Noma (cancrum oris): A scoping literature review of a neglected disease (1843 to 2021)

BackgroundNoma (cancrum oris) is an ancient but neglected and poorly understood preventable disease, afflicting the most disenfranchised populations in the world. It is a devastating and often fatal condition that requires urgent and intensive clinical and surgical care, often difficult to access as most cases of noma occur in resource-limited settings. We conducted a scoping review of the literature published on noma to understand the size and scope of available research on the disease and identify research gaps that need to be addressed to evolve our understanding of how to address this disease. MethodsWe searched 11 databases and collected primary peer reviewed articles on noma in all languages, the final search was conducted on 24(th) August 2021. The oldest manuscript identified was from 28(th) March 1843 and the most recently published manuscript was from 3(rd) June 2021. Search terms included cancrum oris and noma. Data was extracted using a standardised data extraction tool and key areas of interest were identified. The Preferred Reporting Items for Systemic review and Meta-Analyses requirements were followed. ResultsThe review included 147 articles, the majority of the studies (n = 94, 64%) were case reports. Most manuscripts (n = 81, 55%) were published in the 2000s, 49 (33%) were from the 1900s and 17 (12%) from the 1800s. The main areas of interest identified were the history and epidemiology of the disease, noma's clinical progression and aetiology, treatment regimens, mortality rates and the risk factors for the development of noma. ConclusionsNoma has been reported in the literature for hundreds of years; however important gaps in our understanding of the disease remain. Future research should focus on determining the burden and distribution of disease; the true mortality rate, pathogenic cause(s) and the factors that influence prognosis and outcomes after treatment. Author summaryNoma is a devastating and often fatal condition that mainly affects children in severely disenfranchised communities. Noma is preventable and requires urgent basic medical care in the early stages of disease. Once the disease reaches the last stage, sequelae, survivors require expert surgical care, usually difficult to access as most cases of noma occur in resource-limited settings. We conducted a scoping review of the literature published on noma to understand the size and scope of available research on the disease and to identify research priorities that will evolve our understanding of how to eradicate this disease. Our review showed that noma has been reported in the literature for hundreds of years; however several major gaps in knowledge still exist. There is appreciation among the small community of clinicians and researchers involved in noma care and research that these gaps in knowledge impact on the ability to develop and implement sound evidence-based policies and activities aimed at eradicating noma from communities that continue to be afflicted by this ancient disease. The main focus of future research should be to study the burden and distribution of disease; the true mortality rate, and the pathogenic cause(s) and the factors that influence prognosis and outcomes after treatment.