Failure of Mandibular Distraction Osteogenesis in Klippel- Feil Syndrome- 4: A Case Report of a Rare Syndromic Robin Sequence.

Klippel-Feil syndrome-4 (KFS4), a rare autosomal recessive form of Klippel- Feil syndrome, is characterized by facial dysmorphism, nemaline myopathy, and short stature. Only 10 cases of KFS4 have been previously published in the literature. We report a novel case of a 1- month-old girl with known KFS4 and Robin Sequence (RS). At 2 months old, she underwent bilateral mandibular distraction osteogenesis to correct significant airway obstruction. Despite adequate mandibular advancement, the patient failed extubation twice and eventually required a tracheostomy. Due to the multiple anomalies present in KFS4, mandibular distraction osteogenesis may have a decreased likelihood of surgical success.

X-ray therapy safety and awareness education for medical trainees and attending physicians.

INTRODUCTION: Medical imaging involving ionizing radiation is common in the clinical setting. Little is known about the level of radiation safety training for medical trainees and attending physicians. We sought to identify the level of radiation safety knowledge and training at the undergraduate, postgraduate, and attending physician level. METHODS: A 29-question survey was sent by email to two sites in Canada. We pooled the results of medical students, residents, and attending physicians. The primary outcome was to describe the amount of radiation safety training among these groups. The secondary outcomes were to describe the frequency of radiation exposure, level of radiation knowledge, and preferred training method for radiation safety. RESULTS: Of 115 surveys that were properly completed, 31 (26.9%) medical students, 17 (14.7%) residents, and 67 (58.3%) attending physicians responded. A greater number of medical students (41.9%) reported they had zero hours of training time for radiation safety compared to attending physicians (14.9%) (p<0.05). A higher number of attending physicians (47.8%) and residents (64.7%) participated in patient care involving fluoroscopy daily or at least several times per week compared to medical students (3.2%) (p<0.001). Attending physicians had the greatest number of correct responses to radiation safety questions. Online courses and workshops were the preferred training methods. CONCLUSIONS: Radiation safety training is an important component of medical education for medical trainees and attending physicians. Current radiation safety training requirements and procedures at various levels of medical training in Canada should be addressed. Implementing radiation safety education may improve adherence to the radiation safety principles.

Surgical mesh information on YouTubeTM: Evaluating the usage and reliability of videos for patient education.

INTRODUCTION: Patients in search of answers to health-related questions often seek out information on the internet. The current study aimed to evaluate the quality of videos on the topic of mesh pertaining to its use in the treatment of stress urinary incontinence or pelvic organ prolapse. METHODS: A total of 100 videos on the topic of mesh on YouTubeTM were screened in this study. From that, a further 30 were selected for review. Five experts in the medical field reviewed each video anonymously, using two video assessment tools. Video characteristics were collected and evaluated. Videos were assessed based on a Global Assessment Score (GAS) and Patient Education Tool for Audiovisual Materials (PEMAT-A/V) scale for ease of patient access and comprehension. The overall correlation between raters and videos was also compared. RESULTS: The GAS and PEMAT-A/V ratings correlation across multiple raters demonstrated excellent inter-rater reliability. We found that the overall GAS score and recommendation was substandard, and the median PEMAT-A/V understandability score was 70% (poorly understandable). Most videos contained some form of marketing, and a scarce number had reliable sources of information. Evidence of neutrality was low. CONCLUSIONS: Through the expert assessment of videos using quality assessment tools, this study demonstrated the overall variable quality of mesh videos on YouTubeTM and the need for further education regarding patient resources.

Outcomes of Patients with Small Intestine Adenocarcinoma in a Canadian Province: A Retrospective Multi-Center Population-Based Cohort Study.

Background: Small intestine adenocarcinoma is a rare cancer. The current study aims to determine the outcomes of patients with small intestine adenocarcinoma in a Canadian province. Methods: This retrospective population-based cohort study assessed patients with small intestine adenocarcinoma who were diagnosed from 2008 to 2017 in Saskatchewan. A Cox proportional multivariate regression analysis was performed to determine the correlation between survival and exploratory factors. Results: 112 eligible patients with a median age of 73 years and M:F of 47:53 were identified. Overall, 75% had a comorbid illness, and 45% had a WHO performance status >1. Of the 112 patients, 51 (46%) had early-stage disease and 61 (54%) had advanced-stage disease. The median overall survival (mOS) was as follows: stage one, 59 months; stage two, 30 months; stage three, 20 months; and stage four, 3 months (p < 0.001). The median disease-free survival of patients with stage three disease who received adjuvant chemotherapy was 26 months (95% CI:23.1−28.9) vs. 4 months (0.0−9.1) with observation (p = 0.04). Patients who received chemotherapy for advanced disease had a mOS of 10 months (3.5−16.5) vs. 2 months (0.45−3.6) without chemotherapy (p < 0.001). In the multivariate analysis, stage four disease, hazard ratio (HR), 3.20 (1.84−5.40); WHO performance status >1, HR, 2.22 (1.42−3.45); lack of surgery, HR, 2.10 (1.25−3.50); and a neutrophil:lymphocyte ratio of >4.5, HR, 1.72 (1.10−2.71) were significantly correlated with inferior survival. Conclusions: Most patients with small intestine adenocarcinoma were diagnosed with advanced-stage disease. Advanced-stage disease, poor performance status, lack of surgery and a baseline neutrophil:lymphocyte ratio >4.5 were correlated with inferior survival.