An unusual case of noma caused by Klebsiella pnuemoniae and its management.

Noma or cancrum oris is an orofacial gangrene causing progressive mutilating destruction of the infected tissues. It mainly affects malnourished children with poor oral hygiene and concurrent debilitating systemic illnesses. It is a polymicrobial infection and borrelia vincentii and fusobacterium are the most important pathogens known. We present a case of a boy aged 2.5 years with noma where klebsiella was grown and was the initial cause of failure of empiric therapy.

Noma in an HIV infected patient in Guinea-Bissau: a case report.

BACKGROUND: Noma is a multifactorial and multibacterial opportunistic infection that initially causes necrotic gingivitis but rapidly spreads to the nearby orofacial tissue resulting in sloughing and severe deformation of the facial structures. The majority of cases are seen in young children under the age of 6 years. Noma is strongly associated with poverty, malnutrition and immunosuppression, and is often preceded by severe systemic infections such as measles and malaria. Only few cases of noma infection in adults have been described. CASE REPORT: We present here a case report with a 32-year-old Guinean woman who was diagnosed with noma infection and on that occasion discovered that she was HIV-1 seropositive. After treatment with amoxicillin/clavulanic acid and metronidazole for her noma infection the woman was transferred to the national hospital where antiretroviral treatment was initiated. CONCLUSION: Noma is an opportunistic infection and immunodeficiencies such as HIV should always be suspected when presenting in an adult patient.

[Noma/Cancrum oris: a neglected disease]. / La enfermedad de Noma/cancrum oris: una enfermedad olvidada.

Noma is an aggressive orofacial gangrenous pathology that damages hard and soft tissues of the mouth and the face. Throughout the centuries it has been present around the globe, but nowadays it has practically disappeared from developed countries and mainly affects children from the most disadvantaged places, especially in Africa. Noma disease is a multifactorial process; malnutrition, debilitating diseases (bacterial or viral systemic diseases, HIV-associated immunosuppression, etc.) and intraoral infections are some of the factors implied. The characteristic tissue necrosis is produced by a polymicrobial infection. Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, spyrochetes, etc, are some of the species that have been isolated from the affected areas. Without treatment, noma is lethal in a short period of time, and the patients that survive show severe sequelae that hinder their life and interpersonal relationships. The aim of this paper is to unify the existing information and to promote wider knowledge and awareness among the population.

La enfermedad de Noma/cancrum oris: una enfermedad olvidada / Noma/Cancrum oris: a neglected disease

La enfermedad de Noma es una patología gangrenosa agresiva orofacial que daña a tejidos duros y blandos de la boca y de la cara. A lo largo de los siglos ha estado presente en todo el planeta, aunque en la actualidad ha desaparecido prácticamente de los países desarrollados, afectando casi siempre a niños de los lugares más desfavorecidos, especialmente en el continente africano. Es un proceso multifactorial en el que intervienen factores como la malnutrición, las enfermedades debilitantes (infecciones sistémicas bacterianas o víricas, inmunodepresión asociada al VIH, etc.) y las infecciones intraorales. La necrosis tisular característica la origina una infección polimicrobiana. Algunas de las especies que se han aislado de las zonas afectadas son: Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, espiroquetas, etc. Sin tratamiento es letal en poco tiempo, y los pacientes que sobreviven presentan graves secuelas que dificultan su vida y sus relaciones interpersonales. El objetivo de esta revisión es unificar la información existente y promover un mayor conocimiento y concienciación de la población (AU)

Noma is an aggressive orofacial gangrenous pathology that damages hard and soft tissues of the mouth and the face. Throughout the centuries it has been present around the globe, but nowadays it has practically disappeared from developed countries and mainly affects children from the most disadvantaged places, especially in Africa. Noma disease is a multifactorial process; malnutrition, debilitating diseases (bacterial or viral systemic diseases, HIV-associated immunosuppression, etc.) and intraoral infections are some of the factors implied. The characteristic tissue necrosis is produced by a polymicrobial infection. Fusobacterium necrophorum, Prevotella intermedia, Prevotella melaninogenica, Fusobacterium nucleatum, Bacteroides fragilis, Bacillus cereus, Trueperella pyogenes, spyrochetes, etc, are some of the species that have been isolated from the affected areas. Without treatment, noma is lethal in a short period of time, and the patients that survive show severe sequelae that hinder their life and interpersonal relationships. The aim of this paper is to unify the existing information and to promote wider knowledge and awareness among the population (AU)

Noma Neonatorum From Multidrug-Resistant Pseudomonas aeruginosa: An Underestimated Threat?

We present the case of an extremely low birth weight infant with diffuse gingival noma, initially misdiagnosed as thrush. Multidrug-resistant Pseudomonas aeruginosa strain was cultured and treated with systemic and local colistin with complete healing. Noma neonatorum from multidrug-resistant pathogens may appear in neonatal intensive care units. 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. Noma has virtually disappeared from Europe and North America, but it is still common among children and young adults in India, Africa, and South America. Noma is a polymicrobial opportunistic infection related to malnutrition and immune dysfunction. In the neonate, a similar but distinct condition, known as "noma neonatorum" was described in 1977, in which gangrenous lesions involve the mucocutaneous junctions of oral, nasal, and anal area, and, occasionally, the eyelids and the scrotum. The neonatal disease has been linked to Pseudomonas aeruginosa, prematurity, and low birth weight. There is no established treatment, and mortality is almost inevitable in the few reported cases. In this study, we present the first European case of noma neonatorum from a multidrug-resistant strain of P aeruginosa.

Noma affected children from Niger have distinct oral microbial communities based on high-throughput sequencing of 16S rRNA gene fragments.

We aim to understand the microbial ecology of noma (cancrum oris), a devastating ancient illness which causes severe facial disfigurement in>140,000 malnourished children every year. The cause of noma is still elusive. A chaotic mix of microbial infection, oral hygiene and weakened immune system likely contribute to the development of oral lesions. These lesions are a plausible entry point for unidentified microorganisms that trigger gangrenous facial infections. To catalog bacteria present in noma lesions and identify candidate noma-triggering organisms, we performed a cross-sectional sequencing study of 16S rRNA gene amplicons from sixty samples of gingival fluid from twelve healthy children, twelve children suffering from noma (lesion and healthy sites), and twelve children suffering from Acute Necrotizing Gingivitis (ANG) (lesion and healthy sites). Relative to healthy individuals, samples taken from lesions in diseased mouths were enriched with Spirochaetes and depleted for Proteobacteria. Samples taken from healthy sites of diseased mouths had proportions of Spirochaetes and Proteobacteria that were similar to healthy control individuals. Samples from noma mouths did not have a higher abundance of Fusobacterium, casting doubt on its role as a causative agent of noma. Microbial communities sampled from noma and ANG lesions were dominated by the same Prevotella intermedia OTU, which was much less abundant in healthy sites sampled from the same mouths. Multivariate analysis confirmed that bacterial communities in healthy and lesion sites were significantly different. Several OTUs in the Orders Erysipelotrichales, Clostridiales, Bacteroidales, and Spirochaetales were identified as indicators of noma, suggesting that one or more microbes within these Orders is associated with the development of noma lesions. Future studies should include longitudinal sampling of viral and microbial components of this community, before and early in noma lesion development.

[Evoluting form of cancrum oris, about 55 cases collected at the Academic Hospital Yalgado Ouedraogo of Ouagadougou]. / Le noma évolutif, à propos de 55 observations vues au centre hospitalier universitaire Yalgado Ouedraogo de Ouagadougou.

The cancrum oris is still an up to date disease in our environment. The death rate and the after effects of this disease make all together the main interest of this survey. In a retrospective survey carried out from January 2003 to December 2012, we colligated 55 cases of progressive cancrum oris followed at the stomatological and maxillofacial surgery at the Academic Hospital Yalgado OUEDRAOGO. On the epidemiological level, we noticed an impact of 5.5 cases per year. The average age of our patients was about 7.64 with a sex ratio of 1.03. Most of the patients were from an underprivileged family (96.4%). On the clinical level, we noticed that most of the patients consulted only after the gangrene had fallen (89.1%) and were seriously affected (67.3%) with a bad oral and dental hygiene (38.1%). The attacks were mainly jugal (25%) and labial (24.1%). The cancrum oris was in most of the cases associated to broncho pneumonitis, malaria and to HIV infection (31.37%). For the medical treatment, we focused on resuscitation, re nutrition, hydro electrolytic rebalancing and antibiotherapy. The surgical treatment was essentially made on the affected areas, controlled skinning and most often followed by sequestrectomy. 81.8% of the patients recovered completely from the infection, 60% had after effect injuries. We recorded a death rate of 14.5%. In order to overcome this disease we need both national and international support.

Microarray analysis of microbiota of gingival lesions in noma patients.

Noma (cancrum oris) is a gangrenous disease of unknown etiology affecting the maxillo-facial region of young children in extremely limited resource countries. In an attempt to better understand the microbiological events occurring during this disease, we used phylogenetic and low-density microarrays targeting the 16S rRNA gene to characterize the gingival flora of acute noma and acute necrotizing gingivitis (ANG) lesions, and compared them to healthy control subjects of the same geographical and social background. Our observations raise doubts about Fusobacterium necrophorum, a previously suspected causative agent of noma, as this species was not associated with noma lesions. Various oral pathogens were more abundant in noma lesions, notably Atopobium spp., Prevotella intermedia, Peptostreptococcus spp., Streptococcus pyogenes and Streptococcus anginosus. On the other hand, pathogens associated with periodontal diseases such as Aggregatibacter actinomycetemcomitans, Capnocytophaga spp., Porphyromonas spp. and Fusobacteriales were more abundant in healthy controls. Importantly, the overall loss of bacterial diversity observed in noma samples as well as its homology to that of ANG microbiota supports the hypothesis that ANG might be the immediate step preceding noma.

Risk factors for noma disease: a 6-year, prospective, matched case-control study in Niger.

BACKGROUND: Noma is a poorly studied disease that leads to severe facial tissue destruction in children in developing countries, but the cause remains unknown. We aimed to identify the epidemiological and microbiological risk factors associated with noma disease. METHODS: We did a prospective, matched, case-control study in Niger between Aug 1, 2001, and Oct 31, 2006, in children younger than 12 years to assess risk factors for acute noma. All acute noma cases were included and four controls for each case were matched by age and home village. Epidemiological and clinical data were obtained at study inclusion. We undertook matched-paired analyses with conditional logistic regression models. FINDINGS: We included 82 cases and 327 controls. Independent risk factors associated with noma were: severe stunting (odds ratio [OR] 4·87, 95% CI 2·35-10·09) or wasting (2·45, 1·25-4·83); a high number of previous pregnancies in the mother (1·16, 1·04-1·31); the presence of respiratory disease, diarrhoea, or fever in the past 3 months (2·70, 1·35-5·40); and the absence of chickens at home (1·90, 0·93-3·88). After inclusion of microbiological data, a reduced proportion of Fusobacterium (4·63, 1·61-13·35), Capnocytophaga (3·69, 1·48-9·17), Neisseria (3·24, 1·10-9·55), and Spirochaeta in the mouth (7·77, 2·12-28·42), and an increased proportion of Prevotella (2·53, 1·07-5·98), were associated with noma. We identified no specific single bacterial or viral pathogen in cases. INTERPRETATION: Noma is associated with indicators of severe poverty and altered oral microbiota. The predominance of specific bacterial commensals is indicative of a modification of the oral microbiota associated with reduced bacterial diversity. FUNDING: Gertrude Hirzel Foundation.

Bacterial diversity in oral samples of children in niger with acute noma, acute necrotizing gingivitis, and healthy controls.

BACKGROUND: Noma is a gangrenous disease that leads to severe disfigurement of the face with high morbidity and mortality, but its etiology remains unknown. Young children in developing countries are almost exclusively affected. The purpose of the study was to record and compare bacterial diversity in oral samples from children with or without acute noma or acute necrotizing gingivitis from a defined geographical region in Niger by culture-independent molecular methods. METHODS AND PRINCIPAL FINDINGS: Gingival samples from 23 healthy children, nine children with acute necrotizing gingivitis, and 23 children with acute noma (both healthy and diseased oral sites) were amplified using "universal" PCR primers for the 16 S rRNA gene and pooled according to category (noma, healthy, or acute necrotizing gingivitis), gender, and site status (diseased or control site). Seven libraries were generated. A total of 1237 partial 16 S rRNA sequences representing 339 bacterial species or phylotypes at a 98-99% identity level were obtained. Analysis of bacterial composition and frequency showed that diseased (noma or acute necrotizing gingivitis) and healthy site bacterial communities are composed of similar bacteria, but differ in the prevalence of a limited group of phylotypes. Large increases in counts of Prevotella intermedia and members of the Peptostreptococcus genus are associated with disease. In contrast, no clear-cut differences were found between noma and non-noma libraries. CONCLUSIONS: Similarities between acute necrotizing gingivitis and noma samples support the hypothesis that the disease could evolve from acute necrotizing gingivitis in certain children for reasons still to be elucidated. This study revealed oral microbiological patterns associated with noma and acute necrotizing gingivitis, but no evidence was found for a specific infection-triggering agent.

Noma in an Afghani child: a case report.

We present a case of Cancrum Oris, also known as Noma, in a child treated by an Otorhinolaryngologist at a United States-led Joint Forces hospital in Afghanistan. Noma is a deadly, necrotizing infection of the face that is rarely seen in wealthy nations but can cause significant morbidity in third world countries. Through a literature review, we report the incidence, risk factors, clinical features, and proposed treatment for this disease.

Acute necrotic stomatitis (noma) associated with methicillin-resistant Staphylococcus aureus infection in a newly acquired rhesus macaque (Macaca mulatta).

BACKGROUND: A newly acquired rhesus macaque was suffering from rapid destruction of the left cheek caused by necrotizing stomatitis. METHODS: To restore reconstructive surgery and intensive care with antibiotics, wound protection, wound healing agents, and debridement were applied. RESULTS: Staphylococcus aureus and Enterococcus faecalis were isolated from the culture of the lesion, and the antibiotic susceptibility test revealed methicillin-resistant Staphylococcus aureus infection. Vancomycin and ampicillin-sulbactam effectively treated the bacterial infections, and reconstructive surgery was performed once the infection was cleared. Topical application of recombinant human epidermal growth factor (rhEGF) was useful to treat exposed wound of the noma lesion. CONCLUSIONS: Simian noma associated with methicillin-resistant Staphylococcus aureus (MRSA) had not previously been reported in non-human primates. Although noma associated with MRSA is hard to cure because of its rapid and destructive progress, the aggressive therapy used in this study led to the successful resolution of an acute necrotic stomatitis lesion in a rhesus macaque.

Necrobacillosis in humans.

Necrobacillosis, often used synonymously with Lemierre's syndrome, is a form of abscess infection in the peritonsillar area associated with a thrombophlebitis and caused by the strict anaerobic species Fusobacterium necrophorum. The thrombosis formed affects the internal jugular vein, from which the bacteria are seeded out in the bloodstream and cause bacteremia. Septicemia is a common complication with an often fatal outcome. Necrobacillosis is very rare and is referred to as the 'forgotten disease'. It is probably frequently overlooked in clinical practice in its early and milder forms such as tonsillitis (sore throat) and peritonsillar abscess. F. necrophorum frequently participates in these infections and is thus suspected to have an etiological role in Lemierre's syndrome. Similarly, F. necrophorum seems to play an important role in noma (cancrum oris) and this disease is also included in the necrobacillosis complex. Diagnosis of infections of the necrobacillosis complex seeks to disclose F. necrophorum in swab samples or blood culture. The most commonly used therapy is metronidazole in combination with penicillin or amoxicillin. Clindamycin is also an option, especially in cases of penicillin allergy.

An oro-facial disease 'noma (cancrum oris)' in a Japanese monkey (Macaca fuscata): clinical signs, clinicopathological features, and response to treatment.

BACKGROUND: A Japanese monkey developed severe oro-facial lesions that were called noma in humans. Although extensive destruction of both the buccal regions occurred with rapid progress, author successfully treated the lesions with povidone-iodine, enrofloxacin, chymotrypsin, a glycyrrhizin preparation, and a basic fibroblast growth factor. METHODS: Author clinicopathologically investigated this disease during the treatment. RESULTS: In the subcutaneous and muscular tissues, the lesions developed characteristic changes such as dissolving collagen fibers and muscular tissues phagocytosed by giant and epitheloid cells. The monkey showed a notable increase in creatine kinase activities. The present examinations revealed severe invasive findings in muscular tissues, which were accompanied by infections of beta-hemolytic streptococcus Group C. This monkey was negative for simian immunodeficiency virus antibody; however, infection with simian D retrovirus was not ruled out. CONCLUSIONS: Simian noma was a rapidly devastating disease, which destroyed the muscle tissues of oro-facial structure. Nonhuman primates are the only species that develop oro-facial lesions, corresponding to noma in humans.

Noma (cancrum oris) in human immunodeficiency virus infection and acquired immunodeficiency syndrome (HIV and AIDS): clinical experience in Zimbabwe.

PURPOSE: This retrospective study describes the clinical features and management of noma (cancrum oris) in patients with HIV and AIDS. PATIENTS AND METHODS: Records of 48 consecutive patients with noma (cancrum oris) seen between July 2002 and November 2006 were reviewed for age, gender, clinical features, and management. Other reports on noma in HIV and AIDS in Zimbabwe were also reviewed. RESULTS: There were 48 patients included; 35.4% (n = 17) were males, of which 64.7% (n = 11) were children (16 years and younger) and 35.3% (n = 6) were adults; 64.6% (n = 31) were females, out of which 87.1% (n = 27) were children and 12.9% (n = 4) were adults. The average age was 14.2 years (range, 3 to 30 years) for males and 9.2 years (range, 1 to 36 years) for females. The average age for the entire group was 11 years (range, 1 to 36 years). All patients were HIV-positive by the ELISA method. Only 13 patients had CD4 cell and CD8 cell count obtained, ranging from 10 to 594 cells/microL with a CD4/CD8 ratio ranging from 0.02 to 0.45. Only 5 patients had microbiologic investigations conducted, isolating Staphylococcus aureus, Klebsiella species, group D Streptococcus, and group B hemolytic Streptococcus. Isolated cheek defect (37.5%) was most common, followed by the type I and type IV defect (25% each). Administration of antibiotics, nutritional support, wound debridement, and sequestrectomy were conducted before definitive reconstructive surgery. Facial reconstruction was performed using distant and local advancement flaps. No bony reconstruction was performed. Satisfactory results were achieved with minimal infection and flap breakdown. Follow-up was difficult; patients were lost to follow-up within 6 to 12 months after surgery. CONCLUSION: Noma cases are on the increase in line with the current HIV and AIDS epidemic. Female children appear to be more commonly affected than their male counterparts. Reconstructive surgery is possible in patients with low CD4/CD8 ratios because of HIV infection.

Noma neonatorum.

Noma Neonatorum is characterized by a gangrenous process involving mucocutaneous junctions of oral, nasal and anal area and occasionally, the eyelids and scrotum. It is seen during the first few weeks of neonatal life in premature and low birth weight babies. Noma Neonatorum is commonly described with pseudomonas aeruginosa septicemia. A case of Noma Neonatorum associated with E.coli sepsis is described for the first time.