A noma case report: A warning message from Northern Uganda.

Noma, or Cancrum oris, is a severe and rapidly progressing gangrenous infection that primarily affects the face. It is most commonly observed in children living in impoverished conditions, especially in sub-Saharan Africa. Rapid diagnosis and early management are crucial to prevent devastating consequences, such as functional limitations and serious psychological repercussions. Herein, we present a case of an 8-month-old child affected by noma, whose positive outcome is attributed to the prompt recognition by healthcare personnel. In our patient, the condition was likely related to malnutrition and the preceding extraction of a deciduous tooth reported by the mother and probably associated with a traditional Ugandan practice called Ebiino. This is the second case reported in Uganda, and given the limited healthcare access in most of the country, coupled with the high prevalence of poverty and other predisposing factors, it becomes evident that the incidence of noma is underestimated. Noma, as a neglected disease, requires greater awareness within communities and among healthcare professionals. A collective effort is needed to significantly reduce risk factors and promote prevention of this life-threatening disease.

Prevalence and associated risk factors for noma in Nigerian children: a systematic review and meta-analysis.

OBJECTIVES: To determine the prevalence, case-fatality rate, and associated risk-factors of Noma in children in Nigeria. METHODS: Search was conducted in PubMed, Google Scholar, and Cochrane Library databases. Data were extraction using a double-blind approach. Discrepancies were resolved by a third reviewer. Heterogeneity was evaluated using I2 statistics. Random-effects model was used for the meta-analysis and subgroup analysis was conducted. The study quality was evaluated using standard Critical-Appraisal-Checklist. RESULTS: Of the 1652 articles identified, 12 studies that met the inclusion criteria included 871 cases of Noma. Two studies had high-risk of bias and were excluded in the meta-analysis. Pooled prevalence of Noma was 2.95% (95%CI:2.19-3.71; Z = 7.60; p < 0.00001, I2:100.0). Case fatality was reported in one study. Sex-distribution had a male-to-female ratio of 1.1:1. Malnutrition (88.42%, 95%CI:52.84-124.00; I2:100.0), measles (40.60%; 95% CI:31.56-49.65; I2:100.0) and malaria (30.75%; 95% CI:30.06-31.45; I2:100.0) were the most notable associated risk-factors. Prevalence of Noma was non-significantly lower in southern (1.96%,95%CI:1.49-2.44;6 studies) than in northern (4.43%; 95%CI:-0.98-9.83; 4 studies) Nigeria. One study reported the prevalence of Noma in children younger than 5 years. CONCLUSIONS: About every 3 in 100 children in Nigeria had Noma and the prevalence was non-significantly higher in northern than southern Nigeria. Malnutrition, measles, and malaria were major associated risk-factors. Case-fatality rate and prevalence based on different age-groups were inconclusive.

Cancrum oris and hemiparesis in a young female patient-a case report.

Key Clinical Message: Noma is still around today and can be deadly if ignored. Prompt identification and comprehensive care are essential for averting permanent impairments and disfigurements. Abstract: Noma is a rapid developing orofacial gangrene and a disabling disease that primarily affects young children who live in dangerous conditions. Underlying diseases such as HIV/AIDS and malnutrition can enhance the likelihood of Noma's emergence. This is a case of a 9-year-old girl patient who arrived malnourished and with an ulcerating communicating right mandibular soft tissue lesion as well as right hemiparesis which had an acute onset. The patient was likewise HIV positive discovered upon admission, possibly as a result of vertical transmission, and was an ART (antiretroviral therapy) treatment naive patient. A holistic treatment plan was installed and a positive clinical response was observed. Early treatment is key in Noma management.

Understanding noma: WHO's recognition and the path forward in global health.

The World Health Organization's (WHO) designation of noma as a neglected tropical disease (NTD) on 15 December 2023 marks a crucial advancement in global health efforts. This move sheds light on a condition predominantly affecting undernourished children in isolated regions of sub-Saharan Africa. Recognized as the 21st NTD, noma, or cancrum oris, is a serious condition leading to orofacial gangrene. The disease largely impacts young children and those with compromised immune systems, including individuals with human immunodeficiency virus or leukaemia. Determining the exact prevalence of noma is complex, hindered by rapid disease progression, societal stigma and a lack of reporting, especially in impoverished areas. The WHO's acknowledgment is a significant step, emphasizing the need for more in-depth research and resources to address this overlooked disease. It highlights the critical role of multifaceted prevention strategies, including economic empowerment, improved nutrition and enhanced vaccination efforts. This recognition is pivotal in guiding international health initiatives towards better outcomes for some of the most at-risk populations globally.

Noma in an 18 months Toddler: A Rare Case Report with Review of Literature.

Introduction: Noma is a polymicrobial necrotizing infection of the mouth and face which destroys the soft and hard tissues of the oral and paraoral structures. Malnutrition, poverty, lack of basic hygiene, and chronic disease state etc., possess a risk of developing noma. Noma neonatorum is a progressive gangrenous disease affecting the premature infants. Aim: We present a rare case of noma in an 18 months toddler with a chronic debilitating condition predisposing to the disease progression. Results: The necrotizing infection of the face developed after six months of multiple episodes of chronic blood-tinged diarrhea. The facial defect was managed with release of fibrosis and reconstruction with a superiorly based nasolabial flap. Discussion: Noma reflects extreme malnutrition and poverty with a vast range of etiopathological agents. This case emphasizes the need to understand the host risk factors and etiopathologic agent predisposing to this rare opportunistic and dormant but devastating disease, the morbidities associated with it and measures to prevent it.

Noma: a PubMed-based informetric analysis of a neglected tropical orofacial disease in Nigeria.

Noma is an orofacial gangrenous infection commonly affecting malnourished children in the tropical region of the World, particularly the sub-Saharan Africa. Nigeria is a sub-Saharan African country which is among the countries seriously affected by noma. In Nigeria, noma has been classified as a priority disease. However, only very little attention has been focused on noma research in Nigeria, unlike many other priority diseases. This study conducted an informetric analysis of research outputs on noma in Nigeria, using the PubMed database - a world-leading and authoritative database of medical literature. This study revealed that only 26 PubMed-indexed publications on noma (PONs), published between January 1990 and September 2021, were available. A trend analysis of these PONs showed that the average output (outputs from 1999 to 2020 = 23) rate per year (from 1990 to 2020 [31 years]) was 0.74 (23/31). Sokoto (n = 11), Oyo (n = 4) and Kebbi (n = 3) were the top three Nigerian states surveyed on noma in the included PONs. Only 12 publications had international co-authors, of which only few publications had co-authors affiliated with institutions in other African countries: South Africa - 5 publications; and Rwanda - 2 publications. Only two publications were funded. Also, the top five prolific Nigerian authors on noma in Nigeria were affiliated with Noma Children Hospital, Sokoto State, Nigeria. In conclusion, PONs by Nigerian authors is so meagre in quantity. This low output is an issue of medical concern. More research focused on noma in Nigeria is needed through funding and other research capacity-strengthening measures.

Psychosocial aspects of Noma (Cancrum Oris) in sub-Saharan Africa: A scoping review.

Noma is a neglected tropical disease of an underserved population. Our review describes the volume and scope of existing research evidence on psychosocial aspects of Noma in sub-Saharan Africa (SSA). Our literature search was conducted on all original peer-reviewed research articles on the psychosocial aspects of Noma in each country in SSA using PubMed, Google Scholar, and a direct search of reference list of pertinent journal articles. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews guidelines was used to describe the review. A total of six studies were found in four countries of the region. One in three persons with Noma has a mental health condition, indices of social disadvantage and belief in the supernatural causation of the disease are very common. However, despite evidence of enormous need, a huge gap still exists in the area of psychosocial alleviation.

Health-related quality of life in patients with noma (cancrum oris): Case series.

INTRODUCTION AND IMPORTANCE: Cancrum oris (noma) is a destructive disease of orofacial tissues and adjacent structures that progress rapidly, causing functional and cosmetic problems to the patient and ultimately affecting their health-related quality of life. This case series focuses on the impact of the cancrum oris on the health-related quality of life of patients which has been underreported in literature. CASE PRESENTATION: Herein, we present 3 cases (1 pediatric and 2 adult patients) of cancrum oris. One adult male succumbed to the disease while the remaining two are undergoing treatment. We used the modified versions of the Early Childhood Oral Health Impact Scale (ECOHIS) and the University of Washington-Quality of Life (UWQoL) questionnaire for pediatric and adult patients respectively. CLINICAL DISCUSSION: The sequela of cancrum oris affects negatively the quality of life of the patients even after definitive management. Despite this, there is no validated questionnaire to assess the quality of life. The modified versions of the ECOHIS and the UWQoL questionnaires that were used in this case report can be used effectively to assess the HRQoL of the patients. CONCLUSION: Cancrum oris is a neglected disease that affects negatively the health-related quality of life of patients who suffer from it, despite medical intervention.

A Web of Science-Based Bibliometric Analysis of Global Noma Publications.

The World Health Organization recognizes noma as a global health problem and has suggested prioritizing research into this disease. A bibliographic search of original articles published in the Web of Science database up to 2022 was performed. A bibliometric analysis was carried out with the bibliometrix package in R and VOSviewer. We identified 251 articles published in 130 journals. The first publication was in 1975, the highest number of publications was in 2003, and the average number of citations per document was 12.59. The author with the highest number of publications was Enwonwu CO, and the Noma Children's Hospital had the highest number of articles on this topic. Plastic and Reconstructive Surgery was the journal with the most publications, and the study by Petersen PE was the most cited. The country of corresponding authors that had the most publications and the most significant number of total citations was the United States. "Children" and "Reconstruction" were the most used keywords. In conclusion, there are few publications on noma worldwide, confirming the neglected status of this disease. Urgent actions are needed to increase evidence in regard to the epidemiology of noma and public health interventions to mitigate the ravages of this disease.

Noma disease (cancrum oris, orofacial gangrene) in an acute myeloid leukemia patient: a case report.

BACKGROUND: Noma is a rare disease that occurs mainly in malnourished patients in developing countries. Noma starts as facial swelling and gingival necrosis that eventually necrotizes underlying tissues including the jaw bone, leaving severe disfigurement. It is reported extremely rarely in patients with severe immunosuppression or blood dyscrasia. CASE PRESENTATION: The gingivitis that occurred in a 12-year-old Asian female patient with acute myeloid leukemia was getting increasingly worse. Although the proper treatment was done, the patient's condition did not improve, and eventually, a large full-thickness defect was left in the maxillofacial part. CONCLUSIONS: Early diagnosis and management is the only way to prevent the progression, which leads to facial disfigurement. We present a case of noma in a pediatric acute myeloid leukemia patient, in which oral function was restored through surgical intervention.

More than 100 years of neglect: a bibliometric analysis of global research on noma (cancrum oris).

BACKGROUND: Research plays a significant role in quantifying and addressing the burden of disease, improving healthcare delivery and developing evidence-based policy for neglected tropical diseases (NTDs). Since noma is not a recognised NTD by the World Health Organization (WHO), research activity is hypothesised to be low. This study aims to understand patterns and trends of research conducted on noma, to understand the patterns of citations, highlight networks of collaboration and analyse stakeholders in noma research. METHODS: The SCOPUS database was selected and searched. Data analysis was conducted using the bibliometrix package for R in RStudio. Areas of interest included the distribution of research output on noma by year and location, the academic impact of publication output, mapping the major academic community and conducting a thematic analysis of high-frequency keywords. RESULTS: A total of 208 documents were identified. The annual growth rate of publications is 4.3%. The average number of citations per publication was 11.0 (rounded from 10.98) and the average citations per year per publication was 0.57. The USA was the country with the highest percentage 10.6% (n=22) of corresponding authors, followed by Nigeria (6.7% [n=14]), the UK (6.3% [n=13]) and India (5.8% [n=12]). For those papers with multiple authors, there was an average of 3.22 co-authors per document. The collaboration index was 3.3. Upon plotting collaboration networks between authors, only 11 clusters were identified (frequency and intensity of collaboration). Mapping of trending topics showed a focus on skin grafting and transplantation within the literature. CONCLUSIONS: In order to progress towards eventual elimination of noma it is key that more research should be undertaken and more interest and funding should be dedicated to work on noma. Recognition of noma as an NTD by the WHO would be a first step in moving towards increased awareness.

Case report: a rare case of NOMA (cancrum oris) in a Malian woman.

Noma or cancrum oris is a multi-bacterial and opportunistic infection that destroys soft tissue, as well as muscle and bone, and can be fatal. We present a rare case of Noma in a 32-year-old Malian woman, from whom we isolated an Escherichia coli extended-spectrum beta-lactamase.

Model of care, Noma Children's Hospital, northwest Nigeria.

The Nigerian Ministry of Health has been offering care for noma patients for many years at the Noma Children's Hospital (NCH) in Sokoto, northwest Nigeria, and Médecins Sans Frontières has supported these initiatives since 2014. The comprehensive model of care consists of four main components: acute care, care for noma sequelae, integrated hospital-based services and community-based services. The model of care is based on the limited evidence available for prevention and treatment of noma and follows WHO's protocols for acute patients and best practice guidelines for the surgical treatment of noma survivors. The model is updated continually as new evidence becomes available, including evidence generated through the operational research studies performed at NCH. By describing the model of care, we wish to share the lessons learned with other actors working in the noma and neglected tropical disease sphere in the hope of guiding programme development.

Outcomes at 18 mo of 37 noma (cancrum oris) cases surgically treated at the Noma Children's Hospital, Sokoto, Nigeria.

BACKGROUND: Noma is a rapidly progressing infection of the oral cavity frequently resulting in severe facial disfigurement. We present a case series of noma patients surgically treated in northwest Nigeria. METHODS: A retrospective analysis of routinely collected data (demographics, diagnosis and surgical procedures undergone) and in-person follow-up assessments (anthropometry, mouth opening and quality of life measurements) were conducted with patients who had surgery >6 mo prior to data collection. RESULTS: Of the 37 patients included, 21 (56.8%) were male and 22 (62.9%) were aged >6 y. The median number of months between last surgery and follow-up was 18 (IQR 13, 25) mo. At admission, the most severely affected anatomical area was the outer cheek (n = 9; 36.0% of patients had lost between 26% and 50%). The most frequent surgical procedures were the deltopectoral flap (n = 16; 43.2%) and trismus release (n = 12; 32.4%). For the eight trismus-release patients where mouth opening was documented at admission, all had a mouth opening of 0-20 mm at follow-up. All patients reported that the surgery had improved their quality of life. CONCLUSIONS: Following their last surgical intervention, noma patients do experience some improvements in their quality of life, but debilitating long-term sequelae persist.

'I treat it but I don't know what this disease is': a qualitative study on noma (cancrum oris) and traditional healing in northwest Nigeria.

BACKGROUND: Noma, a neglected disease mostly affecting children, with a 90% mortality rate if untreated, is an orofacial gangrene that disintegrates the tissues of the face in <1 wk. Noma can become inactive with early stage antibiotic treatment. Traditional healers, known as mai maganin gargajiya in Hausa, play an important role in the health system and provide care to noma patients. METHODS: We conducted 12 in-depth interviews with caretakers who were looking after noma patients admitted at the Noma Children's Hospital and 15 traditional healers in their home villages in Sokoto state, northwest Nigeria. We explored perceptions of noma, relationship dynamics, healthcare practices and intervention opportunities. Interviews were audiorecorded, transcribed and translated. Manual coding and thematic analysis were utilised. RESULTS: Traditional healers offered specialised forms of care for specific conditions and referral guidance. They viewed the stages of noma as different conditions with individualised remedies and were willing to refer noma patients. Caretakers trusted traditional healers. CONCLUSIONS: Traditional healers could play a crucial role in the early detection of noma and the health-seeking decision-making process of patients. Intervention programmes should include traditional healers through training and referral partnerships. This collaboration could save lives and reduce the severity of noma complications.

Release of Extra Articular Ankylosis of Jaws as a Sequelae of Cancrum Oris with Extensive Gingival Myasis in a Scoliosis Patient: A Rare Case Report.

'Noma' is an allusion to the rapidly destructive course of gangrene (Emmanuel et al. in J Maxillofac Surg 11:160-170, 1983). The pathogenicity is related to the high bacterial load of microorganisms resulting in gangrene that rapidly invades the facial tissues. The infection causes perforating wounds of the lips, cheek, nose, palate and bony fusion of the maxilla and mandible.

Sociodemographic Characteristics of Traditional Healers and Their Knowledge of Noma: A Descriptive Survey in Three Regions of Mali.

Background: Noma can be a lethal disease and causes disfigurement in young children in low-resource countries, particularly in Africa. In these settings, 80% of the population mainly consult traditional healers for healthcare problems. Our study aimed to describe the sociodemographic characteristics of traditional healers and to assess their knowledge of noma. Methods: We conducted a survey among traditional healers in three Malian regions from May 2015 to January 2016 and collected data on sociodemographic characteristics, professional activity, knowledge, and experience of noma and collaboration with modern medicine. Results: Among 770 traditional healers invited to participate, 732 responded (95%) (mean age, 54.5 years). Most were illiterate (66.3%), which was associated with older age (p < 0.001). Although they treated all types of disease, only 10.5% had some knowledge of noma, with regional differences (p < 0.001). Conclusion: Noma is poorly known among traditional healers, especially in remote areas. Our findings suggest a lack of interest among young people for traditional medicine, implying an imminent decrease of healers, and thus the need for national health systems to strengthen and promote access to modern health care. Training programmes to improve the early diagnosis referral of noma patients should include all types of primary health workers.

Noma in a boy with septic shock: a case report.

BACKGROUND: Noma is a rare disease, which is characterized by rapid progression and a high rate of mortality; however, relatively few cases of noma infection accompanied by septic shock in children have been described. Further, most health care professionals have no knowledge of this disease or of its clinical significance. CASE PRESENTATION: Herein, we present a case report of a six-year-old male patient who was diagnosed with noma infection at a Chinese pediatric medical intensive care unit (PMICU), at which time, it was discovered that he had septic shock. Following treatment by continuous renal replacement therapy (CRRT) for septic shock arising from noma, the patient was in generally good condition, and the local wound was seen to be essentially healed five weeks post-admission. CONCLUSION: Noma is an opportunistic infectious disease condition. Treatment of the acute phase of noma predominantly focuses on controlling the infection and improving systemic conditions. In addition, CRRT could be considered as a treatment option for cases that present with noma accompanied by septic shock.

Noma: a reappraisal in Western countries - are HIV-negative immunocompetent adult patients safe?

Noma is an ancient Greek term referring to a gangrenous disease that destroys soft and hard tissues of oral and perioral structures. This disease has been known since ancient times and persisted in Europe until World War 2, while in the present day it is almost exclusively found in sub-Saharan Africa.

Pattern of noma (cancrum oris) and its risk factors in Northwestern Nigeria: A hospital-based retrospective study.

Background: Noma (cancrum oris) remains the scourge of children and the "face of poverty" in Sub-Saharan Africa. Recent data on the burden of noma and its risk factors are needed for evaluating and redesigning interventions for its prevention and control. Objectives: This study aimed to determine the pattern of noma and its risk factors in Northwestern Nigeria. Materials and Methods: It was a retrospective study that looked into cases of noma (cancrum oris) admitted into the Noma Children Hospital, Sokoto, Nigeria, between January 1999 and December 2011. Information on patients' bio-data, the site and severity of lesions, and presence of trismus and its severity were extracted from the patients' case files and analyzed using descriptive statistics. Results: One hundred and fifty-nine (8.3%) of the 1923 patients admitted to the hospital from January 1999 to December 2011 were diagnosed with fresh noma. The mean age of the patients was 3.0 ± 1.4 years, and majority of them, 139 (87.4%) were aged 1-5 years. The soft-tissue lesions essentially involved multiple sites but most commonly the outer and inner cheeks (84.3%). The most common risk factors identified were measles (47.2%) and protein-energy malnutrition (42.1%). There were rises and falls in the prevalence of noma in the period studied. Conclusion: This study showed a high burden of noma in Northwestern Nigeria, mostly among children aged 1-5 years, and with soft-tissue lesions involving multiple sites. Measles and malnutrition were the major risk factors identified, and the disease trend showed a wave-like pattern. There is an urgent need to eliminate the disease in Nigeria through prevention and control of infectious diseases and malnutrition.

RésuméContexte: Le noma (cancrum oris) demeure le fléau des enfants et le "visage de la pauvreté" en Afrique subsaharienne. Des données récentes sur le fardeau du noma et de ses facteurs de risque sont nécessaires pour évaluer et redéfinir les interventions en vue de sa prévention et de son contrôle. Objectifs: Cette étude visait à déterminer la structure du noma et de ses facteurs de risque dans le nord-ouest du Nigeria. Matériels et Méthodes: Il s'agissait d'une étude rétrospective qui a examiné les cas de noma (cancrum oris) admis à l'Hôpital Noma Children, Sokoto, au Nigeria, entre janvier 1999 et décembre 2011. Informations sur les bio-données des patients, le site et la sévérité des les lésions, et la présence de trismus et sa gravité ont été extraites des dossiers des patients et analysées à l'aide de statistiques descriptives. Résultats: Cent cinquante-neuf (8,3%) des 1923 patients admis à l'hôpital entre janvier 1999 et décembre 2011 ont reçu un diagnostic de noma frais. L'âge moyen des patients était de 3.0 ± 1.4 ans et la majorité d'entre eux 139 (87.4%) étaient âgés de 1 à 5 ans. La lésion des tissus mous concernait essentiellement plusieurs sites, mais le plus souvent les joues externe et interne (84.3%). Les facteurs de risque les plus fréquemment identifiés étaient la rougeole (47.2%) et la malnutrition protéino-énergétique (42.1%). Il y avait des augmentations et des chutes de la prévalence du noma dans la période étudiée. Conclusion: Cette étude a montré une forte charge de noma dans le nord-ouest du Nigeria, principalement chez les enfants âgés de 1 à 5 ans, et avec des lésions des tissus mous impliquant plusieurs sites. La rougeole et la malnutrition ont été les principaux facteurs de risque identifiés, et la tendance de la maladie a montré un profil ondulatoire. Il est urgent d'éliminer la maladie au Nigéria grâce à la prévention et au contrôle des maladies infectieuses et de la malnutrition.