| Noma | |
|---|---|
| Other names | cancrum oris, fusospirochetal gangrene, necrotizing ulcerative stomatitis, stomatitis gangrenosa, the "face of poverty" |
 | |
| Stage 3 noma (gangrenous stage) in a young girl | |
| Specialty |
Pediatrics, otorhinolaryngology, dentistry 
|
| Symptoms | Facial edema, fever, gangrene of face, necrotizing gingivitis, difficulty eating, drooling, mouth and cheek pain, swollen lymph nodes, loss of appetite, anemia |
| Complications | Lifelong facial disfigurement, difficulty eating/drinking, social stigma, difficulty speaking |
| Usual onset | age 2-6 years |
| Duration | acute phase lasts 2-4 weeks |
| Risk factors | Poverty, protein malnutrition, recent illness, poor oral hygiene, kwashiorkor, HIV infection, "closely spaced pregnancies", maternal malnutrition |
| Differential diagnosis | oral cancer, trench mouth, leishmaniasis post-kala-azar dermal leishmaniasis, leprosy, lethal midline granuloma, herpetic gingivostomatitis, syphilis, mucormycosis, chemical burns, other types of gangrene |
| Prevention | Adequate childhood nutrition |
| Treatment | Antibiotics, vitamin supplements, disinfectant mouthwash |
| Prognosis | 90% fatality rate without treatment |
| Frequency | 140,000 new cases per year (1998 estimate), may be underreported |
Noma (also known as necrotizing ulcerative stomatitis, gangrenous stomatitis, or cancrum oris) is a rapidly progressive and often fatal infection of the mouth and face. This disease predominantly affects children between the ages of two and six years old in the least developed countries around the world, primarily in the "noma belt" of sub-Saharan Africa. Noma has also been seen in severely immunocompromised people in the developed world.
Signs and symptoms
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.
Staging
The World Health Organization divides noma into five stages: acute necrotizing gingivitis, edema, gangrenous, scarring, and sequelae.
Warning signs
Before the development of noma, children may have simple gingivitis: inflammation and reddening of the gums. Gums bleed when touched or during brushing of the teeth. The WHO recommends that parents and health workers check children's teeth for gingivitis. At this stage, noma can still be prevented. The WHO recommends disinfectant mouthwash; if not available, use warm, salted water that has been boiled. A high-protein diet and patient education on oral hygiene are also important. Vitamin A supplements are recommended as well.
Stage I: Acute necrotizing gingivitis
This is the first stage of noma. The gums are red or reddish-purple and bleed spontaneously. The child has fetid breath and may drool. Painful ulcers of the gums develop, causing trouble eating. If the child is malnourished and has recently been sick with an infectious disease, such as measles or chickenpox, they are at more risk for developing noma. Fever may develop at this stage. This stage has an "indefinite duration." Treatments for this stage and all five stages are listed in the "Treatments" section below.
Stage II: Edema
This stage begins the acute phase of noma. The telltale sign is facial edema (swelling) of the lips, cheeks, eyes, etc. Ulceration of the gums worsens during this stage; ulceration may spread to the mucosa (soft, mucus-producing tissue) of the mouth and nose. Fetid breath persists. The child may feel pain or soreness in their mouth and cheeks. High fever is common at this stage. Drooling persists. Lymphadenopathy (swollen lymph nodes) occur at this stage as well. The child may have a decreased appetite and difficulty healing.
Stage III: Gangrene
This is also in the acute phase of noma. At this point, sequelae will inevitably set in. In this stage, the infection eats away at the soft tissue of the child's face. The gangrene may affect the cheeks, lips, nose, mouth, and nasal and oral cavities. The lesions have well-defined borders and a center of black necrotic tissue. Surrounding skin will be discolored. The necrotic tissue sloughs away over time, leaving holes in the face and the soft tissue. Bones and teeth may be exposed; there may be a hole through the cheek. Facial gangrene will dry over time. The child is apathetic, has little appetite, and has great difficulty eating.
Stage IV: Scarring
The acute phase is over by this point, but treatment is still recommended. This stage lasts one to two weeks. The child may experience trismus (difficulty moving/opening the jaw), scars will form, and any exposed teeth will set in place. Although the acute phase is over, the child's life is still at risk.
Stage V: Sequelae
The disease is over by this point, but sequelae from the gangrenous and scarring stages remain. Tissue may be missing, teeth may still be exposed, and the face is disfigured. Scar tissue has grown in. The child may have difficulty eating, drinking, and speaking. The child may lose teeth; teeth may also have become set in the wrong places. There may still be problems with drooling and with opening/closing the jaw. Reconstructive surgery is an option at this phase. Social reintegration is also very important.
Causes and risks
The underlying causes for this disease are primarily poor oral hygiene and malnutrition. Although the causative organisms are common in many environments, this disease nearly exclusively affects extremely impoverished and malnourished children in tropical regions. Noma is an opporunistic rather than contagious infection.
Noma is often reported as a sequela to acute necrotizing ulcerative gingivitis. 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.
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). Treatment of these organisms can help arrest the infection, but does not restore already-missing or disfigured tissue.
Predisposing factors include:
- 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)
- living in proximity to livestock
Treatment
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, and other antibiotics.
By stage
In all stages of noma, the World Health Organization encourages antibiotics, vitamin A supplements or other nutritional supplements, a high-protein diet, and proper hydration.
The World Health Organization recommends using amoxicillin and metronidazole in tandem to treat stage I noma (acute necrotizing gingivitis), along with the use of chlorhexidine and hydrogen peroxide to clean the mouth and gums.
For stage II noma (edema phase), stage III noma (acute/gangrenous stage), and stage IV noma (scarring phase), the WHO recommends either one of two therapies. The first therapy includes the concurrent use of amoxicillin, clavulanic acid, gentamicin, and metronidazole. The second option includes the concurrent use of ampicillin, gentamicin, and metronidazole. For both options, chlorhexidine mouthwash is advised. For stage III and IV noma, the use of ketamine and honey are both given as options for dressing the lesions.
Reconstruction is usually very challenging and should be delayed until full recovery (usually about one year following initial intervention).
Prognosis
Noma is associated with a very high morbidity, 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. After gangrene sets in, children generally die within one to two weeks of sepsis. 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.
Epidemiology
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. The World Health Organization estimates that 500,000 people are affected, and that 140,000 new cases are reported each year.
History
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 hysician 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.
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. 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.
Since 1970, there has been little research done on noma, with few exceptions. One exception is Cyril Enwonwu, a Nigerian scientist focusing on noma. 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.
Society and culture
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. 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. 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.
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.
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. 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. 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.
See also
Further reading
- Boss K, Marck K (2006). The Surgical Treatment of Noma (in Dutch). Alphen aan den Rijn : Belvédère/Mediadact. ISBN 978-90-71736-31-5.