Noma (disease) - Noma Disease

Noma (also referred to as cancrum oris, fusospirochetal gangrene, necrotizing ulcerative stomatitis, stomatitis gangrenosa) is a rapidly progressive, polymicrobial, often gangrenous infection of the mouth or genitals.

Signs and symptoms

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

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

Causes

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.

It is often reported as a sequela to acute necrotising ulcerative gingivitis. Predisposing factors include:

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

Prognosis

The disease is associated with high morbidity and mortality 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. The WHO estimates that 500,000 people are affected, and that 140,000 new cases are reported each year. The mortality rate is approximately 90 percent.

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. 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.

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).

International initiatives

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 Ethiopian sufferers, and Swiss charity Winds of Hope. There is one dedicated noma hospital in Nigeria, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams. 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. 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 fro m Nepal where the 19 year old girl received free surgical treatment in Chitwan Medical College, Bharatpur; the team of surgeons was led by Dr. Sushil Subedi, Oral and Maxillofacial surgeon and the head of Department.

References