Cancrum oris

Noma (cancrum oris)

• is a severely debilitating orofacial disease
  • is a gangrenous infection that causes rapid, widespread orofacial destruction
• most frequently affects children between 2 and 5 years of age who live in low-income settings in Africa and Asia (2)
  • the World Health Organization (WHO) estimates that 140,000 new cases of noma occur annually (2)
    • affects malnourished children and mainly observed in tropical countries, particularly sub-Saharan Africa (3)
    • was once frequently reported in Europe and North America, although as living conditions improved in these settings, noma has been eradicated (besides a few sporadic cases in immunocompromised individuals)
      • leukaemic patients:
        • often a terminal complicaton of the disease
        • associated with the use of anti-inflammatory and immunosuppressive drugs
  • a mortality rate of approximately 85% and a burden of disease calculated to be a loss of 1-10 million disability-adjusted life year (3)
• cause of noma is unknown, and noma is not contagious (1)
  • main risk factors for noma include chronic malnutrition, a lack of access to healthcare (specifically immunizations), and comorbidities such as measles and human immunodeficiency virus (HIV)
    • measles:
      • especially in the malnourished, typically in children in tropical Africa
      • cranium oris develops from an acute, ulcerative gingivitis
• pathogenesis is a fast-spreading, noncontagious gangrenous infection occurring in the face, often preceded by acute necrotizing gingivitis, and stomatitis (3)
  • rare microbiological studies suggest an opportunistic infection caused by an imbalance in normal intraoral microorganisms
• WHO classifies noma into stages: Stage 0: simple gingivitis, Stage 1: acute necrotizing gingivitis, Stage 2: oedema, Stage 3: gangrene, Stage 4: scarring, and Stage 5: sequelae
• reported mortality rate for untreated patients is 90% within weeks after the onset of first symptoms (1)
  • treatment in the early acute stages with antibiotics, wound debridement, and nutritional support greatly reduces mortality and morbidity
  • late treatment consists of surgical rehabilitation, which is often complex (3)
• survivors of the acute stages of noma face a lifetime of functional challenges such as difficulty eating and speaking

Reference:

• Farley E, Ariti C, Amirtharajah M, et al. Noma, a neglected disease: A viewpoint article. PLoS Negl Trop Dis. 2021;15(6):e0009437. Published 2021 Jun 17. doi:10.1371/journal.pntd.0009437
• World Health Organization (WHO). Noma is a severe disease It is treatable if detected and managed early. Republic of Congo: Brazzaville; 2016.
• Srour ML, Marck K, Baratti-Mayer D. Noma: Overview of a Neglected Disease and Human Rights Violation. Am J Trop Med Hyg. 2017;96(2):268-274. doi:10.4269/ajtmh.16-0718