Noma (cancrum oris): A scoping literature review of a neglected disease (1843 to 2021)

Background Noma (cancrum oris) is an ancient but neglected and poorly understood preventable disease, afflicting the most disenfranchised populations in the world. It is a devastating and often fatal condition that requires urgent and intensive clinical and surgical care, often difficult to access as most cases of noma occur in resource-limited settings. We conducted a scoping review of the literature published on noma to understand the size and scope of available research on the disease and identify research gaps that need to be addressed to evolve our understanding of how to address this disease. Methods We searched 11 databases and collected primary peer reviewed articles on noma in all languages, the final search was conducted on 24th August 2021. The oldest manuscript identified was from 28th March 1843 and the most recently published manuscript was from 3rd June 2021. Search terms included cancrum oris and noma. Data was extracted using a standardised data extraction tool and key areas of interest were identified. The Preferred Reporting Items for Systemic review and Meta-Analyses requirements were followed. Results The review included 147 articles, the majority of the studies (n = 94, 64%) were case reports. Most manuscripts (n = 81, 55%) were published in the 2000s, 49 (33%) were from the 1900s and 17 (12%) from the 1800s. The main areas of interest identified were the history and epidemiology of the disease, noma’s clinical progression and aetiology, treatment regimens, mortality rates and the risk factors for the development of noma. Conclusions Noma has been reported in the literature for hundreds of years; however important gaps in our understanding of the disease remain. Future research should focus on determining the burden and distribution of disease; the true mortality rate, pathogenic cause(s) and the factors that influence prognosis and outcomes after treatment.

First Case of Cancrum Oris (Noma) in an HIV-Positive Patient in Bulgaria

We present a case of a necrotising disease of the orofacial tissues, the so-called noma, in a female patient with AIDS in a stage of severe immune deficiency. To our knowledge, this is the first time a case like that has been reported in Bulgaria. The patient received an antibiotic course of treatment with a triple combination to good effect on the inflammatory process, and began also HAART. Within 3 weeks, the patient’s general condition improved. However, despite the treatment, a demarcation line was formed, and a defect on the lower lip occurred. Noma is a manifestation of severe immune deficiency with lasting adverse consequences for the patient: severe deformation of the maxilo-facial region and functional impairment (i.e. difficulties eating and speaking, and salivary leak).

Rol bacteriano en la gingivitis ulcero necrotizante aguda

La gingivitis ulcero necrotizante aguda (GUNA) es una enfermedad infecciosa del tejido gingival. Se presenta comúnmente como una inflamación y/o ulceración aguda y dolorosa, que afecta principalmente la zona de la papila interdental y puede estar acompañada de halitosis, formación de pseudomembranas y sangrado gingival (1,2). Si no es tratada a tiempo, la GUNA puede ocasionar una rápida destrucción del tejido y transformarse en una periodontitis ulcero necrotizante aguda (PUNA) e incluso en un Cancrum oris (Noma), que puede resultar fatal (3,4).

Cancrum Oris(Noma): An Early Sign of Acute Lymphoblastic Leukemia Relapse

Cancrum oris is an infectious disease, which involves the orofacial tissues and adjacent neighboring structures in its fulminant course. Cancrum oris can occur rarely in chemotherapy-induced neutropenic patients with acute lymphoblastic leukemia. This rare case report reveals that cancrum oris can be a sign of acute lymphoblastic leukemia relapse. It is characterized by gangrenous lesions on skin with fine-needle aspiration cytology smear from the lesion showing infiltration of lymphoblasts. Cancrum oris can occur rarely in chemotherapy-induced neutropenic patients with acute lymphoblastic leukemia, but the presentation as an early sign of extramedullary disease relapse has not been documented in literature. Here we report a case of cancrum oris in a 27-year-old woman who presented with redness and swelling of the upper lip and infranasal area. She had a known case of acute lymphoblastic leukemia diagnosed 6 months back and was on regular multiagent chemotherapy for the same. Clinical examination revealed gangrenous lesions on infranasal, intranasal, and area involving the upper lip. Complete blood count, bone marrow aspiration, and cytology did not show any lymphoblasts. Blood culture and swab culture from the gangrenous area were sterile. Patient did not get better even after 7 days of broad-spectrum antibiotic treatment. With a high degree of clinical suspicion of isolated extramedullary relapse, fine-needle aspiration cytology from marginal and gangrenous area was taken and revealed infiltration of soft tissues by lymphoblasts. After receiving four cycles of multiple chemotherapy drugs, the skin lesions resolved. Treatment of cancrum oris involves the correction of the underlying immune status, antibiotics, and surgical reconstruction. The rare possibility of relapse of acute lymphoblastic leukemia presenting as cancrum oris should be kept in mind in patients with acute lymphoblastic leukemia on chemotherapy with skin manifestations.

‘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.