Noma (disease)

Severe facial disfiguration resulting from gangrenous stomatitis (cancrum oris)

The mucous membranes of the mouth develop ulcers, followed by rapid, painful tissue degeneration and necrosis of the tissues of the bones in the face.[3][9]

The World Health Organization divides noma into five stages: acute necrotizing gingivitis, edema, gangrenous, scarring, and sequelae.[10]

The underlying causes for this disease are primarily poor oral hygiene and malnutrition.[10] Although the causative organisms are common in many environments, this disease nearly exclusively affects extremely impoverished and malnourished children in tropical regions.[1][5] Noma is an opportunistic rather than contagious infection.[4]

Noma is often reported as a sequela or progession of necrotizing periodontal diseases. Although many bacteria are suspected in causing noma, the exact causative agents remain unknown. One study of noma patients in Niger found a correlation between noma and a high proportion of Prevotella intermedia bacteria in the mouth; however, more research is needed on the exact cause. Noma is associated with abnormal mouth microbiota.[3]

Fusobacterium necrophorum and Prevotella intermedia are important bacterial pathogens in this disease process, interacting with one or more other bacterial organisms (such as Treponema denticola, Treponema vincentii, Porphyromonas gingivalis, Tannerella forsythia, Staphylococcus aureus, and certain species of nonhemolytic Streptococcus).[11] Treatment of these organisms can help arrest the infection, but does not restore already-missing or disfigured tissue.[12]

Predisposing factors include:[5][13][14]

• malnutrition
• vitamin deficiency (particularly deficiencies of Vitamin A and Vitamin B)
• contaminated drinking water
• immunodeficiency
• poor hygiene, particularly oral
• recent illness (especially acute necrotizing ulcerative gingivitis, measles, malaria, and severe diarrhea)[2][3]
• living in proximity to livestock

The progression of the disease can be halted with the use of antibiotics and improved nutrition; however, its physical effects are permanent and may require oral and maxillofacial surgery or reconstructive plastic surgery to repair. Treatments for noma in the acute stage include penicillin, sulfonamides,[1] and other antibiotics.

A man with scarring and disfigurement resulting from noma

Noma is associated with a very high morbidity,[6] and a mortality rate of approximately 90 percent. The prognosis is much better with treatment; if children have access to medical care, the mortality rate drops to under 10 percent.[4] After gangrene sets in, children generally die within one to two weeks of sepsis.[1] One doctor suspects that the bacteria eating away at the mouth and face get into the bloodstream, causing septicemia and eventual death. Survivors may have difficulty eating and speaking as well as problems with drooling.[16]

The disease affects mainly children in the poorest countries of Africa, Asia and South America. Most people who acquire this disease are between the ages of two and six years old.[7] The World Health Organization estimates that 500,000 people are affected, and that 140,000 new cases are reported each year.[17]

Noma (sketch from 1836)

Known in antiquity to such physicians as Hippocrates and Galen, noma was once reported around the world, including in Europe and the United States. The disease was well-known in the Netherlands in the 1500s and 1600s. The first clinical description of noma was in 1595 by a Dutch man, Carolus Battus. Dutch surgeon Cornelis van de Voorde first used the term "noma" to describe the disease in 1680. A European scientist, Gabriel Lund, attributed noma to poverty, cramped living conditions, and malnutrition in 1765. English physician John Addington Symmonds linked the disease to previous infection with measles. The first surgical treatment for noma sequelae was performed in 1781. Surgical treatments for sequelae developed throughout the 1800s. In the late 1800s, scientists suspected that noma was caused by bacteria.[1]

With improvements in hygiene and nutrition, noma has disappeared from industrialized countries since the 20th century, except during World War II when it was endemic to the Auschwitz and Belsen concentration camps.[18] The disease and treatments were studied by Berthold Epstein, a Czech physician and forced-labor prisoner who had recommended the study under Josef Mengele's direction.[18]

Since 1970, there has been little research done on noma, with few exceptions. One exception is Cyril Enwonwu, a Nigerian scientist focusing on noma.[1] Nigeria is also home to one of the few hospitals in the world that focuses on treating noma patients: Sokoto Noma Hospital, in the city of Sokoto.[16]

People with noma and noma survivors may face stigma. Some think that noma is a contagious disease, so they avoid noma sufferers and survivors to avoid contracting it.[16] Parents may hide afflicted children within the home because of social stigma, which can prevent them from getting treatment. Some also believe noma may be caused by witchcraft or a curse on the child's parents.[2] Based on one 1997 estimate, roughly 770,000 people worldwide live with noma sequelae. However, "noma is a disease of shame," and children are sometimes hidden in isolation rather than being sent to receive treatment.[4]

In Nigeria, sufferers and their families may seek traditional medicine rather than go to a medical center. In a study of 7,185 noma sufferers across Nigeria, only 19% reported going to a hospital or medical center upon discovering a facial lesion. 47.6% took 1-3 weeks to visit a hospital; the rest took longer to visit a hospital.[10]

Children and other noma survivors in Africa are helped by a few international charitable organizations, such as Facing Africa, a UK registered charity that helps affected Ethiopians, and Swiss charity Winds of Hope.[19] There is one dedicated noma hospital in Nigeria, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams supported by Médecins Sans Frontières. Some of the staff are noma survivors.[16][20] In other countries, such as Ethiopia, international charities work in collaboration with the local health care system to provide complex reconstructive surgery which can give back facial functions such as eating, speaking and smiling. Teams of volunteer medics coming from abroad are often needed to support the local capacity to address the most severe cases, which can be extremely challenging even for senior maxillofacial surgeons.[21] On 10 June 2010, the work of such volunteer surgeons was featured in a UK BBC Two documentary presented by Ben Fogle, Make Me a New Face: Hope for Africa's Hidden Children.[22][23]

• Necrotizing fasciitis
• Noma neonatorum

• Short documentary on noma in Nigeria