Noma (disease)

Not to be confused with Noma neonatorum.

Noma

Other names: Fusospirochetal gangrene, necrotizing ulcerative stomatitis, stomatitis gangrenosa[1]
A man afflicted with noma
Symptoms| Rapidly progressive gangrene of the mouth and face[2]
Usual onset| Age 2 years to 7 years[2]
Causes| Malnutrition[2]
Risk factors| Pre-term births, low birth weight, Measles, malaria, HIV, poor oral hygiene[2]
Prevention| Adequate nutrition, measles vaccination, prevention of malaria and HIV[2]
Treatment| Antibiotics, surgery[2]
Frequency| 30,000–40,000 cases per year (globally)[2]
Deaths| 85%[2]

Noma, also known as cancrum oris, is a rapidly progressive neglected tropical disease that presents with gangrene of the mouth and face resulting in significant facial disfigurement.[2][3]

The cause is opportunistic infection by normal oral flora, on a background of poverty and malnutrition and in association with other diseases including measles, malaria, HIV, and poor oral hygiene.[2] The death rate is around 85%.[2] Prevention requires adequate nutrition, measles vaccination, and prevention of malaria and HIV.[2] Treatment is with antibiotics, and surgery.[2]

Worldwide, the incidence is 30,000–40,000 cases per year.[2] It is generally seen in children age 2-years to 7-years living in sub-Saharan Africa.[2] The condition has been recognised for centuries.[2]

## Contents

* 1 Signs and symptoms
* 2 Causes
* 3 Diagnosis
* 4 Treatment
* 5 Epidemiology
* 6 Society and culture
* 7 References
* 8 External links

## Signs and symptoms[edit | edit source]

The mucous membranes of the mouth develop ulcers, and rapid, painful tissue degeneration ensues, which can degrade tissues of the bones in the face.[4]

* a. Rapidly destructive and fulminatingdisease requiring lengthy antibiotic treatment prior to surgery. b. Arrested advanced disease requiring short course of antibioticsbefore surgical reconstruction (arrow indicates sequestrumformation)

* Noma (sketch from 1836)

## Causes[edit | edit source]

Noma affects extremely impoverished and malnourished children in sub-Saharan countries and other tropical regions; while some bacterial organisms are often found in affected children, these same bacteria are widespread. The underlying causes for this disease are primarily poor sanitation and malnutrition.[2][3]

Fusobacterium necrophorum and Prevotella intermedia are important bacterial pathogens in this disease process, interacting with one or more other bacterial organisms (such as Borrelia vincentii, Porphyromonas gingivalis, Tannerella forsythia, Treponema denticola, Staphylococcus aureus, and certain species of nonhemolytic Streptococcus).[5] Treatment of these organisms can help arrest the infection, but does not restore already-missing or disfigured tissue.

It is often reported as a sequela to acute necrotising ulcerative gingivitis. Predisposing factors include:[3][6][7]

* malnutrition (particularly Vitamin-A and Vitamin-B) or dehydration
* poor hygiene, particularly oral
* unsafe drinking water
* proximity to unkempt livestock
* recent illness
* an immunodeficiency disease, including AIDS
* measles
* smoking

## Diagnosis[edit | edit source]

The diagnosis of this condition in an affected individual is based on physical exam which will show mouth ulcers, and skin ulcers.[8]

## Treatment[edit | edit source]

Known in antiquity to such physicians as Hippocrates and Galen, noma was once reported around the world, including Europe and the United States. With improvements in hygiene and nutrition, noma has disappeared from industrialized countries since the 20th century, except during World War II when it was endemic to Auschwitz and Belsen concentration camps.[9] The disease and treatments were studied by Berthold Epstein, a Czech physician and forced-labor prisoner who had recommended the study under Josef Mengele's direction.[9]

The progression of the disease can be halted with the use of antibiotics and improved nutrition; however, its physical effects are permanent and may require oral and maxillofacial surgery or reconstructive plastic surgery to repair. Reconstruction is usually very challenging and should be delayed until full recovery (usually about one year following initial intervention).[10]

## Epidemiology[edit | edit source]

The disease is associated with high morbidity and mortality.[2] It affects mainly children in the poorest countries of Africa. Children in Asia and some countries of South America are also highly affected. Most children who get the disease are between the ages of two and six years old.[11] The WHO estimates that 500,000 people are affected, and that 140,000 new cases are reported each year.[12] The mortality rate is approximately 90 per cent.[1]

## Society and culture[edit | edit source]

Children and other noma survivors in Africa are helped by a few international charitable organizations, such as Facing Africa, a UK registered charity that helps affected Ethiopian, and Swiss charity Winds of Hope.[13] There is one dedicated noma hospital in Nigeria, the Noma Children Hospital Sokoto, staffed by resident and visiting medical teams supported by Médecins Sans Frontières.[14] In other countries, such as Ethiopia, international charities work in collaboration with the local health care system to provide complex reconstructive surgery which can give back facial functions such as eating, speaking and smiling. Teams of volunteer medics coming from abroad are often needed to support the local capacity to address the most severe cases, which can be extremely challenging even for senior maxillofacial surgeons.[15] On 10 June 2010 the work of such volunteer surgeons was featured in a UK BBC Two documentary presented by Ben Fogle, Make Me a New Face: Hope for Africa's Hidden Children. Recently a case was reported from Nepal where the 19-year-old girl received free surgical treatment in Chitwan Medical College, Bharatpur; the team of surgeons was led by Sushil Subedi, Oral and Maxillofacial surgeon and the head of Department.[16][17]

## References[edit | edit source]

1. ↑ 1.0 1.1 Marck KW (April 2003). "A history of noma, the 'Face of Poverty'". Plastic and Reconstructive Surgery. 111 (5): 1702–7. doi:10.1097/01.PRS.0000055445.84307.3C. PMID 12655218. Archived from the original on 2019-04-04. Retrieved 2021-07-15.
2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 2.17 Srour, ML; Marck, K; Baratti-Mayer, D (8 February 2017). "Noma: Overview of a Neglected Disease and Human Rights Violation". The American journal of tropical medicine and hygiene. 96 (2): 268–274. doi:10.4269/ajtmh.16-0718. PMID 28093536. Archived from the original on 14 August 2022. Retrieved 21 April 2023.
3. ↑ 3.0 3.1 3.2 Enwonwu CO, Falkler WA, Phillips RS (July 8, 2006). "Noma (cancrum oris)". The Lancet. 368 (9530): 147–56. doi:10.1016/S0140-6736(06)69004-1. PMID 16829299. S2CID 10647321.
4. ↑ "AllRefer Health - Noma (Cancrum Oris, Gangrenous Stomatitis)". Archived from the original on 2007-05-28. Retrieved 2007-07-12.
5. ↑ Neville, Brad. Oral and Maxillofacial Pathology (3rd ed.). Saunders Book Company. pp. 062008\. 5.11.
6. ↑ Auluck A, Pai KM (2005). "Noma: Life Cycle of a Devastating Sore - Case Report and Literature Review" (PDF). Journal of the Canadian Dental Association. 71 (10): 757–757c. PMID 16324228. Archived (PDF) from the original on 2021-08-12. Retrieved 2021-07-15.
7. ↑ Enwonwu CO (2006). "Noma--the ulcer of extreme poverty". The New England Journal of Medicine. 354 (3): 221–4. doi:10.1056/NEJMp058193. PMID 16421362.
8. ↑ "Noma: MedlinePlus Medical Encyclopedia". medlineplus.gov. Archived from the original on 31 August 2021. Retrieved 14 September 2021.
9. ↑ 9.0 9.1 Lifton RJ (1986). The Nazi Doctors: Medical Killing and Psychological Genocide. Basic Books. p. 361. ISBN 978-0-465-04905-9.
10. ↑ Neville, Brad. Oral and Maxillofacial Pathology, 3rd Ed. Saunders Book Company, 062008. 5.11.2
11. ↑ "The European Noma-Network". Archived from the original on 2016-03-14. Retrieved 2007-07-12.
12. ↑ Bourgeois DM, Leclercq MH (1999). "The World Health Organization initiative on noma". Oral Dis. 5 (2): 172–174. doi:10.1111/j.1601-0825.1999.tb00085.x. PMID 10522217.
13. ↑ "Winds of Hope". www.windsofhope.org. Archived from the original on 2021-01-26. Retrieved 2021-05-14.
14. ↑ "Noma - a neglected disease". Noma. Archived from the original on 2021-06-12. Retrieved 2021-06-12.
15. ↑ Medical care Archived 2009-04-28 at the Wayback Machine at Project Harar
16. ↑ "Make Me a New Face: Hope for Africa's Hidden Children". BBC. June 2010. Archived from the original on April 8, 2019. Retrieved January 13, 2016.
17. ↑ Fogle, Ben (July 6, 2010). "Ben's Documentary on Noma - BBC2". BenFogle.com. Archived from the original on April 11, 2018. Retrieved January 13, 2010.

## External links[edit | edit source]

Classification|

* ICD-9-CM: 528.1
* MeSH: D009625

|

External resources|

* MedlinePlus: 001342

* v
* t
* e

Bacterial diseases due to gram negative non-proteobacteria (BV4)

Spirochaete|

| Spirochaetaceae|

| Treponema|

* Treponema pallidum
* Syphilis/bejel
* Yaws
* Treponema carateum (Pinta)
* Treponema denticola

|

Borrelia|

* Borrelia burgdorferi/Borrelia afzelii
* Lyme disease
* Erythema migrans
* Neuroborreliosis

* Borrelia recurrentis (Louse borne relapsing fever)
* Borrelia hermsii/Borrelia duttoni/Borrelia parkeri (Tick borne relapsing fever)

Leptospiraceae|

| Leptospira|

* Leptospira interrogans (Leptospirosis)

|

Chlamydiaceae|

| Chlamydia|

* Chlamydia psittaci (Psittacosis)
* Chlamydia pneumoniae

* Chlamydia trachomatis
* Chlamydia
* Lymphogranuloma venereum
* Trachoma

|

Bacteroidetes|

* Bacteroides fragilis
* Tannerella forsythia
* Capnocytophaga canimorsus
* Porphyromonas gingivalis
* Prevotella intermedia

Fusobacteria|

* Fusobacterium necrophorum (Lemierre's syndrome)
* Fusobacterium nucleatum
* Fusobacterium polymorphum

* Streptobacillus moniliformis (Rat-bite fever/Haverhill fever)

* v
* t
* e

Oral and maxillofacial pathology

Lips

* Cheilitis
* Actinic
* Angular
* Plasma cell
* Cleft lip
* Congenital lip pit
* Eclabium
* Herpes labialis
* Macrocheilia
* Microcheilia
* Nasolabial cyst
* Sun poisoning
* Trumpeter's wart

Tongue

* Ankyloglossia
* Black hairy tongue
* Caviar tongue
* Crenated tongue
* Cunnilingus tongue
* Fissured tongue
* Foliate papillitis
* Glossitis
* Geographic tongue
* Median rhomboid glossitis
* Transient lingual papillitis
* Glossoptosis
* Hypoglossia
* Lingual thyroid
* Macroglossia
* Microglossia
* Rhabdomyoma

Palate

* Bednar's aphthae
* Cleft palate
* High-arched palate
* Palatal cysts of the newborn
* Inflammatory papillary hyperplasia
* Stomatitis nicotina
* Torus palatinus

Oral mucosa – Lining of mouth

* Amalgam tattoo
* Angina bullosa haemorrhagica
* Behçet's disease
* Bohn's nodules
* Burning mouth syndrome
* Candidiasis
* Condyloma acuminatum
* Darier's disease
* Epulis fissuratum
* Erythema multiforme
* Erythroplakia
* Fibroma
* Giant-cell
* Focal epithelial hyperplasia
* Fordyce spots
* Hairy leukoplakia
* Hand, foot and mouth disease
* Hereditary benign intraepithelial dyskeratosis
* Herpangina
* Herpes zoster
* Intraoral dental sinus
* Leukoedema
* Leukoplakia
* Lichen planus
* Linea alba
* Lupus erythematosus
* Melanocytic nevus
* Melanocytic oral lesion
* Molluscum contagiosum
* Morsicatio buccarum
* Oral cancer
* Benign: Squamous cell papilloma
* Keratoacanthoma
* Malignant: Adenosquamous carcinoma
* Basaloid squamous carcinoma
* Mucosal melanoma
* Spindle cell carcinoma
* Squamous cell carcinoma
* Verrucous carcinoma
* Oral florid papillomatosis
* Oral melanosis
* Smoker's melanosis
* Pemphigoid
* Benign mucous membrane
* Pemphigus
* Plasmoacanthoma
* Stomatitis
* Aphthous
* Denture-related
* Herpetic
* Smokeless tobacco keratosis
* Submucous fibrosis
* Ulceration
* Riga–Fede disease
* Verruca vulgaris
* Verruciform xanthoma
* White sponge nevus

Teeth (pulp, dentin, enamel)

* Amelogenesis imperfecta
* Ankylosis
* Anodontia
* Caries
* Early childhood caries
* Concrescence
* Failure of eruption of teeth
* Dens evaginatus
* Talon cusp
* Dentin dysplasia
* Dentin hypersensitivity
* Dentinogenesis imperfecta
* Dilaceration
* Discoloration
* Ectopic enamel
* Enamel hypocalcification
* Enamel hypoplasia
* Turner's hypoplasia
* Enamel pearl
* Fluorosis
* Fusion
* Gemination
* Hyperdontia
* Hypodontia
* Maxillary lateral incisor agenesis
* Impaction
* Wisdom tooth impaction
* Macrodontia
* Meth mouth
* Microdontia
* Odontogenic tumors
* Keratocystic odontogenic tumour
* Odontoma
* Dens in dente
* Open contact
* Premature eruption
* Neonatal teeth
* Pulp calcification
* Pulp stone
* Pulp canal obliteration
* Pulp necrosis
* Pulp polyp
* Pulpitis
* Regional odontodysplasia
* Resorption
* Shovel-shaped incisors
* Supernumerary root
* Taurodontism
* Trauma
* Avulsion
* Cracked tooth syndrome
* Vertical root fracture
* Occlusal
* Tooth loss
* Edentulism
* Tooth wear
* Abrasion
* Abfraction
* Acid erosion
* Attrition

Periodontium (gingiva, periodontal ligament, cementum, alveolus) – Gums and tooth-supporting structures

* Cementicle
* Cementoblastoma
* Gigantiform
* Cementoma
* Eruption cyst
* Epulis
* Pyogenic granuloma
* Congenital epulis
* Gingival enlargement
* Gingival cyst of the adult
* Gingival cyst of the newborn
* Gingivitis
* Desquamative
* Granulomatous
* Plasma cell
* Hereditary gingival fibromatosis
* Hypercementosis
* Hypocementosis
* Linear gingival erythema
* Necrotizing periodontal diseases
* Acute necrotizing ulcerative gingivitis
* Pericoronitis
* Peri-implantitis
* Periodontal abscess
* Periodontal trauma
* Periodontitis
* Aggressive
* As a manifestation of systemic disease
* Chronic
* Perio-endo lesion
* Teething

Periapical, mandibular and maxillary hard tissues – Bones of jaws

* Agnathia
* Alveolar osteitis
* Buccal exostosis
* Cherubism
* Idiopathic osteosclerosis
* Mandibular fracture
* Microgenia
* Micrognathia
* Intraosseous cysts
* Odontogenic: periapical
* Dentigerous
* Buccal bifurcation
* Lateral periodontal
* Globulomaxillary
* Calcifying odontogenic
* Glandular odontogenic
* Non-odontogenic: Nasopalatine duct
* Median mandibular
* Median palatal
* Traumatic bone
* Osteoma
* Osteomyelitis
* Osteonecrosis
* Bisphosphonate-associated
* Neuralgia-inducing cavitational osteonecrosis
* Osteoradionecrosis
* Osteoporotic bone marrow defect
* Paget's disease of bone
* Periapical abscess
* Phoenix abscess
* Periapical periodontitis
* Stafne defect
* Torus mandibularis

Temporomandibular joints, muscles of mastication and malocclusions – Jaw joints, chewing muscles and bite abnormalities

* Bruxism
* Condylar resorption
* Mandibular dislocation
* Malocclusion
* Crossbite
* Open bite
* Overbite
* Overeruption
* Overjet
* Prognathia
* Retrognathia
* Scissor bite
* Maxillary hypoplasia
* Temporomandibular joint dysfunction

Salivary glands

* Benign lymphoepithelial lesion
* Ectopic salivary gland tissue
* Frey's syndrome
* HIV salivary gland disease
* Necrotizing sialometaplasia
* Mucocele
* Ranula
* Pneumoparotitis
* Salivary duct stricture
* Salivary gland aplasia
* Salivary gland atresia
* Salivary gland diverticulum
* Salivary gland fistula
* Salivary gland hyperplasia
* Salivary gland hypoplasia
* Salivary gland neoplasms
* Benign: Basal cell adenoma
* Canalicular adenoma
* Ductal papilloma
* Monomorphic adenoma
* Myoepithelioma
* Oncocytoma
* Papillary cystadenoma lymphomatosum
* Pleomorphic adenoma
* Sebaceous adenoma
* Malignant: Acinic cell carcinoma
* Adenocarcinoma
* Adenoid cystic carcinoma
* Carcinoma ex pleomorphic adenoma
* Lymphoma
* Mucoepidermoid carcinoma
* Sclerosing polycystic adenosis
* Sialadenitis
* Parotitis
* Chronic sclerosing sialadenitis
* Sialectasis
* Sialocele
* Sialodochitis
* Sialosis
* Sialolithiasis
* Sjögren's syndrome

Orofacial soft tissues – Soft tissues around the mouth

* Actinomycosis
* Angioedema
* Basal cell carcinoma
* Cutaneous sinus of dental origin
* Cystic hygroma
* Gnathophyma
* Ludwig's angina
* Macrostomia
* Melkersson–Rosenthal syndrome
* Microstomia
* Noma
* Oral Crohn's disease
* Orofacial granulomatosis
* Perioral dermatitis
* Pyostomatitis vegetans

Other

* Eagle syndrome
* Hemifacial hypertrophy
* Facial hemiatrophy
* Oral manifestations of systemic disease

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