Noma: Overview of a Neglected Disease and Human Rights Violation

Abstract

Noma is an orofacial gangrene affecting malnourished children and mainly observed in tropical countries, particularly sub-Saharan Africa. Epidemiological data on noma are scarce, but a current estimate of the global incidence is 30,000-40,000 cases per year, with a mortality rate of approximately 85% and a burden of disease calculated to be a loss of 1-10 million disability-adjusted life years. The etiology of noma is multifactorial with malnutrition as an ever present factor, often in combination with concomitant diseases, such as measles, malaria, and human immunodeficiency virus (HIV), and poor oral hygiene. The pathogenesis is a fast-spreading, noncontagious gangrenous infection occurring in the face, often preceded by acute necrotizing gingivitis, and stomatitis. Rare microbiological studies suggest an opportunistic infection caused by an imbalance in normal intraoral microorganisms. Prevention lies in food security, measles vaccination, prevention of malaria and HIV, including the early detection and treatment of necrotizing gingivitis and stomatitis. Early treatment with antibiotics may prevent gangrene or reduce its extent. Late treatment consists of surgical rehabilitation, which is often complex. However, access to medical care is very limited for noma patients due to the extremely poor conditions in which they live that are frequently located in remote rural areas. The authors support the United Nations Human Rights Council Resolution 19/7 adopted on March 22, 2012 "The right to food," and advocate for the inclusion of noma on the list of neglected tropical diseases to encourage more medical and institutional attention for this often lethal or very mutilating infectious gangrene.

Figure 1.

Acute necrotizing gingivitis (A) and necrotizing stomatitis (B). (A) Acute necrotizing gingivitis (ANG) is considered as an important precursor of noma and is characterized by spontaneous bleeding, ulceration of the papillae, and gingival pain. In this patient, ANG of the upper right dental arch is accompanied by an abundance of calculus and plaque, greyish pseudomembranes and disappearance of gingival papillae. (B) Upper right dental arch: necrotizing stomatitis with destruction of gingival papillae and attached mucosa. Presence of greyish pseudomembranes and exposure of alveolar bone. The lesion shows destruction of gingival mucosa and underlying necrotic bone, a likely precursor of noma.

Figure 2.

Acute noma: edema stage in a child with discolored and brittle hair, a secondary sign of malnutrition.

Figure 3.

Acute noma: necrosis stage. Bluish discoloration of the skin is a sign of underlying necrosis and is visible in this patient through a small hole of already sloughed skin.

Figure 4.

Acute noma: slough and healing. After this slough (A) the first granulation tissue can be observed at the border of the wound as a sign of wound healing (B). Parts of the maxilla and mandible will sequestrate during the following months.

Figure 5.

Noma sequelae. In this patient (same as in Figure 4), the process of healing has resulted in severe wound contracture with ankylosis of the mandible, leading to eating and speech problems, including facial disfigurement.