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.

The Effects of Radial Longitudinal Deficiency on Long-Term Use of the Thumb in Pediatric Patients Following Index Pollicization.

PURPOSE: To assess the effect of radial longitudinal deficiency on the function of pollicized digits as determined by the Thumb Grasp and Pinch (T-GAP) assessment. METHODS: We retrospectively evaluated 25 hands with thumb hypoplasia that underwent index finger pollicization. Patients were followed for an average of 10.4 years. Hands were divided by severity into two groups: no or mild radial longitudinal deficiency (RLD) (Group 1 = 16) and moderate to severe RLD (Group 2 = 9). We collected demographic information and completed physical examination measures, including hand strength, elbow, wrist, and hand range of motion, the Kapandji opposition score, active grasp span, and T-GAP total score. RESULTS: Patients with moderate to severe forms of RLD had stiffer long fingers, lower Kapandji opposition scores, and limited active and passive range of motion for elbow flexion, wrist ulnar deviation, and pollicized thumb interphalangeal flexion. They had shorter forearms, decreased active grasp span, and fewer thumb creases at the interphalangeal thumb joint. In addition, the T-GAP total score was significantly lower when comparing the two groups. Children with mild dysplasia were able to achieve 32% of age-matched normal grasp strength. Patients with more severe radial dysplasia averaged 17% less grasp strength compared with children with mild dysplasia. Patients with moderate to severe RLD also had lower T-GAP total scores and strength measurements if they had limited wrist ulnar deviation. CONCLUSIONS: Individuals with moderate to severe RLD have unique anatomical factors that affect outcomes after pollicization. These individuals use their thumbs for fewer activities, have weaker grasp, and retain more primitive grasp patterns compared with those who have milder forms of RLD. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic 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.

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.

Complications in the 2-Year Postoperative Period Following Pediatric Syndactyly Release.

PURPOSE: Syndactyly surgical release is one of the most common congenital hand surgeries performed by pediatric hand surgeons. The purpose of our study was to evaluate the complications associated with syndactyly release and determine factors that correlate with higher complication rates within the 2-year postoperative period. METHODS: A retrospective chart review was completed for patients who underwent syndactyly release at a single pediatric center between 2005 and 2018. Patients were included if they had a diagnosis of syndactyly and underwent surgical release, and excluded for a diagnosis of cleft hand, incomplete surgical documentation, surgery performed at an outside institution, or follow-up care that did not extend beyond the first postoperative visit. Complications were classified using the Clavien-Dindo (CD) system. RESULTS: Fifty-nine patients met the inclusion criteria, which included 143 webs released in 85 surgeries. A total of 27 complications occurred for the 85 surgeries performed. The severity of complications was CD grade I or II in 23% of surgeries, most commonly unplanned cast changes, and CD grade III in 8% of surgeries. No CD grade IV or V complications occurred. The CD grade III complications included 6 reoperations. The complication rate was higher when performing >1 syndactyly release per surgery. It also was higher for patients undergoing >1 surgical event. Rates of complication per surgery were similar between patients with multiple surgeries compared with those with a single surgery. Concomitant diagnoses and complexity of syndactyly was not associated with a higher complication rate. CONCLUSIONS: Syndactyly release was associated with a complication rate of 31% per surgical event with 44% of these complications related to unplanned cast changes and 8% of complications that required admission or reoperation. Risk factors for complications following syndactyly release include >1web operated on per surgery and undergoing >1 surgical event. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognosis IV.

Cost Comparison of Botulinum Toxin Injections Versus Surgical Treatment in Pediatric Patients With Cerebral Palsy: A Markov Model.

PURPOSE: The purpose of this study was to compare the cost-effectiveness of surgical release to botulinum toxin injections in the treatment of upper-extremity (UE) cerebral palsy (CP). METHODS: A Markov transition-state model was developed to assess the direct and indirect costs as well as accumulated quality-adjusted life-years associated with surgery (surgery group) and continuous botulinum toxin injections (botulinum group) for the treatment of UE CP in children aged 7 to 12 years. Direct medical costs were obtained from institutional billing departments. The number of parental missed workdays associated with each treatment was estimated and previously published regressions were used to calculate indirect costs associated with missed work. Total costs, cost-effectiveness, and incremental cost-effectiveness ratios were calculated. Incremental cost-effectiveness ratios and willingness to pay thresholds were used to make decisions regarding society's willingness to pay for the incremental cost of each treatment given the incremental benefit. RESULTS: The surgery group demonstrated lower direct, indirect, and total costs compared with the botulinum group. Direct costs were $29,250.50 for the surgery group and $50,596.00 for the botulinum group. Indirect costs were $9,467.46 for the surgery group and $44,428.60 for the botulinum group. Total costs were $38,717.96 for the surgery group and $95,024.60 for the botulinum group, a difference of $56,306.64. The incremental cost-effectiveness ratio was -$42,019.88, indicating that surgery is a less costly and more effective treatment and that botulinum injections fall outside the societal willingness to pay threshold. Excluding indirect costs associated with parental missed work during home occupational therapy did not have a significant impact on the model. CONCLUSIONS: Surgery is associated with lower direct, indirect, and total costs, as well as a greater number of accumulated quality-adjusted life-years. Surgery provides a greater benefit at a lower cost, which suggests that botulinum injections should be used sparingly in this population. Treatment with surgery could represent savings of $5.6 to $11.3 billion annually in the United States. TYPE OF STUDY/LEVEL OF EVIDENCE: Economic/Decision Analysis II.

Defining Features of Hand Anomalies in Severe Thumb Hypoplasia: A Classification Modification.

PURPOSE: To investigate morphologic hand anomalies in children with severe but unclassifiable forms of thumb hypoplasia and radial-sided hand deficiency. METHODS: We identified 15 extremities in 13 patients with severe thumb hypoplasia and associated absent radial-sided digits through the Congenital Upper Limb Differences registry. All patients had forearm involvement. Medical records, clinical photographs, and radiographs were evaluated. Radial longitudinal deficiency (RLD) and thumb hypoplasia were classified according to the Bayne and Klug classification and modified Blauth classification, respectively. Unusual or defining associated hand characteristics were identified and categorized. RESULTS: The most common type of forearm abnormality was absence of the radius (Bayne and Klug type IV), which was present in 10 extremities in the cohort. All 15 extremities had absent thumbs with loss of additional digits. In 6 patients, RLD was part of a syndrome (46%). CONCLUSIONS: Severe forms of thumb hypoplasia in RLD are uncommon. We propose a further modification of the Blauth classification of thumb hypoplasia, type VI, for improved communication regarding this severe type of radial deficiency involving the hand. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

A Comparative Analysis of 150 Thumb Polydactyly Cases from the CoULD Registry Using the Wassel-Flatt, Rotterdam, and Chung Classifications.

PURPOSE: Three commonly used classifications for thumb polydactyly are the Wassel-Flatt, Rotterdam, and Chung. The ideal classification system would have high validity and reliability and be descriptive of the thumb anomaly. The purposes of this investigation were to (1) compare the inter- and intrarater reliability of these 3 classifications when applied to a large sample of patients enrolled in the Congenital Upper Limb Differences (CoULD) Registry and (2) determine the prevalence of radial polydactyly types when using the various classifications in a North American population. METHODS: Inter- and intrarater reliability were determined using 150 cases of radial polydactyly presented in a Web-based format to 7 raters in 3 rounds, a preliminary training round and 2 observation rounds. Raters classified each case according to the Wassel-Flatt, Rotterdam, and Chung classifications. Inter- and intrarater reliability were evaluated with the intraclass correlation coefficient (ICC) calculated using 2-way random measures with perfect agreement. RESULTS: For Wassel-Flatt, both the interrater (ICC, 0.93) and the intrarater reliability (ICC, 0.91) were excellent. The Rotterdam classification had excellent reliability for both interrater reliability (ICC, 0.98) and intrarater reliability (ICC, 0.94), when considering type alone. Interrater analysis of the additional subtypes demonstrated a wide range of reliabilities. The Chung classification had good interrater (ICC, 0.88) and intrarater reliability (ICC, 0.77). Within the Wassel-Flatt classification, the most frequent unclassifiable thumb was a type IV hypoplastic thumb as classified by the Rotterdam classification. CONCLUSIONS: The Wassel-Flatt and Rotterdam classifications for radial polydactyly have excellent inter- and intrarater reliability. Despite its simplicity, the Chung classification was less reliable in comparison. The Chung and Rotterdam classification systems capture the hypoplastic subtypes that are unclassifiable in the Wassel-Flatt system. Addition of the hypoplastic subtype to the Wassel-Flatt classification (eg, Wassel-Flatt type IVh) would maintain the highest reliability and classify over 90% of thumbs deemed unclassifiable in the Wassel-Flatt system. CLINICAL RELEVANCE: The Wassel-Flatt and Rotterdam classifications have excellent inter-and intrarater reliability for the hand surgeon treating thumb polydactyly. Addition of a hypoplastic subtype to the Wassel-Flatt (Type 4h) allows classification of most previously unclassifiable thumbs.

What Proportion of Orthopaedic Surgery Residency Programs Have Accessible Parental Leave Policies, and How Generous are They?

BACKGROUND: Parental leave during graduate medical education is a component of wellness in the workplace. Although every graduate medical education program is required by the Accreditation Council for Graduate Medical Education (ACGME) to have a leave policy, individual programs can create their own policies. The ACGME stipulates that "the sponsoring institution must provide a written policy on resident vacation and other leaves of absence (with or without pay) to include parental and sick leave to all applicants." To our knowledge, a review of parental leave policies of all orthopaedic surgery residency programs has not been performed. QUESTION/PURPOSES: (1) What proportion of orthopaedic surgery residency programs have accessible parental (maternity, paternity, and adoption) leave policies? (2) If a policy exists, what financial support is provided and what allotment of time is allowed? METHODS: All ACGME-accredited orthopaedic surgery residency programs in 2017 and 2018 were identified. One hundred sixty-six ACGME-accredited allopathic orthopaedic surgery residency programs were identified and reviewed by two observers. Reviewers determined if a program had written parental leave policy, including maternity, paternity, or adoption leave. Ten percent of programs were contacted to verify reviewer findings. The search was sequentially conducted starting with the orthopaedic surgery residency program's website. If the information was not found, the graduate medical education (GME) website was searched. If the information was not found on either website, the program was contacted directly via email and phone. Parental leave policies were classified as to whether they provided dedicated parental leave pay, provided sick leave pay, or deferred to unpaid Family Medical Leave Act (FMLA) policies. The number of weeks of maternity, paternity, and adoption leave allowed was collected. RESULTS: Our results showed that 3% (5 of 166) of orthopaedic surgery residency programs had a clearly stated policy on their program website. Overall, 81% (134 of 166) had policy information on the institution's GME website; 7% (12 of 166) of programs required direct communication with program coordinators to obtain policy information. Further, 9% (15 of 166) of programs were deemed to not have an available written policy as mandated by the ACGME. A total of 21% of programs (35 of 166) offered designated parental leave pay, 29% (48 of 166) compensated through sick leave pay, and 50% (83 of166) deferred to federal law (FMLA) requiring up to 12 weeks of unpaid leave. CONCLUSIONS: Although 91% of programs meet the ACGME requirement of written parental leave policies, current parental leave policies in orthopaedic surgery are not easily accessible for prospective residents, and they do not provide clear compensation and length of leave information. Only 3% (5 of 166) of orthopaedic surgery residency programs had a clearly stated leave policy accessible on the program's website. Substantial improvements would be gained if every orthopaedic residency program clearly outlined the parental leave policy on their residency program website, including compensation and length of leave, particularly in light of the 2019 American Board of Orthopaedic Surgery changes allowing time away to be averaged over the 5 years of training. CLINICAL RELEVANCE: Parental leave policies are increasingly relevant to today's trainees []. Applicants to orthopaedic surgery today value work/life balance including protected parental leave [].

Improving Our Ability to Predict Resident Applicant Performance: Validity Evidence for a Situational Judgment Test.

Construct: We investigated whether a situational judgment test (SJT) designed to measure professionalism in physicians predicts residents' performance on (a) Accreditation Council for Graduate Medical Education (ACGME) competencies and (b) a multisource professionalism assessment (MPA). Background: There is a consensus regarding the importance of assessing professionalism and interpersonal and communication skills in medical students, residents, and practicing physicians. Nonetheless, these noncognitive competencies are not well measured during medical education selection processes. One promising method for measuring these noncognitive competencies is the SJT. In a typical SJT, respondents are presented with written or video-based scenarios and asked to make choices from a set of alternative courses of action. Interpersonally oriented SJTs are commonly used for selection to medical schools in the United Kingdom and Belgium and for postgraduate selection of trainees to medical practice in Belgium, Singapore, Canada, and Australia. However, despite international evidence suggesting that SJTs are useful predictors of in-training performance, end-of-training performance, supervisory ratings of performance, and clinical skills licensing objective structured clinical examinations, the use of interpersonally oriented SJTs in residency settings in the United States has been infrequently investigated. The purpose of this study was to investigate whether residents' performance on an SJT designed to measure professionalism-related competencies-conscientiousness, integrity, accountability, aspiring to excellence, teamwork, stress tolerance, and patient-centered care-predicts both their current and future performance as residents on two important but conceptually distinct criteria: ACGME competencies and the MPA. Approach: We developed an SJT to measure seven dimensions of professionalism. During calendar year 2017, 21 residency programs from 2 institutions administered the SJT. We conducted analyses to determine the validity of SJT and USMLE scores in predicting milestone performance in ACGME core competency domains and the MPA in June 2017 and 3 months later in September 2017 for the MPA and 1 year later, in June 2018, for ACGME domains. Results: At both periods, the SJT score predicted overall ACGME milestone performance (r = .13 and .17, respectively; p < .05) and MPA performance (r = .19 and .21, respectively; p < .05). In addition, the SJT predicted ACGME patient care, systems-based practice, practice-based learning and improvement, interpersonal and communication skills, and professionalism competencies (r = .16, .15, .15, .17, and .16, respectively; p < .05) 1 year later. The SJT score contributed incremental validity over USMLE scores in predicting overall ACGME milestone performance (ΔR = .07) 1 year later and MPA performance (ΔR = .05) 3 months later. Conclusions: SJTs show promise as a method for assessing noncognitive attributes in residency program applicants. The SJT's incremental validity to the USMLE series in this study underscores the importance of moving beyond these standardized tests to a more holistic review of candidates that includes both cognitive and noncognitive measures.

Women in Orthopaedics: How Understanding Implicit Bias Can Help Your Practice.

Women comprise approximately 50% of medical students; however, only 14% of current orthopaedic residents are women. There are many factors that contribute to the reluctance of female medical students to enter the field including limited exposure to musculoskeletal medicine during medical school, negative perception of the field, lack of female mentors, barriers to promotion, and acceptance by senior faculty. Diversity in orthopaedics is critical to provide culturally competent care. Two pipeline programs, the Perry Initiative and Nth Dimensions, have successful track records in increasing female and underrepresented minorities in orthopaedic surgery residency training. Recognizing and combating implicit bias in orthopaedics will improve recruitment, retention, promotion, and compensation of female orthopaedic surgeons. The purpose of this chapter is to provide an overview of the current status of women in orthopaedics, describe ways to improve diversity in the field, and make surgeons aware of how implicit bias can contribute to discrepancies seen in orthopaedic surgery, including pay scale inequities and women in leadership positions.

Graded motor imagery for women at risk for developing type I CRPS following closed treatment of distal radius fractures: a randomized comparative effectiveness trial protocol.

BACKGROUND: Distal radius fractures (DRF) account for nearly one-fifth of all fractures in older adults, and women experience them 5× as often as men. Most DRF occur with low impact injuries to the wrist with an outstretched hand, and are often managed via closed treatment and cast immobilization. Women sustaining a DRF are at risk for upper limb immobility, sensorimotor changes, edema and type I complex regional pain syndrome (CRPS). Since CRPS onset is likely influenced by alterations in the brain's somatosensory region, a rehabilitation intervention, Graded Motor Imagery (GMI), aims to restore cortical representation, including sensory and motor function, of the affected limb. To date, there are no studies on the use of GMI in reducing risk of or preventing the onset of type I CRPS in women with DRF treated with cast immobilization. Due to a higher likelihood of women with this injury developing type I CRPS, it is important to early intervention is needed. METHODS/DESIGN: This article describes a six-week randomized comparative effectiveness trial, where the outcomes of a modified GMI program (mGMI) + standard of care (SOC) group (n = 33) are compared to a SOC only control group (n = 33). Immediately following cast immobilization, both groups participate in four 1-h clinic-based sessions, and a home program for 10 min three times daily until cast removal. Blinded assessments occur within 1 week of cast immobilization (baseline), at three weeks post cast immbolization, cast removal, and at three months post cast removal. The primary outcomes are patient reported wrist/hand function and symptomology on the Patient Rated Wristand Hand Evaluation, McGill Pain Questionnaire, and Budapest CRPS Criteria. The secondary outcomes are grip strength, active range of motion as per goniometry, circumferential edema measurements, and joint position sense. DISCUSSION: This study will investigate the early effects of mGMI + SOC hand therapy compared to SOC alone. We intend to investigate whether an intervention, specifically mGMI, used to treat preexisiting pain and motor dysfunction might also be used to mitigate these problems prior to their onset. If positive effects are observed, mGMI + SOC may be considered for incorporation into early rehabilitation program. TRIAL REGISTRATION: This trial is registered at ClinicalTrials.gov with identifier NCT02957240 (Approval date: April 20, 2017).

A New, Direct Measure of Thumb Use in Children After Index Pollicization for Congenital Thumb Hypoplasia.

PURPOSE: After index pollicization for congenital thumb hypoplasia, time-based hand dexterity tests do not indicate whether the new thumb is being used by a child. The Thumb Grasp and Pinch assessment (T-GAP) is a new outcome measure that classifies grasp and pinch styles to quantify use of the new thumb. The goal of this study was to establish concurrent validity and construct validity in the T-GAP. METHODS: Data from children treated with index finger pollicization for congenital thumb hypoplasia were retrospectively reviewed. Measures of strength, range of motion, and scores on the Box and Blocks Test (BBT), 9-Hole Peg Test (NHPT), Functional Dexterity Test (FDT), and Task 7 (Heavy Objects) from the Jebsen-Taylor Test (JTT7) were recorded. Patients also completed the T-GAP consisting of 9 age-appropriate tasks, during which grasp patterns were classified. Spearman correlation coefficients were calculated comparing the T-GAP score with scores on the BBT, NHPT, FDT, and JTT7. RESULTS: We evaluated 21 thumbs in 21 children an average of 71.7 months after pollicization surgery (range, 9-175 months). The T-GAP score was significantly correlated with BBT, NHPT, FDT, and JTT7 (R = 0.69, -0.60,-0.59, and -0.60, respectively). The T-GAP score was significantly correlated with tripod pinch, key pinch, and grip strength (R = 0.77, 0.75, and 0.71, respectively) and with opposition and grasp span (R = 0.50 and 0.52, respectively). The T-GAP was the only functional measure correlated with parent and patient satisfaction with thumb function. CONCLUSIONS: Concurrent validity was supported by significant correlations between T-GAP score for all 4 dexterity measures. Construct validity was supported by significant correlations between strength and range of motion of the thumb and T-GAP score. CLINICAL RELEVANCE: This evaluation may help surgeons and therapists better understand results after pollicization and determine whether the new thumb is being incorporated into daily activities.

The Frequency of Carpal Tunnel Syndrome in Hurler Syndrome After Peritransplant Enzyme Replacement Therapy: A Retrospective Comparison.

PURPOSE: Children with Hurler syndrome (HS) develop carpal tunnel syndrome (CTS) owing to glycosaminoglycan deposition secondary to enzyme deficiency. Advancement in the treatment of the underlying enzyme deficiency now commonly includes peritransplant intravenous enzyme replacement therapy (ERT). The primary objective of this study was to determine if the use of limited ERT in addition to hematopoietic stem cell transplantation (HCT) for the treatment of children with HS reduces the incidence of surgical intervention for CTS compared with a cohort of historical controls treated with HCT alone. The secondary objectives were to evaluate the impact of demographic and transplant-related characteristics on the incidence of CTS. Lastly, the results of surgical treatment of CTS in HS are reported. METHODS: Medical records for a historical group of 43 HS patients who underwent HCT alone (group 1) were compared with 31 HS patients who underwent HCT + ERT (group 2). Both groups were compared for genotype, age at transplant, sex, transplant graft source, median/ulnar nerve conduction study parameters as well as the incidence and treatment of CTS. Pre- and postoperative nerve conduction studies were compared for children treated surgically for CTS. RESULTS: The cumulative incidence of CTS at 5 years for HS children treated with HCT + ERT was 51% compared with 47% for HS children treated with HCT alone. The incidence of CTS did not depend upon graft source, age at transplant, or sex. Median nerve conduction velocity for both sensory and motor potentials demonstrated significant improvement after carpal tunnel release. CONCLUSIONS: Although the administration of ERT prior to and for several months after HCT has become routine in our institution, our findings do not suggest this combined therapy is sufficient to decrease the development of CTS. Surgical intervention for median nerve compression remains the treatment of choice for CTS in HS children. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Correlation Between Standard Upper Extremity Impairment Measures and Activity-based Function Testing in Upper Extremity Cerebral Palsy.

BACKGROUND: Although the treatment of cerebral palsy should be based on improving function as assessed by measures of impairment, activity, and participation, the standard indications for surgical treatment of upper extremity cerebral palsy (UECP) are impairment measures, primarily active and passive range of motion (ROM). Recently, validated activity measures have been developed for children with UECP. The purposes of this study were to determine the relationship between impairment and activity measures in this population, and whether measures of activity correlate with each other. METHODS: A total of 37 children, ages 5 to 16 years, who met standard ROM surgical indications for UECP were evaluated with the impairment measures of active and passive ROM and stereognosis, as well as 3 activity measures [Assisting Hand Assessment (AHA), Box and Blocks test, and the Shriners Hospitals Upper Extremity Evaluation Dynamic Positional Analyses (SHUEE DPA)]. Impairment measures were correlated with activity measures using Spearman rank correlation coefficients. RESULTS: Impairment measures showed inconsistent correlation with activity measures. Of the 12 comparisons, only 4 correlated: active forearm supination (ρ=0.47, P=0.003), wrist extension (ρ=0.55, P=0.001), and stereognosis scores (ρ=0.54, P=0.001) were correlated with AHA; and wrist extension was correlated with the SHUEE DPA (ρ=0.41, P=0.01). When the results of activity tests were compared, the AHA was correlated with the Box and Blocks tests (ρ=0.63, P<0.001), and the SHUEE DPA and Box and Blocks tests were correlated with each other (ρ=0.35, P=0.04). CONCLUSIONS: The goal of surgery in UECP is to improve the child's ability to perform activities, and ultimately to participate in life situations. Impairment measures, such as ROM, were inconsistently correlated with validated measures of activity. Some activity measures correlated with each other, although they did not correlate with the same impairment measures. We conclude that impairment measures, including ROM, do not consistently predict functional dynamic ROM used to perform activities for children with UECP. Activity limitation measures may provide more appropriate indicators than impairment measures for upper extremity surgery for this population. LEVEL OF EVIDENCE: Level II-diagnostic.

Idiopathic Fenestrated Complex Syndactyly in a Unique Crisscross Fashion.

This case presents surgical treatment of a unique form of syndactyly: an isolated fenestrated, complex, crisscross syndactyly of the right middle and ring fingers. A 2-year-old boy presented with the ring finger lying dorsal and the middle finger lying volar, with the middle phalanges syndactylized. A surgical release was performed with a subsequent z-plasty, 2 years later, for scar elongation. At the age of 4, he has essentially full function of his hand with minimal limitations. This case demonstrates that 2 digits that were syndactylized in a coronal plane (ring finger dorsal and middle finger volar) can be successfully surgically separated.

The Effect of Treatment on Stereognosis in Children With Hemiplegic Cerebral Palsy.

PURPOSE: To determine if rehabilitation alone or combined with surgery or botulinum toxin injection improved stereognosis in children with hemiplegic cerebral palsy. METHODS: Inclusion criteria were children with spastic hemiplegic cerebral palsy who had stereognosis testing 2 separate times with documentation of intervening treatment. Sixty-three children were included, 30 girls and 33 boys at an average age of 9.1 years (range, 4.4-16.0 years). Twelve standardized objects were used for manual identification. Baseline and postintervention stereognosis results were recorded for the hemiplegic and the dominant limb of each patient. The patients were separated into 3 groups based on intervening treatment: surgery with rehabilitation (27 patients), botulinum toxin injection with rehabilitation (19 subjects), and rehabilitation alone (7 subjects). Results were also analyzed by patient age group. RESULTS: Baseline testing of the hemiplegic limb revealed that 27 patients (43%) exhibited severe stereognosis impairment (0-4 objects identified correctly), 18 (28%) were moderately impaired (5-8 objects), 13 (21%) were mildly impaired (9-11 objects), and 5 (8%) had intact stereognosis (12 objects). There was no statistically significant difference in change in stereognosis scores postintervention among the 3 different treatment groups or between patients who had surgery and those who did not have surgery. There was no statistically significant difference in stereognosis function or postintervention change based on patient age at time of testing. CONCLUSIONS: In this study, 92% of children with spastic hemiplegic cerebral palsy had stereognosis impairment with a wide spectrum of severity. After operative or nonoperative treatment interventions, stereognosis as a secondary outcome measure was not changed. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.

Male and female differences in musculoskeletal disease.

Gender differences exist in the presentation of musculoskeletal disease, and recognition of the differences between men and women's burden of disease and response to treatment is key in optimizing care of orthopaedic patients. The role of structural anatomy differences, hormones, and genetics are factors to consider in the analysis of differential injury and arthritic patterns between genders.

Development and validation of a basic arthroscopy skills simulator.

PURPOSE: The purpose of our study was to develop a low-fidelity surgical simulator for basic arthroscopic skills training, with the goal of creating a pretrained novice ready with the basic skills necessary for all joint arthroscopic procedures. METHODS: A panel of education, arthroscopy, and simulation experts designed and evaluated a basic arthroscopic skills training and testing box. Task deconstruction was used to create 2 modules, which incorporate core skills common to all arthroscopic procedures. Core metrics measured were time to completion, number of trials to steady state, and number of errors. Face validity was evaluated using a questionnaire. Construct validity was examined by comparing 8 medical students with 8 expert orthopaedic surgeons. RESULTS: Surgeons were faster than students on both module 1 (P = .0013), simulating triangulation skills, and module 2 (P = .0190) simulating object manipulation skills. Surgeons demonstrated fewer errors (6.9 errors versus 28.1; P = .0073). All surgeons were able to demonstrate steady state (i.e., perform 2 trials that were within 10% of each other for time to completion and errors) on both modules within 3 trials on each module. Only 2 novices were able to demonstrate steady state on either module, and both did so within 3 trials. Furthermore, face validity of the skills trainer was shown by the expert arthroscopists. CONCLUSIONS: We describe a basic arthroscopy skills simulator that has face and construct validity. Our expert panel was able to design a simulator that differentiated between experienced arthroscopists and novices. CLINICAL RELEVANCE: Surgical simulation is an important part of efficient surgical education. This simulator shows good construct and face validity and provides a low-fidelity option for teaching the entry-level arthroscopist.

Management of the spastic wrist and hand in cerebral palsy.

Research from the last 5 years on the pathophysiology and treatment of upper extremity sequelae of cerebral palsy (CP) is presented. The development of new treatments of CP-affected limbs, utilizing the brain's inherent neuroplasticity, remains an area of promising and active research. Functional magnetic resonance imaging scans have evaluated the role of neuroplasticity in adapting to the initial central nervous system insult. Children with CP appear to have greater recruitment of the ipsilateral brain for motor and sensory functions of the affected upper limb. Studies have also shown that constraint-induced movement therapy results in localized increase in gray matter volume of the sensorimotor cortex contralateral to the affected arm targeted during rehabilitation. Recent therapy interventions have emphasized the role of home therapy programs, the transient effects of splinting, and the promise of constraint-induced movement therapy and bimanual hand training. The use of motion laboratory analysis to characterize the movement pattern disturbances in children with CP continues to expand. Classification systems for CP upper limb continue to expand and improve their reliability, including use of the House Classification, the Manual Ability Classification System, and the Shriner's Hospital Upper Extremity Evaluation. Surgical outcomes have greater patients' satisfaction when they address functional limitations, also in addition to aesthetics, which may improve patients' self-esteem. Surgical techniques for elbow, wrist, fingers, and thumb continue to be refined. Research into each of these areas continues to expand our understanding of the nervous system insults that cause CP, how they may be modified, and how hand surgeons can continue to serve patients by improving their upper limb function and aesthetics.