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Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome and Hirschsprung disease (PCWH) is a rare manifestation of Waardenburg-Shah syndrome associated with mutations in theSOX10gene. The phenotypic expression is variable, thus presenting a diagnostic challenge. Clinical manifestations of PCWH may mimic other neurocutaneous syndromes. A thorough history, careful physical examination, appropriate imaging studies and an index of suspicion are needed to diagnose this condition. We describe an adolescent girl with skin hypopigmentation and blue irides associated with sensorineural hearing loss, Hirschsprung disease, as well as seizures with neurological signs, and discuss the challenges in diagnosing PCWH.

Patient engagement technologies (PETs) guide patients through perioperative care, but little is known about their costs-benefits.Retrospective cohort study of patients undergoing elective colorectal, cardiac, thoracic surgery 2015-2020. PET was implemented 2018. Patients were propensity-matched in pre-PET, PET, non-PET groups. Costs of surgical encounter and 30 days post-discharge, mortality, length-of-stay, readmissions, complications, satisfaction were compared.Overall, 4,373 patients underwent surgery and 607 (13.9%) patients enrolled in the PET. PET patients did not have increased costs in any specialty. Colorectal PET patients' variable costs of surgical encounter were $102 lower than non-PET, $1495 lower than pre-PET (p = 0.03). Thoracic PET patients' total costs of surgical encounter were $9224 lower than non-PET, $2187 lower than pre-PET (p = 0.03). Thoracic PET patients had lower mean LOS (2.4 days, 5.1 non-PET, 3.1 pre-PET, p = 0.03). PET patient satisfaction ranged 86.0%-97.8%.Use of a PET did not increase costs and was associated with benefits for patients undergoing elective surgery.

The NCCN Guidelines for Lung Cancer Screening recommend criteria for selecting individuals for screening and provide recommendations for evaluation and follow-up of lung nodules found during initial and subsequent screening. These NCCN Guidelines Insights focus on recent updates to the NCCN Guidelines for Lung Cancer Screening.

Objectives: To identify reporting guidelines related to surgical technique and propose recommendations for areas that require improvement. Study Design and Setting: A protocol-guided scoping review was conducted. A literature search of MEDLINE, the EQUATOR Network Library, Google Scholar, and Networked Digital Library of Theses and Dissertations was conducted to identify surgical technique reporting guidelines published up to December 31, 2021. Results: We finally included 55 surgical technique reporting guidelines, vascular surgery (n = 18, 32.7%) was the most common among the clinical specialties covered. The included guidelines generally showed a low degree of international and multidisciplinary cooperation. Few guidelines provided a detailed development process (n = 14, 25.5%), conducted a systematic literature review (n = 13, 23.6%), used the Delphi method (n = 4, 7.3%), or described post-publication strategy (n = 6, 10.9%). The vast majority guidelines focused on the reporting of intraoperative period (n = 50, 90.9%). However, of the guidelines requiring detailed descriptions of surgical technique methodology (n = 43, 78.2%), most failed to provide guidance on what constitutes an adequate description. Conclusion: Our study demonstrates significant deficiencies in the development methodology and practicality of reporting guidelines for surgical technique. A standardized reporting guideline that is developed rigorously and focuses on details of surgical technique may serve as a necessary impetus for change.

Background Radiation pneumonitis (RP) is a dose-limiting and potentially fatal toxicity of thoracic radiotherapy most often seen in patients treated for primary lung cancer. The purpose of this study was to identify predictors of in-hospital death among lung cancer patients admitted for acute RP in the Healthcare Cost and Utilization Project (HCUP) database. Materials and Methods The HCUP National Inpatient Sample database was queried from 2012 through 2016 to capture adult lung cancer patients admitted to the hospital with a principal diagnosis of acute RP. Multivariate logistic regression modeling and χ2 tests were used to determine predictors of in-hospital death. Results Of the 882 patients with lung cancer admitted for RP, 67 patients (7.6%) died during the hospitalization and 90 patients (10.2%) required mechanical ventilation. Of those requiring mechanical ventilation, 38 patients (42.2%) died. The average age at hospitalization was 70.4 years (range, 35-90). Of those factors associated with death on univariate analysis, interstitial lung disease (odds ratio [OR] = 6.14; 95% confidence interval [CI], 1.9-19.4; P = .002), pulmonary hypertension (OR = 3.1; 95% CI, 1.6-6.2; P = .001), diabetes mellitus (OR = 2.0; 95% CI, 1.1-3.3; P = .013), and more affluent Zip Code (OR = 1.9; 95% CI, 1.1-3.2; P = .021) remained statistically significant on multivariate logistic regression. Conclusion In the largest reported cohort of patients with lung cancer hospitalized with a principal diagnosis of acute RP, the presence of interstitial lung disease, pulmonary hypertension, diabetes mellitus, and more affluent Zip Code were associated with in-hospital death. Comorbid diagnoses may be useful for risk-stratified management of inpatients with RP.

BACKGROUND: Little is known about the effect of the coronavirus disease 2019 (COVID-19) pandemic and the outbreak response measures on door-to-balloon time (D2B). This study examined both D2B and clinical outcomes of patients with STEMI undergoing primary percutaneous coronary intervention (PPCI).Methods and Results:This was a retrospective study of 303 STEMI patients who presented directly or were transferred to a tertiary hospital in Singapore for PPCI from October 2019 to March 2020. We compared the clinical outcomes of patients admitted before (BOR) and during (DOR) the COVID-19 outbreak response. The study outcomes were in-hospital death, D2B, cardiogenic shock and 30-day readmission. For direct presentations, fewer patients in the DOR group achieved D2B time

Background Many patients undergoing general thoracic surgery can be discharged on the same day as chest tube removal, but some are not, leading to increased resource utilization. This study assesses the frequency and duration of extended length of stay (ELOS) after tube removal and identifies risk factors for ELOS. Methods We retrospectively reviewed all adult patients undergoing general thoracic surgery at a tertiary referral medical center captured in the Society of Thoracic Surgeons General Thoracic Surgery Database and obtained detailed clinical data on chest tube management from August 2013 to April 2017. Pre-operative demographics, procedures, diagnoses, comorbidities, hospital service category, and lab values were examined to identify risk factors associated with ELOS after chest tube removal using multivariable generalized linear regression models. Results One thousand and four hundred seventy patients had ≥1 chest tubes placed at the time of operation and discharged after chest tube removal: anatomic lung resection (34%), wedge resection (29%), decortication (16%), and other (21%). Fifty-one percent of these patients were male, 81% were white, and the mean age was 59 years (SD: 15 years). One-third of the patients had prior cardiothoracic operations. Twenty-three percent of these patients had ELOS, defined as discharge ≥1 calendar day after chest tube removal with a median additional hospital stay of 3 days (interquartile range, 2-7 days). A multivariable regression model demonstrated that risk factors for ELOS included being admitted to an oncology or transplant service, undergoing decortication procedure, active smoking, and increased disability. Conclusions Patients with obesity, more severe disability, or actively smoking, undergoing, decortication, admitted to transplant and oncology services were more likely to experience ELOS. These factors should be considered when identifying appropriate patient groups for fast-track algorithms.

OBJECTIVES Surgical management of spontaneous pneumothorax typically involves wedge resection and mechanical pleurodesis. It is unclear whether combining mechanical and chemical pleurodesis can further reduce the recurrence rate. We have performed a meta-analysis of studies comparing the combined approach with mechanical pleurodesis alone. METHODS A comprehensive search of the existing literature was performed using PubMed, EMBASE and Web of Science for all types of studies that compared combined pleurodesis to a single approach. We used the Cochrane Risk of Bias Tool and Strengthening The Reporting of OBservational Studies in Epidemiology (STROBE) to assess the quality of the studies. Relative risk of pneumothorax recurrence was calculated, and the differences between the studies were examined. The primary outcome was the recurrence of pneumothorax. RESULTS Of 2301 eligible studies, 5 studies were included. Five hundred sixty-one patients who received combined pleurodesis were compared to 286 patients who received mechanical pleurodesis only. Patients treated with combined intervention had a 63% lower risk of developing a recurrent pneumothorax compared to single intervention [relative risk 0.37, 95% confidence interval (CI) 0.18–0.76; P = 0.006]. There were no statistically significant differences in the length of stay (standardized mean difference −0.17, 95% CI −0.39 to 0.05, P = 0.138), the duration of postoperative air leak (standardized mean difference 0.17, 95% CI −1.14 to 1.47, P = 0.804) or the duration of postoperative chest tube drainage (standardized mean difference −0.07, 95% CI −0.27 to 0.12, P = 0.471). CONCLUSIONS This meta-analysis demonstrated that combined intervention with mechanical and chemical pleurodesis for spontaneous pneumothorax may be more effective in preventing recurrence than mechanical pleurodesis alone. These findings will provide some guidance to surgeons in the decision-making process.

Return to Work (RTW) programmes have become imperative in manpower scarce countries. This paper describes a RTW programme in a Singapore tertiary hospital, reports patient outcomes and discusses the practicality and effectiveness of the programme. Seventy-three workers participated in the programme over a two-year period. A statistically significant increase in work ability and self-perceived overall health status from first contact with worker (baseline) to discharge was observed. Continued programme participation till first RTW was associated with higher work ability and self-perceived overall health status at baseline. The RTW Coordinator-anchored multidisciplinary model which provided holistic support to the worker and addressed stakeholder interests were central to the programme's success. Greater awareness of RTW programme benefits will improve sustained participation. Our RTW programme features, implementation experiences and participant reported effectiveness may inform the development of improved return to work models.