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.

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.

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 [].

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.

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.

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.

Affected and contralateral hand strength and dexterity measures in children with hemiplegic cerebral palsy.

PURPOSE: To determine how the affected hemiplegic hand and contralateral dominant hand in children with hemiplegic cerebral palsy compare with age-matched norms for grip strength, pinch strength, and dexterity. METHODS: We enrolled 37 children with hemiplegic cerebral palsy (26 boys; average age, 9.8 y). Grip and pinch strength and Box and Blocks Test for dexterity were measured in both hands. Affected and contralateral hands results were analyzed and compared with each other and with norms for age and sex. RESULTS: Affected hands had significantly less grip and pinch strength than the contralateral hands. Subjects transported significantly fewer blocks in one minute with the Box and Blocks Test (mean, 10.8 blocks) with the affected hand than the contralateral hand. Compared with normative values, affected-side grip and pinch strengths were significantly less, whereas contralateral hand grip and pinch strengths were similar. Dexterity in both affected and contralateral hands was significantly less than normative values. Decreased dexterity in the contralateral hand was correlated with decreased nonverbal intelligence quotient. CONCLUSIONS: Dexterity of the contralateral hand is diminished in children with hemiplegia. Assessment of the contralateral hand may reveal opportunities for therapeutic intervention that improve fine motor function. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Epidemiology of congenital upper limb anomalies in a midwest United States population: an assessment using the Oberg, Manske, and Tonkin classification.

PURPOSE: To examine the relative presentation frequency of children with upper limb congenital anomalies at 3 Midwestern referral centers using the Oberg, Manske, and Tonkin (OMT) classification and to assess the utility of this new classification system. METHODS: 641 individuals with 653 congenital upper extremity anomalies were identified at 3 hospitals in 2 large metropolitan areas during a 1-year interval. Patients were identified prospectively and the specific upper extremity anomaly and any associated syndromes were confirmed using medical records and radiographs. We applied the OMT classification that categorizes anomalies using a dysmorphology outline as malformations, dysplasias, deformations, and syndromes, and assessed its utility and ease of use. RESULTS: There were 480 extremities (74%) with a limb malformation including 184 involving the entire limb. Arthrogryposis was the most common of these (53 extremities). Anomalies affecting only the hand plate accounted for 62% (296) of the malformations. Of these, radial polydactyly (15%) was the most common specific anomaly, followed by symbrachydactyly (13%) and cleft hand (11%). Dysplasias were noted in 86 extremities; 55 of these were multiple hereditary exostoses. There were 87 extremities with deformations and 58 of these were trigger digits. A total of 109 children had a syndrome or association. Constriction ring sequence was most common. The OMT was straightforward to use and most anomalies could be easily assigned. There were a few conditions, such as Madelung deformity and symbrachydactyly, that would benefit from clarification on how to best classify them. CONCLUSIONS: Malformations were the most common congenital anomalies in the 653 upper extremities evaluated over a 1-year period at 3 institutions. We were able to classify all individuals using the OMT classification system.

Hand function with touch screen technology in children with normal hand formation, congenital differences, and neuromuscular disease.

PURPOSE: To measure and compare hand function for children with normal hand development, congenital hand differences (CHD), and neuromuscular disease (NMD) using a function test with touch screen technology designed as an iPhone application. METHODS: We measured touch screen hand function in 201 children including 113 with normal hand formation, 43 with CHD, and 45 with NMD. The touch screen test was developed on the iOS platform using an Apple iPhone 4. We measured 4 tasks: touching dots on a 3 × 4 grid, dragging shapes, use of the touch screen camera, and typing a line of text. The test takes 60 to 120 seconds and includes a pretest to familiarize the subject with the format. Each task is timed independently and the overall time is recorded. RESULTS: Children with normal hand development took less time to complete all 4 subtests with increasing age. When comparing children with normal hand development with those with CHD or NMD, in children aged less than 5 years we saw minimal differences; those aged 5 to 6 years with CHD took significantly longer total time; those aged 7 to 8 years with NMD took significantly longer total time; those aged 9 to 11 years with CHD took significantly longer total time; and those aged 12 years and older with NMD took significantly longer total time. CONCLUSIONS: Touch screen technology has becoming increasingly relevant to hand function in modern society. This study provides standardized age norms and shows that our test discriminates between normal hand development and that in children with CHD or NMD. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.

Dorsal carpal wedge osteotomy in the arthrogrypotic wrist.

PURPOSE: To assess the outcome of patients who underwent dorsal carpal wedge osteotomy (DCWO) for the treatment of wrist flexion deformities causing functional limitations resulting from arthrogryposis. METHODS: We performed a retrospective chart review of consecutive patients treated with DCWO between 1996 and 2009 by a single surgeon. Follow-up of greater than 1 year (mean, 45 mo; range, 12-108 mo) was available in 12 patients (20 wrists). RESULTS: All 12 patients' parents reported subjective improvement in position and appearance, and in performing activities of daily living. Wrist extension was significantly increased (mean, 43°), wrist flexion was significantly decreased (mean, 34° from neutral), and there was no significant change in wrist motion arc. We saw significantly greater improvement in wrist extension in children operated on at 7 years of age or greater and in patients treated concomitantly with an extensor carpi ulnaris tendon transfer. Complications included 1 infection. CONCLUSIONS: The excessively flexed wrist in children with arthrogryposis can safely and effectively be improved with DCWO, which in turn facilitates independence in activities of daily living and school-related tasks as reported by parents. For patients older than 7 years of age at the time of surgery, and for patients treated with concomitant extensor carpi ulnaris transfer at the time of DCWO, we found greater recovery of wrist extension. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Congenital Upper-Limb Differences: A 6-Year Literature Review.

➤ The Oberg-Manske-Tonkin (OMT) classification of congenital hand and upper-limb anomalies continues to be refined as our understanding of the genetic and embryonic etiology of limb anomalies improves.➤ We have conducted an evaluation of graft and graftless techniques for syndactyly reconstruction; strengths and drawbacks exist for each technique.➤ Treatment for radial longitudinal deficiency remains controversial; however, radialization has shown promise in early follow-up for severe deformities.➤ Recent emphasis on psychosocial aspects of care has demonstrated that children with congenital upper-limb differences demonstrate good peer relationships and marked adaptability.