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BackgroundIntrathecal nusinersen administration, a fundamental step in the treatment of spinal muscular atrophy, is challenging in children. AimsThis retrospective monocentric analysis of prospectively collected data evaluated the feasibility of needleless general anesthesia exclusively with sevoflurane, without imaging guidance, for children undergoing nusinersen administration in a 24-month period. MethodsClinical data included demographics, type of spinal muscular atrophy, presence and severity of scoliosis. Primary outcome was defined by the number of predefined sentinel adverse events related to anesthesia. Secondary outcomes were assessed by duration of the procedure, number of lumbar puncture attempts, and number of failures. Other measures included number and type of moderate, minor and minimal adverse events, as well as number and type of puncture-related adverse events. Results116 patients (mean age: 8.7 (SD 6.9) years; with scoliosis: 49.1%) underwent 250 lumbar punctures; two cases of prolonged desaturation, considered as sentinel adverse events, (0.8%) were recorded during anesthesia (primary outcome). None of the patients underwent orotracheal intubation nor required an unplanned admission in the Pediatric Intensive Care Unit. No patient required an unplanned or prolonged hospitalization after the procedure. Mean number of puncture attempts was 1.6 (SD 1.3), and mean duration of the procedure was 14.1 (SD 8.3) minutes. No failure in the drug administration occurred (secondary outcomes). ConclusionIn this single-center experience, needleless general anesthesia with inhaled sevoflurane without imaging guidance has been shown to be feasible for children with spinal muscular atrophy undergoing lumbar puncture for nusinersen administration.

Background: Both intracavitary electrocardiography (IC-ECG) and ultrasound (US) have been proven to be safe and accurate for intraprocedural tip location during central venous catheterization, and both are known to be easily applicable and feasible in pediatric patients. Though, no prospective clinical study has directly compared the two methods as regards their applicability, feasibility, and procedural time. Methods: This study prospectively enrolled all children requiring a central venous access device in non-emergency situations, during a period of 1 year. All devices were inserted according to a well-defined insertion bundle including both IC-ECG and US-based tip location. The primary endpoint of the study was to compare the two methods in terms of applicability, feasibility and time required. Results: This study included 100 consecutive central venous catheterizations in children of age ranging from 1 month to 18 years. The applicability of IC-ECG based tip location was 98% and its feasibility 100%; the time required for IC-ECG was 1.9 ± 2 min. The applicability of US-based tip location was 96% and its feasibility was 100%; the maneuver required 2.2 ± 3 min. Conclusions: US is an appropriate alternative method for intraprocedural tip location in children. The combined use of US and IC-ECG (both maneuvers being accurate, inexpensive, cost-effective, non-invasive, and equally fast to perform) should be recommended for tip location in pediatric patients, and it will avoid completely the use of fluoroscopy or of post-procedural x-ray.