Noma (ORPHA:2700)
Noma is a gangrenous disease that causes severe destruction of the soft and osseous tissues of the face.
Epidemiology
Its exact prevalence is unknown. The disease was present in the Western world up until the start of the 20th century, but it now mainly affects children between 2 and 6 years of age living in the poorest regions of the world. Rare cases of noma have been described in adults with severe immunodeficiency (individuals with AIDS or myelopathy, or those being treated with immunosuppressants) in Africa and in the Western world.
Clinical description
In addition to the severe facial destruction, children with noma often present with rhinolalia aperta, uncontrollable drooling, and socially handicapping halitosis. The most debilitating sequela is permanent jaw constriction. Spontaneous resolution of the disease is associated with the formation of extremely dense and fibrous scars that may lead to osseous ankylosis between the mandible and maxilla or the mandible and the malar bone. The buccal opening in these children is limited to between 0 and 10 mm making intake of solid foods problematic or impossible. The risk factors associated with noma are malnutrition, intercurrent disease such as rubella or malaria, poor oral hygiene, and the presence of a lesion of the gingival mucosa (an early sign of necrotising gingivitis).
Etiology
The aetiology remains unknown. A bacterial aetiology has long since been suggested but carrying out extensive studies in the regions most affected by the disease is problematic. The few studies reported have indicated that bacteria from the Prevotella and Fusobacteria groups may play a role in the disease.
Management and treatment
The management of acute noma is aimed at improving the health status (introduction of a balanced diet and vitamin supplements, and sufficient hydration) of affected individuals and providing topical care. Antibiotics are recommended to prevent the often lethal complications associated with infection and to help limit extension of the lesion. If the lesion is still intrabuccal and limited, antibiotics may also prevent progression of the disease in some cases. Once the cicatrisation stage is complete, stepwise reconstructive surgery (aimed at relieving the stricture of the mouth and then reconstruction of the mandible and maxilla, cheek, lips and nose) can be considered. In addition to reconstruction of the face of affected children, reconstructive surgery can be used to allow improvement of impaired function (elocution, salivary continence, buccal opening).
Prognosis
Noma is associated with significant morbidity and mortality, often due to complications such as generalised sepsis, intracerebral septic emboli, bronchial aspiration or inanition. The disease develops very rapidly and in isolated regions medical care is often only sought after the lesions are well established. Delayed treatment will not allow a return to status quo ante. The disease leads to social exclusion of affected children: they are rarely admitted into schools for fear of contamination (which in reality does not occur) and are often rejected by their families and village, who continue to view noma as a malediction.

                            Last update: 
                            March 2007


                            - Expert reviewer(s): 
                            Dr Denise BARATTI-MAYER