seen for simultaneous correction of genu valgum and transphyseal ACL reconstruction. Treatment of both pathologies in a concomitant surgery can be considered in the appropriate population, with expected results comparable to each procedure in isolation. LEVEL OF EVIDENCE: Level IV-case series.

Best practice guidelines regarding prenatal evaluation and delivery of patients with skeletal dysplasia.

BACKGROUND: Skeletal dysplasia comprises a heterogeneous and collectively common group of inherited disorders of development, growth, and maintenance of the human skeleton. There is potential for increased perinatal morbidity and mortality in pregnant women who themselves have skeletal dysplasia, and for affected fetuses where skeletal dysplasia is suspected in utero. OBJECTIVE: We sought to establish guidelines for perinatal health care professionals who should be aware of these risks, to optimize maternal and child health pregnancy outcomes through best prenatal and delivery management practices. STUDY DESIGN: A panel of 13 multidisciplinary international experts participated in a Delphi process, which comprised consideration of thorough literature review and a list of 54 possible care recommendations subject to 2 rounds of anonymous voting and a face-to-face meeting. Those recommendations with >80% agreement were considered as consensual. RESULTS: During the first round, consensus was reached to support 30 out of the 54 statements. After the panel discussion, the group reached consensus on 40 statements. These statements include guidelines for the evaluation and treatment of pregnant women with skeletal dysplasia and for the unborn child with or suspected to have skeletal dysplasia. CONCLUSION: Consensus-based best practice guidelines are provided as a minimum of standard care to minimize associated health risks, and improve clinical outcomes for patients with skeletal dysplasia.

Early-Onset Spinal Deformity in Skeletal Dysplasias: A Multicenter Study of Growth-Friendly Systems.

PURPOSE: Severe, early-onset spinal deformity is common in patients with skeletal dysplasia. These deformities often present at young ages and are associated with significant pulmonary dysfunction. The objective of this study is to verify the effectiveness of growth-friendly spinal instrumentation systems in promoting growth in patients with skeletal dysplasia and early-onset kyphoscoliosis. METHODS: A retrospective, multicenter comparative cohort study was performed. Twenty-three patients identified to have a skeletal dysplasia (SKD) were evaluated for diagnosis, age at treatment, gender, and type of growing rod construct (spine vs. rib constructs). Patients were matched by age and construct type with similarly treated patients with early-onset scoliosis (CON) without skeletal dysplasia. Radiographic parameters including maximum coronal and sagittal Cobb angle with levels, T1-S1 height, and T1-T12 height were measured. RESULTS: T1-T12 (12.8 vs. 15.2 cm, p = .01) and T1-S1 (21.2 vs. 24.5 cm, p = .05) heights were significantly shorter for the SKD group at implantation, and kyphosis tended to be more severe in children with SKD (p = .80 and .07, respectively). Kyphosis did not improve with treatment. Scoliosis improved (p < .01), and ΔT1-T12 and ΔT1-S1 significantly increased in both groups (p < .01). Complication rates were similar between the two groups; however, patients with SKD had more intraoperative monitoring changes and hardware failures (p < .005). CONCLUSION: Although patients with SKD start with shorter spine lengths, gains in spine length appear to be comparable to other forms of EOS. Neuromonitoring changes and implant failures are more common in the SKD group. SIGNIFICANCE: The effectiveness of growth-friendly techniques in promoting growth in early-onset spinal deformities in patients with skeletal dysplasia has not been previously studied. We report the first comprehensive review of this topic. Growth-friendly techniques are an appropriate treatment option in this patient population.

Outcomes and Return to Sport After Ulnar Collateral Ligament Reconstruction in Adolescent Baseball Players.

BACKGROUND: The number of ulnar collateral ligament (UCL) reconstructions in adolescent athletes has increased over the past 2 decades. Clinical results in this population have not been well studied. PURPOSE/HYPOTHESIS: The purpose of this study was to evaluate the outcomes and return to sport after UCL reconstruction in a large group of adolescent baseball players. We hypothesized that excellent clinical outcomes and high rates of return to sport would be observed in this population at a minimum 2-year follow-up. STUDY DESIGN: Case series; Level of evidence, 4. METHODS: We reviewed 140 adolescent (aged ≤19 years) baseball players who underwent UCL reconstruction with the American Sports Medicine Institute (ASMI) technique by a single surgeon. Medical records were reviewed for patient demographics, injury characteristics, operative details, and surgical complications. Patient-reported outcomes were assessed using the Conway scale, the Andrews-Timmerman (A-T) score, the Kerlan-Jobe Orthopaedic Clinic (KJOC) score, and a 0- to 100-point subjective scale for elbow function and satisfaction. Return to sporting activity was assessed using a custom-designed questionnaire. RESULTS: The mean age at the time of surgery was 18.0 years (range, 13-19 years), and the mean follow-up was 57.9 months (range, 32.4-115.4 months). Over half (60%) of patients were high school athletes. The mean duration of symptoms before surgery was 6.9 months (range, 0.5-60.0 months). Partial tears were identified in 57.9% of patients, and 41.3% of patients had preoperative ulnar nerve symptoms. Graft type included the ipsilateral palmaris in 77.1% of patients. Concomitant procedures were performed in 25% of patients. Outcomes on the Conway scale were "excellent" in 86.4% of patients. The mean A-T and KJOC scores were 97.3 ± 6.1 and 85.2 ± 14.6, respectively. Mean patient satisfaction was 94.4. Overall, 97.8% of patients reported returning to sport at a mean of 11.6 months (range, 5-24 months), and 89.9% of patients returned to sport at the same level of competition or higher. A total of 11.6% of patients went on to play professional baseball. CONCLUSION: UCL reconstruction with the ASMI technique is an effective surgical option in adolescents, with excellent outcome scores. At a minimum of 2-year follow-up, nearly 90% of patients returned to their preinjury level of sport.

Best practices in peri-operative management of patients with skeletal dysplasias.

Patients with skeletal dysplasia frequently require surgery. This patient population has an increased risk for peri-operative complications related to the anatomy of their upper airway, abnormalities of tracheal-bronchial morphology and function; deformity of their chest wall; abnormal mobility of their upper cervical spine; and associated issues with general health and body habitus. Utilizing evidence analysis and expert opinion, this study aims to describe best practices regarding the peri-operative management of patients with skeletal dysplasia. A panel of 13 multidisciplinary international experts participated in a Delphi process that included a thorough literature review; a list of 22 possible care recommendations; two rounds of anonymous voting; and a face to face meeting. Those recommendations with more than 80% agreement were considered as consensual. Consensus was reached to support 19 recommendations for best pre-operative management of patients with skeletal dysplasia. These recommendations include pre-operative pulmonary, polysomnography; cardiac, and neurological evaluations; imaging of the cervical spine; and anesthetic management of patients with a difficult airway for intubation and extubation. The goals of this consensus based best practice guideline are to provide a minimum of standardized care, reduce perioperative complications, and improve clinical outcomes for patients with skeletal dysplasia.