The Effect of Adoption on Functioning and Psychosocial Well-Being in Patients With Congenital Upper-Extremity Differences.

PURPOSE: Adoption status has been investigated in numerous populations, identifying negative and positive effects on patient outcomes. This study compared parent-reported outcomes in adopted and nonadopted individuals with congenital upper-extremity (UE) anomalies to determine whether there was a measurable difference in parent perceptions of psychosocial health and functioning. METHODS: The Congenital Upper Limb Differences registry identified adopted individuals from a large cohort of patients. Each adopted patient was matched to 2 nonadopted patients by sex, age, and diagnosis of UE difference using the Oberg-Manske-Tonkin classification. Parent-reported scores on functional and psychosocial subjective outcomes were compared between the 2 cohorts using the Pediatric Outcome Data Collection Instrument. RESULTS: Ninety-one adopted pediatric patients with congenital UE differences were identified. Sixty had complete matches for comparison, and the remaining 31 patients were not able to be matched and were excluded from the analysis. Pediatric Outcome Data Collection Instrument scores for the UE function domain were worse in the adopted cohort as judged by minimally clinically important difference. In contrast, scores for Pediatric Outcome Data Collection Instrument domains of transfer and mobility, sports and physical function, pain and comfort, and happiness did not exceed the minimally clinically important difference, although the adopted cohort scores were all relatively lower than the nonadopted cohort scores. CONCLUSIONS: In pediatric patients with UE differences, adoption status is seen to affect only the parent-reported domain of UE function. While differences by specific congenital diagnosis call for additional investigation, overall, these findings should be reassuring to families planning to adopt children with UE differences, and they should be supported in their expectations of UE functioning. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic III.

Outcomes of Pediatric and Adolescent Carpal Tunnel Release.

PURPOSE: To investigate carpal tunnel syndrome (CTS) presentation and long-term outcomes of carpal tunnel release (CTR) in children and adolescents. METHODS: All pediatric and adolescent patients who underwent CTR between February 2003 and June 2018 were identified. Patients were grouped by etiology: lysosomal storage disease (11 hands), idiopathic (6 hands), acute traumatic (7 hands), delayed traumatic (5 hands) and tumorous (2 hands). Medical records were reviewed for presenting symptoms and preoperative treatments. Final outcomes were assessed via phone interviews, chart review, the Boston Carpal Tunnel Questionnaire (BCTQ), and Patient-Reported Outcomes Measurement Information System (PROMIS) scores. RESULTS: All 25 patients (31 hands) identified were included in the study; median age at surgery was 12.7 years (range, 2.5-23.3 years). Eighteen patients completed surveys at a median of 4.7 years after surgery (range, 8 months-16 years). Common presenting symptoms in lysosomal storage disease were numbness/tingling (7 hands); pain was only reported in 1 hand. The most frequent indication for acute traumatic CTR (7 hands) was palmar hand swelling at the carpal tunnel (4 hands). Delayed traumatic and idiopathic CTS most often presented with numbness/tingling (4 hands and 6 hands, respectively) and pain (3 hands, 4 hands, respectively). Of the original 4 lysosomal storage disease surgeries included in long-term follow-up, all experienced gradual recurrence of symptoms after years of relief (range, 3-14 years). Two patients underwent revision CTR and were symptom-free at follow-up. All patients with acute traumatic and tumorous etiologies had full resolution of symptoms. Delayed traumatic and idiopathic etiologies frequently experienced recurrent or recalcitrant symptoms (4 of 5 and 3 of 6 surgeries, respectively). CONCLUSIONS: Carpal tunnel release often alleviates symptoms in children with lysosomal storage disease for years to decades. Carpal tunnel release successfully relieves symptoms in acute traumatic cases, but is not always sufficient to relieve symptoms associated with delayed traumatic etiologies. Approximately half of patients with idiopathic CTS experience recalcitrant or recurrent symptoms. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Radiographic and Clinical Outcomes of the Shoulder in Long-Term Follow-Up of Brachial Plexus Birth Injury.

PURPOSE: The purpose of this study was to examine radiographic and functional outcomes of the shoulder in brachial plexus birth injury (BPBI) patients at long-term follow-up. METHODS: Medical records were reviewed at the Shriners Hospital in St. Louis to identify patients older than 18 years with a diagnosis of BPBI. Twenty-five patients were included in this study. Evaluations included radiographic examination, clinical examination, and outcome measures including the Mallet classification, visual analog scale (VAS) for shoulder pain, American Shoulder and Elbow Shoulder score (ASES), quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH), Simple Shoulder Test (SST), and Patient-Reported Outcomes Measurement Information System (PROMIS) domain measures. RESULTS: The mean age of the patients included was 44 years. Average follow-up was 37 years. No patient had primary nerve reconstruction surgery. Thirteen patients had a history of shoulder surgery at an average age of 6 years. There was a wide spectrum of deformity seen radiographically. Fifty-five percent of patients had evidence of glenoid retroversion, posterior humeral translation, or a biconcave glenoid. An additional 20% of patients had complete posterior dislocation of the humeral head. Despite the degenerative changes seen on radiographs, the mean VAS for pain was low, at 2. The mean ASES shoulder score was 54. The mean QuickDASH score was 30. Mean PROMIS Physical Function, Pain Interference, Depression, and Anxiety scores were near the means for the general population, with similar outcomes in patients treated with shoulder surgery compared with those treated without surgery. CONCLUSIONS: Adult BPBI patients experience minimal shoulder pain despite deformity and degenerative changes on shoulder radiographs. Whereas there are significant deficits in range of motion and function, PROMIS scores in adult BPBI patients are normal. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Outcomes of Pediatric Syndactyly Repair Using Synthetic Dermal Substitute.

PURPOSE: Full-thickness skin grafts are classically used for areas of skin deficit in syndactyly reconstruction surgery. However, skin grafting requires additional time under anesthesia and includes donor site morbidity. Synthetic dermal substitute has produced favorable web creep and scar appearance outcomes in syndactyly reconstruction. We sought to validate these results using objective outcome measures. METHODS: All patients who had undergone syndactyly reconstruction with synthetic dermal substitute with 1-year follow-up were identified. We included 23 webs in 16 patients; 2 were lost to follow-up. Median age at surgery was 27 months; 7 patients were female. Five webs were revision surgeries. Two patients had a diagnosis of amniotic constriction band, and 3 had symbrachydactyly. All webs were assessed from blinded clinical photos. Outcome measures included web creep assessment (range, 0-5) and Vancouver Scar Scale. RESULTS: Of 21 webs, 2 had minimal creep and 16 had none; 3 had clinically relevant creep (grade 2). Vancouver Scar Scale scores for all webs averaged 1.19 (0 is normal). There was normal vascularity in 20 webs, pigmentation was normal in 17 webs, skin pliability was normal in 13 webs, and scar height was flat in 15 webs. On the visual analog scale assessment, surgeons rated the appearance of the 21 webs as an average of 8.8 (range, 5-10). There were no postoperative complications. CONCLUSIONS: Synthetic dermal substitute is an effective, efficient, and visually satisfactory option for coverage of skin deficits in syndactyly reconstruction. The current heterogeneous sample revealed that this approach can provide satisfactory outcomes for patients, families, and surgeons. Although no surgical complications were noted, the rate of integration and healing has yet to be determined. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Characterization of Hand Anomalies Associated With Möbius Syndrome.

PURPOSE: To investigate the distinguishing morphological characteristics of the upper extremities in children with Möbius syndrome. METHODS: Twenty-seven involved extremities in 14 patients with a diagnosis of Möbius syndrome were identified at 2 institutions. Medical records, radiographs, and clinical photographs were evaluated. Congenital hand differences were classified according to the Oberg, Manske, and Tonkin classification, and hands with symbrachydactyly were classified by the Blauth and Gekeler classification. The presence of other congenital anomalies was catalogued. RESULTS: There was bilateral involvement in 93% of patients with congenital hand anomalies. Twelve patients demonstrated congenital hand anomalies and 2 patients had been diagnosed with arthrogryposis. Among the 12 patients with congenital hand anomalies, 21 hands were classifiable as symbrachydactyly by the Oberg, Manske, and Tonkin classification and could be categorized by the Blauth and Gekeler classification. Short finger type was the most common subtype of symbrachydactyly, present in 13 hands. Eleven of these 13 patients (85%) were primarily affected on the radial side of the hand. Proximal arm involvement was identified in 2 patients with symbrachydactyly, both of whom had Poland syndrome and an absent pectoralis major. CONCLUSIONS: Symbrachydactyly in Möbius syndrome differs from the typical presentation of symbrachydactyly. Characteristically, there is a bilateral presentation with a strong predilection for radially based brachydactyly. These described characteristics may help the hand surgeon appropriately assess patients, especially those with radial-sided symbrachydactyly. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.

Intraobserver and Interobserver Reliability of the Oberg-Manske-Tonkin (OMT) Classification: Establishing a Registry on Congenital Upper Limb Differences.

BACKGROUND: The purpose of this investigation was to determine the reliability of the Oberg-Manske-Tonkin (OMT) classification system applied to patients enrolled in a prospective, multicenter cohort study of congenital upper limb differences. Our hypothesis was that the OMT classification would exhibit high intraobserver and interobserver reliability and thus would be an appropriate tool for the new Congenital Upper Limb Differences registry. METHODS: Four pediatric hand surgeons independently reviewed the medical records, clinical photographs, and radiographs of 60 randomly selected patients previously enrolled in the Congenital Upper Limb Differences registry. Patient's information was summarized and shared electronically (PowerPoint). Each rater classified each congenital anomaly according to the OMT classification system. Responses were recorded using a web-based data capture tool (REDCap). Three weeks later, the surgeons repeated the process. Intraobserver reliability for each rater was assessed using Fleiss' κ. Interobserver reliability was assessed using percent exact agreement (when all 4 raters were in agreement) as well as Fleiss' κ. RESULTS: Using the OMT classification, intraobserver reliability for the 4 pediatric hand surgeons showed almost perfect agreement, with κ values in the range of 0.89 to 0.93. Interobserver reliability demonstrated substantial agreement, with κ value of 0.79 (95% confidence interval, 0.77-0.82) in the first reading and 0.80 (95% confidence interval, 0.77-0.83) in the second reading. The highest possible agreement (κ=1) was seen for the following diagnoses: congenital dislocation of radial head (OMT I.A.2.v), Madelung deformity (I.A.2.vii), radial polydactyly (I.B.2.iii), triphalangeal thumb (I.B.2.iv), Kirner deformity (I.B.4.vi), and osteochondromatosis (3.B.4.i). CONCLUSIONS: The OMT classification of congenital upper limb differences exhibits substantial to almost perfect intraobserver and interobserver reliability among pediatric hand surgeons at different institutions. The OMT is therefore well-suited for classification of congenital upper limb anomalies in patients enrolled in a multicenter registry. LEVEL OF EVIDENCE: Level II-diagnostic.

Long-Term Outcomes of Huber Opposition Transfer for Augmenting Hypoplastic Thumb Function.

PURPOSE: This study was conducted to report the functional outcomes of the Huber opposition transfer (abductor digiti minimi muscle) in types II and IIIA hypoplastic thumbs. METHODS: Patients who had undergone a Huber opposition transfer with at least 5 years of follow-up were included in this study. There were 21 thumbs included; 12 returned for a detailed evaluation and 9 were included with a medical record review. Outcome measures included range of motion and pinch strength; Pediatric Outcomes Data Collection Instrument (PODCI) and Patient-Reported Outcomes Measurement Information System (PROMIS) scores were collected on those who could return. There were 15 type II and 6 type IIIA thumbs. RESULTS: Range of motion was significantly less than normal for both the interphalangeal and the metacarpophalangeal joints. For the returning cohort, key and tripod pinch were 44% and 65% of normal. The median Kapandji score was 9 (range, 6-10). The PODCI scores were high for global, upper extremity function, happiness, and pain. The PROMIS scores were similar to normal, except for parent reports of physical function. For all included patients, there was a revision surgery rate of 22%, primarily related to persistent instability. CONCLUSIONS: At a minimum 5-year follow-up, the Huber opposition transfer for types II and IIIA thumbs was shown to provide good subjective outcomes, despite limited range of motion and strength. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Distal Humerus External Rotation Osteotomy for Hand Position in Arthrogryposis.

PURPOSE: In the amyoplasia type of arthrogryposis, a reverse pronated grasp pattern is often seen. We hypothesized that repositioning the hands, through distal humerus external rotation osteotomies (DHO), would allow for palm-to-palm grasp without arm cross-over and would improve function and parent/patient satisfaction. METHODS: The medical records of all patients treated surgically for arthrogryposis were reviewed at the Shriners Hospital for Children, St. Louis, MO. From 2012 to 2014, 9 patients (14 extremities) had undergone a DHO. All patients had preoperative and postoperative video recordings of functional activities and we assessed functional changes after osteotomies. Preoperative upper extremity position was graded as 1, palms facing midline; 2, palm facing posterior; and 3, palms facing away from midline. Postoperative Pediatric Outcomes Data Collection Instrument (PODCI) questionnaires were obtained and parent satisfaction was evaluated. RESULTS: Mean patient age at the time of surgery was 6.5 years. Five patients underwent bilateral DHOs. All patients had 3 or fewer additional procedures on an upper extremity during the study period. All patients had an improved resting posture of the upper extremity after DHO surgery, with a mean change of 51° (range, 15°-90°). Grasp pattern was altered in 13 extremities; there was a change in hand position of at least 1 grade and 5 had complete change from 3 to 1, palms facing away from midline to facing toward midline. There was a wide range in postoperative PODCI scores for function, but Happiness scores were high, mean 89 (range, 60-100). Parents universally stated the procedure improved the child's function "a great deal." There were 2 complications: 1 periprosthetic humerus facture with recurrence of the internal rotation and 1 patient with scarring of the triceps requiring tenolysis. CONCLUSIONS: The DHO is an effective procedure for correcting the internal rotation position of the upper extremity in arthrogryposis and the surgery improves hand opposition with minimal complications. Universally, there was perceived improved function with high postoperative PODCI Happiness scores. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Parental Assessment of Status of Congenital Upper Limb Differences: Analysis of the Pediatric Outcomes Data Collection Instrument.

PURPOSE: To determine the range of the Pediatric Outcomes Collection Instrument (PODCI) scores for children with a wide variety of congenital upper limb differences and to examine the scoring effect of the patient's surgical history, family history, severity of involvement, and syndromic associations. METHODS: We reviewed the PODCI scores for 109 patients, aged 2-18 years, treated for nontraumatic upper extremity conditions. Charts were reviewed for sex, age, extent of limb involvement, laterality, family history, surgical history, and syndrome association. All patients were classified based on the Oberg, Manske, Tonkin classification with general categories of malformation, deformation, or dysplasia. RESULTS: Of 109 patients, 80 (73%) had a malformation, 12 (11%) had a deformation, and 17 (16%) had a dysplasia. The cohort as a whole had a happiness PODCI score that was similar to the normal population, yet a lower (worse) PODCI score for upper extremity and global function. Patients with a dysplasia had a higher upper extremity function scores than those with malformations or deformations, but they had similar happiness and global function scores. Complete upper limb involvement and lower extremity involvement statistically lowered the PODCI score within our study cohort, whereas a positive family history and syndromic association increased PODCI scores. CONCLUSIONS: This study showed that there was a similar level of perceived happiness between children/adolescents with congenital upper extremity conditions compared with the normal pediatric population based on PODCI scores. In contrast, the perceived upper extremity and global function was significantly decreased in patients with congenital differences compared with normal individuals. This investigation also revealed that the extent of upper extremity involvement, lower extremity involvement, family history, and syndromic association may affect PODCI scores as independent variables and should be taken into consideration in studies of upper extremity congenital anomalies. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Outcomes of Therapy and Ulnar Nerve Transposition for Elbow Stiffness After Pediatric Medial Epicondyle Fractures.

Purpose: Following medial epicondyle fractures, a subset of pediatric patients has persistent limitations in elbow motion. This study soughted to understand the patient characteristics of this group and to assess the effectiveness of intensive therapy and ulnar nerve transposition in improving elbow range of motion and patient-reported outcomes. Methods: A cohort of 31 pediatric patients with stiffness after elbow trauma was narrowed to 8 pediatric patients (7 female) ranging in age from 9 to 14 years, who were diagnosed with medial epicondyle fractures and underwent intensive therapy and ulnar nerve transposition with or without elbow joint release. We collected demographic and objective data as well as subjective data including Patient-Reported Outcome Measurement Information System (PROMIS) scores before and after ulnar nerve transposition. Results: Following initial intensive therapy, elbow range of motion improved by an average of 56°, and 7 of the 8 patients reached a functional motion arc of 100°. Subsequently, following ulnar nerve surgery with or without elbow release, motion improved by an average of 22°, and 5 of the 8 patients demonstrated improvement from this intervention. Surgery led to improvements in subjective outcomes with an improvement in PROMIS mobility scores by an average of 9 points, pain interference by 6 points, and upper extremity scores by 3 points. Based on a previously determined minimally important difference of three points, these indicate significant clinical improvements. Conclusions: A subset of pediatric patients with persistent stiffness following medial epicondyle fractures may benefit from additional interventions, including intensive therapy, transposition of the ulnar nerve, and open capsular release. However, not all patients were improved after ulnar nerve surgery, and the identification and treatment of ulnar nerve irritability may not fully resolve preoperative symptoms in all patients. Type of study/level of evidence: Therapeutic IV.

Barriers to mammography among inadequately screened women.

Mammography use has increased over the past 20 years, yet more than 30% of women remain inadequately screened. Structural barriers can deter individuals from screening, however, cognitive, emotional, and communication barriers may also prevent mammography use. This study sought to identify the impact of number and type of barriers on mammography screening status, and to examine whether number and type of barriers are different for never-screened and off-schedule women. A total of 182 women aged 40 years or older completed a computer kiosk facilitated survey as part of a larger patient navigator intervention. Logistic regression analysis indicated that breast cancer knowledge predicted whether a woman had ever had a mammogram (odds ratio [OR] = 1.04, 95% confidence interval [CI] = 1.02-1.06, p = .0003), while the number of emotional, structural, and communication barriers predicted whether a woman was on-schedule for mammograms (OR = 0.79, 95% CI = 0.65-0.95, p = .0127). The results suggest that to increase the use of mammography at recommended regular intervals, interventions should be tailored toward current screening status.

Levetiracetam for benign epilepsy of childhood with centrotemporal spikes-three cases.

Benign epilepsy with centrotemporal spikes (BECTS), also known as benign rolandic epilepsy (BRE) of childhood represents 15% of all childhood epilepsies [Handbook of Epilepsy Treatment (2000)]. A majority of these patients do no require treatment; however, in those cases where treatment is justified, the most efficacious medication with a benign safety profile should be selected. We present three clinical cases of otherwise healthy children with BECTS who were treated only with levetiracetam. All three of these children remain seizure-free and are experiencing no reported side effects.