Syndromic Involvement of Patients Presenting With Congenital Upper Limb Anomalies: An Analysis of 4,317 Cases.

PURPOSE: This study investigated the patterns of syndromic involvement for patients with congenital upper limb anomalies (CULAs). We hypothesize that patients with CULAs will present with predictable syndromic patterns. METHODS: This retrospective study queried the multicenter Congenital Upper Limb Differences (CoULD) Registry. Of the 4,317 patients enrolled, 578 (13%) reported one or more syndromes. Syndromes were confirmed to be recognized by the Online Mendelian Inheritance in Man. Demographics were reviewed and compared with the full CoULD registry group. Syndromes reported by five or more patients were examined to determine the type of CULA according to Oberg/Manske/Tonkin classifications. RESULTS: Of the 578 children with one or more reported syndromes, 517 had Online Mendelian Inheritance in Man recognized syndromes (cohort A), In cohort A, 58 syndromes were each represented by a single patient within the registry. Forty-eight syndromes in cohort A were reported by two or more patients, which accounted for 461 of the total patients with reported syndromes. However, VACTERL and Poland syndromes were the most commonly reported syndromes. Patients with CULAs and syndromes frequently exhibited bilateral involvement (61%), compared with the entire CoULD group (47%) and other orthopedic (50%) and medical conditions (61%) compared with the entire CoULD group (24% and 27%, respectively). Additionally, they exhibited a lower frequency of family history of a congenital orthopedic condition (21%) or a family member with the same CULA (9%) compared with the entire CoULD group (26% and 14%, respectively). CONCLUSIONS: Associated syndromes were recorded in 578 patients (13%) in the CoULD registry as follows: 58 syndromes represented by a single patient, 48 by 2 or more patients, and 23 syndromes by 5 or more patients. Rare syndromes that are only represented by a single patient are more likely to be unknown by a pediatric hand surgeon, and consultation with a geneticist is advised. TYPE OF STUDY/LEVEL OF EVIDENCE: Differential Diagnosis/Symptom Prevalence Study IV.

Early Surgical Results of Pediatric Unicondylar Phalangeal Fractures.

PURPOSE: Conventional teaching dictates that timely diagnosis and expeditious treatment of unicondylar phalangeal fractures are essential to restore articular congruity, which allows for optimal range of motion and function. The objective of this study was to compare the radiographic and clinical results of surgical treatment of acute and chronic unicondylar phalanx fractures in pediatric patients. METHODS: Thirty-four patients with unicondylar phalangeal fractures underwent surgical intervention at a pediatric tertiary care center from 2004 to 2016. A fracture was defined as acute if an interval of 4 weeks or less had elapsed between the date of injury and date of surgery and chronic if an interval of more than 4 weeks had elapsed between the date of injury and date of surgery. Preoperative and postoperative radiographs were assessed. The joint alignment was determined by measuring the angle between the longitudinal axis of the phalanx and a line tangential to the articular condyles on anteroposterior radiographs. RESULTS: There were 24 patients (71%) with acute and 10 (29%) with chronic fractures. An oblique volar fracture pattern was most common in both the groups. The average joint alignment obtained from the anteroposterior radiographs at presentation was 6° in the acute group and 12° in the chronic group. The surgical procedures performed included closed reduction and percutaneous pinning, open reduction and internal fixation, osteoclasis or osteotomy and fixation, and corrective advancement osteotomy. The posttreatment alignment was within 3° of neutral in 21 patients (88%) with acute injury and 8 (80%) with chronic injury. The average interphalangeal joint range of motion at the final clinic visit was 1°-92° in the acute group and 4°-85° in the chronic group. One patient with chronic injury developed avascular necrosis after treatment with corrective osteotomy. CONCLUSIONS: The patterns and characteristics of unicondylar phalanx fractures are similar between pediatric patients and adults. The radiographic alignment and digital motion are improved with surgical reduction and fixation, regardless of chronicity. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Donor-Site Morbidity for Iliac Crest Harvesting for Pediatric Scaphoid Nonunion.

PURPOSE: Screw fixation with iliac crest bone grafting (ICBG) is a well-studied treatment for pediatric scaphoid nonunions. Studies in adults, as well as in pediatric spine fusions, have demonstrated high rates of complications with ICBG, including longer-term donor site pain. We hypothesized that in pediatric patients undergoing ICBG for scaphoid nonunion, the donor site complication rate would be lower than that reported in other populations. METHODS: Records of patients ages 0-18 years at a single institution undergoing surgical reconstruction for scaphoid nonunion from 1995 to 2016 were reviewed. Patient and surgical variables were recorded, including how ICBG was harvested. Donor site complications were recorded, including donor site pain beyond 30 days after surgery, infection, peri-incisional or lower extremity numbness at any point after surgery and reoperation at the donor site at any time point after surgery. RESULTS: During the study period, 119 wrists in 117 patients underwent internal fixation and ICBG for scaphoid nonunion. The average age was 16 years; mean follow-up was 1 year. The majority of wrists (73, 62.9%) underwent harvest of both outer and inner tables of the iliac crest; 38 (31.9%) had only outer table harvested; 5 (4.3%) had only cancellous graft harvested. Ten wrists (8.4%) had a donor site complication. The most common donor site complication was donor site pain beyond 30 days after surgery (5, 4.2%), followed by numbness (4, 3.4%). No infections, seromas, or reoperations at the donor site occurred. In comparison to those subjects who did not experience complications, we found no difference based on the age at surgery or the type of graft used. Female patients were more likely to have a recorded complication than males. CONCLUSIONS: Donor site morbidity for iliac crest grafting in pediatric patients undergoing scaphoid nonunion surgery appears to be lower than that previously reported in adult patients. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Do Nails and Nubbins Matter? A Comparison of Symbrachydactyly and Transverse Deficiency Phenotypes.

PURPOSE: Transverse deficiency (TD) and symbrachydactyly may be difficult to distinguish due to shared phenotypes and a lack of pathognomonic features. The 2020 Oberg-Manske-Tonkin classification update modified these anomalies to include "with ectodermal elements" for symbrachydactyly and "without ectodermal elements" for TD as a defining differentiating characteristic. The purpose of this investigation was to characterize ectodermal elements and the level of deficiency and to examine whether ectodermal elements versus the level of deficiency was a greater determining factor for Congenital Upper Limb Differences (CoULD) surgeons making the diagnosis. METHODS: This was a retrospective review of 254 extremities from the CoULD registry with a diagnosis of symbrachydactyly or TD by pediatric hand surgeons. Ectodermal elements and the level of deficiency were characterized. A review of the registry radiographs and photographs was used to classify the diagnosis and compare it with the diagnosis given by the pediatric hand surgeons. The presence/absence of nubbins versus the level of deficiency as the determining factor to differentiate the pediatric hand surgeons' diagnosis of symbrachydactyly (with nubbins) versus TD (without nubbins) was analyzed. RESULTS: Based on radiographs and photographs of the 254 extremities, 66% had nubbins on the distal end of the limb; of the limbs with nubbins, nails were present on 51%. The level of deficiency was amelia/humeral (n = 9), <1/3 transverse forearm (n = 23), 1/3 to 2/3 transverse forearm (n = 27), 2/3 to full forearm TD (n = 38), and metacarpal/phalangeal (n = 103). The presence of nubbins was associated with a four times higher likelihood of a pediatric hand surgeon's diagnosis of symbrachydactyly. However, a distal deficiency is associated with a 20-times higher likelihood of a diagnosis of symbrachydactyly than a proximal deficiency. CONCLUSIONS: Although both the level of deficiency and ectodermal elements are important, the level of deficiency was a greater determining factor for a diagnosis of symbrachydactyly versus TD. Our results suggest that the level of deficiency and nubbins should both be described to help provide greater clarity in the diagnosis of symbrachydactyly versus TD. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

Corrective Osteotomy for Symptomatic Radial Head and Neck Malunions in the Skeletally Immature.

BACKGROUND: Radial head and neck fracture malunion can lead to pain, stiffness, deformity, and functional limitations. This study analyzed the early radiographic and functional outcomes of corrective osteotomy for symptomatic radial head and/or neck malunion in skeletally immature patients. METHODS: Thirteen pediatric patients treated with corrective osteotomy for symptomatic radial head and/or neck malunion from 2004 to 2018 were identified. Radiographic union, range of motion, pain, and complications were recorded. Functional outcomes were assessed using the Broberg and Morrey Elbow Scale. RESULTS: The average age at the time of surgery was 12.3 years (range: 8.6 to 16.3). The mean time from injury to osteotomy was 7.5 months (range: 2 to 24.1 mo). Before osteotomy, mean radial head-shaft angulation was 23 degrees (range: 5 to 57 degrees), mean displacement was 76% (range: 0 to 55), average elbow flexion contracture was 18 degrees (range: 0 to 60 degrees), and average elbow flexion was 112 degrees (range: 95 to 135 degrees). After a mean follow-up of 14.3 months, 12 of 13 osteotomies (92%) healed; 1 patient had a painless nonunion. The number of patients reporting pain decreased from 9 to 2 ( P =0.012). Radiographic alignment and range of motion improved in all patients. Mean angulation and displacement improved to 8 degrees (range: 2 to 15 degrees, P =0.001) and 3% (range: 0 to 18 degrees, P =0.001), respectively. Mean elbow flexion contracture was reduced to 5 degrees (range: 0 to 30 degrees, P =0.008), and the average elbow flexion increased to 128 degrees (range: 100 to 135 degrees, P <0.001). Three patients underwent implant removal for complications, including heterotopic ossification and avascular necrosis. Physeal arrest did not lead to any adverse sequelae. The sole case of avascular necrosis had a reduced range of motion but no pain. The mean postoperative Broberg and Morrey score was 91.1; 7 patients had excellent postoperative scores, 4 good, and 2 fair. CONCLUSIONS: Corrective osteotomy for symptomatic malunion of the radial head and/or neck in skeletally immature patients can improve elbow alignment, motion, pain, and function. Preservation of the soft-tissue attachments to the proximal epiphysis and rigid internal fixation are critical components of this surgical technique. LEVEL OF EVIDENCE: Therapeutic Level IV.

Descriptive Epidemiology of Isolated Distal Radius Fractures in Children: Results From a Prospective Multicenter Registry.

INTRODUCTION: More than 1 in 4 pediatric fractures involves the distal radius. Most prior epidemiologic studies are limited to retrospective, single center investigations, and often include adults. This study aims to describe the contemporary epidemiology of pediatric distal radius fractures using prospectively collected data from a multicenter Pediatric Distal Radius Fracture Registry. METHODS: Patients aged 4 to 18 years diagnosed with a distal radius fracture from June 2018 through December 2019 at 4 tertiary care pediatric centers were screened and enrolled in this prospective longitudinal cohort study. Patients were excluded if they presented with bilateral distal radius fractures, polytrauma, or re-fracture. Demographic information, mechanism of injury, fracture characteristics, associated injuries, and procedural information were recorded. All radiographs were reviewed and measured. Descriptive statistics and bivariate analyses were performed. RESULTS: A total of 1951 patients were included. The mean age was 9.9±3.3 years, and 61.3% of patients were male ( P <0.001). Most injuries occurred during a high-energy fall (33.5%) or sports participation (28.4%). The greatest proportion of fractures occurred during the spring months (38.5%). Torus fractures (44.0%) were more common than bicortical (31.3%) or physeal (21.0%) fractures. Of the physeal fractures, 84.3% were Salter-Harris type II. Associated ulnar fractures were observed in 51.2% of patients. The mean age at injury was higher for patients with physeal fractures (11.6±2.9 y) than patients with torus or bicortical fractures (9.4±3.1 and 9.6±3.1 y, respectively; P <0.001). Thirty-six percent of distal radius fractures underwent closed reduction and 3.3% underwent surgical fixation. Patients treated with closed reduction were more likely to be male (68.7% vs. 57.2%; P <0.001), obese (25.3% vs. 17.2%; P <0.001), and have bicortical fractures (62.2% vs. 14.5%; P <0.001). CONCLUSIONS: Distal radius fractures in children have a male preponderance and are most likely to occur in the spring months and during high-energy falls and sports. Physeal fractures tend to occur in older children while torus and bicortical fractures tend to occur in younger children. LEVEL OF EVIDENCE: Level I-prognostic.

Social Deprivation and Congenital Upper Extremity Differences-An Assessment Using PROMIS.

PURPOSE: Social deprivation, a measure of socioeconomic status, has been shown to negatively affect perceptions of orthopedic conditions and outcomes of treatment. The objective of this study was to assess whether social deprivation correlates with subjective assessment of function in pediatric patients with congenital hand differences. METHODS: Patients enrolled in the Congenital Upper Limb Differences (CoULD) registry were assessed using the Patient-Reported Outcomes Measurement Information System (PROMIS). The PROMIS scores for Pain Interference, Peer Relations, Anxiety, Depression, and Upper Extremity (UE) function were obtained for all patients 5 years and older at initial presentation. Social deprivation was determined by the Area Deprivation Index (ADI); the index ranges from 0 to 100 with higher scores being the most deprived. The PROMIS scores were correlated with the ADI for all patients. RESULTS: Three hundred seventy-five pediatric patients with congenital UE differences were evaluated. Average age was 11 years, 56% were female, and 55% had bilateral involvement. Overall, PROMIS scores were within 1 SD of normal for Peer Relations, Pain, Depression, and Anxiety. However, child-reported scores for UE function (39) were more than 1 SD below the national average (50). The mean ADI for the cohort was lower than the national average, indicative of less deprivation, with 14% of patients in the most deprived national quartile. Children in the highest ADI quartile reported PROMIS scores that reflected higher Pain Interference (41 vs 45), lower Peer Relations (55 vs 50), higher Anxiety (44 vs 49), and higher Depression (43 vs 47) than children in the lowest ADI quartile. CONCLUSIONS: The PROMIS scores were normal for psychosocial measures in children with congenital hand differences when evaluated as an entire cohort. However, child self-reported PROMIS scores for Pain Interference, Peer Relations, Anxiety, and Depression were worse in more socially deprived areas, suggesting more psychosocial challenges in these children. CLINICAL RELEVANCE: Pediatric patients with congenital upper extremity differences in areas of higher social deprivation report lower psychosocial well-being. The care of these individuals must be considered within the context of their environment because they may be more at risk for negative outcomes secondary to environmental and societal stressors.

Changes in superior displacement, angulation, and shortening in the early phase of healing for completely displaced midshaft clavicle fractures in adolescents: results from a prospective, multicenter study.

BACKGROUND: Progressive displacement of diaphyseal clavicle fractures has been observed in adult patients, at times necessitating a change from nonoperative to operative treatment. Whether this occurs in adolescent patients has not been well investigated. The purpose of this study was to assess the rate and extent of progressive clavicle fracture displacement in adolescent patients following injury and during the early stages of healing. METHODS: This was a multicenter study evaluating prospective data that had previously been collected as part of a larger study evaluating the functional outcomes of adolescent clavicle fractures. A consecutive series of completely displaced diaphyseal clavicle fractures in patients aged 10-18 years treated at 1 of 3 tertiary-care pediatric trauma centers was included; all fractures underwent standardized imaging within 2 weeks of the date of injury and during the course of healing (5-20 weeks after injury). Measurements of clavicle shortening, superior displacement, and angulation were performed using validated techniques. Progressive displacement and/or interval improvement in fracture alignment, as well as the subsequent need for surgical intervention, was noted. Patient demographic and radiographic parameters were assessed as possible risk factors for interval displacement. RESULTS: One hundred patients met the inclusion criteria. Mean end-to-end shortening, cortex-to-cortex shortening, superior displacement, and angulation at the time of injury were 24 mm, 15 mm, 15 mm, and 7°, respectively. At a mean of 10 weeks after injury, the fracture alignment improved across all 4 measurements for the overall cohort, with mean improvements of 3.5 mm in end-to-end shortening, 3.3 mm in cortex-to-cortex shortening, 2.1 mm in superior displacement, and 2° in angulation. By use of a clinical threshold of a change in shortening or displacement of 10 mm or change in angulation of 10°, 26% of fractures improved, 4% worsened, and 70% remain unchanged. Patients with more severe fractures were more likely to have improved alignment than were patients with less displaced fractures (P < .001). No patient underwent surgical intervention for progressive displacement. CONCLUSION: Significant early improvements in fracture alignment were observed in a substantial percentage of adolescent patients with completely displaced clavicle fractures. Among the most severely displaced fractures, shortening improved approximately 6 mm and angulation improved approximately 9°. In 4% of cases, increased displacement was observed, but this tended to be mild, and in no cases did it prompt surgical intervention. This finding indicates that the true final deformity after an adolescent clavicle fracture is commonly less than that present at the time of injury.

Early Results of Surgical Treatment of Triangular Fibrocartilage Complex Tears in Children and Adolescents.

PURPOSE: To investigate the clinical results and patient-reported outcomes following surgical treatment for triangular fibrocartilage complex (TFCC) tears in the pediatric and adolescent population. METHODS: We reviewed 149 patients with 153 arthroscopy-confirmed TFCC tears. Mean age at surgery was 15.5 years (range, 7-19 years). There were 86 females. Plain radiographs and magnetic resonance imaging were used to characterize bony and soft tissue pathology. Mayo Modified Wrist Score (MMWS) and Patient-Reported Outcomes Measurement Information System (PROMIS) Upper Extremity Short Form assessed functional outcomes. Median patient follow-up was 21.8 months (IQR:5.9-55.4). RESULTS: Pre-operatively, all patients had wrist pain or instability with activities. The median pre-operative MMWS was 80 (interquartile range [IQR], 65-90). Fifty-six (35%) presented with positive ulnar variance. Concomitant pathology included distal radioulnar joint (DRUJ) instability (14%), ulnocarpal impaction (20%), ulnar styloid nonunion (33%), and distal radius growth arrest (30%). On arthroscopy, there were 15 (10%) isolated 1A, 79 (52%) 1B, 1 (1%) 1C, 30 (20%) 1D tears, and 25 (16%) cases of multiple tears. Twenty-six percent of wrists underwent TFCC debridement, 68% arthroscopy-assisted repair, 6% both for combined tears. Fifty-one percent of wrists underwent bony procedures-most commonly ulnar-shortening osteotomy to achieve neutral ulnar variance (40%) and symptomatic ulnar styloid nonunion excision with concomitant TFCC repair (39%). At final follow-up, pain, wrist range of motion, DRUJ stability, ulnar variance, and MMWS (median, 95 [IQR, 86.5-100]) improved significantly. The median PROMIS T-score at final follow-up was 57 (IQR, 45-57). The MMWS was better in those with concomitant bony procedures at index surgery than those with only repair or debridement of TFCC tears. CONCLUSIONS: Most pediatric TFCC tears are posttraumatic and peripheral. Surgical treatment of TFCC tears and concomitant pathology in the pediatric and adolescent population results in decreased pain, improved motion and stability, and excellent functional outcomes in the majority of patients. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Outcomes of Late Microsurgical Nerve Reconstruction for Brachial Plexus Birth Injury.

PURPOSE: Microsurgical nerve reconstruction has been advocated between 3 and 9 months of life in select patients with brachial plexus birth injury (BPBI), yet some patients undergo indicated surgery after this time frame. Outcomes in these older patients remain poorly characterized. We analyzed outcomes of nerve reconstruction performed after 9 months of age and hypothesized that (1) Active Movement Scale (AMS) scores improve after surgery, and (2) there are no differences in AMS scores between patients undergoing nerve transfers versus those undergoing nerve grafting. METHODS: From 2000 to 2014, 750 patients at 6 U.S. centers were prospectively enrolled in a multicenter database. We included patients treated with nerve reconstruction after 9 months of age with minimum 12 months' follow-up. Patients were evaluated using AMS scores. To focus on the results of microsurgery, only outcomes prior to secondary surgery were analyzed. We analyzed baseline variables using bivariate statistics and change in AMS scores over time and across treatment groups using linear mixed models. RESULTS: We identified 32 patients (63% female) with median follow-up of 29.8 months. Median age at microsurgery was 11.2 months. Twenty-five (78%) had an upper trunk injury. Compared with before surgery, total AMS scores improved modestly at 1 year and 2 or more years follow-up. At 1 year follow-up, AMS scores improved for shoulder function (abduction, external rotation) and elbow flexion. Between-group comparisons found no differences in total AMS scores or AMS subscales between graft and transfer groups at 1 year or 2 or more years after surgery, so we cannot recommend one strategy over the other based on our findings. CONCLUSIONS: Overall, nerve reconstruction in patients with BPBI after 9 months of age resulted in improved function over time. There was no difference in outcomes between nerve transfer and nerve graft groups and 1 or 2 or more years follow-up. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Convergent Validity of PODCI and PROMIS Domains in Congenital Upper Limb Anomalies.

PURPOSE: This study evaluates the perceived functional and psychosocial impact of upper limb congenital anomalies prior to surgical intervention, utilizing the Pediatric Outcomes Data Collection Instrument (PODCI) and Patient-Reported Outcomes Measure Information System (PROMIS) domains. We hypothesized that scores will be in the normal range and that the 2 outcomes measures will have strong convergent validity. METHODS: A multicenter prospectively collected database of congenital upper limb patients, the CoULD (Congenital Upper Limb Differences) study group, was utilized. Demographic information was collected, and anomalies were classified by the Oberg-Manske-Tonkin (OMT) classification. Scores for PODCI subscales of Upper Extremity (UE) function, Pain/comfort, and Happiness and PROMIS domain of UE function, Pain, Depression, Anxiety, and Peer relations were collected. Ceiling and floor effects and convergent validity for PODCI and PROMIS domains were calculated. RESULTS: Three hundred fifty-nine patients, average age 10 years and 55% male, were included. Two hundred forty-one patients had a malformation of the entire limb and 231 had a hand plate malformation. Four patients had a deformation, 118 dysplasia, and 45 a syndrome. There was no difference between the PODCI and the PROMIS ceiling or floor effects for the UE domains. The ceiling effect for PROMIS Pain domain (46%) was similar to the floor effect of the PODCI Pain subscale and no difference was seen between the floor effect of PODCI Happiness and PROMIS Depression domains. Convergent validity was obtained for the UE and Pain domains, and also between PODCI Happiness and PROMIS Depression subscales. CONCLUSIONS: The PROMIS domains for UE function, Pain, and Depression are comparable with PODCI scores in congenital upper extremity anomalies. CLINICAL RELEVANCE: Given the large burden placed on patients with the lengthy PODCI questionnaire, consideration of replacing the PODCI with the more quickly obtained PROMIS scores may reduce patient burden and provide similar information in this population.

Congenital pseudarthrosis of the clavicle: surgical decision making and outcomes.

BACKGROUND: Congenital pseudarthrosis of the clavicle (CPC) is a rare entity in which the primary ossification center of the clavicle fails to coalesce. The natural history of CPC is unknown, and there is controversy regarding surgical vs. conservative treatment. METHODS: A retrospective review of 47 pediatric patients treated for CPC was performed. The Quick Disabilities of the Arm and Shoulder (QuickDASH) survey and the Patient Reported Outcomes Measurement Information System (PROMIS) upper extremity domain were used to assess overall patient satisfaction, function, and quality of life after treatment. RESULTS: Twenty-four of 47 (51%) patients underwent surgical treatment. Of these, 9 patients (38%, 9/24) underwent surgery at <18 months of age using suture fixation alone, whereas the older 15 surgical patients (15/24, 62%) were treated with plate fixation. The younger surgical cohort had a nonunion rate of 43% (3/7) compared with 13% (2/15) in the older cohort. All surgical patients had resolution of preoperative symptoms. Eleven (11/24, 46%) surgical subjects responded to the follow-up survey. Upper extremity function normalized according to the QuickDASH survey (score of 0 for all subjects). The median PROMIS upper extremity domain score was 55, which was also in the normal range. CONCLUSIONS: This series of CPC patients improves our understanding of treatment options and outcomes of surgical treatment. All surgical patients had resolution of preoperative symptoms. Patients treated surgically with stable fixation at an older age had higher rates of union than those treated in infancy with suture fixation. Patient-reported outcomes were favorable overall.

Early Results of Single-plug Autologous Osteochondral Grafting for Osteochondritis Dissecans of the Capitellum in Adolescents.

BACKGROUND: Osteochondral autologous transplantation surgery (OATS) has been advocated for unstable osetochondritis dissecans (OCD) lesions of the adolescent capitellum, though limited information is available regarding clinical and radiographic results in North American patients. We hypothesize that single-plug OATS is safe and effective in alleviating pain and restoring function in unstable OCD. METHODS: Twenty-eight patients with unstable OCD treated with single-plug OATS were evaluated. Mean age at surgery was 14.2 years; there were 14 males. Etiology of OCD was presumed to be sports participation, including baseball (n=5) and gymnastics (n=11). Indications for surgery included unstable, deep OCD lesions; 2 lesions were uncontained, and 3 patients (11%) had OATS after failed prior surgery. OATS was performed by an anconeus muscle-splitting approach; donor grafts were harvested from the lateral femoral condyle by small arthrotomy. Functional outcomes were quantified using the Timmerman instrument. Median clinical and radiographic follow-up was 6.3 months (range, 5.0 to 27.0 mo) and 5.7 months (range, 5.0 to 26.7 mo), respectively. Furthermore, all patients returned functional questionnaires at a median of 9 months postoperatively (range, 5 to 27 mo). RESULTS: Of the 26 patients who reported preoperative tenderness, 19 (73%) patients had no tenderness at most recent clinical follow-up (P=0.02). Of 18 patients with restricted elbow motion preoperatively, 13 had achieved full range of motion (P=0.10). Both elbow flexion and extension improved significantly [flexion: median change (interquartile range)=10 degrees (0 to 10 degrees), P=0.009; extension: 0 degree (-5 to 0 degrees), P <0.001). On postoperative magnetic resonance imaging, 86% (P<0.001) of elbows had restoration of articular congruity and 93% had complete graft incorporation. Objective [median change (interquartile range)=5 degrees (0 to 15 degrees)], subjective [25 degrees (15 to 40 degrees)], and overall [35 degrees (15 to 45 degrees)] Timmerman scores improved significantly (P=0.001, <0.001, and <0.001, respectively). Of the 13 patients with >6 months follow-up, 9 patients (69%) had returned to their primary sport (P=0.27) and 100% had returned to general sports participation. There were no postoperative complications. At final follow-up, all donor knees were asymptomatic with full motion and strength. CONCLUSION: Single-plug OATS is safe and effective in improving pain and elbow function in adolescents with unstable OCD, with high return to sports rates and little donor-site morbidity. LEVEL OF EVIDENCE: Level IV-case series.

Surgical Reconstruction of Missed Monteggia Lesions in Children.

BACKGROUND: Chronic Monteggia lesions in children may cause pain, deformity, decreased range of motion, and neurological symptoms. Numerous surgical techniques have been advocated to reconstruct long-standing Monteggia injures in efforts to maximize long-term upper limb function. The purpose of this investigation was to assess the clinical and radiographic results of a modified surgical technique for missed Monteggia fracture-dislocations. METHODS: A retrospective evaluation of 52 patients who underwent surgical reconstruction of missed Monteggia fracture-dislocations at a tertiary pediatric hospital was performed. The median patient age at the time of surgery was 6.8 years, and the median time from injury to surgery was 12.9 weeks. Electronic medical records, including clinic notes, radiographic images, and operative reports, were reviewed for study analysis. Median clinical and radiographic follow-up was 19.1 months. RESULTS: The median elbow range of motion improved from 108 degrees of flexion and 5 degrees short of full extension preoperatively to 140 degrees of flexion (P<0.001) and full extension (P=0.10) postoperatively. Forearm range of motion also improved from a median of 80 degrees of pronation and 58 degrees of supination preoperatively to 80 degrees of pronation (P=0.54) and 80 degrees of supination (P<0.001) postoperatively. Congruent radiocapitellar alignment was maintained in 39 patients (75%). Nine patients (17%) had redislocation of the radiocapitellar joint, and 4 patients (8%) had radiographic resubluxation. Six of the 9 patients who experienced redislocation underwent early revision and achieved uncomplicated longer term results. Patients who received repair of the native annular ligament were more likely to achieve lasting radiocapitellar joint stability (P=0.03) when compared with patients who received annular ligament reconstruction or if the annular ligament was not addressed. CONCLUSION: Meaningful improvements in elbow motion and radiocapitellar stability can be safely achieved in the majority of children following surgical reconstruction of missed Monteggia lesions. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Growth Disturbance Following Intra-articular Distal Radius Fractures in the Skeletally Immature Patient.

INTRODUCTION: The purpose of this study was to characterize the incidence of growth disturbance following intra-articular distal radius fractures in skeletally immature patients and to assess early radiographic and functional outcomes. METHODS: A retrospective investigation of pediatric patients with intra-articular distal radius fractures between 1997 and 2012 at a single institution was performed. Pathologic fractures and fractures in patients with closed physes were excluded. In total, 28 patients (24 males, 4 females), with a mean age of 13.8 years and mean follow-up of 31.7 months, met inclusion criteria. Fractures were categorized according to the Salter-Harris classification, and all radiographs were assessed for evidence of physeal disturbance. Information regarding treatment and early clinical results were obtained from a medical record review. Functional outcomes using the Disabilities of the Arm, Shoulder, and Hand (DASH) and Modified Mayo Wrist Score (MMWS) were collected. Fisher exact test was used to compare the incidence of physeal arrest in the study population to previously published rates of physeal arrest in extra-articular fractures involving the distal radius. Because the data were not parametrically distributed, the Mann-Whitney-Wilcoxon test was used to compare those who did and did not develop physeal arrest. RESULTS: Of the 28 patients, 9 (32%) sustained Salter-Harris III fractures and 19 (68%) sustained Salter-Harris IV fractures. Growth disturbance occurred in 12 (43%) patients, comprised of 3 Salter-Harris III fractures and 9 Salter-Harris IV fractures; 7 of these patients underwent surgical intervention to address deformity. All 4 children age 10 years or younger had growth arrests that underwent subsequent procedures for a skeletal rebalancing of the wrist. No significant differences in DASH or MMWS were seen in the short term between patients who did or did not have physeal arrest. CONCLUSIONS: Intra-articular distal radius fractures in skeletally immature patients have a considerably higher rate of physeal growth arrest than extra-articular physeal fractures. Following acute management aimed at restoring and preserving anatomic physeal and articular alignment, follow-up radiographs should be obtained to evaluate for physeal arrest in skeletally immature children. Patients and families should be counseled regarding the high rate of growth disturbance and the potential need for deformity correction in the future, particularly in younger children. LEVEL OF EVIDENCE: IV-case series.

Patient-reported Outcomes in Arthrogryposis.

BACKGROUND: Little is known about patient-reported health status in children and adolescents with arthrogryposis. Utilizing the Patient-Reported Outcome Measurement Information System (PROMIS) and Pediatric Outcomes Data Collection Instrument (PODCI) questionnaires, we investigated functional and psychosocial measures in arthrogryposis. METHODS: A total of 118 patients with arthrogryposis were identified from a prospective longitudinal cohort (the Congenital Upper Limb Difference Registry) from 2014 to 2018. Demographics and patient-reported outcome measures were evaluated, including the PROMIS [upper extremity (UE) function, pain, depression, anxiety, and peer relations] and PODCI questionnaires (UE function, pain, happiness, and global function). RESULTS: A total of 29 arthrogrypotic patients had complete PROMIS and PODCI data. This cohort was divided into distal arthrogryposis and amyoplasia groups, with 15 and 14 patients in each group, respectively. There were 8 males in the distal arthrogryposis group with a median age of 9 years and 7 males in the amyoplasia group with a median age of 8 years. For both cohorts, the median UE function PROMIS scores were significantly below population norms, 31 for distal arthrogryposis and 22 for amyoplasia. PODCI UE function was statistically lower for amyoplasia compared with the distal arthrogryposis cohort. PROMIS pain, depression, anxiety, and peer relations were in the normal range for both amyopasia and distal arthrogryposis. Median PODCI pain and happiness ranged from 85 to 88 for all patients with no statistical difference between groups. CONCLUSIONS: Arthrogryposis patients have lower UE function scores compared with population normals, but they have emotional states that are consistent with populations norms. Amyoplasia patients were functionally worse than distal arthrogryposis patients. LEVELS OF EVIDENCE: Level II.

Current Trends and Variations in the Treatment of Unicameral Bone Cysts of the Humerus: A Survey of EPOS and POSNA Members.

BACKGROUND: A variety of treatment methods have so far been described for unicameral bone cysts (UBC). However, to the best of our knowledge, no particular consensus has yet been reached on when to operatively treat a patient with a humeral UBC. Therefore, members of the European Pediatric Orthopedic Society (EPOS) and Pediatric Orthopedic Society of North America (POSNA) were surveyed to characterize current treatment preferences. METHODS: An online electronic questionnaire was sent out to all registered EPOS and POSNA members. The survey comprised 45 questions related to the diagnosis, treatment, and follow-up characteristics of patients with UBCs of the humerus. Particular questions related to the nonoperative or surgical treatment of pathologic proximal humerus and humeral shaft fractures were also included. RESULTS: In total, 444 participants (132 EPOS and 292 POSNA members) responded, of whom 400 were actively involved in UBC treatment. The preferred diagnostic modalities to confirm the diagnosis of a UBC in the humerus were radiographs (88%), MRI in cases of questionable diagnosis (58%) or CT scan (8%). For painless UBCs 67% prefer no treatment at all except when the fracture risk is deemed high (then 53% recommend surgery); 71% of respondents would treat painful UBCs with surgery. Most common surgical techniques comprise curettage (45%), artificial bone substitutes (37%), corticosteroid injection (29%), or intramedullary stabilization (eg, rodding; 24%).Fractured, nondisplaced and mildly displaced proximal humerus UBCs and mildly displaced pathologic humerus shaft fractures are all preferably treated nonoperatively (94%, 91%, 83%, respectively). Severely displaced pathologic proximal humerus fractures are treated less often conservatively (36%) than surgically (40%), and severely displaced humerus shaft fractures are preferably treated surgically (63%) by intramedullary stabilization (60%). CONCLUSIONS: There is great variation among EPOS and POSNA members with regards to the diagnosis and treatment of UBCs in the humerus. Although some consensus on general treatment principles is seen, specific surgical treatment indications vary.Prospective randomized-controlled studies are needed to evaluate the outcomes of the different surgical approaches compared with nonoperative strategies. LEVEL OF EVIDENCE: Level V-expert opinion.

Functional Elbow Range of Motion in Children and Adolescents.

BACKGROUND: The functional elbow range of motion in children and adolescents has not been previously reported. In adults, a functional motion arc of 30 to 130 degrees of elbow flexion and 50 degrees of pronation to 50 degrees of supination is well established. Contemporary tasks such as cellular phone use and keyboarding require greater elbow flexion and pronation than the functional motion arc. Whether or not this is true in children has yet to be established. We hypothesize that to perform functional and contemporary tasks, children and adolescents use a greater range of elbow motion and forearm rotation as compared with adults. METHODS: Twenty-eight subjects performed 8 functional tasks and 4 contemporary tasks. Kinematic data were captured using a 3-dimensional motion analysis system as previously described. Mean and SD was collected for elbow flexion, extension, pronation, and supination. Unpaired t tests were performed to compare elbow kinematics of children 6- to 11-year-old (n=14) to that of adolescents 12- to 17-year-old (n=14), with a significance criterion of P-value <0.05. RESULTS: The mean arc of motion to achieve functional tasks was 28 to 146 degrees of elbow extension/flexion and 54 degrees of supination to 65 degrees of pronation. Contemporary tasks utilized 40 to 148 degrees of elbow extension/flexion and 49 degrees of supination to 65 degrees of pronation. A greater supination/pronation arc (107 degrees) and greater elbow flexion (148 degrees) were used to bring a cellular phone to the ear, whereas greater pronation (65 degrees) was used to type on a keyboard. Statistically significant differences were observed between children and adolescents in each of the 12 tasks, with the exception of using a fork. CONCLUSIONS: A motion arc of 30 to 130 degrees of elbow flexion and 50 to 50 degrees of pronation/supination is sufficient to achieve most positional and functional tasks in children and adolescents. However, specific contemporary tasks such as the use of a cellular phone and typing on a keyboard utilize more elbow flexion and pronation. Awareness of greater use of flexion and pronation to achieve contemporary tasks may help guide surgeons in the care of patients with posttraumatic elbow and forearm deformities and contractures. LEVEL OF EVIDENCE: Level II-investigation of a diagnostic test.

The Frequency of Mediastinal Injury in Acute Posterior Sternoclavicular Dislocations: A Multicenter Study.

BACKGROUND: Acute posterior sternoclavicular dislocations (APSCD) are rare injuries that historically have prompted concern for injury to the great vessels and other mediastinal structures from initial trauma or subsequent treatment, resulting in the recommendation that a thoracic or vascular surgeon be present or available during operative treatment. The objectives of the study were to characterize the demographic, clinical, and radiographic characteristics of a large series of APSCDs in skeletally immature patients and to describe the rate and nature of any vascular or mediastinal complications that occurred during treatment. METHODS: Following Institutional Review Board approval, records of consecutive patients under 25 years of age treated for APSCD were collected from each of 6 participating centers. Only acute injuries (sustained fewer than 10 days before presentation) were included. Patient demographics, injury mechanism, associated mediastinal injuries, and need for thoracic/vascular surgery were recorded. Mediastinal structures injured or compressed by mass effect were specifically characterized by review of preoperative computed tomography imaging. RESULTS: Review identified 125 patients with a mean age of 14.7 years; 88% were male. APSCD most commonly resulted from a sporting injury (74%) followed by falls from standing height (10%) and high-energy motor vehicle trauma (10%). The most common finding on cross-sectional imaging was compression without laceration of the ipsilateral brachiocephalic vein (50%). Eleven patients had successful closed reduction, and 114 (90%) had open reduction and internal fixation, with 25 failed or unstable closed reductions preceding open treatment. There were no vascular or mediastinal injuries during reduction or fixation that required intervention. CONCLUSIONS: In this multicenter series of 125 APSCDs no injuries to the great vessels/mediastinal structures requiring intervention were identified. Although more than half of patients had evidence of extrinsic vascular compression at the time of injury, careful open reduction of acute injuries can be safely performed. Although vascular injuries following APSCD seem to be quite rare, vascular complications can be catastrophic. Treating providers should consider these data and their own institutional resources to maximize patient safety during the treatment of APSCD. LEVEL OF EVIDENCE: Level III-therapeutic case control study.

Management of Fasciotomy Incisions After Acute Compartment Syndrome: Is Delayed Primary Closure More Feasible in Children Compared With Adults?

BACKGROUND: Recent adult literature has demonstrated that in the setting of acute compartment syndrome (ACS), if fasciotomy wounds are not closed after the first debridement, they are unlikely to be closed via delayed primary closure (DPC). The purpose of this study was to report the success of DPC through serial debridement in children with fasciotomy wounds secondary to ACS and to determine whether length of hospital stay is negatively affected by adopting a DPC strategy. METHODS: We identified all patients treated with fasciotomy for ACS (aged 0 to18 y). Patient, injury, and treatment characteristics were summarized by fasciotomy treatment type. Patients were grouped as: primary closure, DPC, and flap or skin graft (F/SG). For patients who required additional debridements after initial fasciotomy, treatment success was defined as closure by DPC (without requiring F/SG). Multivariable logistic regression was used to determine factors associated with additional surgeries, complications, and treatment success. RESULTS: A total of 82 children underwent fasciotomies for ACS. Fifteen (18%) patients were treated with primary closure at the time of their initial fasciotomy and were excluded from the remainder of the analysis, 48 (59%) patients underwent DPC, and 19 (23%) patients were treated with F/SG. The majority of delayed fasciotomy wounds were successfully closed by DPC (48/67, 72%) and the rate of successful closure remained consistent with each successive operative debridement. There were no differences across DPC and F/SG groups with respect to age, method of injury, or injury severity. Patients who underwent F/SG remained in the hospital for an average of 12 days compared with 8 days for those who underwent DPC (P<0.001). CONCLUSIONS: In the setting of ACS, pediatric fasciotomy wounds that are not closed after the first postfasciotomy debridement still have a high likelihood of being closed through DPC with serial surgical debridement. In children, persisting with DPC strategy for fasciotomy closure after ACS is more successful than it is in adults. LEVEL OF EVIDENCE: Level III.