To review developmental surgical anatomy and technical nuances related to pediatric skull base surgery. Retrospective, single-center case series with literature review. MD Anderson Cancer Center. Patients undergoing pediatric skull base surgery. Review developmental anatomy of the pediatric skull base as it relates to technical nuance of various surgical approaches and insight gained from a 25-year institutional experience with this unique patient population. Thirty-nine patients meeting these criteria were identified over a 13-year period from 2003 to 2016 and compared to a previously reported earlier cohort from 1992 to 2002. The most common benign pathologies included nerve sheath tumors (11%), juvenile nasopharyngeal angiofibromas (9.5%), and craniopharyngiomas (4.8%). The most common malignancies were chondrosarcoma (11%), chordoma (11%), and rabdomyosarcoma (11%). Varied surgical approaches were utilized and were similar between the two cohorts save for the increased use of endoscopic surgical techniques in the most recent cohort. The most common sites of tumor origin were the infratemporal fossa, sinonasal cavities, clivus, temporal bone, and parasellar region. Gross total resection and postoperative complication rates were similar between the two patient cohorts. Pediatric skull base tumors, while rare, often are treated surgically, necessitating an in depth understanding of the anatomy of the developing skull base.
SUMMARYPurpose: Surgical resection of single, dominant, epileptogenic lesions in patients with tuberous sclerosis complex (TSC) is now accepted as an effective therapy. However, patients with symptomatic tubers in eloquent cortex are sometimes not offered surgery because of the concern for postoperative neurologic morbidity. In this study, we examine our results in children undergoing surgery for resection of tubers and associated seizure foci in rolandic and perirolandic cortex. Methods: Between 1998 and 2008, 52 pediatric patients (mean age 4 years) with TSC underwent epilepsy surgery at the NYU Comprehensive Epilepsy Center. Fifteen of these patients underwent multistage surgery for invasive mapping of seizure foci and surrounding functional cortex followed by resection of tubers/seizure foci in or near rolandic cortex. Data were retrospectively collected and neurologic outcomes were tabulated.Results: Postoperatively, four patients (27%) had either new hemiparesis or worsening of a preexisting hemiparesis. However, all patients were back to their neurologic baselines at 3-month follow-up, yielding no permanent postoperative deficits. The modified Engel outcome was class I in nine patients (60%), class II in three patients (20%), class III in two patients (13%), and class IV in one patient (7%) after 40 months mean follow-up. Discussion: Surgical resection of tubers and associated epileptogenic foci in rolandic and perirolandic cortex in children with TSC is feasible, with low neurologic morbidity, and yields good seizure control. These results suggest that tubers and perituberal epileptogenic foci can be safely resected even in eloquent regions because of reorganization of functional cortex or because these lesions contain no neurologic function.
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