The Incidence of Hearing Loss in Trisomy 21

Abstract

OBJECTIVE: 1) Define the role of preoperative investigations for an accurate diagnosis of site/extension of the skull base defect (SBD). 2) Identify different solutions in performing duraplasty, choosing among available techniques/materials. 3) Evaluate the efficacy and safety of the endoscopic endonasal approach in pediatric age. METHOD: From 2000 to 2010, 26 patients (14 males, 12 females, mean age 8.9 years) affected by SBD referred to our Institute. The diagnostic work-up provided endoscopic examination and neuroimaging with CT and MR. Intraoperative intrathecal fluorescein test was performed during surgery to display the defects site/s and ensure a watertight duraplasty. Follow-up was carried out with periodic endoscopic and MR evaluation. RESULTS: Pediatric SBDs represent 9.6% of our experience in cerebrospinal fluid leaks treatment. 14 patients were affected by spontaneous defects, 12 by traumatic defects (2 iatrogenic, 10 accidental). 21 patients were treated with a pure endoscopic technique, 5 patients underwent a combined, endoscopic endonasal and craniotomic surgical approach. Duraplasty was performed with single/double layer (11) or multilayer (15) technique, depending on the site. The most employed materials were autologous. No intra-and postoperative complications were observed. Mean follow up was 5.5 years. CONCLUSION: SBDs are rare conditions, which require surgical treatment in order to avoid serious complications. Our experience highlights that an accurate preoperative assessment of the defect is mandatory to guarantee the proper selection of the surgical technique. The use of dedicated instruments grants a minimally invasive surgical approach, which decreases the risk of functional/aesthetic strings. Larger and longer studies are needed.