Long-Term Follow-up of Untreated Adult Patients with Spondylothoracic Dysostosis (Jarcho-Levin Syndrome).

BACKGROUND: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history. METHODS: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed. RESULTS: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points). CONCLUSIONS: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered. LEVEL OF EVIDENCE: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Opioid-Sparing Multimodal Analgesia Efficacy in Hispanic Patients Undergoing Total Knee Arthroplasty.

Adequate pain control after total knee arthroplasty (TKA) has been associated with improved patient mobility and satisfaction, and is a task that has historically relied on opioids. Multimodal analgesic regimens can be considered a therapeutic alternative for postoperative pain control to avoid opioid consumption and its adverse side effects. This study aimed to evaluate the use of a multimodal analgesia protocol based on nonsteroidal anti-inflammatory drugs (NSAIDs) and acetaminophen as an alternative to reduce opioid consumption in Hispanic patients undergoing primary TKA. Methods: An institutional review board-approved, randomized controlled trial evaluated postoperative pain control after TKA in a Hispanic population. The experimental opioid-sparing group received 30-mg ketorolac intravenously every 6 hours and 1-g acetaminophen orally every 6 hours as the patient requested. The experimental group had the option to use morphine and oxycodone combined with acetaminophen as a rescue therapy. The control group received opioids in the form of 0.1-mg/kg morphine intravenously every 6 hours and/or oxycodone combined with acetaminophen (2.5 and 325 mg), 2 tablets orally every 6 hours, as the patient requested. Sociodemographic, operative, and immediate postoperative data and pain intensity measurements according to the numerical rating scale (NRS) were compared between groups. Analgesic efficacy was assessed according to the NRS scores at 12, 24, and 48 hours postoperatively. Results: Eighty-one Hispanic patients met inclusion criteria: 42 patients in the experimental group and 39 patients in the control group. None of the patients in the experimental group requested oxycodone combined with acetaminophen as a rescue therapy. Only 2 patients in the experimental group used a mean of 3.0 ± 1.4 mg of rescue morphine; in comparison, the control group consumed a mean of 28.0 ± 7.9 mg of morphine and 64.8 ± 26.0 mg of oxycodone per patient. A pain intensity decrease was observed in the experimental and control groups, with no postoperative evaluation differences at 12 hours (6.7 ± 2.9 compared with 5.9 ± 2.8; p = 0.209), 24 hours (6.2 ± 2.0 compared with 6.1 ± 2.2; p = 0.813), and 48 hours (4.7 ± 2.1 compared with 4.6 ± 1.7; p = 0.835). Conclusions: The use of a multimodal analgesic regimen based on intravenous ketorolac and oral acetaminophen was adequate in reducing postoperative pain and opioid consumption in Hispanic patients undergoing TKA. Level of Evidence: Therapeutic Level I. See Instructions for Authors for a complete description of levels of evidence.

Practice Variation Among Hispanic American Orthopedic Surgeons in the Management of Geriatric Distal Radius Fracture.

INTRODUCTION: There is a controversy in the management of distal radius fractures (DRF) and its criteria for surgical intervention on geriatric patients. The American Academy of Orthopedic Surgeons (AAOS) developed evidence-based guidelines for treatment of DRF. The aim of this study was to evaluate the current practice of Hispanic orthopedic surgeons in the management of geriatric DRF and examine their adherence to AAOS guidelines based on years of surgical experience. MATERIAL & METHODS: A survey was emailed to all orthopedic surgeons who live in Puerto Rico and treated DRF in their daily practice. Responses concerning demographic, management and clinical scenarios were evaluated. For each clinical scenario, treatment of choice was selected with the same fracture in a geriatric and young adult patient. Comparison between years of surgical experience and adherence to the AAOS guidelines was performed. RESULTS: A total of 65 surgeons responded the survey with 65% having >15 years in practice. A high consensus with AAOS guidelines for DRF was found. Use of preoperative radiographs was reported in all respondents, with an additional 12% routine use of preoperative computed tomography scans. Seventy-seven percent of respondents did not allow any range of motion (ROM) at immediate postoperative period, while 23% allowed active or passive ROM. Use of postoperative therapy was reported in 72.3%. Correlation between years of surgical experience showed a higher use of Vitamin C postoperatively for prophylaxis of Complex Regional Pain Syndrome among surgeons <15 years (P = 0.01). A general consensus trend toward operative fixation was noted among geriatric and young adult patients with the same fracture type in all clinical scenarios. DISCUSSION AND CONCLUSIONS: This survey demonstrates a practice variation toward surgical management of geriatric DRF among Hispanic orthopedic surgeons; despite their compliance with the AAOS AUC guidelines. The geriatric DRF management does not vary significantly among years of surgical experience.