The Impact on Families of Children With Congenital Upper Extremity Differences.

PURPOSE: To investigate the impact on caregivers of caring for a child with congenital upper extremity differences. METHODS: In this cross-sectional study, caregivers of patients enrolled in the multi-institutional Congenital Upper Limb Difference (CoULD) registry were contacted. Demographic information and the Impact on Family Scale (IOFS), a validated measure of perceived caregiver strain, were collected. Patient-reported outcome measures from the CoULD registry, the Pediatric Outcomes Data Collection Instrument (PODCI), and Patient-Reported Outcomes Measurement Information System (PROMIS) were also analyzed for correlation with IOFS. RESULTS: Two hundred ninety-nine caregivers participated. Factors with significantly stronger impact on family included public insurance; bilateral upper extremity involvement; household income of $20,000-40,000; additional musculoskeletal diagnosis; and a single adult caregiver household. There was a significantly increased subcategory of IOFS-Finance score for distant travel to see the surgeon. Additionally, all categories of the PODCI (upper extremity, mobility, sports, pain, happiness, and global) demonstrated a negative correlation with IOFS. PROMIS upper extremity and peer relations also demonstrated an inverse relationship with IOFS, whereas PROMIS pain interference had a positive correlation with IOFS. The overall IOFS for children with CoULDs was greater than previously reported for children with brachial plexus birth injury, and less than cerebral palsy and congenital heart disease. CONCLUSIONS: Caregivers of children with congenital upper extremity differences report a significant impact on family life. Socioeconomic factors, such as economically disadvantaged or single-caregiver households, and clinical factors, such as bilateral upper extremity involvement, correlate with greater family impact. These findings represent opportunities to identify at-risk families and underscore the importance of caring for the whole family through a multidisciplinary approach. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic II.

Patient and Caregiver Impressions of the Impact of Madelung Deformity: A CoULD Registry Analysis.

PURPOSE: This study seeks to investigate demographics of patients with Madelung deformity in a large, geographically diverse sample and understand patient and caregiver perceptions of the impact of this condition. We hypothesized that patients with untreated Madelung deformity have greater pain and lower function compared to the normal population but are less affected than the chosen control group, namely, patients with proximal radioulnar synostosis (PRUS). METHODS: This retrospective study queried the Congenital Upper Limb Differences (CoULD) Registry, a multicenter registry of patients treated in tertiary care pediatric hospitals. We searched patients enrolled as of July 2022 and identified 3,980 total patients and 66 (1.7%) with a diagnosis of Madelung deformity. We reviewed demographics and Patient-Reported Outcomes Measurement Information System (PROMIS; peer relations, depressive symptoms, pain interference, and upper extremity function domains) scores at time of enrollment. We used a matched cohort comparison with propensity scoring for 50 patients with Madelung deformity and 50 patients with PRUS (control cohort). RESULTS: Patients with Madelung deformity presented at an average age of 13.1 years (± 2.1 years). Ninety-eight percent were female, and 82% were White. Seventy-four percent had distal radius-only deformity. Upper extremity PROMIS scores in both the Madelung deformity and the PRUS groups were significantly "worse" than normal, confirming our hypothesis. The Madelung deformity and PRUS cohort scores were not consistently different from one another. PROMIS scores from all other domains, including pain interference, were similar to, or better than normal for both groups, disproving the second part of our hypothesis. CONCLUSIONS: Patients with Madelung deformity averaged 13 years of age and were nearly all female, and the majority had only distal involvement of the radius. Patients with Madelung deformity had lower function based on PROMIS scores, similar to the control cohort, whereas all other PROMIS measures were similar to or better when compared to normal values. Pain interference scores in both cohorts were lower than normal values. Patients with Madelung deformity have decreased function, similar to the comparative cohort of patients with PRUS, but do not present with increased pain. TYPE OF STUDY/LEVEL OF EVIDENCE: Symptom prevalence III.

Syndactyly Reconstruction Technique Utilizing Skin Substitute.

Syndactyly reconstruction often times leaves areas of skin deficit that require coverage for healing. Numerous techniques have been reported to address the deficits, including graftless technique, which utilizes mobilization of adjacent skin for coverage, full-thickness skin grafting, and skin substitute grafts. The technique described here demonstrates the ease of the use of skin substitutes for coverage in syndactyly reconstruction and reports the expected outcomes.

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.

Study Characteristics and Impact of Pediatric Orthopaedic Society of North America Annual Meeting's "Best Papers" From 2009 to 2019.

OBJECTIVE: Award-winning abstracts are selected every year at the Pediatric Orthopaedic Society of North America (POSNA) annual meeting as "best paper" or poster. It is unknown how many achieve publication in peer-reviewed journals and the impact they have. We sought to determine the characteristics, including the level of evidence (LoE), publication rates, and the impact of award-winning abstracts on pediatric orthopaedic surgery practice from 2009 to 2019. METHODS: Award-winning abstracts or "best papers" from 2009 to 2019 were retrospectively reviewed from the POSNA website from abstract publication to manuscript publication. A search across Pubmed was used to match abstracts to their publications by comparing author names, titles, study design and methodology, results, and conclusions. Area of focus, abstract authors, institutions, publication status, LoE, time to publication, authors of publication, journals of publication, and the journal's latest Hirsch Index and impact factor were recorded. RESULTS: There have been 54 "best papers" at the POSNA annual meeting from 2009 to 2019. Of those, 39 have been published across 17 different journals for a publication rate of 72%. The average time from abstract presentation to publication was 21.2 months with a range of 0 to 121 months. Of the published award-winning abstracts, 64% (25) were published within 2 years, 87% (34) within 3 years, and 95% (37) within 4 years. Out of the published abstracts, 26% (10) were in the Journal of Pediatric Orthopaedics , 23% (9) were in the Journal of Bone and Joint Surgery , and 10% (4) were in the Journal of Child Orthopaedics . The median number of abstract authors was 4 and increased to a median of 6 authors once published. Most award-winning abstracts had a LoE of 3. The average journal impact factor for all publications was 4; the average Hirsch Index for the corresponding author was 29.9, and the average number of citations for a publication was 41 with a range of 0 to 270. CONCLUSIONS: The majority of the "best papers" presented at POSNA annual meetings from 2009 to 2019 were published in peer-reviewed journals within 2 years of presentation, with approximately half being published in the Journal of Pediatric Orthopaedics or Journal of Bone and Joint Surgery . The publication rate of "best papers" at the POSNA annual meeting was found to be higher than rates reported for abstracts presented at the annual meetings of POSNA, American Society for Surgery of the Hand and European Pediatric Orthopaedic Society, but similar to the rates observed for American Association of Hip and Knee Surgeons, American Academy of Orthopaedic Surgeons, and Orthopaedic Trauma Association. Most of the selected "best papers" at the POSNA annual meeting are published and have a substantial impact on pediatric orthopaedic surgery practice. LEVEL OF EVIDENCE: Level IV.

Outcomes of Therapy and Ulnar Nerve Transposition for Elbow Stiffness After Pediatric Medial Epicondyle Fractures.

Purpose: Following medial epicondyle fractures, a subset of pediatric patients has persistent limitations in elbow motion. This study soughted to understand the patient characteristics of this group and to assess the effectiveness of intensive therapy and ulnar nerve transposition in improving elbow range of motion and patient-reported outcomes. Methods: A cohort of 31 pediatric patients with stiffness after elbow trauma was narrowed to 8 pediatric patients (7 female) ranging in age from 9 to 14 years, who were diagnosed with medial epicondyle fractures and underwent intensive therapy and ulnar nerve transposition with or without elbow joint release. We collected demographic and objective data as well as subjective data including Patient-Reported Outcome Measurement Information System (PROMIS) scores before and after ulnar nerve transposition. Results: Following initial intensive therapy, elbow range of motion improved by an average of 56°, and 7 of the 8 patients reached a functional motion arc of 100°. Subsequently, following ulnar nerve surgery with or without elbow release, motion improved by an average of 22°, and 5 of the 8 patients demonstrated improvement from this intervention. Surgery led to improvements in subjective outcomes with an improvement in PROMIS mobility scores by an average of 9 points, pain interference by 6 points, and upper extremity scores by 3 points. Based on a previously determined minimally important difference of three points, these indicate significant clinical improvements. Conclusions: A subset of pediatric patients with persistent stiffness following medial epicondyle fractures may benefit from additional interventions, including intensive therapy, transposition of the ulnar nerve, and open capsular release. However, not all patients were improved after ulnar nerve surgery, and the identification and treatment of ulnar nerve irritability may not fully resolve preoperative symptoms in all patients. Type of study/level of evidence: Therapeutic IV.

Trapeziectomy with Ligament Reconstruction/ Suspensionplasty Compared to Suture Tape Suspensionplasty for the Surgical Treatment of Advanced Thumb Carpometacarpal Osteoarthritis.

Purpose: Trapeziectomy with tendon reconstruction/suspensionplasty (TRS) is the most commonly performed surgical procedure in the United States for treatment of thumb carpometacarpal (CMC) osteoarthritis (OA). Trapeziectomy with suture tape suspensionplasty (STS) has been used recently at the study institution as an alternative surgical treatment option with perceived benefits of earlier return to function and reduced operative time. The purpose of this study was to compare patient outcomes following TRS versus STS for treatment of thumb CMC OA. Methods: All patients who underwent primary, isolated TRS or STS for treatment of thumb CMC OA between 1/1/2014 and 9/1/2020 were analyzed. We assessed demographics and preoperative and postoperative patient-rated outcome scores including Patient-reported outcomes measurement information system scores as well as pain outcomes, satisfaction, and appearance at a mean of 2.6 years after surgery (minimum 6 months). Time to return to work and activities was compared between groups. Bivariate statistics compared outcomes between groups. Results: Ninety-four patients were included in the final study cohort, of which 53 underwent TRS and 41 underwent STS. There were no differences in preoperative, postoperative, or final patient-rated outcome scores between groups. Patients reported high global and appearance satisfaction scores at final follow-up in both groups. Mean tourniquet time was 15 minutes (26%) shorter and return to work was on average 3 weeks faster for the STS group. Conclusions: There were no differences in postoperative patient-rated outcome scores between the STS and TRS groups. The STS group had a shorter surgical time and faster return-to-work after surgery. Type of study/level of evidence: Therapeutic III.

Caregiver Versus Surgeon Concerns in New Diagnosis of Congenital Hand and Upper Extremity Anomalies.

PURPOSE: The initial office visit can be a stressful time for families with children with congenital upper extremity differences. These families may have concerns that differ from the information that pediatric hand surgeons believe is important to discuss. The purpose of this study was to compare topics that families and surgeons believe are most important to address at the initial office visits to better inform and guide that visit. METHODS: Qualitative interviews were conducted with 15 pediatric hand surgeons and 17 caregivers of children with congenital upper extremity differences. Interviews were conducted using semi-structured guides. Interviews were recorded, transcribed, and analyzed using thematic analysis to identify recurring themes from both study groups. RESULTS: Overarching themes were similar between the groups and included diagnosis/cause, treatment, psychosocial considerations, future outcomes, and further resources. However, emphasis among these themes differed, with surgeons emphasizing the cause and events leading up to the diagnosis and caregivers preferring to discuss next steps and treatment options. Surgeons also discussed feelings of grief or guilt more commonly than the caregivers. CONCLUSIONS: Although surgeons emphasize the cause and psychosocial impact of a newly diagnosed congenital upper extremity difference at the initial office visit, caregivers mostly focused on the future and next steps that can be taken. CLINICAL RELEVANCE: Surgeons should ensure to address these issues so that families are comfortable with the treating surgeon and believe that their concerns are being adequately addressed.

Effect of Race and Geography on Patient- and Parent-Reported Quality of Life for Children With Congenital Upper Limb Differences.

PURPOSE: Patient beliefs about health and disability are shaped by many social factors and are a key determinant in their ultimate outcome. We hypothesized that pediatric and parent-reported outcome measures regarding a child's congenital upper limb difference will be affected by geographic location, parent education, sex, ethnicity, race, age, and presence of additional medical comorbidities. METHODS: Patients enrolled in the multicenter Congenital Upper Limb Difference registry were included. Age, sex, race, ethnicity, medical comorbidities, highest level of parental education, area deprivation index, and geographic region were recorded. Patient-Reported Outcomes Measurement Information System (PROMIS) in the pediatric and parent-reported domains of upper extremity, anxiety, pain interference, peer relationships, and depressive symptoms were collected. RESULTS: The only difference between geographic regions in the United States in pediatric and parent-reported PROMIS was that parents in the Midwest reported higher upper extremity function scores in children with upper limb differences than the West. Black patients demonstrated higher scores in parent and child-reported domains of depression, pain, and anxiety, and lower scores in upper extremity function than White and Asian peers. Additionally, children with medical comorbidities also demonstrated worse outcomes in multiple PROMIS domains. There was no difference in scores based on sex, parent education, and ethnicity. CONCLUSIONS: In children with congenital upper limb differences, race and additional medical comorbidities have an impact on patient- and parent-reported PROMIS outcome measures in multiple domains, with Black children and those with additional medical comorbidities scoring lower than their peers. CLINICAL RELEVANCE: As we strive to develop a health care system that provides equitable care to all patients, providers who care for children with upper limb differences should be aware that race and additional medical comorbidities can negatively affect patient- and parent-reported PROMIS outcome measures.

Reliability of the Masada Classification for Forearm Involvement in Patients With Hereditary Multiple Osteochondromas (HMO).

BACKGROUND: Classifications describing forearm lesions in patients with Hereditary Multiple Osteochondromatosis (HMO) have been used to recommend surgical intervention and stratify outcomes; however, there is no consensus on which classification offers greater reliability. The purpose of this study was to determine the reliability of the Masada classification and newer classifications among pediatric hand surgeons. METHODS: One hundred one patients with HMO between June 2014 and October 2019 were enrolled in the Congenital Upper Limb Differences (CoULD) Registry. Of those, 67 patients with 101 forearms were included. Four pediatric hand surgeons from the CoULD study group undertook an online evaluation. Each rater classified radiographs according to the Masada classification. Six weeks later, raters were asked to reclassify images according to the Masada, Gottschalk, and Jo classifications. Rater agreement for these classifications was assessed by estimating Fleiss kappa along with a 95% CI. RESULTS: Interrater agreement for Masada classification after the first reading was poor (κ=0.35; 95% CI=0.30-0.41) across all raters. Interrater agreement across the 4 raters decreased for the Masada classification from the first to the second reading (κ=0.35 vs 0.21; P <0.001). Intrarater agreement for the Masada classification ranged from 0.32 to 0.63 from the first to the second study reading. Gottschalk and Jo classifications yielded significantly better interrater agreement compared with Masada (κ=0.43 vs 0.21; P <0.001). Unclassifiable cases were highest in the Masada classification (34% to 44%) and lower in the Jo (17%) and Gottschalk (14%) classifications. CONCLUSION: Despite wide use, the Masada classification was found to have low reliability when classifying forearm deformities in HMO. Gottschalk offered more options for location, yet lacked deformity description including radial head dislocation. Jo classification offered more locations than Masada and incorporated radial head dislocation in some patterns. Based on the shortcomings in all 3 classification systems, the development of a more inclusive and reliable classification is warranted. LEVEL OF EVIDENCE: Level II; Diagnostic.

Firearm Injuries to the Wrist and Hand in Children and Adults: An Epidemiologic Study.

BACKGROUND: Gunshot wounds (GSWs) to the wrist and hand are a major health and economic burden in the United States. There are few studies examining the circumstances and epidemiological factors surrounding these injuries. This study assesses the epidemiological factors, as well as the shooting circumstances, injury details, and surgical management of wrist and hand GSWs in children and adults. METHODS: Medical records and radiographs were reviewed for all patients with ballistic injury to the wrist or hand treated at an urban academic level 1 trauma center from 2016 to 2019. Fisher exact and Pearson χ2 tests were used to assess differences between groups. RESULTS: Two hundred forty-nine victims (29 children, 220 adults) with complete documentation were identified. Among 180 victims with known shooting circumstances, 132 (70%) were shot by another person and 110 (65%) were injured by intentional gunfire. Eighty-seven victims (35%) suffered a concurrent GSW to another body region. Metacarpal fracture was the most commonly diagnosed bony injury (37%), followed by proximal phalanx fracture (25%). One hundred twenty-nine victims (52%) underwent surgery following their injuries. Nerve discontinuity was diagnosed in 27 victims (11%), while 20 victims (8%) had vascular disruption. There was no significant difference between children and adult victims' type of fracture, concurrent injuries, rates of surgery, or in the most common fracture fixation method. CONCLUSIONS: Most wrist and hand GSW victims were injured due to intentional, non-self-inflicted gunfire. Most patients present with hand fractures, and fortunately, nerve and vascular disruptions are uncommon.

Cost-Effectiveness of Pediatric Hand International Medical Missions.

PURPOSE: Limited access and resources in low- and middle-income countries leave many individuals deprived of medical care. Surgical mission trips offer a solution to provide sound surgical care to underserved areas but require a sizable financial support. Previous analyses of such trips have not included values of donated supplies and costs borne by the host country. We hypothesized that the orthopedic mission trips, utilizing the World Pediatric Project (WPP) model, can be executed in a cost-effective manner according to the World Health Organization thresholds even when considering cost to the organizations and host country. METHODS: World Pediatric Project records for the most recent pediatric upper extremity orthopedic mission trips of 2016, 2018, and 2019 were obtained. Cost estimates were based on documentation from each of the mission trips. Total costs included the costs borne by the WPP, estimates of the value of donated supplies, and costs borne by the host country. The cost-effectiveness of the surgical mission trips was determined by the total cost and potential benefit of performing the orthopedic surgeries using disability-adjusted life years averted. RESULTS: Three separate mission trips to St. Vincent and the Grenadines were analyzed. Forty-five pediatric patients had received surgical care. The cost was calculated to be $431.50 per disability-adjusted life years averted when only the WPP costs are considered; including donated supplies and cost borne by the host country in the total cost, the cost was $6898.10 per disability-adjusted life years averted. After comparing the cost values to the per capita gross domestic product of St. Vincent and the Grenadines, $7,463.54, the WPP mission trips were determined to be cost-effective according to the WHO-CHOICE thresholds in all 5 categories. CONCLUSIONS: Orthopedic medical mission trips can provide cost-effective surgical treatments for the upper extremity even when the costs to the organization and host country and trip donations are considered. TYPE OF STUDY/LEVEL OF EVIDENCE: Economic/Decision Analysis III.

Outcomes of Acute Repair Versus Nonrepair of Zone I Flexor Digitorum Profundus Tendon Injuries.

PURPOSE: The aim of this study was to determine whether the clinical results of zone I flexor digitorum profundus (FDP) tendon injuries managed with acute surgical repair are comparable to the clinical results of those managed without repair (eg, primary FDP excision or observation). METHODS: Patients aged ≥18 years presenting to a level 1 trauma center between 2015 and 2020 with zone I FDP tendon injury were identified with retrospective chart review. We assessed the following data: age, sex, physical therapy visits, surgical intervention, surgical complications (including infection, repeat surgery after the primary intervention, and rupture of repair), and patient-reported outcomes measurement information system scores. RESULTS: Twenty-six patients met the inclusion criteria. Group 1 (N = 15 patients, 23 fingers) patients were treated with acute surgical repair. Group 2 (N = 11 patients, 11 fingers) patients were managed without surgical repair, including FDP excision (N = 7) or observation alone (N = 4). In group 1, the average distance from the distal palmar crease to fingertip at the final follow-up was 1.6 cm (range, 0-4 cm). Fourteen of the 15 patients participated in >3 therapy visits. The following complications occurred: 4 fingers with rerupture (2 patients), 4 fingers with surgical wound dehiscence (2 patients), 3 infections (2 patients), and 4 repeat surgeries for these complications. In group 2, the average distance from the distal palmar crease to fingertip at the final follow-up was 1.1 cm (range, 0.5-3 cm). There were no infections, episodes of wound dehiscence, or repeat surgeries. At the final follow-up, both groups showed clinically meaningful improvement on Patient Reported Outcomes Measurement Information System (PROMIS) upper extremity, pain interference, and physical function scores, with similar PROMIS domain scores between groups. CONCLUSIONS: Patients treated without FDP tendon repair had similar outcomes to, and fewer complications than, patients treated with acute tendon repair. Our data suggest that the notable commitment of health care costs, time, and adherence to protocols/restrictions after surgical repair may not confer functional benefit. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Foveal Triangular Fibrocartilage Complex Tears: Recognition of a Combined Tear Pattern.

PURPOSE: Triangular fibrocartilage complex (TFCC) injuries are categorized most commonly by the Palmer and the Atzei and Luchetti classifications. Combined tears are reported less frequently, and the classification of these tears is unclear. In this study, we present a series of patients with combined central disc and foveal TFCC tears that do not fit into either of these classifications. METHODS: Seventeen patients from 2 institutions presented with a combined central and foveal TFCC tear, confirmed by wrist arthroscopy between September 2017 and December 2020. Patient demographics, injury mechanism, associated injuries, clinical findings, magnetic resonance imaging results, surgeon description of tears, and treatment rendered were evaluated. RESULTS: Patients were predominantly female (76%). Mean age was 40 years with a bimodal age distribution (7/17 patients <35 and 10/17 >45 years). Eleven patients (65%) sustained the injury from a fall. All had some degree of distal radioulnar joint (DRUJ) instability on clinical examination and almost half (8/17) had a history of a distal radius or ulnar styloid fracture during the initial injury. The foveal tear was confirmed by direct visual assessment and a positive hook test result; a suction test result commonly was positive as well. All patients underwent debridement of the central tear, and 16 of 17 underwent foveal TFCC repair with 1 having an irreparable tear. CONCLUSIONS: We present a series of patients with combined central disc and foveal TFCC tears, a pattern that does not fit current classification schemes. All patients exhibited some degree of instability of the DRUJ and almost half had a history of distal radius or ulnar styloid fracture. Identification of a central tear of the TFCC, together with even subtle DRUJ instability, should lead to consideration of a coexistent foveal tear. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

Current Concepts in Upper-Extremity Motion Analysis: Room To Grow?

Kinematic motion analysis (KMA) is well established in the assessment of gait and lower-extremity kinematics; however, its application to upper-extremity (UE) pathology has been limited. This review provides a concise overview of information related to the KMA technology that is pertinent to the clinician. Advantages of KMA for UE assessment are discussed, along with barriers to implementation. An example of KMA used for perioperative assessment of a patient undergoing a distal humerus osteotomy for the correction of arthrogrypotic internal rotation deformity is provided to illustrate its clinical feasibility. Kinematic motion analysis has exciting potential to advance the evaluation and management of UE disorders; however, broad application will require validation and standardization of UE-specific KMA protocols in addition to decreased logistical and cost burdens.

The Oberg, Manske, and Tonkin Classification of Congenital Upper Limb Anomalies: A Consensus Decision-Making Study for Difficult or Unclassifiable Cases.

PURPOSE: An ideal classification system promotes communication and guides treatment for congenital upper limb differences (CULDs). The Oberg, Manske, and Tonkin (OMT) classification utilizes phenotypic presentation and knowledge of developmental biology for the classification of CULDs. In this consensus decision-making study, we hypothesized that CULDs that are difficult to classify would be identically classified by a group of experienced pediatric hand surgeons. METHODS: An international consortium of 14 pediatric hand surgeons in 3 countries contributed a group of 72 difficult-to-classify CULD cases. These were identified from the clinical practices of the surgeons and from associated registries. Through a Delphi-type process, repeated efforts were made to obtain consensus for the correct OMT classification of each case utilizing clinical images and radiographs. RESULTS: The first round of discussion yielded a universal consensus for 57 cases. The remaining 15 cases continued to be put through additional rounds of the Delphi-type process. The repeat classification and discussion resulted in a final yield of 93% complete consensus in classification by the OMT. The primary challenge in diagnosis was differentiating cleft hand from ulnar longitudinal deficiency, identified as group A. Five cases were in this group, yet 2 remained without a clear consensus. Another controversial group, group B, was termed "brachy-polydactyly" and consisted of 3 cases where diagnoses varied between sympolydactyly, symbrachydactyly, or complex syndactyly. CONCLUSIONS: The Delphi-type process was feasible and effective and allowed a 93% consensus in the diagnosis of difficult-to-classify cases by the OMT Classification. There remain limitations and controversies with the OMT system, especially when classifying hands with less than 5 skeletal digits, syndactyly, and those with diagnostic overlap between ulnar longitudinal deficiency and cleft hand and those considered "brachypolydactyly." An improved understanding of the underlying etiology may be needed to determine the final diagnosis in difficult-to-classify conditions. CLINICAL RELEVANCE STATEMENT: A consensus-seeking approach is effective and feasible in addressing difficult-to-classify CULDs.

Functional Outcomes and Health-Related Quality of Life of Adults With Congenital Below-Elbow Amputation in North America.

PURPOSE: Congenital below-elbow amputation (BEA) is a common upper-extremity anomaly and generally encompasses 2 diagnoses, symbrachydactyly and transverse deficiency. Little is known about the physical, mental, and social well-being of adults with congenital BEA. A deeper understanding of longitudinal outcomes within this population may help guide family conversations and counseling for patients with congenital BEA. METHODS: The Shriners Hospitals for Children Health Outcomes Network was queried to identify all patients currently >18 years of age who had been seen as a child between 1975 and 2019 for congenital BEA at 1 of 20 Shriners Hospitals across North America. A unique health survey examining physical functioning, mental health, social outcomes, and health-related quality of life was constructed and sent by mail or in electronic form to eligible patients. RESULTS: A total of 64 questionnaires were completed. Patients ranged between 18 and 34 years of age, and 70% were female. Nearly two-thirds of patients (64%) reported that a prosthesis was not required and only 14% reported daily prosthetic use. Although respondents reported below-average Patient-Reported Outcomes Measurement Information System (PROMIS) upper-extremity scores, there were no differences in Short-Form 12 or Quick Disabilities of the Arm, Shoulder, and Hand scores relative to the US general population. Study participants had lower PROMIS Pain Intensity and higher PROMIS satisfaction with social roles and activities scores than the US general population, translating to clinically meaningful differences. CONCLUSIONS: Although adults with congenital BEA report lower upper-extremity functional scores than the general population, they report no clear differences from normative values in self-efficacy, psychosocial well-being, health-related quality of life, or global life satisfaction. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

An Analysis of Associated Conditions and the Relationship Between the Severity of Hand Manifestations With That of the Forearm in Ulnar Longitudinal Deficiency.

PURPOSE: A deeper investigation of medical and musculoskeletal conditions in patients with ulnar longitudinal deficiency (ULD) is needed. The association between the severity of the manifestations of ULD in the hands and forearms has not been firmly established. The purpose of this study was to describe the medical and musculoskeletal conditions associated with ULD and examine the relationship between hand and forearm anomalies. METHODS: The Congenital Upper Limb Differences registry was queried for all patients with a diagnosis of ULD, as defined by the Oberg-Manske-Tonkin classification system, between 2014 and 2020. The patients' demographic information, medical and musculoskeletal comorbidities, radiographs, and clinical images were reviewed. The participants were classified using the Bayne, Cole and Manske, and Ogino classification systems. RESULTS: Of 2,821 patients from the Congenital Upper Limb Differences registry, 75 patients (2.7%) with ULD (14 bilateral), with 89 affected extremities, were included. Hand anomalies were present in 93% of the patients. Approximately 19% of the patients had an associated medical comorbidity, and 20% of the patients had an associated musculoskeletal condition. Cardiac anomalies were present in 8.0% of the patients, and 12% of the patients had a lower extremity abnormality. Radial head dislocation was observed in 13 of 18 patients with Bayne type II or III ULD compared with 8 of 43 patients with other types of unilateral ULD. There was a significant positive association among the Bayne and Ogino, Bayne and Cole/Manske, and Ogino and Cole/Manske classification systems in patients with unilateral ULD. CONCLUSIONS: Associated medical and musculoskeletal conditions are common in patients with ULD, of which cardiac and lower extremity abnormalities are most frequently observed. There is a significant positive association between the severity of forearm anomalies and that of hand anomalies in patients with unilateral ULD. All patients with ULD should undergo a thorough cardiac evaluation by their pediatrician or a pediatric cardiologist. TYPE OF STUDY/LEVEL OF EVIDENCE: Symptom prevalence study III.

The Impact of Isolated Versus Multiple Osteochondromas: Analysis of the CoULD Registry.

PURPOSE: The burden of upper extremity (UE) osteochondromas on function and self-perception among pediatric patients is unclear. The purpose of our study was to study the impact of osteochondromas in comparison to population norms and to evaluate solitary versus multiple osteochondromas on subjective UE function as measured by patient rated outcomes. METHODS: We utilized the CoULD (Congenital Upper Limb Differences) Registry to review all pediatric patients presenting with osteochondromas between January 2014 and February 2021. Demographic information was collected and patients were classified as having either single or multiple osteochondromas. Patient-Reported Outcome Measurement Information System (PROMIS) and Pediatric Outcomes Data Collection Instrument (PODCI) tools were utilized for assessment. Scores for PODCI subscales of UE function, Pain/comfort, and Happiness and PROMIS domains of UE Function, Pain, Depression, Anxiety, and Peer Relations were reviewed. Differences between groups were analyzed using the Student t test. RESULTS: Ninety-nine patients met inclusion criteria for the study with an average age of presentation of 9.3 years and 61 patients (62%) were male. Overall, patients demonstrated worse UE Function as well as greater Anxiety and Depression in comparison to the population normals on PROMIS assessment. Patients also demonstrated worse patient and parent reported PODCI UE, Sports and Physical Functioning, Pain/Comfort and Global Functioning scores compared with population norms but demonstrated better than average happiness scores. Patients with multiple osteochondromas demonstrated greater PROMIS pain interference and more disability in PODCI Sports and Physical Functioning, Pain/Comfort and Global Functioning compared with those with solitary osteochondromas. CONCLUSION: Patients with UE osteochondromas have worse overall function in comparison to population norms, exceeding established minimally clinically important difference values. In addition, patients with multiple osteochondromas reported more pain and poorer physical function than those with solitary osteochondromas. Physicians should be alert to the physical and psychosocial burden of this disease. LEVEL OF EVIDENCE: Level II-prognostic.