Medulloblastoma is the most common form of brain malignancy of childhood. The mainstay of epidemiological data regarding childhood medulloblastoma is derived from case series, hence population-based studies are warranted to improve the accuracy of survival estimates. To utilize a big-data approach to update survival estimates in a contemporary cohort of children with medulloblastoma. We performed a population-based retrospective observational cohort study utilizing the Surveillance, Epidemiology, and End Results Program database that captures all children, less than 20 years of age, between 1973 and 2012 in 18 geographical regions representing 28% of the US population. We included all participants with a presumed or histologically diagnosis of medulloblastoma. The main outcome of interest is survivors at 1, 5 and 10 years following diagnosis. A cohort of 1735 children with a median (interquartile range) age at diagnosis of 7 (4-11) years, with a diagnosis of medulloblastoma were identified. The incidence and prevalence of pediatric medulloblastoma has remained stable over the past 4 decades. There is a critical time point at 1990 when the overall survival has drastically improved. In the contemporary cohort (1990 onwards), the percentage of participants alive was 86, 70 and 63% at 1, 5 and 10 years, respectively. Multivariate Cox-Regression model demonstrated Radiation (HR 0.37; 95% CI 0.30-0.46, p < 0.001) and Surgery (HR 0.42; 95% CI 0.30-0.58, p < 0.001) independently predict survival. The probability of mortality from a neurological cause is <5% in patients who are alive 8 years following diagnosis. The SEER cohort analysis demonstrates significant improvements in pediatric medulloblastoma survival. In contrast to previous reports, the majority of patients survive in the modern era, and those alive 8 years following initial diagnosis are likely a long-term survivor. The importance of minimizing treatment-related toxicity is increasingly apparent given the likelihood of long-term survival.
The reported success of ETV/CPC for infantile hydrocephalus is higher in sub-Saharan Africa than developed nations. Large long-term prospective multi-center observational studies addressing patient-important outcomes are required to further evaluate the efficacy and safety of this re-emerging procedure.
Background In the pediatric population, bony defects of the extremities pose a significant challenge for which free vascularized fibular grafts (FVFGs) represent a valuable reconstructive option. The purpose of this study was to explore surgical outcomes, complications, and long-term functionality of FVFG for this patient group.
Methods Using MEDLINE and PubMed databases, studies were identified of pediatric extremity reconstruction using FVFG which reported functional outcomes and/or complications. The operative logs at a tertiary pediatric center were then reviewed for cases of FVFG between January 2000 and 2017. Demographic characteristics, surgical indications, operative details, graft survival, bony union, complications, and functionality of the reconstruction were recorded.
Results A total of 366 studies were identified with 23 ultimately meeting inclusion criteria in the systematic review. In the institutional series, 29 patients were included with mean age of 9.7 years (1–17 years). Indications for reconstruction included malignant bone tumor (n = 11), osteomyelitis (n = 9), congenital pseudoarthrosis (n = 6), and osteofibrous dysplasia (n = 3). Major postoperative complications included graft nonunion (24.1%), fracture (17.2%), and hardware failure (17.2%). Thirteen patients (44.8%) experienced delayed complications, while two (6.8%) experienced an immediate complication. Complications occurred in the donor site in 2 cases and the recipient site in all 13 cases. Long-term graft survival was achieved in 27 patients (93.1%), and 23 (79.3%) had full functional recovery, with an average Musculoskeletal Tumor Society score of 90% (60–100%). Mean follow-up was 5.17 years (2–12 years).
Conclusion This review and institutional series demonstrate the versatility of FVFG to facilitate limb reconstruction in large defects or serve as a salvage option in complex cases. While immediate and donor-site complications are uncommon, delayed recipient-site complications are frequent, and patients and families should be counseled regarding this expectation. Though technically challenging, excellent long-term graft viability and functional recovery can be achieved in the large majority of patients.
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