Noma A69.00

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

aquatic cancer; cancrum oris; Chancrum oris; Cheek burn; Gangrene infectious of the mouth; infectious gangrene of the mouth; Stomatitis gangraenosa; Water crayfish

History
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Battus, 1595; Boot, 1649; van de Voorde, 1680; Lund, 1765; Coates, 1826; Richter, 1828

Definition
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A foudroyant infectious disease that occurs very rarely in industrialized countries. It often begins as a gingival infection, spreads rapidly and leads to progressive ulceration of the mouth and cheek area. In most cases, the infectious tissue destruction proceeds without a major defensive reaction. This results in large tissue defects of the facial region, which often include the cheek and lip regions, exposing the dental apparatus. Some authors regard the disease as fusospirochetosis.

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Occurrence/Epidemiology
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Worldwide 100.000-300.000 diseases/year. In endemic areas (Sub-Sahara, Sahel) the incidence is 1-12/1,000 inhabitants/year. In the Federal Republic of Germany it occurs practically only sporadically in migrants or as a consequence of other infectious diseases such as typhoid, measles or HIV/AIDS.

Etiopathogenesis
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Oral mixed infection (acute necrotizing gingivitis), especially colonization of the cavum oris with Fusobacterium necrophorum, F. nucleatum, Prevotella intermedia (> 80% of patients), Actinomyces spp. (40-80% of patients), Bacteroides, Streptococci spp. and Staphylococcus spp. Borrelia vincenti is regularly found in the diseased tissue.

Predisposing factors: malnutrition, immunosuppression (e.g. measles, varicella, HIV infection, CMV infection, herpes simplex), poor oral hygiene, living conditions with close contact to pets.

Manifestation
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Especially for undernourished infants and school children (mostly 2nd-8th year of age) in Africa, South East Asia, South America.

Clinical features
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Ulcerative stomatitis or swelling of the affected area of the face, bleeding gums, loss of teeth. Transition of the inflammation to the bone: sequestrating osteitis, ulceration of the cheeks, necrosis of the cheek area.

General therapy
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Treatment of basic diseases, therapy of malnutritional conditions with protein-rich food and food additives, especially high doses of vitamins and trace elements (especially zinc).

External therapy
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Moist compresses with antiseptics such as potassium permanganate (light pink), antibiotic ointments.

Internal therapy
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Penicillins are effective in early stages, better antibiosis after antibiogram (mixed infection). Also effective are tetracyclines, cephalosporins or ciprofloxacin. If necessary, glucocorticoids such as prednisolone (e.g. Decortin H) for glucocorticoid substitution.

Operative therapie
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For limited defects in the lip and cheek area, excision and covering with flap plasty. Cooperation with oral and maxillofacial surgeons and plastic surgeons.

Progression/forecast
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In addition to rapid mortality (70-90% of untreated cases), milder courses are also known (Farley E et al. 2019).

Note(s)
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"Noma" was derived from the Greek term "nomein" (to devour).

Literature
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  1. Baratti-Mayer D et al (2003) Noma: an "infectious" disease of unknown aetiology. Lancet Infect Dis 3: 419-43
  2. Battus C (1595) Handboeck of the Chirurgijen. Abraham Ende, Isaak Canin, Dordrecht, pp. 339-343
  3. Berthold P (2003) Noma: a forgotten disease. Dent Clin North Am 47: 559-574
  4. Blumer G, MacFarlane A (1901) An epidemic of noma: report of sixteen cases. On J Med Sci 122: 527
  5. Boat A (1649) Observationes Medicae de Affectibus Omissis. Thomas Newcomb, London, p.15
  6. Coates bra (1826) Description of the gangrenous ulcer of the mouths of children. North Am Med Surg J 2: 1
  7. Erdmann D et al (1998) Reconstruction of severe facial defects due to noma. Surgeon 69: 1257-1262
  8. Farley E et al (2019) 'I treat it but I don't know what this disease is': a qualitative study on noma (cancrum oris) and traditional healing in northwest Nigeria. Int Health pii: ihz066
  9. Lund G (1765) Om Sjukdomen NOMA, och Chinchina Barkens nytta emot densamma. Kongl Vetenskaps Acad Handl 26: 36
  10. Marck KW (2003) Cancrum oris and noma: some etymological and historical remarks. Br J Plast Surgery 56: 524-527
  11. Naidoo S et al (2004) Oro-facial manifestations in paediatric HIV: a comparative study of institutionalized and hospital outpatients. Oral Dis 10: 13-18
  12. Rotbart HA et al (1986) Noma in children with severe combined immunodeficiency. J Pediatr 109: 596-600
  13. Judge AL (1828) The water crayfish of the children. TCF Enslin, Berlin, S. 1-3
  14. Stingl P (2000) Noma in Africa. The face of poverty. MMW Progress Med 142: 34
  15. of de Voorde C (1680) Nieuw Lichtende Fakkel der Chirurgie or Hedendaagze Heel-Konst: III Van de Noma, or Rotting Ulceratie. Wilhelmus Goeree, Middelburg, S. 538-541

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Last updated on: 29.10.2020