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Noma

Cancrum Oris

Noma (cancrum oris, gangrenous stomatitis) is a severe gangrenous disease of orofacial tissues seen in malnourished children.


Presentation

Before necrosis the following are common presentations [7]:

These are followed by rapid, painless and extensive necrosis of the oral cavity which should involve the cheek, nose and palate bones. There may be necrosis of the genitalia and mucocutaneous gangrene may be seen during the neonatal period.

Gangrene
  • Noma (cancrum oris, gangrenous stomatitis) is a severe gangrenous disease of orofacial tissues seen in malnourished children.[symptoma.com]
  • The many historical synonyms for this disease together with other historical data indicate that orofacial gangrene in children was a common affection in Europe in previous centuries.[ncbi.nlm.nih.gov]
  • Old antibiotics may help.Noma (cancrum oris) is a devastating gangrenous disease that leads to destruction of facial tissue with significant morbidity and mortality in children and young adults.[ncbi.nlm.nih.gov]
  • This case report describes a baby with noma, an orofacial gangrenous infection.[ncbi.nlm.nih.gov]
  • Noma neonatorum is a gangrenous process that occurs in the oral, nasal or anal area and occasionally the eyelids and scrotum of the newborn.[ncbi.nlm.nih.gov]
Dutch
  • In the sixteenth and seventeenth centuries, Dutch chirurgeons clearly described noma as a clinical entity and realized that the popular name "water canker" was not sufficient, because this quickly spreading ulceration in the faces of children was different[ncbi.nlm.nih.gov]
  • Jean Ziegler and his collaborators embarked on a thorough process of research and consultations with NGOs working in the field of Noma, human rights and humanitarian organizations – among which CARE International, Dutch Noma Foundation, Ecumenical Advocacy[righttofood.org]
Lymphadenopathy
  • A 2-year-6-month old, appropriately immunised, well-thriving boy, symptomatic for the past 6 months, presented with recurrent fever, progressive pallor, lymphadenopathy and a raw area on the right cheek, with discharging sinus.[ncbi.nlm.nih.gov]
Intravenous Administration
  • The signs and symptoms were controlled only after the intravenous administration of 500 mg tid of imipenem/cilastatin sodium and 2 g qd of vancomycin.[ncbi.nlm.nih.gov]
Down Syndrome
  • Apart from these, noma has also been reported in association with cyclic neutropenia, herpetic stomatitis, leukemia, Down's syndrome and Burkett's disease.[ncbi.nlm.nih.gov]
Ecthyma
  • We report here a 2.5-year-old male child with community-acquired Pseudomonal sepsis showing the characteristic lesions of ecthyma gangrenosum. The child had development of gangrenous changes of the nose and face - the 'cancrum oris' or 'Noma'.[ncbi.nlm.nih.gov]
  • Our case is consistent with previous descriptions of noma neonatorum; however, we question the distinction between noma neonatorum and a neonatal presentation of ecthyma gangrenosum.[ncbi.nlm.nih.gov]
Ankylosis
  • Noma defects of the anterolateral face are often associated with fibrous or bony ankylosis fusing the mandibula to the skull base.[ncbi.nlm.nih.gov]
  • A rare case of a male patient who suffered from noma with sequelae of ankylosis in bilateral temporomandibular joints for 52 years and a neoplasm in the right commissure of the lips for 1 year, was operated on to resolve the ankylosis, remove the neoplasm[ncbi.nlm.nih.gov]
  • After successful microvascular soft tissue free flap reconstruction, we now included skeletal restoration and mandibular ankylosis release into the initial step of complex noma surgery.[ncbi.nlm.nih.gov]
  • This is a report of a presumed case of noma that resulted in bony ankylosis of the maxilla and mandible. Three-dimensional shaded surface CT reconstruction images were especially useful in demonstrating the architecture of the abnormal bone.[ncbi.nlm.nih.gov]
  • Underlined is the functional and organic aspect of this disease, in particular the distortion of the face, which commonly involves the full thickness of the cheek skin and bone, mandibular ankylosis and craniofacial dismorphisms, and the modern approach[ncbi.nlm.nih.gov]
Distractibility
  • The distraction was achieved with a bone distractor, originally prepared for distraction osteoneogenesis of the mandible.[ncbi.nlm.nih.gov]
Incontinence
  • Patients who survive noma generally suffer from its sequelae, including serious facial disfigurement, trismus, oral incontinence, and speech problems.[ncbi.nlm.nih.gov]

Workup

Diagnosis is reliant on swabs and cultures for organisms [8]. The result often shows Borrielia vincentii and fusiform bacilli. In rapidly progressing cases, anaerobes may be detected.
To determine the extent of the condition, facial x-rays and CT scans can be used.

Streptococcus Bovis
  • Streptococcus bovis type II and E coli were isolated from the noma and Streptococcus bovis, Bacteroides fragilis, and Bacteroides asacharolyticus from the peritoneal fluid.[ncbi.nlm.nih.gov]
Eikenella
  • ., Eikenella corrodens, Fusobacterium spp., Gemella haemoylsans, and Neisseria spp.[ncbi.nlm.nih.gov]

Treatment

Treatment involves the following [9]:

  • Fluid resuscitation
  • Antibiotics
  • Enteral feeding
  • Wound debridement

The last stage of treatment in some cases is plastic surgery which involves facial reconstruction and the possible repair of temporomandibular joint.

Prognosis

The clinical course for each case varies a great deal and the mortality and morbidity rate is high as 70-90% of cases die [6]. It remains to be seen if this high mortality is as a result of clinical manifestations or as a result of poor or inaccurate diagnosis.

Etiology

Fusobacterium necrophorum and Prevotella intermedia are believed to play a role in the development of this disease [2]. The interactions that bring about the disease remain unclear but it is believed to develop following acute necrotising fasciitis.

The following are risk factors documented for the condition [3]:

Epidemiology

The condition is a disease of children as 80% of cases are seen in patients less than 10 years old [4]. It is common in developing countries, most especially in the poorest areas of Africa, parts of Asia and parts of South America. A total of 100,000 people are affected per year by this condition.

Sex distribution
Age distribution

Pathophysiology

Noma refers to a severe disfiguring gangrene of the face and mouth which first starts as gingival ulcer before it spreads rapidly through the tissues in the mouth and face [5]. Unlike what is obtainable with other facially infectious processes, noma can disrupt anatomic barriers by spreading through the bone and muscle instead of progressing along anatomic spaces of the head and neck. The gangrene that results may involve the maxilla and the mandible and also extend to the infra-orbital margins as well as the nose.

Prevention

The main preventive measures include proper nutrition, cleanliness as well as sanitation [10].

Summary

This condition refers to the spontaneous necrosis of both the soft and hard tissues in the oral cavity [1]. The condition was first described in the mid-eighteenth century by Tourdes. It is a rapidly progressive and polymicrobial infection that is found when the immune system is compromised.

Patient Information

Noma is a type of disease which destroys the mucous membranes of the mouth as well as other tissues. It is seen mostly in malnourished children in parts of the world known for uncleanliness and living closely with animals.

The main cause is unknown however it I believed to be as a result of bacteria known as fusospirochetal organisms. The disorder is most potent when the child has had illnesses such as measles, scarlett fever, tuberculosis, cancer and other forms of immunocompression.

Treatment is often with the aid of antibiotics but the chance of survival is often very poor most of the time.

References

Article

  1. Enwonwu CO, Falkler WA Jr, Phillips RS. Noma (cancrum oris). Lancet 2006; 368:147.
  2. Evrard L, Laroque G, Glineur R, Daelemans P. Noma: clinical and evolutive aspect. Acta Stomatol Belg 1996; 93:17.
  3. Tempest MN. Cancrum oris. Br J Surg 1966; 53:949.
  4. Marck KW. A history of noma, the "Face of Poverty". Plast Reconstr Surg 2003; 111:1702.
  5. Boot A. Observationes Medicae de Affectibus Omissis. London: Thomas Newcomb, 1649.
  6. Tourdes J. Du noma ou du sphacele de la bouche chez les enfants. Thesis. Strasbourg, 1848.
  7. Baratti-Mayer D, Pittet B, Montandon D, et al. Noma: an "infectious" disease of unknown aetiology. Lancet Infect Dis 2003; 3:419.
  8. Van Damme, PA. Essay noma. Lancet 2006; 368:561.
  9. Leclercq MH. Can dentistry tackle inequality? The challenge of noma. Fdi world 1999; 2:9.
  10. Bourgeois DM, Leclercq MH. The World Health Organization initiative on noma. Oral Dis 1999; 5:172.

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Last updated: 2017-08-09 18:25