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Synonyms: noma (from Greek: to devour); gangrenous stomatitis; face of poverty (flourishes where poverty is rife)
This was described in the mid-eighteenth century by Tourdes. It describes spontaneous necrosis of the soft and hard tissues of the oral cavity. Other oral problems are outlined in the related separate article Problems in the Mouth.
Epidemiology
A disease of children (80% of patients are less than 10 years old), it is seen in developing countries, especially the poorest areas of Africa, some parts of Asia and South America.[1] The World Health Organization (WHO) estimates that 100,000 people are affected per year - of which 80% are children in Africa.
Some cultures do not treat the disease, as it is considered taboo. This results in a barrier to detection of the disorder and to its appropriate management.
Aetiology
The cause is unknown but it may develop secondary to acute necrotising fasciitis.
Risk factors
- Poverty.
- Malnourishment.
- Immunosuppression.
- Poor oral hygiene.
- Poor sanitation.
- Living in close proximity to domestic animals.
- Measles - common in tropical Africa following an ulcerative gingivitis.
- Typhoid.
- Bacillary dysentery.
- Tuberculosis.
- Whooping cough.
- Leukaemia - a terminal manifestation.
Presentation
- Prior to necrosis:
- Poor oral hygiene is nearly always present.
- Excessive salivation.
- Malodour from the mouth.
- Grey discoloration.
- Gingival ulcer formation.
- Followed by rapid, painless and extensive necrosis of the oral cavity, which can involve the cheek, nose, palate and bones.
In 'noma pudendi' there is necrosis of the genitalia and, in 'noma neonatorum', mucocutaneous gangrene occurs during the neonatal period.[2]
Investigations
- Swabs and culture for organisms - Borrelia vincentii and fusiform bacilli are commonly found, as are anaerobes in rapidly progressing cases.[3]
- Facial X-rays and CT scan to determine the extent of involvement.
Management
- Resuscitation - airway protection may be needed; fluid resuscitation to prevent dehydration.
- Antibiotics.
- Enteral feeding.
- Patients usually require wound debridement.
- Later treatment requires plastic surgery with facial reconstruction and possible repair of temporomandibular joint.[4]
Complications
- Dehydration
- Sepsis
- Airway compromise
- Facial disfigurement
- Psychological stress
Prognosis
The clinical course varies with each case and there is a high morbidity and mortality rate. WHO estimates that 70-90% of cases die.[4]
Further reading and references
Peterson PE; Cases of Noma reported in regions of the world, World Health Organization
Parikh TB, Nanavati RN, Udani RH; Noma neonatorum. Indian J Pediatr. 2006 May73(5):439-40.
Paster BJ, Falkler Jr WA Jr, Enwonwu CO, et al; Prevalent bacterial species and novel phylotypes in advanced noma lesions. J Clin Microbiol. 2002 Jun40(6):2187-91.
Enwonwu CO; Noma--the ulcer of extreme poverty. N Engl J Med. 2006 Jan 19354(3):221-4.