The Hidden Burden of Craniosynostosis in Brazil’s Unified Health System: A 10-Year Retrospective Analysis of the Disease’s Diagnoses and Surgical Operations

BACKGROUND: Patients with craniosynostosis, a congenital anomaly involving early cranial suture fusion, face numerous challenges in low-income countries and middle-income countries. This study analyzed craniosynostosis diagnoses and operations in Brazil's Unified Health System (SUS) and identified associated predictors. METHODS: The authors examined craniosynostosis diagnoses and operations from 2012 to 2022 using the DATASUS database. With an estimated incidence of 1 in 2500 live births, the authors forecasted expected diagnosis volumes and utilized a decision-tree model to distinguish between syndromic and nonsyndromic diagnoses. The authors predicted the necessary operations for each group and evaluated workforce capacity trends in neurosurgery and plastic surgery. The authors assessed the implementation impact of the 2015 National Policy for Comprehensive Care for People with Rare Diseases. FINDINGS: From 2012 to 2022, 3337 diagnoses and 2120 operations related to craniosynostosis operations were recorded. However, the authors identified a gap of 9204 diagnoses and 2886 operations not performed in this period. The authors found that, for every 100 additional neurosurgeons, there were 9.2 more diagnoses ( P =0.012) and 5.2 more operations ( P =0.044); for plastic surgeons, each additional 100 corresponded to 5.2 more diagnoses ( P =0.016) and 3.0 more operations ( P =0.037). Diagnosis rates increased significantly postpolicy ( P <0.001), but operative rates did not ( P =0.071). INTERPRETATION: Although diagnoses and operations increased over time, Brazil lags behind forecasted needs. Optimizing integrated policy implementation and proactively incorporating rare diseases in the health care system are essential to improving access to craniosynostosis care.