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
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]
Noma (cancrum oris, gangrenous stomatitis) is a severe gangrenous disease of orofacial tissues seen in malnourished children. [symptoma.com]
- 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]
- Intravenous Administration
The signs and symptoms were controlled only after the intravenous administration of 500 mg tid of imipenem/cilastatin sodium and 2 g qd of vancomycin. [ncbi.nlm.nih.gov]
Skin
- Ecthyma
Our case is consistent with previous descriptions of noma neonatorum; however, we question the distinction between noma neonatorum and a neonatal presentation of ecthyma gangrenosum. [ncbi.nlm.nih.gov]
Psychiatrical
- Frigidity
He smokes a pipe and watches American movies from the 80s while trawling the frigid waters of the Limfjord. [world.time.com]
As the sun lowered over Copenhagen’s frigid harbor on February 9, Noma’s senior staff huddled outdoors in one of their still-unheated greenhouses for an emergency meeting. [vanityfair.com]
- Distractibility
The distraction was achieved with a bone distractor, originally prepared for distraction osteoneogenesis of the mandible. [ncbi.nlm.nih.gov]
‘It’s distracting, unserious. But with writing the journal, I tested out these new ways of operating. I’ve put speakers in all our kitchens.’ He also closed the banquet room. ‘Banquets are very lucrative but it’s factory labour,’ says Redzepi. [metro.co.uk]
Just eating and observing the food takes up so much energy that trying to make conservation with strangers distracts you from your main objective, which is enjoying the experience. [scallionpancake.com]
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.
Microbiology
- Pseudomonas
The disease is caused by Pseudomonas aeruginosa and usually affects premature ill babies during the first few weeks of life. A full-term neonate with nasal and scrotal noma is uncommon and is therefore reported. [ncbi.nlm.nih.gov]
- Streptococcus Bovis
Streptococcus bovis type II and E coli were isolated from the noma and Streptococcus bovis, Bacteroides fragilis, and Bacteroides asacharolyticus from the peritoneal fluid. [ncbi.nlm.nih.gov]
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.