RESUMO
Background: The study aimed to estimate the prevalence of major congenital anomalies of the alimentary system and the abdominal wall in Thailand using a nationwide hospital discharge database from the National Health Security Office (2017-2020). Methods: The study extracted data from records with International Classification of Diseases-10 (ICD-10) codes related to esophageal malformation (ESO), congenital duodenal obstruction (CDO), jejunoileal atresia (INTES), Hirschsprung's disease (HSCR), anorectal malformation (ARM), abdominal wall defects (omphalocele (OMP) and gastroschisis (GAS)), and diaphragmatic hernia from the database with patient age selection set to less than 1 year. Results: A total of 2539 matched ICD-10 records were found in 2376 individuals over the 4-year study period. Concerning foregut anomalies, the prevalence of ESO was 0.88/10 000 births, while that of CDO was 0.54/10 000 births. The prevalence figures of INTES, HSCR, and ARM were 0.44, 4.69, and 2.57 cases per 10 000 births, respectively. For abdominal wall defects, the prevalences of OMP and GAS were 0.25 and 0.61 cases/10 000 births, respectively. The mortality in our cases was 7.1%, and survival analysis found that associated cardiac defects had a statistically significant influence on survival in most anomalies studied. In HSCR, both Down syndrome (DS) (hazard ratio (HR)=7.57, 95% confidence interval (CI)=4.12 to 13.91, p<0.001) and cardiac defects (HR=5.82, 95% CI=2.85 to 11.92, p<0.001) were significantly associated with poorer survival outcomes. However, only DS (adjusted HR=5.55, 95% CI=2.63 to 11.75, p<0.001) independently predicted worse outcomes by multivariable analysis. Conclusions: Our analysis of the hospital discharge database found that the prevalence of gastrointestinal anomalies in Thailand was lower than that reported in other countries, except for HSCR and anorectal malformations. Associated Down syndrome and cardiac defects influence the survival outcomes of these anomalies.
RESUMO
BACKGROUND: We evaluated the survival outcomes following hepatic resection as a treatment modality in pediatric patients with hepatoblastoma at a single institution, and to identify radiological parameters associated with poorer survival outcomes. METHODS: This was a retrospective cohort study. Medical records were reviewed, pertaining to pediatric patients diagnosed with hepatoblastoma who underwent surgical resection at a university hospital in Thailand between 2004 and 2021. Radiological parameters, clinical factors, and pathological data were also collected. Survival analysis was performed, and prognostic factors were identified using logistic regression analysis. RESULTS: Forty-two suitable patients were identified. Three cases with incomplete data were excluded, resulting in 39 cases being analyzed. Except for two, all patients received preoperative chemotherapy following the Thai Pediatric Oncology Group regimen. The two- and five-year overall survival rates were 78.0% and 70.9%, respectively. Upon analysis, the radiological parameters associated with poorer survival were poor response to neoadjuvant chemotherapy, presence of metastasis, post-chemotherapy tumor diameter, Post treatment extent of disease (POSTTEXT) Stage IV disease, presence of portal vein involvement, and presence of residual disease; poor neoadjuvant-response, portal vein involvement, and metastasis were independently associated with worse outcomes. In patients with non-metastatic hepatoblastoma who had at least a 25% reduction in size following neoadjuvant chemotherapy, the 5-year survival rate was 90.9% (95% CI 50.8-98.6%). CONCLUSIONS: Although preoperative evaluation of the tumor extent staging did not significantly affect survival, portal vein involvement as per POSTTEXT staging, stable or increasing tumor size, and metastasis following neoadjuvant chemotherapy were associated with poor overall survival. LEVEL OF EVIDENCE: IIB.