Presentation
Before necrosis the following are common presentations [7]:
- Poor oral hygiene
- Excessive salivation
- Malodour from the mouth
- Grey discoloration
- Gingival ulcer formation
These are followed by rapid, painless and extensive necrosis of the oral cavity which should involve the cheek, nose and palate bones. There may be necrosis of the genitalia and mucocutaneous gangrene may be seen during the neonatal period.
Entire Body System
- Gangrene
Noma (cancrum oris, gangrenous stomatitis) is a severe gangrenous disease of orofacial tissues seen in malnourished children. [symptoma.com]
The many historical synonyms for this disease together with other historical data indicate that orofacial gangrene in children was a common affection in Europe in previous centuries. [ncbi.nlm.nih.gov]
- Fishing
The kitchen has glass ceilings so that chefs get to see daylight; the main dining room is a soothing oak cocoon accented with dried fish and seaweed. Eventually, there will be greenhouses, rooftop gardens, a sauna and vegetable plots. [theguardian.com]
The room is kitted out with floor-to-ceilings windows, there's dried seaweed and herbs that hang from wooden beams, dried-out fish carcasses are strung from one wall and servers set tables with handmade ceramic cups. [gq-magazine.co.uk]
Where traditional recipes rely on the naturally occurring enzymes in fish intestines to break down the fish into a sauce, we use koji to recreate a similar process. [noma.dk]
Abstract When assessing the relationship between direction and orientation in drawings by young children and adults, Taguchi and Noma used a fish-drawing task. [ncbi.nlm.nih.gov]
- Fever
The child had experienced occasional cough and fever and also had erupting tooth buds. These tooth buds had been seen as the cause of the cough and fever by the traditional herbalist; hence, they were extracted. [ncbi.nlm.nih.gov]
- Hunting
It’s not that Noma discovered nature – Ferran and Albert [Adrià] had highlighted it at elBulli – but René sort of emerged from the Nordic woods with a clutch of mushrooms and a duck-hunting knife and put Copenhagen and Nordic cooking on the map.” [theguardian.com]
For the past five years, visitors from Copenhagen and Aarhus have been wading into the chilly waters of a sound called Limfjorden to hunt the indigenous and rare European flat-shelled oysters that some consider the best in the world. [newsweek.com]
Buyers eyeing Bieber’s Sydney hideaway Buyers are on the hunt for prestige property on the lower north shore, with the luxury Mosman home pop megastar Justin Bieber rented in 2017 firmly on the radar. [news.com.au]
This went on for a while, until Redzepi, stuck in Greenland after a hunting trip, had a breakthrough: he realized that by relying on wild ingredients foraged from the Nordic countryside he could create something new — cooking that, according to him, speaks [thepointmag.com]
- Swelling
The next stage is extremely painful when the cheeks or lips begin to swell and the victim’s general condition deteriorates. [facingafrica.org]
If the ulcer is left untreated, it progresses rapidly to involve the cheek or lip; swelling is often the earliest externally visible sign of disease. [nejm.org]
It also makes a swell glaze for roasted potatoes or a lovely stand-in for hot chocolate. My partner in crime was Brian "I belong to several fermentation groups on Facebook" Gojdics. [wired.com]
At the same time, there is swelling of the corresponding part of the face. Untreated, the swollen skin will become gangrenous and will perforate, within a week, leaving a hole in the face. Noma is thought to lead to death in 70–90 per cent of cases. [righttofood.org]
Screening for gingivitis: Gums: redness, pain, bleeding Hyper salivation, drooling Bad breath Anorexia Gingival ulceration Facial swelling / oedema. [ennonline.net]
Gastrointestinal
- Vomiting
Health officials said diners at Copenhagen's Noma restaurant fell sick over a five-day period in February, suffering from vomiting and diarrhoea. [bbc.co.uk]
“World’s Best Restaurant Hit by Vomiting Bug,” said Huffington Post. “Restaurant Leaves Bad Taste with Guests,” giggled the Financial Times. [world.time.com]
"It won't hurt you, but the yeast itself might taste like vomit," he said before walking it back a little. "OK, how about 'it can have unpleasurable flavor profiles?'" He skimmed off the offending yeast and it was good as new. [wired.com]
- Diarrhea
It involves the extraction of tooth buds in babies with common childhood illnesses such as fever, cough, and diarrhea. It is thought that the tooth buds are responsible for the ailments seen in these infants. [ncbi.nlm.nih.gov]
Witness Gawker’s headline: “‘Best Restaurant in the World’ Offers Ants, Roe, and Horrible Diarrhea Illness.” Redzepi says he understands the edge he walks. “We’re a restaurant that’s known for perfection, and suddenly there’s a stain on us. [world.time.com]
Noma is usually preceded by a debilitating illness, such as measles, malaria, diarrhea, tuberculosis, or necrotizing ulcerative gingivitis. [nejm.org]
Workup
Diagnosis is reliant on swabs and cultures for organisms [8]. The result often shows Borrielia vincentii and fusiform bacilli. In rapidly progressing cases, anaerobes may be detected.
To determine the extent of the condition, facial x-rays and CT scans can be used.
Treatment
Prognosis
The clinical course for each case varies a great deal and the mortality and morbidity rate is high as 70-90% of cases die [6]. It remains to be seen if this high mortality is as a result of clinical manifestations or as a result of poor or inaccurate diagnosis.
Etiology
Fusobacterium necrophorum and Prevotella intermedia are believed to play a role in the development of this disease [2]. The interactions that bring about the disease remain unclear but it is believed to develop following acute necrotising fasciitis.
The following are risk factors documented for the condition [3]:
- Poverty
- Malnourishment
- Immunosuppression
- Poor oral hygiene
- Poor sanitation
- Living too close to domestic and unkempt animals
- Leukaemia
- Measles
- Typhoid
- Tuberculosis
- Bacillary dysentery
Epidemiology
The condition is a disease of children as 80% of cases are seen in patients less than 10 years old [4]. It is common in developing countries, most especially in the poorest areas of Africa, parts of Asia and parts of South America. A total of 100,000 people are affected per year by this condition.
Pathophysiology
Noma refers to a severe disfiguring gangrene of the face and mouth which first starts as gingival ulcer before it spreads rapidly through the tissues in the mouth and face [5]. Unlike what is obtainable with other facially infectious processes, noma can disrupt anatomic barriers by spreading through the bone and muscle instead of progressing along anatomic spaces of the head and neck. The gangrene that results may involve the maxilla and the mandible and also extend to the infra-orbital margins as well as the nose.
Prevention
The main preventive measures include proper nutrition, cleanliness as well as sanitation [10].
Summary
This condition refers to the spontaneous necrosis of both the soft and hard tissues in the oral cavity [1]. The condition was first described in the mid-eighteenth century by Tourdes. It is a rapidly progressive and polymicrobial infection that is found when the immune system is compromised.
Patient Information
Noma is a type of disease which destroys the mucous membranes of the mouth as well as other tissues. It is seen mostly in malnourished children in parts of the world known for uncleanliness and living closely with animals.
The main cause is unknown however it I believed to be as a result of bacteria known as fusospirochetal organisms. The disorder is most potent when the child has had illnesses such as measles, scarlett fever, tuberculosis, cancer and other forms of immunocompression.
Treatment is often with the aid of antibiotics but the chance of survival is often very poor most of the time.
References
- Enwonwu CO, Falkler WA Jr, Phillips RS. Noma (cancrum oris). Lancet 2006; 368:147.
- Evrard L, Laroque G, Glineur R, Daelemans P. Noma: clinical and evolutive aspect. Acta Stomatol Belg 1996; 93:17.
- Tempest MN. Cancrum oris. Br J Surg 1966; 53:949.
- Marck KW. A history of noma, the "Face of Poverty". Plast Reconstr Surg 2003; 111:1702.
- Boot A. Observationes Medicae de Affectibus Omissis. London: Thomas Newcomb, 1649.
- Tourdes J. Du noma ou du sphacele de la bouche chez les enfants. Thesis. Strasbourg, 1848.
- Baratti-Mayer D, Pittet B, Montandon D, et al. Noma: an "infectious" disease of unknown aetiology. Lancet Infect Dis 2003; 3:419.
- Van Damme, PA. Essay noma. Lancet 2006; 368:561.
- Leclercq MH. Can dentistry tackle inequality? The challenge of noma. Fdi world 1999; 2:9.
- Bourgeois DM, Leclercq MH. The World Health Organization initiative on noma. Oral Dis 1999; 5:172.