Joseline Haizel-Cobbina, Anne Heukwa-Tefoung, Soumya Pahari, Ikeoluwa Lagunju, Lori C Jordan, Philip Aldana, Michael C Dewan
The vast majority of pSCD and pSCD|MMS burden resides in Africa, wherein SCD-related infrastructure is often limited. Sufficient capacity building for diagnostic accuracy and screening coupled with maximal medical therapy for pSCD, and robust neurosurgical infrastructure is required to improve longitudinal SCD care, mitigate neurologic complications and enhance functional outcomes. Derived from a limited body of published literature, these estimates should be considered exploratory.
OBJECTIVE: The aims of the study were twofold: 1) quantify the global burden of pSCD|MMS, and 2) estimate the number of children who warrant specialist evaluation for possible surgical revascularization.
METHODS: A systematic review was conducted following PRISMA guidelines. Clinical data extracted included number of patients with pSCD, pSCD-MMS, and those who underwent surgical revascularization. Using weighted proportions and 2021 Institute for Health Metrics and Evaluation (IHME) global estimates for pSCD, for each WHO region we estimated the burden of pSCD|MMS, and the volume of pSCD|MMS patients who underwent surgical revascularization.
RESULTS: Of the 6,845 studies identified, 14 were included. Nine (64%) studies were from AMR, 2 (14%) studies from EMR, 1 study (7%) from EUR, 1 study (7%) from AFR, and 1 (7%) multicenter study from AMR and EUR. Of the 2045 pSCD patients between the ages of 1-21 years identified, 1.5% were diagnosed with pSCD|MMS. Of 279 pSCD|MMS patients, 49.8% underwent surgical revascularization. The projected number of pSCD|MMS cases worldwide was 36,587, equating to 18,219 potentially benefiting from surgical revascularization. Across WHO regions, the African region harbors the largest number of projected pSCD|MMS cases (32,189) including 16,030 candidates for surgical consideration. Projections should be interpreted cautiously given the limited number and geographic distribution of the available studies.
CONCLUSION: The vast majority of pSCD and pSCD|MMS burden resides in Africa, wherein SCD-related infrastructure is often limited. Sufficient capacity building for diagnostic accuracy and screening coupled with maximal medical therapy for pSCD, and robust neurosurgical infrastructure is required to improve longitudinal SCD care, mitigate neurologic complications and enhance functional outcomes. Derived from a limited body of published literature, these estimates should be considered exploratory.