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Etiology and Pathology

The exact cause of cancrum oris is unknown but there appear to be three factors instrumental in its development: (1) a predisposing state of debilitation and malnutrition; (2) a specific infection of the mouth usually due to a spirochete of the Treponema (Borrelia) vincentii type; and (3) a precipitating acute systemic febrile illness, such as measles, chickenpox, or malaria.

Noma predominantly afflicts children between 2 and 6 years of age with poor general health and lowered resistance to infection. The child is usually markedly undernourished and anemic with a serious protein and vitamin deficiency (especially vitamin A). Well-nourished, healthy children are not at risk for noma. Other predisposing factors at any age include poor oral hygiene, immunodeficiencies, and trauma to the orofacial soft tissues. Additional risk factors in neonates include intrauterine growth retardation, prematurity, and infants who are small for their gestational age.

Noma develops as a complication of ulcerative gingivitis (Vincent's disease). The organisms usually incriminated in its development in children and adults represent a mixed flora, including spirochetes of the Treponema vincentii type and fusiform bacilli such as Fusobacterium fusiforme. Other organisms that have been identified in the lesions include alpha-hemolytic streptococci, Staphylococcus aureus, Klebsiella pneumoniae, Candida albicans, Bacteroides melaninogenicus and anaerobic Corynebacterium species. There are a variety of other bacterial invaders in the infected slough. In neonates Pseudomonas aeruginosa is usually cultured from the lesion and 86% of neonates will have a positive blood culture for Pseudomonas. This bacterium is also often cultured from the orofacial region of children and adults, but positive blood cultures are much less common.

It is known that poor oral hygiene and acute herpes stomatitis with apthous ulcer predispose to the development of Vincent's disease. Ulcerating gingivitis is rare in North American and European children, although it may be seen in young adults. However, in Africa and other areas of the tropics, it is a common condition in young children, especially those who are undernourished. In 1952, Jelliffe reported 53 cases of cancrum oris seen in Nigeria. He noted that malnutrition played a major role in causing this condition, which he called "infective gangrene of the mouth." He thought gingivitis was the first stage, followed by infective necrosis of the maxilla and/or mandible and then a second stage of gangrene of adjacent or overlying facial soft tissues. Other investigators have since confirmed these findings.

The pathological process of noma is basically that of apthous ulceration of the gingiva, leading to exposure of the underlying bony structures and spread of the lesion through the soft tissues of the cheek and jaw with extensive loss of facial tissue (Figs. 39.1, 39.2 and 39.8). Initially, there is an acute ulcerative gingivitis which begins in an area of periodontal stagnation adjacent to a carious tooth (it does not occur in an edentulous mouth). The gingivitis may be present for several weeks or months as a subacute process and then may disappear. However, at any time, an acute general illness may cause a flare-up of the gingivitis, with extension into the adjacent soft tissues that progresses to gangrene and extensive tissue necrosis. As ulceration of the gum proceeds, the underlying maxilla or mandible is exposed and the teeth become loose. Bony sequestration complicates virtually every case of noma. In some patients the process stops with the development of osteomyelitis of the upper or lower jaw.

Fig.39.1 Noma in a young African child showing clear-cut edges of the sloughed, gangrenous soft tissues of the upper lip and central maxilla. A pot belly and umbilical hernia are also present, as in many African children.

Fig. 39.2 Noma with extensive erosion of the lips and cheek, exposing the underlying mandible. AFIP 74-19325. (From D.H. Connor and F.W. Chandler, eds. Pathology of Infectious Diseases. Appleton & Lange, Stamford, Conn., 1997).

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