Noma (cancrum oris): An unresolved global challenge

Abstract

Noma (canrum oris) is a mutilating necrotizing disease of uncertain etiology, but it is accepted that it is caused primarily by a polybacterial infection with secondary ischemia. The consequent necrotizing fasciitis, myonecrosis, and osteonecrosis results in destruction of facial structures with severe functional impairment and disfigurement. It most frequently affects children, particularly in sub-Saharan Africa, who are malnourished or debilitated by systemic conditions including but not limited to malaria, measles, and tuberculosis; and less frequently debilitated HIV-seropositive subjects. In the vast majority of cases, in susceptible subjects, noma is preceded by necrotizing stomatitis. However, it has been reported, albeit rarely, that noma can arise without any preceding oral lesions being observed. Noma is not recurrent and is not transmissible.

Figure 1

Pathogenic events in noma, and its clinical course

Figure 2

Necrotizing oral diseases in HIV‐seropositive patients. In these cases the lesions did not progress to noma. (A) Necrotizing gingivitis of the interdental papillae and the marginal gingiva (J Oral Pathol Med 2014; 43:1‐6, reproduced with permission24). (B) Necrotizing periodontitis of the marginal periodontal attachment apparatus (J Oral Pathol Med 2014; 43:1‐6, reproduced with permission24). (C‐E) Necrotizing stomatitis of the alveolar mucosa (J Oral Pathol Med 2014; 43:1‐6, reproduced with permission24). (F) Necrotizing stomatitis of the left buccal mucosa (SADJ 2014; 69:468‐470, reproduced with permission35)

Figure 3

Necrosis of the right‐side of the lower lip in an HIV‐seropositive highly active antiretroviral treatment‐naïve child with a CD4 + T cell count of 13 cells/mm³. The parents reported that this lesion had developed quite rapidly (SADJ 2014; 69:468‐470, reproduced with permission35)

Figure 4

(A) Necrosis of the left buccal mucosa in a 43‐y‐old HIV‐seropositive female with a CD4 + T cell count of 4 cells/mm³, already involving the skin. According to the patient the lesion seen in (A) involving the skin of the cheek (B) developed within 3 d of the buccal mucosal lesion being noticed

Figure 5

Noma of the right cheek of uncertain duration. (A) Maxillary molars; (B) mandibular molars; (C, D) necrotizing periodontitis/stomatitis; (E) mandibular bone exposed by necrotizing stomatitis. Note the remarkable, characteristic circular shape of the full‐thickness defect (J Oral Pathol Med 2014; 43:1‐6, reproduced with permission24)

Figure 6

(A) Mandibular and maxillary necrotizing gingivitis, with early necrotizing stomatitis of the labial mucosa (arrow) apparently from contact with the gingival lesions, in a 16‐y‐old HIV‐seropositive adolescent with a CD4 + T cell count of 17 cells/mm³, who had been on highly active antiretroviral treatment for the previous 4 y. (B) Severe necrotizing stomatitis of the left buccal mucosa in the same patient. The gingival, labial, and buccal lesions developed rapidly. The patient was admitted to hospital and penicillin, metronidazole, and gentamycin were administered with fluid replacement and daily irrigation of the intra‐oral lesions with saline. A semi‐fluid high‐protein diet was given. The lesions were debrided, and dressed daily with bismuth‐iodoform‐paraffin paste. The patient was referred to the local HIV clinic to optimize the highly active antiretroviral treatment regimen. (C) 5 d after admission to hospital, the left buccal mucosa was healing satisfactorily and the necrotizing gingivitis had resolved. The patient was discharged from hospital, but was lost to follow‐up (SADJ 2014; 69:468‐470, reproduced with permission35)

Figure 7

(A) Severe necrotic destruction of the nose, lips, and cheeks extending to the infra‐orbital margin, and denudation and necrosis of the anterior mandible and anterior maxilla of a 6‐y‐old HIV‐seropositive highly active antiretroviral treatment‐naïve child with a CD4 + T cell count of 6 cells/mm³. (B) The appearance 2 wk after hospital admission: much of the necrotic soft tissue has been removed. (C) All the necrotic soft tissue removed. (D) The appearance 3 mo after resection of all the necrotic soft tissue and bone with extensive scarring around the defect. (E) Computed tomography image showing the extent of the damage to the mandible and maxilla (Head Neck Pathol 2013; 7:188‐192, reproduced with permission8)

Figure 8

A 32‐y‐old malnourished HIV‐seropositive female with noma and a CD4 + T cell count of 118 cells/mm³, who had been on highly active antiretroviral treatment for 6 y. (A) A well‐defined, oval, full thickness defect of the lip below the vermilion border. (B) Necrotizing periodontitis affecting the anterior mandibular teeth with necrotizing stomatitis of the labial mucosa. (C) Posteriorly extending necrotizing stomatitis of the left buccal mucosa. (D) The sublabial scar 8 wk after admission to hospital and 6 wk after suturing. The patient was satisfied despite the aesthetically imperfect result. (E) Healed left posterior buccal mucosa 8 wk after treatment (AIDS Res Hum Retroviruses 2014; 30: 213‐216, reproduced with permission34)