Background: Temporal bone meningoencephalocele (TB-MEC) diagnosed with or following cholesteatoma is a rare yet complex neurotological scenario. This study aims to characterize the clinical features and treatment strategies to facilitate clinicians’ decision-making and patients’ counseling. Methods: We conducted a retrospective case series with a systematic literature review of the past 3 decades. For the literature review, PRISMA guidelines were followed, with articles sourced from PubMed and EMBASE. The study examined demographics, clinical aspects, imaging findings, surgical approaches, and postoperative outcomes. Results: We identified 75 cases (n=72 from the literature and n=3 original). The majority of the patients with TB-MEC had prior cholesteatoma surgeries (79%), mostly canal wall down (CWD) mastoidectomy, with a relatively high complication rate (15%) at presentation. TB-MECs were incidentally discovered intraoperatively in 39.5% of the cases. Most (65%) of the preoperatively diagnosed had major defects (>1cm). Tegmen mastoidum was involved in 68%. Surgical treatment employed trans-mastoid approach in 72%, including minor and major defects, whereas middle fossa or combined approaches were reserved for major defects only. Autologous grafts, mainly temporalis fascia, were the primary choice for reconstruction (98%), regardless of defect sizes or surgical approach. Single surgery was mostly sufficient, irrespective of defect size (100% and 93% for minor and major defects), and complications were minimal. Conclusions: Despite advancements in imaging, TB-MED are still discovered intraoperatively at a substantial rate. High-risk cases are patients with prior complications and multiple past surgeries, mainly CWD. Although previously described complications were ominous, today, a single-stage procedure yields positive outcomes.

Background: Given the good prognosis of low-risk papillary thyroid microcarcinomas (lrPTMCs) accurate risk stratification is valuable to optimize management: active surveillance vs. surgery. BRAFV600E testing was associated with increased recurrence risk, hence AS seems reasonable for mutation-negative lrPTMC. However, when considering AS, patient perception is highly important as adherence and emotional aspects are challenging. In this study, we aimed to model the contribution of BRAFV600E testing for the management of PTMCs when tailored to the patient perspective. Methods: We developed a Markovian model to predict the role of BRAFV600E in prioritizing between hemithyroidectomy (HT) and active surveillance (AS) for lrPTMCs. We used a simulated cohort of lrPTMCs, with probabilities of each strategy driven from previous literature. Outcomes were measured as quality-of-life years (QALYs). One- and two-way sensitivity analyses were conducted to ascertain model robustness. Results: We found that the optimal strategy (e.g., that would maximize QALYs) varies according to BRAFV600E status for patients without a preset predilection between AS to HT. Using one-way sensitivity analysis, we found that the two main variables that have the strongest impact on the decision are the utility of AS and the utility of a disease-free state after HT. Two-way sensitivity analysis demonstrated that BRAFV600E status can define the optimal strategy for patients in the middle zone of the utility range (e.g., patients without clear preference). Conclusions: Our model suggests that BRAFV600E status can facilitate decision-making regarding AS vs. HT for patients without preset predilection. Our model supports further real-life studies of BRAFV600E testing for PTMCs.