Health-related quality of life in 205 children with arthrogryposis multiplex congenita.

Purpose: Arthrogryposis multiplex congenita (AMC) describes a heterogeneous group of rare congenital conditions. Health-related quality of life (HRQL) may be reduced in AMC due to broadly heterogeneous physical impairments and participation limitations. This study described HRQL in children and youth with AMC, compared HRQL between child self- and parent-proxy reports, and identified factors associated with better/worse HRQL.

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.

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.

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.

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.

Review and critique of current testing protocols for upper-limb prostheses: a call for standardization amidst rapid technological advancements.

This article provides a comprehensive narrative review of physical task-based assessments used to evaluate the multi-grasp dexterity and functional impact of varying control systems in pediatric and adult upper-limb prostheses. Our search returned 1,442 research articles from online databases, of which 25 tests-selected for their scientific rigor, evaluation metrics, and psychometric properties-met our review criteria. We observed that despite significant advancements in the mechatronics of upper-limb prostheses, these 25 assessments are the only validated evaluation methods that have emerged since the first measure in 1948.

Association of Parity and Previous Birth Outcome With Brachial Plexus Birth Injury Risk.

Objective: To evaluate the association of maternal delivery history with a brachial plexus birth injury risk in subsequent deliveries and to estimate the effect of subsequent delivery method on brachial plexus birth injury risk.

Methods: We conducted a retrospective cohort study of all live-birth deliveries occurring in California-licensed hospitals from 1996 to 2012. The primary outcome was recurrent brachial plexus birth injury in a subsequent pregnancy.

Team Approach: The Care of Children with Hand and Upper Limb Differences.

» Pediatric hand and upper limb differences include a wide range of conditions that may be genetic, part of a syndrome, or arise from birth trauma or an unknown cause.» Because of the variety of conditions and complexity of care requiring professionals from multiple disciplines, the Pediatric Hand Team is similar in purpose to the coordinated multidisciplinary care provided by Craniofacial Panels for children with craniofacial anomalies. Pediatric hand surgeons are trained to lead and coordinate the care of children with these differences, and the Pediatric Hand Team includes occupational and/or certified hand therapists, child life specialists, geneticists and genetic counselors, prosthetists and orthotists, pediatric physical medicine and rehabilitation physicians, pediatric orthopaedic surgeons, pediatric anesthesiologists, and social workers and psychologists.

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.

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.

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.

Self-concept development in children with limb differences: A scoping review.

Having a visible physical difference, such as a limb difference, can have a significant impact on a child's psychosocial development, as children with limb differences may experience negative psychosocial sequelae. The aim of this scoping review was to investigate the findings of literature pertaining to self-concept of children with limb differences. Using the PRISMA ScR guidelines, a literature search was conducted in Embase, CINAHL, PsycINFO and PubMed databases.

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.

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.

Clinical Manifestations of Constriction Band Syndrome.

Background: Constriction band syndrome (CBS) is a congenital limb anomaly with varying presentation. We sought to characterize the clinical manifestations of CBS by analyzing a large cohort of patients. Our secondary aim was to evaluate potential risk factors for CBS.

A review of upper limb pediatric prostheses and perspectives on future advancements.

Many complex factors affect whether a child with a congenital upper limb deficiency will wear a prosthetic limb. Ultimately, for a child to wear and use their prosthesis, it must facilitate the effective performance of daily tasks and promote healthy social interactions. Although numerous pediatric devices are available, most provide a single open-close grasp (if a grasping function is available at all) and often offer nonanthropomorphic appearances, falling short of meeting these criteria.

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.

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.

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.

Posterior Elbow Capsulotomy and Triceps Lengthening for Elbow Extension Contracture in Children with Arthrogryposis Multiplex Congenita.

Unlabelled: Posterior elbow capsulotomy plus triceps lengthening facilitates passive elbow flexion in children with arthrogryposis multiplex congenita, allowing independent function for activities of daily living, such as feeding and self-care of the face and hair.

Description: The posterior aspect of the distal end of the humerus and the olecranon are identified by palpation and exposed via a curvilinear incision over the posterior aspect of the elbow. Identifying the osseous landmarks can be challenging in some patients.

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.

Microsurgery for Brachial Plexus Injury Before Versus After 6 Months of Age: Results of the Multicenter Treatment and Outcomes of Brachial Plexus Injury (TOBI) Study.

Background: Infants with more severe brachial plexus birth injury (BPBI) benefit from primary nerve surgery to improve function. The timing of the surgery, however, is controversial. The Treatment and Outcomes of Brachial Plexus Injury (TOBI) study is a multicenter prospective study with the primary aim of determining the optimal timing of this surgical intervention.