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]. / Le Noma.

Noma is a necrotizing ulcerative stomatitis known since Antiquity. It occurs mostly in poor countries, the Sahel countries being the most affected. Each year, several hundred thousand cases are reported. Noma affects especially malnourished children who are less than 6 years old and rarely adults with acquired immunodeficiency (HIV, cancer). Ulcerative lesion is occurring rapidly due to the production of endotoxins by bacteria from oral commensal, telluric and animal origin. Necrotic debridement leads to huge defects: loss of soft tissue (skin, nerves, vessels, eye), bone (maxilla, mandible) and teeth. Death occurs rapidly in a few weeks in 80 % of the cases. In case of survival, the consequences are functional, aesthetic, psychological and social. The goal of the treatment in the acute phase is the patient's survival and the fight against limited mouth opening. The management of the phase of sequela is an anaesthetic, surgical and physiotherapy challenge. Its purpose is the social reintegration of the patient.

Paul Tessier facial reconstruction in 1970 Iran, a series of post-noma defects.

BACKGROUND: Paul Tessier was a pioneering plastic surgeon who founded craniofacial surgery and had an international influence in the field of reconstructive surgery. We reviewed his techniques in the reconstruction of post-noma defects in Iran in the late 1970s. PATIENTS AND METHODS: We studied a series of 23 patients operated on by Tessier from 1974 to 1978 in Iran (property of Association Française des Chirurgiens de la Face). They all suffered from noma in childhood with major facial defects. RESULTS: Ten suffered from simple lip and cheek defects, nine also from nose defects and four from extensive facial defects. Abbe flaps were used in 15 patients to reconstruct the lips completed by commissuroplasty in six patients. Nose defects were reconstructed with nasofrontal flaps (ten cases). The outer cheek was reconstructed with a rotation flap (four cases), or with a frontotemporal flap (six cases). The inner cheek was reconstructed using a Barron-Tessier myocutaneous flap (ten cases). Of the 23 patients, partial flap necrosis occurred in five cases. CONCLUSIONS: Tessier was a pioneering plastic surgeon who used local flaps to reconstruct these important facial defects. He had a high rate of success, although nowadays local flaps are commonly replaced by free flaps.

Mouth ulcers: a deadly disease for children from the sixteenth to eighteenth centuries.

INTRODUCTION: Two processes of vastly different clinical characteristics-what we know today as "Aphthous Stomatitis" and "noma"-occupied the minds of dentists and doctors from the sixteenth to eighteenth centuries. The aim of this article was to look at their knowledge-concerning the differential diagnosis and treatment of these two processes. MATERIALS AND METHODS: Publications of the Spanish physicians and dentists from the sixteenth to eighteenth centuries are reviewed and analysed. RESULTS: The great difference as to how both evolved and about their-consequences led to serious efforts being made to establish a-differential diagnosis and their respective aetiology and treatment. CONCLUSION: While the first goal can be clearly said to have been achieved, practitioners only had the resources offered by authors of antiquity and the Middle Ages to explain the aetiology and apply therapeutic treatment, since microbial theories of disease were not developed until the end of the nineteenth century and antibiotic treatment had to wait until the twentieth century.

[Antonino Longo: the school of Concetti and the birth of paediatrics in Catania]. / Antonino Longo:la scuola del Concetti e la nascita della pediatria a Catania.

The biography of Antonino Longo gives us the opportunity to gain knowledge in, even in everyday life, Luigi Concetti's school of paediatrics in Rome, the state of the art in some infectious diseases,and the reality of paediatrics in Catania where Longo founded University teachings and organised health care for children.

Cancrum oris and noma: some etymological and historical remarks.

Necrotising ulcerative stomatitis is used in the International Statistical Classification of the WHO for orofacial gangrene in children, that is known in medical literature as cancrum oris or noma. The many historical synonyms for this disease together with other historical data indicate that orofacial gangrene in children was a common affection in Europe in previous centuries. The etymological and historical backgrounds of the names "noma" and "cancrum oris" indicate that "cancrum oris" is based on the incorrect use of the Latin term "cancer oris" and maybe on tradition, for which reasons the use of "noma" as medical term for necrotising ulcerative stomatitis should be preferred.

A history of noma, the "Face of Poverty".

Noma (necrotizing ulcerative stomatitis, stomatitis gangrenosa, or cancrum oris) is a devastating orofacial gangrene that occurs mainly among children. The disease has a global yearly incidence of 140,000 cases and a mortality rate of approximately 90 percent. Patients who survive noma generally suffer from its sequelae, including serious facial disfigurement, trismus, oral incontinence, and speech problems. The medical history of noma indicates that the disease was already known in classical and medieval civilizations in Europe. In the sixteenth and seventeenth centuries, Dutch chirurgeons clearly described noma as a clinical entity and realized that the popular name "water canker" was not sufficient, because this quickly spreading ulceration in the faces of children was different from "cancer." In the eighteenth century, awareness that noma is related to poverty, malnutrition, and preceding diseases such as measles increased in northwestern Europe. In the first half of the nineteenth century, extensive surgical procedures were described for the treatment of the sequelae of noma. At the end of that century, noma gradually disappeared in the Western world because of economic progress, which gave the poorest in society the opportunity to feed their children sufficiently. Only in the twentieth century were effective drugs (sulfonamides and penicillin) against noma developed, as well as adequate surgical treatment for the sequelae of noma. These modes of treatment remain inaccessible for the many present-day victims of noma because of their extreme poverty. The only truly effective approach to the problem of noma throughout the world is prevention, namely, combating the extreme poverty with measures that lead to economic progress. In the meantime, medical doctors in the Western world should not forget their own history and ignore this global health problem; rather, they should face "the face of poverty" with the eyes of mercy and concern suited to their profession.

[Noma: Dutch history of a nearly forgotten disease]. / Noma: de Nederlandse geschiedenis van een inmiddels bijna vergeten aandoening.

Noma is an orofacial gangrene that may develop in malnourished and debilitated children. It was once a common disorder in the Netherlands. The medical history of noma has some important Dutch contributions. In 1595, Carel Baten was the first to describe noma as a clinical entity and in 1680, Cornelis van de Voorde coined the name 'noma' for this orofacial gangrene, thereby replacing the term 'water cancer' which was then in common usage. One of the first facial reconstruction operations on a noma patient was performed by Leendert Stelwagen in 1779. Noma gradually disappeared in the Netherlands during the second half of the nineteenth century due to an improved nutritional status amongst the poorest in society. Yet in other parts of the world approximately 110,000 children a year still die from this disorder, which has been largely forgotten in the Western world.