Moving a missing hand: children born with below elbow deficiency can enact hand grasp patterns with their residual muscles.

Children with a unilateral congenital below elbow deficiency (UCBED) have one typical upper limb and one that lacks a hand, ending below the elbow at the proximal/mid forearm. UCBED is an isolated condition, and affected children otherwise develop normal sensorimotor control. Unlike adults with upper limb absence, the majority of whom have an acquired loss, children with UCBED never developed a hand, so their residual muscles have never actuated an intact limb. Their ability to purposefully modulate affected muscle activity is often assumed to be limited, and this assumption has influenced prosthetic design and prescription practices for this population as many modern devices derive control signals from affected muscle activity. To better understand the motor capabilities of the affected muscles, we used ultrasound imaging to study 6 children with UCBED. We examined the extent to which subjects activate their affected muscles when performing mirrored movements with their typical and missing hands. We demonstrate that all subjects could intentionally and consistently enact at least five distinct muscle patterns when attempting different missing hand movements (e.g., power grasp) and found similar performance across affected and typically developed limbs. These results suggest that although participants had never actuated the missing hand they could distinctively and consistently activate the residual muscle patterns associated with actions on the unaffected side. These findings indicate that motor control still develops in the absence of the normal effector, and can serve as a guide for developing prostheses that leverage the full extent of these children's motor control capabilities.

Neuromuscular Dysfunction in Clubfeet Associated With Constriction Band Syndrome.

INTRODUCTION: Constriction band syndrome (CBS) is a congenital limb anomaly frequently associated with clubfoot. Clubfeet in CBS patients may be associated with peroneal nerve dysfunction in the involved lower extremity; however, the etiology of this neuromuscular dysfunction is not clear. We sought to characterize the distribution of constriction bands on lower extremities with clubfoot and determine if neuromuscular deficit (NMD), defined here as having absent ankle dorsiflexion, was associated with ipsilateral proximal bands. Our secondary aim was to compare the treatment and outcomes of clubfeet with NMD to those without NMD. METHODS: We performed a retrospective review of all patients with CBS and clubfoot presenting to our facility between January 1, 1998 and December 31, 2018. Treatment with the Ponseti method, at least 1 year of follow-up at this facility, and a detailed physical exam describing lower extremity neuromuscular function and the presence and location of constriction bands were required for inclusion in the study cohort. RESULTS: Twenty children with 26 clubfeet were included. Forty-six percent (12/26) of the clubfeet had NMD. Clubfeet with and without NMD had ipsilateral thigh or leg constriction bands at similar rates [42% (5/12) vs. 43% (6/14), P =0.106], and the majority (7/12) of clubfeet with NMD did not have an ipsilateral thigh or leg band. While children with an NMD clubfoot tended toward more casts, relapses, and surgical procedures, these differences did not reach statistical significance. The use of a daytime AFO beyond age four was higher in the NMD clubfeet [58% (7/12) vs. 14% (2/14), P =0.04]. CONCLUSION: Clubfeet with neuromuscular deficits may occur in the absence of proximal ipsilateral constriction bands, suggesting they may be caused by mechanisms other than direct damage from visible constriction bands to underlying nerves. They can also coexist with arthrogrypotic conditions. Clubfeet with an NMD tended toward more casts, relapses, and surgeries than those without NMD, but these differences did not reach statistical significance. These patients often elect long-term use of a daytime AFO.

Maternal Epidemiology of Brachial Plexus Birth Injuries in California: 1996 to 2012.

OBJECTIVE: This study aimed to evaluate the incidence of brachial plexus birth injury (BPBI) and its associations with maternal demographic factors. Additionally, we sought to determine whether longitudinal changes in BPBI incidence differed by maternal demographics. STUDY DESIGN: We conducted a retrospective cohort study of over 8 million maternal-infant pairs using California's Office of Statewide Health Planning and Development Linked Birth Files from 1991 to 2012. Descriptive statistics were used to determine BPBI incidence and the prevalence of maternal demographic factors (race, ethnicity, age). Multivariable logistic regression was used to determine associations of year, maternal race, ethnicity, and age with BPBI. Excess population-level risk associated with these characteristics was determined by calculating population attributable fractions. RESULTS: The incidence of BPBI between 1991 and 2012 was 1.28 per 1,000 live births, with peak incidence of 1.84 per 1,000 in 1998 and low of 0.9 per 1,000 in 2008. Incidence varied by demographic group, with infants of Black (1.78 per 1,000) and Hispanic (1.34 per 1,000) mothers having higher incidences compared with White (1.25 per 1,000), Asian (0.8 per 1,000), Native American (1.29 per 1,000), other race (1.35 per 1,000), and non-Hispanic (1.15 per 1,000) mothers. After controlling for delivery method, macrosomia, shoulder dystocia, and year, infants of Black (adjusted odds ratio [AOR] = 1.88, 95% confidence interval [CI] = 1.70, 2.08), Hispanic (AOR = 1.25, 95% CI = 1.18, 1.32), and advanced-age mothers (AOR = 1.16, 95% CI = 1.09, 1.25) were at increased risk. Disparities in risk experienced by Black, Hispanic, and advanced-age mothers contributed to a 5, 10, and 2% excess risk at the population level, respectively. Longitudinal trends in incidence did not vary among demographic groups. Population-level changes in maternal demographics did not explain changes in incidence over time. CONCLUSION: Although BPBI incidence has decreased in California, demographic disparities exist. Infants of Black, Hispanic, and advanced-age mothers are at increased BPBI risk compared with White, non-Hispanic, and younger mothers. KEY POINTS: · The incidence of BPBI has decreased over time.. · Demographic disparities in BPBI incidence and risk exist.. · Infants of Black, Hispanic, and advanced age mothers are at greatest risk of BPBI..

The Green Transfer-Long-Term Results.

PURPOSE: The purpose of this study was to determine the long-term results of the Green transfer (flexor carpi ulnaris to extensor carpi radialis brevis) for patient-reported outcomes, wrist position, and range of motion. METHODS: We re-examined 13 patients from a previous prospective study involving surgery for hemiplegia that included a Green transfer. The average follow-up was 8 years with the range from 5 to 11 years. The wrist range of motion and the postoperative position of the wrists were measured. The surgical outcomes were measured via the Pediatric Orthopedic Data Collection Instrument, the Shriner's Hospital Upper Extremity Evaluation, Pediatric Quality of Life, and visual analog score for appearance from the patient and the parent. RESULTS: At this follow-up, only 7 of the 13 patients had a wrist position near neutral with the ability to flex and extend the wrist. Wrist range of motion was improved in four, decreased in four, and stayed the same in five patients. In contrast to these positional wrist results, statistically significant improvements were noted in several aspects of the Pediatric Orthopedic Data Collection Instrument, visual analog scores, and Shriner's Hospital Upper Extremity Evaluation scores. CONCLUSIONS: Long-term follow-up of the flexor carpi ulnaris to extensor carpi radialis brevis tendon transfer in hemiplegic patients reveals the results to be variable but favorable from a patient-reported outcome standpoint. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Long-Term Outcomes of Biceps Rerouting for Flexible Supination Contractures in Children With Brachial Plexus Birth Injuries.

PURPOSE: Forearm supination contractures occur in 7% of children with brachial plexus birth injuries (BPBI). Biceps rerouting is proposed when pronation has deteriorated but is passively correctable to at least 0° (neutral). The purpose of this investigation was to evaluate long-term outcomes of biceps rerouting for this indication, including magnitude and maintenance of correction, complications, and subsequent osteotomy. METHODS: We conducted a retrospective review of all children with BPBI and forearm supination contractures treated with biceps rerouting alone, for the above indications, from 1993 to 2017 with at least 2 years follow-up. Demographic information, BPBI characteristics, surgical details, and ranges of motion were obtained from medical records. Pre- and postoperative active pronation (AP) and supination (AS), elbow flexion contracture, and arc of forearm rotation (Arc) were analyzed using linear mixed-effect models. RESULTS: Twenty-five children (13 females; 13 left forearms; 15 global BPBI) underwent biceps rerouting at age 7 ± 3 years and were followed for 6 ± 3 years. Before surgery, the mean AP and AS were 6° ± 29° and 62° ± 27°, respectively. At the final follow-up, the mean AP, AS, and Arc were 39° ± 36°, 18° ± 34°, and 57° ± 42°, respectively. AP was significantly improved and AS was significantly decreased by 2 years after surgery and at the final follow-up. Neither Arc nor elbow flexion contracture changed significantly. Two of 25 (8%) children underwent subsequent forearm osteotomy. CONCLUSIONS: Biceps rerouting in children with BPBI improves the forearm position when pronation is deteriorating by shifting the arc from supination to pronation without decreasing the arc of motion or worsening elbow flexion contractures. There is a low risk of complications and a limited need for subsequent forearm osteotomy. These results are maintained over time. When performed before passive pronation is reduced beyond neutral, this procedure may prevent severe supination contractures and reduce the need for forearm osteotomy. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Comparison of Different Methods of Measuring Finger Range of Motion via Telehealth.

PURPOSE: This study examined the accuracy and reliability of measuring total motion of the fingers via telehealth using the following three different methods: (1) goniometry, (2) visual estimation, and (3) electronic protractor. Measurements were compared with in-person measurement, which was assumed to be the reference standard. METHODS: Thirty clinicians measured finger range of motion from prerecorded videos of a mannequin hand with articulating fingers, which was posed in extension and flexion that simulated a telehealth visit, using a goniometer with results blinded to the clinician (blinded goniometry), visual estimation, and an electronic protractor, in random order. Total motion was calculated for each finger and for all four fingers in sum. The experience level, familiarity with measuring finger range of motion, and opinions of measurement difficulty were assessed. RESULTS: Measurement with the electronic protractor was the only method equivalent to the reference standard within 20°. Remote goniometer and visual estimation did not fall within the acceptable error margin of equivalence, and both underestimated total motion. Electronic protractor also had the highest interrater reliability (intraclass correlation [upper limit, lower limit], 0.95 [0.92, 0.95]); goniometry (intraclass correlation, 0.94 [0.91, 0.97]) was nearly identical, whereas visual estimation (intraclass correlation, 0.82 [0.74, 0.89]) was much lower. Clinicians' experience and familiarity with range of motion measurements had no relationship with the findings. Clinicians reported visual estimation as the most difficult (80%) and electronic protractor as the easiest method (73%). CONCLUSIONS: This study showed that traditional in-person forms of measurement underestimate finger range of motion via telehealth; a new computer-based method (ie, electronic protractor) was found to be more accurate. CLINICAL RELEVANCE: The use of an electronic protractor can be beneficial to clinicians measuring range of motion in patients virtually.

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.

Patient-reported Outcomes for Children With Unilateral Congenital Below Elbow Deficiency.

BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS ® ) is a validated tool used to evaluate different domains of function in patients with chronic health conditions. This tool has not been validated in children with unilateral congenital below elbow deficiency (UCBED). The purpose of this study was to determine whether PROMIS discerns functional impairment for children with UCBED and whether children with UCBED differ from the general population with respect to PROMIS outcomes. We hypothesized that children with UCBED report mild impairment in upper extremity function but normal mobility, pain interference and peer relations. METHODS: A retrospective chart review of children aged 5 to 17 years with a diagnosis of UCBED who completed a PROMIS questionnaire at their clinic visit at the [blinded locations] was conducted between April 1, 2017 and March 31, 2020. The mean PROMIS scores of UCBED patients were compared with that of the general reference population. Mann Whitney and ANOVA tests were used to explore the differences across the PROMIS upper extremity function domain by arm length and prosthesis use. RESULTS: Fifty-five children (28 boys) with a mean age of 11±3.6 years met the inclusion criteria. Children with UCBED had similar PROMIS scores as the reference population in mobility (51.9±6.2), peer relations (53.5±9.4), and pain interference (40.1±7.2), with mild impairment in the upper extremity function (44.3±10.7). Compared with the 8 to 17-year-old cohort, the parent-proxy (5 to 7-year-old group) reported significantly more upper extremity function impairment (31.3±5.9) vs (48.0±8.8) ( P =0.000). The two age groups did not differ with respect to mobility, pain interference and peer relations. CONCLUSIONS: Our study confirms previous findings that children with UCBED report upper extremity function, peer relationships, pain interference, and mobility, similar to the reference population. In addition, parents of younger children with UCBED report more upper extremity functional impairment than is self-reported by older children with UCBED. LEVEL OF EVIDENCE: Prognostic Level III (comparison with reference population).

Patient-reported Outcomes Measurement Information System (PROMIS) Scores for Children With Brachial Plexus Birth Injury.

BACKGROUND: Patient-reported Outcomes Measurement Information System (PROMIS) for pediatrics is a validated patient-reported or parent-proxy-reported outcomes assessment tool used to evaluate health-related quality of life in children and adolescents with chronic medical conditions. The health-related quality of life of children with brachial plexus birth injury (BPBI) as measured by PROMIS is not well understood. We hypothesized that children with BPBI would report impaired upper extremity (UE) function but normal mobility, pain interference, and peer relationships compared with a reference pediatric population, and that UE function PROMIS scores would be associated with BPBI severity and patient age. METHODS: This is a retrospective cohort study of 180 children with BPBI ages 5 to 17 years old who responded to 4 pediatric PROMIS domains (mobility, pain interference, peer relationships, and UE function) between April 2017 and April 2019. Responses were converted to a T score, which allows comparison with a reference pediatric population (mean reference score=50). Multivariable linear regression was used to quantify the association between PROMIS scores and age, sex, Narakas type, and composite Mallet score. RESULTS: Children with BPBI had normal PROMIS mobility (49.6±8.5), pain interference (44.6±9.7), and peer relationships (52.4±10.6) scores, but reported mild impairment in UE function (40.8±12.1). Age (P<0.0001) and Narakas type (P=0.02) were associated with PROMIS UE function scores, but sex and composite Mallet scores were not. There were no significant associations between the other PROMIS domains and age, sex, Narakas Type, or composite Mallet scores. CONCLUSIONS: Children with BPBI reported PROMIS scores for mobility, pain interference, and peer relationships similar to the reference population but impairment in UE function. Reported UE function decreased with increasing disease severity and increased with age. These PROMIS domains seem to be useful tools for the clinician to evaluate children with BPBI and better understand the challenges they face. Further study is needed to assess their utility in measuring the effects of treatment interventions. LEVEL OF EVIDENCE: Level III.

Thumb Hypoplasia Occurring in Patients With Preaxial Polydactyly.

PURPOSE: Thumb polydactyly and thumb hypoplasia are generally regarded as separate clinical entities. However, several case reports indicate that hypoplasia of both the thumb and the radius can occur in patients with thumb polydactyly and improved understanding of the genetics of the developing upper limb may give an embryologic explanation for this occurrence. Our hypothesis was that patients with preaxial polydactyly can have ipsilateral thumb hypoplasia that may not be recognized until after surgical reconstruction of the extra digit. METHODS: We searched our surgical database for all procedures performed on patients with a diagnosis of preaxial polydactyly between 2002 and 2014. We reviewed the medical record for demographic data, surgical procedures, and follow-up information. In addition, all available radiographs were reviewed. Through this, we identified patients with a diagnosis of ipsilateral thumb hypoplasia, including when in the course of treatment the diagnosis was made, and any related subsequent procedures. RESULTS: We reviewed 132 patients who underwent reconstruction of thumb polydactyly, 10 of whom were identified as having evidence of ipsilateral thumb hypoplasia, an incidence of 8.2%. The diagnosis of thumb hypoplasia was made before surgery in 3 of the 10 patients. One additional patient was noted to have a duplicate thumb on one side and a hypoplastic thumb on the contralateral side. CONCLUSIONS: This study supports the hypothesis that children with preaxial polydactyly can have ipsilateral thumb hypoplasia that may not be noted before surgery. In this study group, 8% of patients with preaxial polydactyly had ipsilateral hypoplasia. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Outcomes of Botulinum Toxin Injection for Shoulder Internal Rotation Contractures in Infants with Brachial Plexus Birth Injury.

PURPOSE: Shoulder internal rotation contractures (IRC) are common sequela of brachial plexus birth injuries (BPBI). Botulinum toxin A (BTX-A) injection into targeted muscles has been described to facilitate functional improvement at the shoulder joint and prevent glenohumeral dysplasia. The purpose of this study was to assess the outcomes of BTX-A injections on shoulder IRC in children with BPBI. METHODS: We conducted a retrospective analysis of 47 children with shoulder IRC due to BPBI, who were treated with BTX-A. Shoulder passive external rotation in adduction and Active Movement Scale external rotation scores were recorded before and after BTX-A injection. We also recorded the number of children who underwent secondary surgical balancing procedures to improve shoulder motion after BTX-A injection. RESULTS: Mean age at the time of injection was 12 months (range, 5-23 months). Subjects demonstrated a significant increase in passive external rotation of 46° (range, 10° to 90) at 4 months; an average improvement of 18° (range, -30° to 80°) persisted at 11 months after injection. A total of 28 patients (60%) underwent subsequent external rotation tendon transfer. At 5-year follow-up, 7 patients (15%) had adequate functional shoulder range of motion and did not undergo external rotation tendon transfer. CONCLUSIONS: Botulinum toxin A injections result in improvement in IRC due to BPBI, which is sustained beyond the expected half-life of 3 months. As many as 15% of patients who have this treatment avoid external rotation tendon transfer. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic IV.

Early Practice Patterns of Hand Surgeons: An American Board of Orthopaedic Surgery Database Study.

PURPOSE: To investigate the variety of cases that are performed by newly graduated fellowship-trained orthopedic hand surgeons. METHODS: All cases submitted by board-eligible orthopedic surgeons taking part II of their board examination between 2004 and 2013 were obtained from the database of the American Board of Orthopaedic Surgery (ABOS). Each case was then categorized based on the fellowship training of the treating surgeon and whether it was a hand surgery case. Each hand surgery case was then further categorized into 1 of the 8 surgical categories used by the ABOS to determine eligibility for the Subspecialty Certificate in Orthopaedic Surgery of the Hand (SCOSH). RESULTS: During our study period, 6,854 orthopedic surgeons submitted 858,146 cases to the ABOS. Fellowship-trained hand surgeons made up 13% of all surgeons, and 24% of all submitted cases were hand surgery cases. Based on the cases submitted, 44% of hand surgeons were not on track to become eligible for the SCOSH. The most common reason for not reaching eligibility was failure to meet the minimum requirements in 1 of either the congenital, the skin and wound problems, the contracture and joint stiffness, the microvascular, or the tumor categories. CONCLUSIONS: A large proportion of fellowship-trained orthopedic hand surgeons are not on track to meet the minimum eligibility requirements for the ABOS SCOSH during their part II case collections. Their case profile is not sufficiently diverse to be considered an active hand surgery practice for the purpose of SCOSH eligibility. CLINICAL RELEVANCE: This study highlights 1 possible reason why over one-third of young hand surgeons do not obtain their subspecialty certification.

Neonatal Magnetic Resonance Imaging Without Sedation Correlates With Injury Severity in Brachial Plexus Birth Palsy.

PURPOSE: Which infants with brachial plexus birth palsy (BPBP) should undergo microsurgical plexus reconstruction remains controversial. The current gold standard for the decision for plexus reconstruction is serial clinical examinations, but this approach obviates the possibility of early surgical treatment. We hypothesize that a new technique using 3-dimensional volumetric proton density magnetic resonance imaging (MRI) without sedation can evaluate the severity of BPBP injury earlier than serial clinical examinations. METHODS: Infants were prospectively enrolled prior to 12 weeks of age and imaged using 3 Tesla MRI without sedation. Clinical scores were collected at all visits. The imaging findings were graded based on the number of injured levels and the severity of each injury, and a radiological score was calculated. All infants were followed at least until the decision for surgery was made based on clinical examination. RESULTS: Nine infants completed the MRI scan and clinical follow-up. The average Toronto score at presentation was 4.4 out of 10 (range, 0-8.2); the average Active Movement Scale score was 50 out of 105 (range, 0-86). Four infants required surgery: 2 because of a flail limb and Horner syndrome and 2 owing to failure to recover antigravity elbow flexion by age 6 months. Radiological scores ranged from 0 to 18 out of a maximum score of 25. The average radiological score for those infants who required surgery was 12 (range, 6.5-18), whereas the average score for infants who did not require surgery was 3.5 (range, 0-8). CONCLUSIONS: Three-dimensional proton density MRI can evaluate spinal nerve roots in infants without the need for radiation, contrast agents, or sedation. These data suggest that MRI can help determine the severity of injury earlier than clinical examination in infants with BPBP, although further study of a larger sample of infants with varying severity of disease is necessary. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic II.

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.

Radiographic Outcomes of Hemiepiphyseal Stapling for Distal Radius Deformity Due to Multiple Hereditary Exostoses.

BACKGROUND: Multiple hereditary exostoses (MHE) affect the distal forearm in 30% to 60% of patients with this condition. Many surgical interventions have been described, but treatment is controversial. We present the radiographic results of low risk, minimally invasive hemiepiphyseal stapling of the distal radius to correct the joint angulation caused by MHE through growth modulation. METHODS: A retrospective review was performed on 18 forearms affected by MHE that underwent radial hemiepiphyseal stapling with follow-up of ≥2 years. The radial articular angle, carpal slip, ulnar tilt, lunate subsidence, and metaphyseal epiphyseal angle were measured on preoperative and final postoperative radiographs and compared. RESULTS: Statistically significant (P<0.05) improvements were found in 4 of 5 measurements, including the radial articular angle, carpal slip, ulnar tilt, and metaphyseal epiphyseal angle. No statistical difference was found in lunate subsidence (P=0.84). CONCLUSION: Hemiepiphyseal stapling of the radial side of the distal radius is a simple and effective method for correcting the radiographic deformity of the distal radius and may be an attractive option for the treatment of the distal radial deformity that is characteristic of the disease. LEVEL OF EVIDENCE: Level IV­therapeutic case series.

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.

Late reconstruction of brachial plexus birth palsy.

Brachial plexus birth palsy (BPBP) presents to the physician on a clinical spectrum, and may substantially impair the child. Potential interventions to improve function for the child with BPBP include physical therapy, microsurgical nerve reconstruction and nerve transfers, soft-tissue balancing and reconstruction with musculotendinous transfers, and osteotomies. Some interventions, such as nerve reconstruction, are best performed in infancy; others, such as muscle transfers and osteotomies, are performed to treat manifestations of this condition that appear later in childhood. Although controversy continues to exist regarding the natural history and surgical management of these patients, recent literature has improved our understanding of surgical indications, anticipated outcomes, and potential complications. On the basis of current evidence, we present here the recommendations for surgical intervention in the upper extremity of children with BPBP, and encourage early referral to a brachial plexus specialist to establish care.

Understanding the capacity of children with congenital unilateral below-elbow deficiency to actuate their affected muscles.

In recent years, commercially available dexterous upper limb prostheses for children have begun to emerge. These devices derive control signals from surface electromyography (measure of affected muscle electrical activity, sEMG) to drive a variety of grasping motions. However, the ability for children with congenital upper limb deficiency to actuate their affected muscles to achieve naturalistic prosthetic control is not well understood, as compared to adults or children with acquired hand loss. To address this gap, we collected sEMG data from 9 congenital one-handed participants ages 8-20 years as they envisioned and attempted to perform 10 different movements with their missing hands. Seven sEMG electrodes were adhered circumferentially around the participant's affected and unaffected limbs and participants mirrored the attempted missing hand motions with their intact side. To analyze the collected sEMG data, we used time and frequency domain analyses. We found that for the majority of participants, attempted hand movements produced detectable and consistent muscle activity, and the capacity to achieve this was not dissimilar across the affected and unaffected sides. These data suggest that children with congenital hand absence retain a degree of control over their affected muscles, which has important implications for translating and refining advanced prosthetic control technologies for children.

Disruption of the radial/ulnar axis:congenital longitudinal deficiencies.

Radial, ulnar, and central deficiencies represent a spectrum of abnormalities in the development of the upper limb. Radial longitudinal deficiency is often associated with abnormalities in other organ systems, such as cardiac and renal, and so requires a comprehensive medical evaluation. On the other hand, ulnar longitudinal deficiency tends to be associated only with other musculoskeletal abnormalities. In all of these conditions, there is a high incidence of ipsilateral thumb abnormalities. Given the importance of the thumb in overall hand function, abnormalities of the thumb often guide treatment for these conditions. Surgical treatment of the wrist and forearm in radial longitudinal deficiency is controversial, as will be outlined in this review.