Noma (also known as 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 opportunistic rather than contagious infection.
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.