Three decades of progress from surgery to medical therapy for isolated neuroaxis BRAF V600E-positive Langerhans cell histiocytosis management: illustrative case.

BACKGROUND: "Langerhans cell histiocytosis" (LCH) is a term that encompasses single-system or multisystem disorders traditionally characterized by a proliferation of clonal CD1a+/CD207+ myeloid-derived histiocytes. In most cases of LCH, mitogen-activated protein kinase (MAPK) pathway somatic mutations lead to near universal upregulation of phosphorylated extracellular signal-regulated kinase expression. The clinical manifestations of LCH are numerous, but bone involvement is common. Intracranial lesions, especially as isolated manifestations, are rare. OBSERVATIONS: The authors presented the case of a long-term survivor of exclusive intracranial LCH that manifested with isolated craniofacial bone and intraparenchymal central nervous system recurrences, which were managed with 3 decades of multimodal therapy. The patient was initially diagnosed with LCH at age 2 years, and the authors documented the manifestations of disease and treatment for 36 years. Most of the patient's treatment course occurred before the discovery of BRAF V600E. Treatments initially consisted of chemotherapy, radiosurgery, and open resections for granulomatous LCH lesions. Into young adulthood, the patient had a minimal disease burden but still required additional radiosurgical procedures and open resections. LESSONS: Surgical treatments alleviated the patient's immediate symptoms and allowed for tumor burden control. However, surgical interventions did not cure the underlying, aggressive disease. In the current era, access to systemic MAPK inhibitor therapy for histiocytic lesions may offer improved outcomes.

Predictors and Outcomes of Ischemic Stroke After Cardiac Surgery.

BACKGROUND: Stroke is a major complication after cardiac surgery causing increased morbidity and mortality. There are limited data on outcomes of patients with large vessel occlusion after cardiac surgery. METHODS: Patients who underwent index cardiac surgeries as defined by The Society of Thoracic Surgeons (STS) from 2010 to 2017 were reviewed from a prospectively maintained database. All patients with neurologic deficits were identified, and only patients with ischemic strokes were included. RESULTS: A total of 10,250 patients underwent cardiac surgical procedures. Postoperative stroke with neurologic deficits occurred in 221 patients (2.16%). Of these, 53 patients (24%) had large vessel occlusion. Patients who had a postoperative stroke were older and more likely to be female. These patients had higher STS predicted mortality and longer bypass time, cross-clamp time, total intensive care unit stay, and total hospital stay. Operative mortality was significantly higher for patients who had postoperative stroke (14.93% vs 2.15%, P < .001). Kaplan-Meier survival estimates demonstrated worse survival for the large vessel occlusion cohort at 1 year (54.72% vs 75%, P = .002). Predictors of stroke included increasing age, known cerebrovascular disease, diabetes mellitus, and emergent operative status. The most significant predictors of operative mortality included emergent operative status and New York Heart Association stage IV heart failure. There was no difference in 30-day, 1-year, and 5-year mortality between the intervention group and the medically managed patients in the large vessel occlusion cohort. CONCLUSIONS: Stroke is a devastating complication after cardiac surgery that increases operative morbidity and mortality. Stroke with large vessel occlusion was associated with worse survival. However, early intervention did not impart a survival benefit.

Evaluation of a simulation-based mini-elective on medical student interest in cardiac surgery.

BACKGROUND: Integrated cardiothoracic (CT) surgery training programs are an increasingly popular pathway to train CT surgeons. Identifying and engaging medical students early is important to generate interest and ensure highly qualified applicants are aware of opportunities provided by a career in CT surgery. METHODS: An optional CT surgery "mini-elective" was developed for preclinical medical students consisting of five 2-hour sessions covering major procedures in cardiac surgery. Each session had an inital 1 hour lecture immediatly followed by a hands on simulation component. Sessions were taught by CT surgery faculty and residents. A precourse and postcourse survey was administered to identify interest in and awareness of the field of CT surgery. RESULTS: There were 22 students enrolled in the course who provided precourse surveys, while 21 provided postcourse surveys. CT surgery was a career consideration for 95.4% of students who took the mini-elective. nine percent of the students who had either scrubbed or observed a CT case precourse, increased to 33.3% postcourse (P = .11). With regards to mentorship, 23.8% felt they could easily find a mentor in CT surgery precourse, increasing to 66.7% postcourse (P = .01). Eighty-one percent of students reported that the mini-elective significantly increased their CT knowledge over the standard cardiovascular curriculum, and 100% of those completing the course were "extremely satisfied" with the experience. CONCLUSIONS: A CT surgery mini-elective increased awareness and interest in the field among preclinical medical students. Longitudinal exposure and mentorship provided in programs such as this will be key to the continued recruitment of high-quality medical students to the field.