The Impact on Families of Children With Congenital Upper Extremity Differences.

PURPOSE: To investigate the impact on caregivers of caring for a child with congenital upper extremity differences. METHODS: In this cross-sectional study, caregivers of patients enrolled in the multi-institutional Congenital Upper Limb Difference (CoULD) registry were contacted. Demographic information and the Impact on Family Scale (IOFS), a validated measure of perceived caregiver strain, were collected. Patient-reported outcome measures from the CoULD registry, the Pediatric Outcomes Data Collection Instrument (PODCI), and Patient-Reported Outcomes Measurement Information System (PROMIS) were also analyzed for correlation with IOFS. RESULTS: Two hundred ninety-nine caregivers participated. Factors with significantly stronger impact on family included public insurance; bilateral upper extremity involvement; household income of $20,000-40,000; additional musculoskeletal diagnosis; and a single adult caregiver household. There was a significantly increased subcategory of IOFS-Finance score for distant travel to see the surgeon. Additionally, all categories of the PODCI (upper extremity, mobility, sports, pain, happiness, and global) demonstrated a negative correlation with IOFS. PROMIS upper extremity and peer relations also demonstrated an inverse relationship with IOFS, whereas PROMIS pain interference had a positive correlation with IOFS. The overall IOFS for children with CoULDs was greater than previously reported for children with brachial plexus birth injury, and less than cerebral palsy and congenital heart disease. CONCLUSIONS: Caregivers of children with congenital upper extremity differences report a significant impact on family life. Socioeconomic factors, such as economically disadvantaged or single-caregiver households, and clinical factors, such as bilateral upper extremity involvement, correlate with greater family impact. These findings represent opportunities to identify at-risk families and underscore the importance of caring for the whole family through a multidisciplinary approach. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic II.

Suture Suspension Arthroplasty for Thumb Carpometacarpal Arthritis Reconstruction: 12- to 14-Year Follow-up.

BACKGROUND: Many surgical procedures have been described for the treatment of thumb carpometacarpal (CMC) joint osteoarthritis, with significant variation. To date, none has proven to be superior. The purpose of this study was to report long-term follow-up results of suture suspension arthroplasty (SSA). METHODS: The SSA technique uses a single incision, trapeziectomy, and an intra-articular suture suspension sling anchored into the insertions of the flexor carpi radialis (FCR) and abductor pollicis longus (APL), which serves to stabilize the base of the thumb metacarpal, correct subluxation deformity, and maintain arthroplasty space. Ninety of 153 SSA reconstructions (59% recall) were evaluated at long-term follow-up (mean, 12.6 years). Data were analyzed for functional outcomes, including preoperative and postoperative grip and pinch strength, radiographs, complications, and postoperative Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) scores. RESULTS: The mean age at date of surgery was 61.7 ± 7.6 years (82% women). Significant improvement was noted in grip strength (preoperative mean, 25.0 kg; postoperative mean, 28.0 kg; P < .0001), key pinch (preoperative, 4.2 kg; postoperative, 5.1 kg; P < .0001), and tip pinch (preoperative, 2.9 kg; postoperative, 3.6 kg; P < .0001). Radiographic subsidence averaged 35% (0-90). Postoperative QuickDASH scores (mean, 6.6; range, 0-50) revealed good to excellent pain relief and function. One revision was performed, and postoperative FCR rupture occurred in 3 reconstructions. CONCLUSIONS: The SSA technique for thumb CMC arthritis reconstruction yields good to excellent long-term clinical outcomes. Potential advantages of the SSA include short operative time, a single incision, minimal cost, and no need for tendon harvesting, pin fixation, or implantable hardware.

Osteoarticular allograft reconstruction after distal radius tumor resection: Reoperation and patient reported outcomes.

BACKGROUND: The aims of this study are to evaluate the rate of wrist joint preservation, allograft retention, factors associated with reoperation and to report the patient reported outcomes after osteoarticular allograft reconstruction of the distal radius. METHODS: Retrospective chart review identified 33 patients who underwent distal radius resection followed by osteoarticular allograft reconstruction, including 27 giant cell tumors and 6 primary malignancies. Ten patients with a preserved wrist joint completed the QuickDASH, PROMIS-CA physical function, and Toronto extremity salvage score (TESS) at a median of 13 years postoperatively. RESULTS: The allograft retention rate was 89%, and an allograft fracture predisposed to conversion to wrist arthrodesis. The reoperation rate was 55% and 36% underwent wrist arthrodesis at a median of 4.2 years following index surgery. The use of locking plate fixation was associated with lower reoperation and allograft fracture rates. Patients reported a median QuickDASH of 10.2 (range: 0-52.3), a mean PROMIS physical function of 57.8 (range: 38.9-64.5) and the median TESS was 95.5 (range: 67.0-98.4). CONCLUSION: Osteoarticular allograft reconstruction results in acceptable long-term patient reported outcomes, despite a high revision rate. Allograft fixation with locking plates seems to reduce the number of reoperations and allograft fractures, along with reduction in wrist arthrodesis rates.

A Desmoid Tumor Involving the Subscapularis Muscle: A Case Report.

CASE: A 31-year-old woman developed worsening pain and paresthesia in the neck, shoulder, and arm over a period of 6 years. Magnetic resonance imaging revealed a soft-tissue mass in the subscapular region, with likely involvement of the scapula and the subscapularis muscle. The mass was resected, and the final histologic diagnosis was desmoid-type fibromatosis. CONCLUSION: Desmoid tumors with subscapularis muscle involvement are exceedingly rare. Although limited range of motion is the more common presentation for these tumors, this case demonstrates that desmoid tumors may present with primarily neurologic symptoms.

Baroreflex Sensitivity Impairment During Hypoglycemia: Implications for Cardiovascular Control.

Studies have shown associations between exposure to hypoglycemia and increased mortality, raising the possibility that hypoglycemia has adverse cardiovascular effects. In this study, we determined the acute effects of hypoglycemia on cardiovascular autonomic control. Seventeen healthy volunteers were exposed to experimental hypoglycemia (2.8 mmol/L) for 120 min. Cardiac vagal baroreflex function was assessed using the modified Oxford method before the initiation of the hypoglycemic-hyperinsulinemic clamp protocol and during the last 30 min of hypoglycemia. During hypoglycemia, compared with baseline euglycemic conditions, 1) baroreflex sensitivity decreases significantly (19.2 ± 7.5 vs. 32.9 ± 16.6 ms/mmHg, P < 0.005), 2) the systolic blood pressure threshold for baroreflex activation increases significantly (the baroreflex function shifts to the right; 120 ± 14 vs. 112 ± 12 mmHg, P < 0.005), and 3) the maximum R-R interval response (1,088 ± 132 vs. 1,496 ± 194 ms, P < 0.001) and maximal range of the R-R interval response (414 ± 128 vs. 817 ± 183 ms, P < 0.001) decrease significantly. These findings indicate reduced vagal control and impaired cardiovascular homeostasis during hypoglycemia.

Osteosarcoma: prognosis plateau warrants retinoblastoma pathway targeted therapy.

Osteosarcoma (OS) is the most common primary bone cancer in children and adolescents, affecting ~560 young patients in the United States annually. The term OS describes a diverse array of subtypes with varying prognoses, but the majority of tumors are high grade and aggressive. Perhaps because the true etiology of these aggressive tumors remains unknown, advances in OS treatment have reached a discouraging plateau, with only incremental improvements over the past 40 years. Thus, research surrounding the pathogenesis of OS is essential, as it promises to unveil novel therapeutic targets that can attack tumor cells with greater specificity and lower toxicity. Among the candidate molecular targets in OS, the retinoblastoma (RB) pathway demonstrates the highest frequency of inactivation and thus represents a particularly promising avenue for molecular targeted therapy. This review examines the present thinking and practices in OS treatment and specifically highlights the relevance of the RB pathway in osteosarcomagenesis. Through further investigation into RB pathway-related novel therapeutic targets, we believe that a near-term breakthrough in improved OS prognosis is possible.