Michelle A. James

Type III-A hypoplastic thumb.

Thirteen type III-A hypoplastic thumbs were reviewed. The abnormalities included those found in type II hypoplasia (narrow thumb-index web space, hypoplastic thenar muscles, unstable metacarpophalangeal joint, as well as extrinsic tendon abnormalities); all had stable carpometacarpal joints. They are differentiated from type III-B hypoplastic thumbs, which have an unstable carpometacarpal joint due to a deficient base of the metacarpal. The extrinsic tendon abnormalities included absent extensor pollicis longus tendon, absent or aberrant flexor pollicis longus tendon, and a tendon interconnection between the flexor pollicis longus and extensor aponeurosis. Twelve of the thumbs had surgical reconstruction. None was treated with ablation and index pollicization.

Impact of Prostheses on Function and Quality of Life for Children with Unilateral Congenital Below-the-Elbow Deficiency

BACKGROUND Children with unilateral congenital below-the-elbow deficiency present a dilemma to clinicians. Parents want the child to have a prosthesis and, because it seems that the deficiency will cause functional problems, one is customarily prescribed for infants. Use of the prosthesis is then encouraged throughout childhood. However, these children frequently abandon the prosthesis. There are no evidence-based guidelines regarding prescription of prostheses or standard methods for assessing use and function. METHODS A multicenter outcomes study was done to assess the quality of life and function of 489 children with a unilateral congenital below-the-elbow deficiency; 321 wore a prosthesis, and 168 did not. The Unilateral Below-the-Elbow Test (UBET) was designed, validated, and administered to these children along with several outcomes measures, including the Pediatric Outcomes Data Collection Instrument (PODCI), the Pediatric Quality of Life Inventory (PedsQL), and the Prosthetic Upper Extremity Functional Index (PUFI). RESULTS Use of a prosthesis was not associated with any clinically relevant differences in PODCI or PedsQL scores. Non-wearers performed either the same as or better than wearers on the UBET. When queried (with use of the PUFI) about performance of various tasks, non-wearers scored themselves higher than wearers. Children with a unilateral congenital below-the-elbow deficiency scored the same as or higher than the general population on the PedsQL. They scored significantly lower than the general population on the PODCI Upper Extremity Physical Function Domain and higher on the Happiness Domain, but the differences were small. CONCLUSIONS Prostheses may help with social acceptance or may be useful as tools for specialized activities, but they do not appear to improve function or quality of life, which are nearly normal for children with unilateral congenital below-the-elbow deficiency regardless of whether they wear a prosthesis. These findings call into question the standard practices of fitting infants with prostheses and encouraging young children to wear the prosthesis.

Characteristics of patients with hypoplastic thumbs

The records of 98 patients with 160 hypoplastic thumbs seen between 1923 and 1993 were reviewed to determine the salient characteristics of this population. Physical findings, photographs, and x-ray films were studied. Sixty-three percent of patients were male, and 63% had bilateral thumb hypoplasia, 59% had radial dysplasia, and 86% had other anomalies. An associated syndrome, most commonly the vertebral, anal, tracheosophageal, renal, and radial limb anomalies association or Holt-Oram syndrome, was present in 44%. Patients with spine, genitourinary, or gastrointestinal anomalies were most likely to have the vertebral, anal, tracheosophageal, renal, and radial limb anomalies association, those with cardiac anomalies were most likely to have Holt-Oram syndrome, and those with lower extremity anomalies were most likely to have a different syndrome. One hundred thirty-nine thumbs were classifiable, using a modification of Blauths classification; 19% were types 1 and 2, 23% were type 3, and 58% were types 4 and 5. One hundred seven operations were performed on 63 upper limbs, including 24 thumb reconstructions and 35 pollicizations. These patients must be carefully examined for bilaterality, other anomalies, and syndromes. Classification using a modification of Blauths criteria is useful and helps the surgeon determine a treatment plan.

Measuring Patient Satisfaction in Orthopaedic Surgery

In addition to their wish to understand the clinical results of orthopaedic interventions, clinicians, patients, and payers are increasingly interested in patient satisfaction, both with the process of care and with outcomes. The construct of satisfaction is complex and depends on the context in which care takes place, including the nature of treatment, its setting, and most importantly the expectation of patients prior to treatment. The characteristics of scales that are effective measures of satisfaction are the same as those of all effective measurement instruments--i.e., reliability, validity, and responsiveness. Measurement of patient satisfaction may be especially important in evaluations of established procedures and processes so that the value of those procedures and processes to patients can be more completely understood.

The association of radial deficiency with thumb hypoplasia

BACKGROUND Congenital longitudinal deficiencies of the radius and thumb are known to be associated with one another; however, the details of their relationship are unknown. The purpose of this study was to determine whether increased severity of radial deficiencies is associated with increased severity of thumb deficiencies and to review the relationship between radial deficiency and reconstructibility of a hypoplastic thumb. METHODS Radiographs and charts of 227 affected upper extremities of 139 patients with radial longitudinal deficiency were reviewed. The associated thumb deficiency was classified according to a modification of the Blauth and Schneider-Sickert scheme and the radial deficiency was classified according to a modification of the Bayne and Klug criteria for 191 extremities of 119 patients. RESULTS The severity of the thumb deficiency was directly proportional to the severity of the radial deficiency (p < 0.0001). Half of the extremities had either a thumb deficiency or thumb and carpal deficiencies without radial deficiency. Two-thirds (sixty-three) of the ninety-five limbs with a normal radius had a thumb that could be surgically reconstructed. Seventy-one (91%) of seventy-eight extremities with a thumb amenable to surgical reconstruction had a radius that did not require surgical reconstruction. All extremities with a radial and/or carpal deficiency had a thumb deficiency. Forty-eight (94%) of fifty-one extremities with complete absence of the radius had a thumb that was not reconstructible. CONCLUSIONS This study supports the growing body of evidence that the components of radial longitudinal deficiency represent a progressive spectrum of upper extremity abnormalities, and a distal progression of severity, with distal structures likely to be more involved than proximal structures.

Kinematic assessment of the upper extremity in brachial plexus birth palsy

Children with brachial plexus birth palsy (BPBP) may have shoulder external rotation and abduction weakness that can restrict activities of daily living (ADLs). Static range of motion measurements may not measure ADL restrictions. Motion analysis has been used to quantify gait limitations and measure changes associated with treatment. The purpose of this study was to determine whether upper extremity motion analysis (UEMA) can measure the differences in shoulder motion during ADLs between children with BPBP and normal children. Following a previously described UEMA protocol, 55 children with BPBP and 51 normal children (control group) were studied. Kinematic data of selected ADLs were collected before surgery. UEMA was used to measure statistically significant differences between children with BPBP and control subjects for all planes of shoulder motion in all activities tested. The authors conclude that UEMA can discriminate between children with BPBP and control subjects during selected ADLs, and suggest that UEMA can also be used to measure the effects of surgical interventions in children with BPBP.

Assessment of children with brachial plexus birth palsy using the Pediatric Outcomes Data Collection Instrument.

The purpose of this study was to determine whether the Pediatric Outcomes Data Collection Instrument (PODCI) measures differences in function between children with brachial plexus birth palsy (BPBP) who are candidates for shoulder tendon surgery and age-matched controls. The PODCI was administered prospectively to 23 children with BPBP who were candidates for shoulder tendon surgery. Their results were compared with published PODCI data for control subjects, and factors associated with function within the BPBP cohort were determined. Children in the BPBP cohort had significantly lower PODCI scores in upper extremity function, sports, and global function than control subjects. Limited active shoulder external rotation was significantly associated with lower functional scores. The PODCI measures diminished upper extremity function in children with BPBP who are candidates for shoulder tendon surgery, thereby showing promise as a tool for measuring baseline function and postoperative functional gains for children with BPBP.

The Unilateral Below Elbow Test: a function test for children with unilateral congenital below elbow deficiency.

The Unilateral Below Elbow Test (UBET) was developed to evaluate function in bimanual activities for both the prosthesis wearer and non-wearer. Nine tasks were chosen for each of four age-specific categories defined by development stages of hand function (2-4y, 5-7y, 8-10y, and 11-21y). Two scales, Completion of Task and Method of Use, were designed to rate performance. To measure reliability, four occupational therapists scored samples of videotaped UBET performances. For Completion of Task, an interval scale, agreement in scoring was measured with interclass correlation coefficients (ICC; n=9; five females, four males). For Method of Use, a nominal scale, chance-adjusted association was calculated with Cohens kappa coefficients (interobserver n=198; 111 females, 87 males; intraobserver n=93; 56 females, 37 males). For Completion of Task, the average ICC was 0.87 for the prosthesis-on condition, and 0.85 for the prosthesis-off condition. ICCs exceeded 0.80 for eight out of nine tasks for the two older age groups, but for only five out of nine tasks in the younger age groups. Higher inter- and intraobserver kappa coefficients for Method of Use resulted when scoring children with their prostheses on versus off. The oldest age group had lower kappa values than the other three groups. The UBET is recommended for the functional evaluation of Completion of Task in children with unilateral congenital below elbow deficiency with and without their prostheses. Method of Use scoring can evaluate individuals for directed therapy interventions or prosthetic training.

Posterior Elbow Capsulotomy with Triceps Lengthening for Treatment of Elbow Extension Contracture in Children with Arthrogryposis

BACKGROUND Flexion of one elbow is essential to enable children with arthrogryposis to achieve independent function such as self-feeding and self-care of the face and hair. We analyzed the outcomes of posterior elbow capsulotomy with triceps lengthening for the treatment of elbow extension contractures in a series of children with arthrogryposis multiplex congenita. METHODS The medical records of all children with arthrogryposis who had been followed for a minimum of two years after treatment with elbow capsulotomy and triceps lengthening were retrospectively reviewed. The postoperative range of motion and ability to reach the mouth were compared with the preoperative status. RESULTS Posterior capsulotomy with triceps lengthening was performed in twenty-nine elbows of twenty-three children with an average age of thirty-five months (range, seven months to thirteen years). The average duration of follow-up was 5.4 years. The arc of motion of all twenty-nine elbows improved from an average of 32 degrees (range, 0 degrees to 75 degrees) preoperatively to an average of 66 degrees (range, 10 degrees to 125 degrees) at the time of final follow-up. All children were able to reach the mouth using passive assistance (e.g., table-push, trunk-sway, and cross-arm techniques), and twenty-two children were able to feed themselves independently. No child underwent subsequent tendon transfer surgery. CONCLUSIONS Elbow capsulotomy with triceps lengthening successfully increases passive elbow flexion and the arc of elbow motion of children with arthrogryposis, enabling hand-to-mouth activities. In contrast to studies in which tendon transfer surgery was used to increase elbow flexion, none of the children in this series underwent subsequent tendon transfer surgery.

Tendon transfers and releases for the forearm, wrist, and hand in spastic hemiplegic cerebral palsy

Cerebral Palsy (CP) is a static disorder of movement and posture secondary to an insult to the developing central nervous system. The peripheral manifestations and functional impairments of this condition vary in severity from mild-to-profound. In hemiplegic CP, 1 side of the body is affected more than the other. Spastic hemiplegia is the most common type and that for which upper extremity surgery is most indicated. Treatment options range from physical therapy and splinting to botulinum toxin A injections (Botox) to tendon transfers to arthrodeses. This article will discuss the indications, preoperative evaluation, our preferred surgical technique, and postoperative protocol for the most commonly used tendon transfers in the upper extremity in spastic hemiplegia.