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

BACKGROUND: Noma (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. METHODS: We searched 11 databases and collected primary peer reviewed articles on noma in all languages, the final search was conducted on 24th August 2021. The oldest manuscript identified was from 28th March 1843 and the most recently published manuscript was from 3rd 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. RESULTS: The 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. CONCLUSIONS: Noma 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.

[Noma/Cancrum oris: a neglected disease]. / La enfermedad de Noma/cancrum oris: una enfermedad olvidada.

Noma is an aggressive orofacial gangrenous pathology that damages hard and soft tissues of the mouth and the face. Throughout the centuries it has been present around the globe, but nowadays it has practically disappeared from developed countries and mainly affects children from the most disadvantaged places, especially in Africa. Noma disease is a multifactorial process; malnutrition, debilitating diseases (bacterial or viral systemic diseases, HIV-associated immunosuppression, etc.) and intraoral infections are some of the factors implied. The characteristic tissue necrosis is produced by a polymicrobial infection. Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, spyrochetes, etc, are some of the species that have been isolated from the affected areas. Without treatment, noma is lethal in a short period of time, and the patients that survive show severe sequelae that hinder their life and interpersonal relationships. The aim of this paper is to unify the existing information and to promote wider knowledge and awareness among the population.

La enfermedad de Noma/cancrum oris: una enfermedad olvidada / Noma/Cancrum oris: a neglected disease

La enfermedad de Noma es una patología gangrenosa agresiva orofacial que daña a tejidos duros y blandos de la boca y de la cara. A lo largo de los siglos ha estado presente en todo el planeta, aunque en la actualidad ha desaparecido prácticamente de los países desarrollados, afectando casi siempre a niños de los lugares más desfavorecidos, especialmente en el continente africano. Es un proceso multifactorial en el que intervienen factores como la malnutrición, las enfermedades debilitantes (infecciones sistémicas bacterianas o víricas, inmunodepresión asociada al VIH, etc.) y las infecciones intraorales. La necrosis tisular característica la origina una infección polimicrobiana. Algunas de las especies que se han aislado de las zonas afectadas son: Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, espiroquetas, etc. Sin tratamiento es letal en poco tiempo, y los pacientes que sobreviven presentan graves secuelas que dificultan su vida y sus relaciones interpersonales. El objetivo de esta revisión es unificar la información existente y promover un mayor conocimiento y concienciación de la población (AU)

Noma is an aggressive orofacial gangrenous pathology that damages hard and soft tissues of the mouth and the face. Throughout the centuries it has been present around the globe, but nowadays it has practically disappeared from developed countries and mainly affects children from the most disadvantaged places, especially in Africa. Noma disease is a multifactorial process; malnutrition, debilitating diseases (bacterial or viral systemic diseases, HIV-associated immunosuppression, etc.) and intraoral infections are some of the factors implied. The characteristic tissue necrosis is produced by a polymicrobial infection. Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, spyrochetes, etc, are some of the species that have been isolated from the affected areas. Without treatment, noma is lethal in a short period of time, and the patients that survive show severe sequelae that hinder their life and interpersonal relationships. The aim of this paper is to unify the existing information and to promote wider knowledge and awareness among the population (AU)

Noma: life cycle of a devastating sore - case report and literature review.

Noma (cancrum oris) is an orofacial gangrene, which during its fulminating course causes progressive and mutilating destruction of the infected tissues. The disease occurs mainly in children with malnutrition, poor oral hygiene and debilitating concurrent illness. Noma is well documented in the literature, but because most patients do not report to a doctor until the disease is at an advanced stage, its onset and progression remain a mystery. This case report, with a survey of recent relevant literature, highlights the different stages in the development of tissue necrosis, including onset and progression, with an emphasis on the need for early diagnosis and prompt treatment.

Noma: an "infectious" disease of unknown aetiology.

Noma (cancrum oris) is a devastating gangrenous disease that leads to severe tissue destruction in the face and is associated with high morbidity and mortality. It is seen almost exclusively in young children living in remote areas of less developed countries, particularly in Africa. The exact prevalence of the disease is unknown, but a conservative estimate is that 770000 people are currently affected by noma sequelae. The cause remains unknown, but a combination of several elements of a plausible aetiology has been identified: malnutrition, a compromised immune system, poor oral hygiene and a lesion of the gingival mucosal barrier, and an unidentified bacterial factor acting as a trigger for the disease. This review discusses the epidemiology, clinical features, current understanding of the pathophysiology, and treatment of the acute phase and sequelae requiring reconstructive surgery. Noma may be preventable if recognised at an early stage. Further research is needed to identify more exactly the causative agents.

[Noma: 'the face of poverty']. / Noma: 'the face of poverty'.

Noma (cancrum oris, water cancer) is an orofacial gangrene that may develop in malnutritioned children debilitated by concomitant diseases as measles. It has been hypothesized that the gangrene originates from acute necrotizing gingivitis, common in malnourished children in developing countries. In previous centuries noma was common in the Western world. The affection disappeared when economical progress permitted the poorest to feed their children sufficiently. The yearly incidence of noma in the world is estimated as 140,000. The mortality is +/- 90%. The facial deformities of those who survive noma are generally serious. Beside facial mutilations, oral functions are hampered (trismus, oral incontinence, impaired speech). Both prevention (improved feeding, measles vaccination) as the treatment of those who contract and survive noma are a challenge for the 'global village' of the modern world.

Cancrum oris: management, incidence, and implications of human immunodeficiency virus in Zambia.

The University Teaching Hospital is situated in the Zambian capital, Lusaka. In a 15-year period (1979 to 1993), 81 child patients with cancrum oris were admitted to the pediatric plastic surgery unit. There were 29 boys and 52 girls, of whom 58 were below 3 years of age. The majority of them were from certain provinces where the population is comparatively much lower than in other provinces of Zambia. The dietary habits in cancrum orisprone provinces are quite different than those of other provinces of Zambia. Of 81 patients, 3 refused surgery, 11 died during early medical treatment, and 12 died following early minor surgery. A total of 55 patients had reconstructive surgery by one of the authors (Nath). Problems encountered during management, such as anesthesia, trismus, and choice of appropriate flaps, are discussed in this paper. The implication of human immunodeficiency virus is also addressed.

Noma: a review.

Cancrum oris or noma is a condition not well known in western Europe and North America. It is, however, a relatively common cause of mortality and disability in children of undeveloped areas of Africa, Asia, and South America. This paper describes the experience at Galmi Hospital, in the sub-Saharan region of South Africa in the Niger Republic, with 50 operative patients out of a group of 300 who were referred. An extensive review of the literature is presented describing the epidemiological impact of the disease, the characteristics of the lesions, the pathogenesis, symptoms, sequelae, differential diagnosis, and preoperative preparation. Additionally, we review approaches to anesthesia, methods of reconstruction, and the most common causes of complications and mortality. Numerous photographs illustrating the devastating consequences of this problem are presented.