Noma (disease)

Noma
Other names: Fusospirochetal gangrene, necrotizing ulcerative stomatitis, stomatitis gangrenosa[1]
A man afflicted with noma
Symptoms	Rapidly progressive gangrene of the mouth and face[2]
Usual onset	Age 2 years to 7 years[2]
Causes	Malnutrition[2]
Risk factors	Pre-term births, low birth weight, Measles, malaria, HIV, poor oral hygiene[2]
Prevention	Adequate nutrition, measles vaccination, prevention of malaria and HIV[2]
Treatment	Antibiotics, surgery[2]
Frequency	30,000–40,000 cases per year (globally)[2]
Deaths	85%[2]

Noma, also known as cancrum oris, is a rapidly progressive neglected tropical disease that presents with gangrene of the mouth and face resulting in significant facial disfigurement.^[2][3]

The cause is opportunistic infection by normal oral flora, on a background of poverty and malnutrition and in association with other diseases including measles, malaria, HIV, and poor oral hygiene.^[2] The death rate is around 85%.^[2] Prevention requires adequate nutrition, measles vaccination, and prevention of malaria and HIV.^[2] Treatment is with antibiotics, and surgery.^[2]

Worldwide, the incidence is 30,000–40,000 cases per year.^[2] It is generally seen in children age 2-years to 7-years living in sub-Saharan Africa.^[2] The condition has been recognised for centuries.^[2]

Signs and symptoms

The mucous membranes of the mouth develop ulcers, and rapid, painful tissue degeneration ensues, which can degrade tissues of the bones in the face.^[4]

• 
  a. Rapidly destructive and fulminatingdisease requiring lengthy antibiotic treatment prior to surgery. b. Arrested advanced disease requiring short course of antibioticsbefore surgical reconstruction (arrow indicates sequestrumformation)
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  Noma (sketch from 1836)

Causes

Noma affects extremely impoverished and malnourished children in sub-Saharan countries and other tropical regions; while some bacterial organisms are often found in affected children, these same bacteria are widespread. The underlying causes for this disease are primarily poor sanitation and malnutrition.^[2][3]

Fusobacterium necrophorum and Prevotella intermedia are important bacterial pathogens in this disease process, interacting with one or more other bacterial organisms (such as Borrelia vincentii, Porphyromonas gingivalis, Tannerella forsythia, Treponema denticola, Staphylococcus aureus, and certain species of nonhemolytic Streptococcus).^[5] Treatment of these organisms can help arrest the infection, but does not restore already-missing or disfigured tissue.

It is often reported as a sequela to acute necrotising ulcerative gingivitis. Predisposing factors include:^[3][6][7]

• malnutrition (particularly Vitamin-A and Vitamin-B) or dehydration
• poor hygiene, particularly oral
• unsafe drinking water
• proximity to unkempt livestock
• recent illness
• an immunodeficiency disease, including AIDS
• measles
• smoking

Diagnosis

The diagnosis of this condition in an affected individual is based on physical exam which will show mouth ulcers, and skin ulcers.^[8]

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 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 Auschwitz and Belsen concentration camps.^[9] 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.^[9]

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. Reconstruction is usually very challenging and should be delayed until full recovery (usually about one year following initial intervention).^[10]

Epidemiology

The disease is associated with high morbidity and mortality.^[2] It affects mainly children in the poorest countries of Africa. Children in Asia and some countries of South America are also highly affected. Most children who get the disease are between the ages of two and six years old.^[11] The WHO estimates that 500,000 people are affected, and that 140,000 new cases are reported each year.^[12] The mortality rate is approximately 90 per cent.^[1]

Society and culture

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 Ethiopian, and Swiss charity Winds of Hope.^[13] 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.^[14] 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.^[15] 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. Recently a case was reported from Nepal where the 19-year-old girl received free surgical treatment in Chitwan Medical College, Bharatpur; the team of surgeons was led by Sushil Subedi, Oral and Maxillofacial surgeon and the head of Department.^[16][17]

References

External links