2 research outputs found
Oral and craniofacial manifestations of Ellis-Van Creveld syndrome: A systematic review
Aim A systematic literature review on oral and craniofacial manifestations of Ellis-Van Creveld syndrome was performed. Methods From 2 databases were selected 74 articles using as key words "Ellis-Van Creveld" AND "Oral" OR "Craniofacial" OR "Dental" OR "Malocclusion". Prisma protocol was used to create an eligible list for the screening. Data were collected in a table to compare the clinical aspects found. Results From the first research emerged 350 articles, and only 72 of them were selected. Conclusion Through this analysis oral and cranio-facial manifestations of Ellis-Van Creveld syndrome were pointed out. Management of Ellis-Van Creveld syndrome requires a multidisciplinary approach involving different clinicians; dentists play an important role in early diagnosis and treatment of the patients
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Clinical and Imaging Outcomes After Trigeminal Schwannoma Radiosurgery: Results From a Multicenter, International Cohort Study
BACKGROUND AND OBJECTIVESAn international, multicenter, retrospective study was conducted to evaluate the long-term clinical outcomes and tumor control rates after stereotactic radiosurgery (SRS) for trigeminal schwannoma. METHODSPatient data (N = 309) were collected from 14 international radiosurgery centers. The median patient age was 50 years (range 11-87 years). Sixty patients (19%) had prior resections. Abnormal facial sensation was the commonest complaint (49%). The anatomic locations were root (N = 40), ganglion (N = 141), or dumbbell type (N = 128). The median tumor volume was 4 cc (range, 0.2-30.1 cc), and median margin dose was 13 Gy (range, 10-20 Gy). Factors associated with tumor control, symptom improvement, and adverse radiation events were assessed. RESULTSThe median and mean time to last follow-up was 49 and 65 months (range 6-242 months). Greater than 5-year follow-up was available for 139 patients (45%), and 50 patients (16%) had longer than 10-year follow-up. The overall tumor control rate was 94.5%. Tumors regressed in 146 patients (47.2%), remained unchanged in 128 patients (41.4%), and stabilized after initial expansion in 20 patients (6.5%). Progression-free survival rates at 3 years, 5 years, and 10 years were 91%, 86%, and 80 %. Smaller tumor volume (less than 8 cc) was associated with significantly better progression-free survival (P = .02). Seventeen patients with sustained growth underwent further intervention at a median of 27 months (3-144 months). Symptom improvement was noted in 140 patients (45%) at a median of 7 months. In multivariate analysis primary, SRS (P = .003) and smaller tumor volume (P = .01) were associated with better symptom improvement. Adverse radiation events were documented in 29 patients (9%). CONCLUSIONSRS was associated with long-term freedom (10 year) from additional management in 80% of patients. SRS proved to be a valuable salvage option after resection. When used as a primary management for smaller volume tumors, both clinical improvement and prevention of new deficits were optimized