Noma (disease)

Noma (disease)
Classification and external resources
Noma (1836)
ICD-10	A69.0
ICD-9	528.1
DiseasesDB	30727
MedlinePlus	001342
MeSH	D009625

Noma^[1] also known as cancrum oris or gangrenous stomatitis, is a gangrenous disease^[2] leading to tissue destruction of the face, especially the mouth and cheek.^[3][4]

Causes

Noma is a rapidly progressive, polymicrobial, opportunistic infection that occurs during periods of compromised immune function. Fusobacterium necrophorum and Prevotella intermedia are thought to be key players in the process and interact with one or more other bacterial organisms (such as Borrelia vincentii, Porphyromonas gingivalis, Tannerella forsynthesis, Treponema denticola, Staphylococcus aureus, and nonhemolytic Streptococcus spp).^[5]

The reported predisposing factors include:^[5]

• Malnutrition or dehydration
• Poor oral hygiene
• Poor sanitation
• Unsafe drinking water
• Proximity to unkempt livestock
• Recent illness
• Malignancy
• An immunodeficiency disorder, including AIDS

Noma was observed in Nazi concentration camps in World War II, and was studied by Josef Mengele. In many cases a recent debilitating illness (usually measles and sometimes herpes simplex, varicella (chicken pox), scarlet fever, malaria, tuberculosis, gastroenteritis, and bronchopneumonia) precedes the appearance of noma as well as cancers such as leukemia.^[5] In many instances the infection begins as necrotizing ulcerative gingivitis (NUG). Early presentation is unclear as noma is often well progressed at initial presentation.^[5]

Noma, unlike most infections, is able to spread through anatomic barriers such as muscle.^[5]

In the developed world, noma has virtually disappeared except for an occasional case related to immunosuppressive conditions such as HIV infection, severe combined immunodeficiency syndrome, or intense immunosuppressive therapy.^[5] The World Health Organization (WHO) estimates the global yearly incidence to be approximately 140,000, of which 100,000 are between 1 and 7 years old and living in sub-Saharan Africa.^[5]

Presentation and prognosis

A man suffering from noma

The mucous membranes of the mouth develop ulcers, and rapid, painless tissue degeneration ensues, which can degrade tissues of the bones in the face.^[6]

In a condition sometimes called noma pudendi, noma can also cause tissue damage to the genitals.

The disease is associated with high morbidity and mortality^[7] and mainly affects children under the age of twelve in the poorest countries of Africa. Children in Asia and some countries of South America are also affected. Most children who get the disease are between the ages of two and six years old.^[8] The WHO estimates that 500,000 people are affected, and that 140,000 new cases are reported each year.^[9] Estimates claim that 80 - 90% of noma cases die from the disease.

Treatment

Known in antiquity to such physicians as Hippocrates and Galen, noma was once reported around the world, including Europe and the United States. With the improvement in hygiene and nutrition, noma has disappeared from industrialized countries since the 20th Century, except during World War II when it was endemic to Auschwitz and Belsen concentration camps.^[10] The disease and treatments were studied by Berthold Epstein, a Czech inmate physician who had recommended the study to Josef Mengele,^[10] the Nazi doctor.

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 reconstructive plastic surgery to repair. Reconstruction is usually very challenging and it should be delayed until full recovery (usually about one year following initial intervention). ^[11]

Children and other noma survivors in Africa are helped by a few international charitable organizations. There is one dedicated noma hospital in Nigeria, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams. In other countries, like 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.^[12] On 10 June 2010 the work of such volunteer surgeons was featured in a UK BBC2 documentary presented by Ben Fogle titled 'Make me a new face - Hope for Africa's hidden children'.^[13][14]

References

1. ^ From Ancient Greek νομή - nomē "spreading (of sores)" (literally "pasturage"), from νέμειν - nemein "to distribute".
2. ^ "noma" at Dorland's Medical Dictionary
3. ^ Enwonwu CO (2006). "Noma--the ulcer of extreme poverty". N. Engl. J. Med. 354 (3): 221–4. doi:10.1056/NEJMp058193. PMID 16421362. 
4. ^ Enwonwu CO, Falkler WA, Phillips RS (2006). "Noma (cancrum oris)". Lancet 368 (9530): 147–56. doi:10.1016/S0140-6736(06)69004-1. PMID 16829299. 
5. ^ ^{a b c d e f g} Neville, Brad. Oral and Maxillofacial Pathology (3rd ed.). Saunders Book Company. pp. 062008. 5.11. 
6. ^ "AllRefer Health - Noma (Cancrum Oris, Gangrenous Stomatitis)". http://health.allrefer.com/health/noma-info.html. Retrieved 2007-07-12. 
7. ^ Barmes DE, Enwonwu CO, Leclercq MH, Bourgeois D, Falkler WA. The need for action against oro-facial gangrene (noma). Trop Med Int Health 1997; 2: 1111-1114.
8. ^ "The European Noma-Network". http://www.noma-project.de/htmlversion/indexhtml.html. Retrieved 2007-07-12. 
9. ^ Bourgeois DM, Leclercq MH. The World Health Organization initiative on noma. Oral Dis 1999; 5: 172-174.
10. ^ ^{a b} Lifton, Robert Jay (1986). The Nazi Doctors: Medical Killing and Psychological Genocide. Basic Books. p. 361. ISBN 0465049052. 
11. ^ Neville, Brad. Oral and Maxillofacial Pathology, 3rd Edition. Saunders Book Company, 062008. 5.11.2
12. ^ Project Harar - Medical care
13. ^ Make Me a New Face: Hope for Africa's Hidden Children at bbc.co.uk
14. ^ Ben Fogle » Blog Archive » Ben’s Documentary on Noma - BBC2

The Surgical Treatment of noma by Kurt Boss and Klaas Marck. ISBN 978-90-71736-31-5

External links

• The fight against noma
• Fondation Sentinelles
• NOMA-HILFE-SCHWEIZ(German)
• Graphic Noma Images
• All Refer Health article on Noma
• Project Harar
• The European Noma Network
• Les Enfants du Noma(French)
• Facing Africa
• The Geneva Study Group on Noma
• The Dutch Noma Foundation
• Bertrand Piccard Winds of Hope Foundation
• Gegen Noma e.V. - Against Noma organisation (German)
• Noma awareness ad on YouTube "Just €10 can save a life" (German)