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.

Radiographic Features of the Metacarpal in Blauth Type III Thumb Hypoplasia.

PURPOSE: The purpose of this study was to investigate whether radiographs can be used to aid in the determination of Blauth IIIA and IIIB thumbs. METHODS: Six pediatric hand surgeons were asked to evaluate the radiographs of 77 thumbs and classify the thumb as IIIA or IIIB and indicate which morphologic features influenced their decision. Quantitative measurements and ratios of radiographs were obtained and compared between IIIA and IIIB thumbs. RESULTS: The radiographic features selected for type IIIA thumbs include near-normal length and near-normal width and for type IIIB thumbs, abnormally short, tapered proximal end, and round proximal end. The six surveyed surgeons reached consensus in 82% (63/77) of thumbs, and this matched the enrolling surgeon's classification in 77% (59/77) cases. The ratio of the length of the thumb metacarpal compared with the length of the index metacarpal was different between IIIA and IIIB thumbs (66% ± 0.08% and 46% ± 0.18%, respectively). The ratio of the width of the thumb metacarpal shaft at its narrowest aspect to the width of the thumb metacarpal base was notably different between IIIA and IIIB (68% ± 0.13% and 95% ± 0.28%, respectively). CONCLUSIONS: Near-normal length and near-normal width of the metacarpal were used to predict IIIA and abnormally short, abnormally narrow, and a round or tapered base of the metacarpal were used to predict IIIB classification. The length of the thumb metacarpal relative to the index metacarpal is on average 66% of the length of the index metacarpal in IIIA thumbs compared with 46% in IIIB thumbs. The width of the shaft of the thumb metacarpal at its narrowest is 68% of the width of the thumb metacarpal base in IIIA thumbs, indicating a flared base. In IIIB thumbs, the shaft width was on average 95% of the base width, indicating a tapered base. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic level III.

Seasonal Variation in Parental Satisfaction With Pediatric Orthopaedics.

BACKGROUND: The degree of parental satisfaction with health care is determined by the family's characteristics and expectations. Many aspects of human physiology and behavior have seasonal rhythms. The purpose of the present study was to determine whether parental satisfaction scores vary across the year in a pediatric orthopaedic outpatient population. METHODS: We retrospectively reviewed a total of 22,951 parental satisfaction scores related to outpatient pediatric orthopaedic encounters between October 2015 and April 2019. Parental satisfaction was measured using the provider subdomain of the shortened version of the Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS). Families were stratified according to various pediatric orthopaedic subspecialty services. RESULTS: The satisfaction scores of all pediatric orthopaedic encounters combined did not vary significantly across the year (P=0.8745). When the families were stratified into groups by pediatric orthopaedic subspecialty clinic, variation in seasons was not associated with statistically significant variation in satisfaction scores for the elective surgery, sports medicine, trauma/fracture care, and hand/upper extremity services. However, satisfaction with the spine service was significantly lower in the winter compared with the summer and fall (73.9±3.8 vs. 83.5±5.3 and 82.6±3.6, respectively; P=0.0147). CONCLUSIONS: The provision of pediatric spine care in a region with 4 distinct seasons received lower parental satisfaction scores during the winter than during the summer and fall. This seasonal variation is an additional source of bias in the measurement of satisfaction with health care. LEVEL OF EVIDENCE: Level II-retrospective study.

The Utility of the Early Postoperative Follow-up and Radiographs After Operative Treatment of Supracondylar Humerus Fractures in Children.

BACKGROUND: Supracondylar humerus (SCH) fractures are common elbow injuries in pediatric patients. The American Academy of Orthopedic Surgeons published guidelines for the standard of care in the treatment of displaced SCH fractures, however, no recommendations for follow-up care were made. With the recent push to eliminate unnecessary radiographs and decrease health care costs, many are questioning postoperative protocols. The purpose of our study was to evaluate the utility of the 1-week follow-up appointment after closed reduction and percutaneous pinning (CRPP) of displaced SCH fractures. METHODS: A retrospective review performed at a single institution from 2014 to 2016 included patients under 14 years of age with a closed, displaced SCH fracture treated with CRPP. Exclusion criteria included patients without complete clinical or radiographic follow-up. Variables examined included time to initial follow-up, change in treatment plan after 1-week x-rays, complications, demographics, fracture type, pin number and configuration, reduction parameters, immobilization, time to pin removal, duration of casting, and clinical outcome. RESULTS: A total of 412 patients were divided into 2 groups based on time to initial follow-up. Overall, 368 had an initial follow-up at 7 to 10 days (group 1) and 44 at 21 to 28 days (group 2). There was no difference in age, sex, fracture type, pin configuration, or a number of pins between groups. Statistically significant findings included time to initial follow-up and days to pin removal (group 1 at 26.2 d vs. group 2 at 23.8 d), type of immobilization (group 1 with 5% circumferential casts and group 2 with 70%), and time to surgery (26.2 vs. 62.9 h, respectively). There was no significant difference in complication rates and only a 0.5% rate of change in management in group 1. CONCLUSIONS: Early postoperative follow-up and radiographs did not change the patient outcome and might be eliminated in children with displaced SCH fractures treated with CRPP. Given the current focus of on efficiency and cost-effective care, eliminating the 1-week postoperative appointment would improve appointment availability and decrease medical cost. LEVEL OF EVIDENCE: Level III-Therapeutic.

Shoulder Release and Tendon Transfer following Neonatal Brachial Plexus Palsy: Gains, Losses, and Midline Function.

BACKGROUND: Shoulder release and tendon transfer is frequently performed to address persistent weakness from neonatal brachial plexus palsy. Although postoperative improvements in motion are well described, associated deficits are poorly documented, and functional assessments are lacking. Loss of ability to reach midline can occur with surgery and may result in impairment. The purpose of this study was to comprehensively assess the gains, losses, functional changes, and patient-reported outcome associated with the authors' surgical approach. METHODS: Consecutive patients undergoing surgery with 2-year follow-up were included (n = 30). Prospectively recorded assessments by therapists were reviewed. Changes were assessed by t test and Wilcoxon rank sum (p < 0.05). RESULTS: Active external rotation and abduction improved and internal rotation diminished. Aggregate modified Mallet score increased with improvements in all subscales, except that hand to spine was unchanged and hand to belly decreased. Functional assessment using the Brachial Plexus Outcome Measure revealed an increase of aggregate score, with no decline in any subscales. Improvements were in hand to back of head, forward overhead reach, holds plate with palm up, opening large container, and strings bead. Aggregate patient self-report of appearance and function increased (from 18 to 23). Loss of ability to reach midline occurred in three patients (10 percent) who had extended Erb or total palsy and preoperative limitations of internal rotation. CONCLUSIONS: Secondary reconstruction rebalances shoulder motion by increasing external rotation and abduction and reducing internal rotation. In this study, a conservative surgical approach results in overall improvement in task-based abilities and self-reported outcomes and preservation of internal rotation within a functional range. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Orthopaedic Manifestations in Turner Syndrome.

Turner syndrome is one of the most common chromosomal anomalies occurring in live-born females. It has been extensively reviewed in the medical literature, yet little has been discussed regarding the skeletal manifestations that present to the orthopaedic surgeon. It is important for the orthopaedic surgeon to be familiar with the clinical findings and comorbid conditions in Turner syndrome because they may be the first line of diagnosis when a patient presents for short stature, scoliosis, or slipped capital femoral epiphysis. Recent studies have identified the short stature homeobox gene as the main cause of the skeletal differences in patients with Turner syndrome, affecting longitudinal bone growth. Skeletal deformities including short stature, delayed skeletal maturation, angular deformity of the limbs, spinal deformity, and early-onset osteoporosis have been associated with Turner syndrome. This article will review the skeletal manifestations of Turner syndrome and propose guidelines for the treatment and monitoring of these patients. LEVEL OF EVIDENCE:: Level V.

Laboratory predictors for risk of revision surgery in pediatric septic arthritis.

BACKGROUND: Reported complications of pediatric septic arthritis range from minor growth abnormalities to potentially life-threatening conditions and death; some children require multiple surgeries for eradication of infection. The purpose of this study is: (1) to determine the failure rate of a single surgical incision and drainage (I&D) in pediatric septic arthritis, (2) to identify risk factors for failure which are detectable at the time of initial presentation, and (3) to trend post-operative C-reactive protein (CRP) values to see if there is a difference between children who fail a single I&D and those who do not. METHODS: The medical records for 105 children who underwent operative management of septic arthritis were retrospectively reviewed. Single and multivariate analyses were performed. RESULTS: Eighty-four children required one surgical intervention [mean age 5.18 years (±4.01); 38 females (45 %), 46 males (55 %)], 21 children required revision surgery [mean age 8.16 years (±4.54); 4 females (19 %), 17 males (81 %)], and the overall rate of revision surgery was 20 %. Delayed diagnosis (p = 0.015), elevated CRP at presentation (p = 0.000), positive blood culture (p = 0.000), and age (p = 0.009) were all associated with revision surgery in bivariate analysis. In multivariate analysis, CRP at presentation and positive blood culture were significant risk factors for revision surgery (p = 0.005 and p = 0.025, respectively). Additionally, markedly elevated CRP levels on post-operative days (POD) 1-4 were each independently significant risk factors for requiring multiple surgeries (all p < 0.000). Fever, elevated erythrocyte sedimentation rate, and leukocyte count were not risk factors for multiple surgeries. CONCLUSIONS: In this study, a positive blood culture or marked elevation in CRP at presentation or on POD 1-4 were associated with revision surgery. These findings may help improve surgical planning for both the initial surgery in order to avoid revisions, as well as revision surgery, should it be indicated. LEVEL OF EVIDENCE: III.