Noma Disease

Noma is a devastating oral gangrene that rapidly destroys the soft and hard facial tissues, occurring mainly in children aged 2 to 6.

If detected on time, it is treatable with antibiotics. However, because it is not known by healthcare professionals, most cases go untreated, rapidly evolving and leading, in 90% of cases, with the death of the child within just two weeks.

For those few who survive, the malformations will very likely remain for life, bringing stigma and isolation.

The disease is preceded as a simple gengivitis. The first stage is characterized by a necrotizing gengivitis with fetid odor, pain and possible bleeding, that is followed by edema in the cheeks. 

Up to stage 2, the disease is treatable with antibiotics. However, when these are not provided, it can evolve within a week to Stage 3. From this stage, the life of the child is at risk. Debridement and wound dressing is needed along with nutrition and hydratation. 

In 90% of cases, after stage 3 the child will die. If he survives, reconstructive surgery will be needed to repare the scars, which sometimes impede talking or eating, but which in most cases lead to stigmatization and poor social conditions.

In recent years, noma has been reported in many countries around the world, but primarily in low and middle income countries in Africa and Asia. In Western countries, esporadic cases occur in immunocompromised adults.

Incidence, as estimated by WHO in 1998, is of 140.000 new cases / year. However, this is very likely an under estimation, since a cluster randomized prevalence study in North West Nigeria estimated a 0,18% of children aged 0-15 . 

Mortality estimates are subjected to the same scarcity of data, but it its undoubtedly very high. According to recent reports it reaches 90% after the edema stage if not treated.

In Mozambique, the last case reported dates from 2009. However, this does not mean that there are no cases, but rather that they go underported, silenced by poverty. The children  are usually seen by traditional healers first, and by the time the remedies proof not effective, the disease is too advanced and the child dies before reaching the hospital, were it could have been reported. Thus, the neglect of the disease continues in a vicious cycle of poverty and ignorance.

The phatogenesis of the disease is still unkown, but there is a consensus on its multifactorial nature.

There has not been a specific pathogen consistently isolated across noma patients, but rather an imbalance of the oral microbiome. 

It is not contagious, nor recurrent, and the risk factors found are: being less than 6 years of age, being malnourished, not having the full vaccination and having recently had another infection.

1595: First case of noma is described by a dutch surgeon and named "water canker"

17th Century

The disease is common, English surgeons name it cancrum oris (oral cancer). Dutch surgeons belief it is different from cancer and name it noma (from the greek "pasture land" something that spreads quickly").

18th Century

The disease is related to malnourishment, poverty and preceding diseases, predominantly measles

19th Century

Surgery procedures are developed. In 1828, the book on noma “Der Waserkrebs der Kinder” is published, and would be the only one for the next 173 years.

In the second half of the century noma disappears in Europe and North America thanks to economic progress and thus no famine, evidencing that poverty is an imperative.

20th Century

World War I

Noma having disappeared for years, oldiers living in trenches suffered from its precurso: Acute Necrotizing Ulcerative Gengivitis, then called Vincent stomatitis and thereafter Trench mouth. It was attributed to poor oral hygene, vitamin deficiency, somoking, psychic stress due to war.

Wolrd War II

Cases were seen again in Nazi exterminations camps linked to the atrocious living conditions. The archives being untreaceable, survivor's memoires indicate that there was a Noma Department within the hospital at the Gypsy Camp in Birkenau, where large numbers of patients with noma were treated by Berthold Epstein, a prisoner, under the supervision of SS physician Josef Mengele. Intriguingly, cases were only reported at the Gypsy camp, although conditions were not worse than for other ethnicities.