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Noma and noma neonatorum.

Noma and noma neonatorum are rare gangrenous diseases that result in mutilating loss of tissue in the oronasal region. Noma usually occurs in patients between the ages of 2 and 5 years who are malnourished, have suffered a precedent illness, or are in some way immunodeficient, or all of the above. The gangrenous slough is thought to be caused by a mixed infection of oral bacterial pathogens. The disease may be fatal when it occurs in a severely debilitated patient. Noma neonatorum produces somewhat similar appearing lesions in the neonate. The infectious organism is usually Pseudomonas and the disease is generally accompanied by a life-threatening pseudomonal sepsis. Both diseases are rare in North America. Patients with noma and noma neonatorum were treated at the Children's Hospital and Medical Center, Seattle, WA. We present these cases and a literature review.

Bacterial Infections

Noma neonatorum: an unusual case of noma involving a full-term neonate.

Noma neonatorum is a gangrenous process that occurs in the oral, nasal or anal area and occasionally the eyelids and scrotum of the newborn. The disease is caused by Pseudomonas aeruginosa and usually affects premature ill babies during the first few weeks of life. A full-term neonate with nasal and scrotal noma is uncommon and is therefore reported.

Humans

Necrotising infection of the orofacial tissues in neonates (noma neonatorum). Case report.

Noma neonatorum should be differentiated from noma, in that it is typically a disease of seriously ill premature infants whose birth weight was low, and is caused by Pseudomonas aerugenosa septicaemia. We know of only two case reports of noma neonatorum involving newborn infants born at full term, so we report here another case of noma neonatorum in a neonate born at full term. In addition we describe the differences between noma neonatorum and noma (cancrum oris), a clinically related entity.

Diagnosis, Differential

Noma: a neglected scourge of children in sub-Saharan Africa.

Poverty is the single most important risk indicator for noma (cancrum oris), a severe gangrene of the soft and hard tissues of the mouth, face, and neighbouring areas. The risk factors associated with an increased probability of noma developing include the following: malnutrition, poor oral hygiene, and a state of debilitation resulting from human immunodeficiency virus (HIV) infection, measles, and other childhood diseases prevalent in the tropics. There are many similarities between noma and necrobacillosis of the body surface of wallabies (Macropus reforgriseus), and it is proposed that noma results from oral contamination by a heavy load of Bacteroidaceae (particularly Fusobacterium necrophorum) and a consortium of other microorganisms. These opportunistic pathogens invade oral tissues whose defences are weakened by malnutrition, acute necrotizing gingivitis, debilitating conditions, trauma, and other oral mucosal ulcers. The current escalation in the incidence of noma in Africa can be attributed to the worsening economic crisis in the region, which has adversely affected the health and well-being of children through deteriorating sanitation, declining nutritional status and the associated immunosuppression, and increased exposure to infectious diseases. Prevention of noma in Africa will require measures that address these problems, and most importantly, eliminate faecal contamination of foods and water supplies.

Africa South of the Sahara

[Noma or cancrum oris: etiopathogenic and nosologic aspects].

Noma is a gangrenous disease that usually begins in the mouth and is characterized by rapid necrotizing destruction of soft tissue and underlying bone. The disease, which is associated with a strong putrid odor suggestive of mixed bacterial infection, develops only in predisposed persons, especially children whose natural defenses have been weakened by poor socio-economic living conditions. However a few atypical cases have recently been reported in adults in whom the main risk factor seemed to be immunodepression. The increasing number of wars in the Third World and the AIDS epidemic raise the likelihood that the number of cases of this disease will rise sharply particularly since the incidence has been grossly underestimated for many years. It now seems clear that a combination of local and systemic risk factors are implicated in the etiopathogenesis of noma with the common denominator being a weakened immune system. This would account for the fulminating course of the infectious process due to one or more opportunistic microbial or cytopathogenic agents or even to a still undetermined immunopathologic reaction that lead to massive tissue destruction. Poor understanding of the etiopathogenesis of noma is a major factor perpetuating the nosologic problems posed by this disease. Some authors add to the confusion by including a range of gangrenous diseases under the heading of noma. It is important that the term noma be applied only to necrotizing processes that begin in the mouth and present the characteristic features and course.

Adult

[Progressive noma: apropos of 27 cases seen at the National Hospital Center of Ouagadougou].

This retrospective study objects to report epidemiological profile, clinical display and take care of progressive noma at the National Hospital Center of Ouagadougou. We assign the term noma to the gangrenous gingivo-stomatitis occurring in a prone environment, leading in a few days into a sharp ulcer in the soft parts, accompanied by halitosis. This definition excludes noma like-lesions. 27 cases have been observed in the ENT department and the Paediatrics between 1991 and 1995. They were 17 boys and 10 girls aging between 2 and 8 years and most of them coming from families with modest income (76%). The consultation delay averaged 8 days. The research for associated pathologies showed 63% energizing and protein malnutrition. We only observed unilateral localisations and no case of extra-facial one. At the stage we received our patients, the gangrenous was formed and the aspect evoked noma right away. Quite a lot needs to be done for precocious diagnosis because it is at the beginning phase that best evolution is obtained. Even though no evidence of the responsibility of a specific pathogenic germ was established so far, nevertheless, the assumption that has more support remains that of a bacterial infection in a prone environment. The vital prognosis of the progressive noma appears to be much linked with intensive care and feeding again. The hope to see this poverty-linked pathology under control is essentially based on prevention: medical population information, medical personnel's awareness for early diagnosis and best take care of these patients, improvement of the living conditions of the people and the health care services.

Burkina Faso

Noma neonatorum: Its aetiopathogenesis.

Noma neonatorum, a gangrenous process affecting the nose, lips, mouth, anal region, and occasionally the scrotum and eyelids, affects neonates, especially low-birth-weight and premature ill babies, and is usually fatal 1-3 days after onset. In 35 cases of noma neonatorum Pseudomonas aeruginosa was isolated from blood-culture (86.3%), gangrenous areas (96.0%), rectal swabs (58.3%), and cerebrospinal fluid (60.0%). Blood-vessels in the deep cutis or subcutis were affected and the gangrenous process extended superficially. Noma in older children and adults is caused by fusospirochaetosis but noma neonatorum appears to be due to P. aeruginosa septicaemia.

Anal Canal

Noma in an unusual case of perforated appendicitis with pneumoperitoneum.

Noma is a very rare gangrenous infection of the oral cavity usually associated with debilitating diseases, immunosuppression, or malnutrition. Its development in a previously healthy 13-year-old boy with perforated appendicitis is presented. Streptococcus bovis type II and E coli were isolated from the noma and Streptococcus bovis, Bacteroides fragilis, and Bacteroides asacharolyticus from the peritoneal fluid. A transient impaired immune cellular function was found and may have contributed to the development of the noma in this child.

Adolescent

Noma neonatorum.

Noma neonatorum is a gangrenous process involving oral, nasal and anal area and occasionally, the eyelids and scrotum. It is seen during the first few weeks of neonatal life. The disease is caused by P. aeruginosa and is usually fatal. Noma neonatorum should be distinguished from the regular noma as it comprises a distinct age group, clinical course, microbiology and prognosis.

Age Factors

Noma in a full-term neonate.

Noma is an uncommon gangrenous process usually affecting malnourished children. A full-term neonate with orofacial noma, bilateral choanal atresia, and transient neutropenia with B cell deficiency is reported. This unusual appearance of noma in a well-nourished newborn might be related to the combination of choanal atresia and transient immune deficiency.

Agranulocytosis

Noma in a nonhuman primate.

A lesion in Macaca cyclopis which appears to conform to defined characteristics of noma in human beings has been reported. Clinical features in common include the gangrenous appearance of the lesion, the association with necrotizing ulcerative gingivitis, and the massive destruction of soft tissue and bone in the oronasofacial regions. Systemic features in common include debilitation of the host, leukocytosis, and depression of cellular immunologic responses. Microbiologic studies revealed the presence of organisms commonly found in necrotizing ulcerative gingivitis. The detection of true noma in nonhuman primates may now allow the opportunity for study of the etiology, pathophysiology, and therapy of this condition for human benefit.

Animals

Noma (cancrum oris) in human immunodeficiency virus/acquired immune deficiency syndrome patients: report of eight cases.

PURPOSE: This article describes the clinical features and management of noma (cancrum oris) in HIV/AIDS patients. PATIENTS AND METHODS: Eight HIV/AIDS patients were studied. Clinical presentation was different in all of the cases. Conservative debridement under local anesthesia with an antibiotic was performed. RESULTS: Satisfactory resolution of the acute phase of this dangerous infection was achieved. CONCLUSION: Minimal but thorough debridement of necrotic tissue along with lavage with povidone-iodine is effective in controlling this infective process. However, long-term sequelae of noma are not possible because of the underlying HIV infection from which the patients inevitably soon succumbs.

Adult

Cancrum oris (noma) in children.

Cancrum oris, noma or gangrenous stomatitis is a disease which affects primarily undernourished and immunosuppressed young children. Frequent in underdeveloped countries, it also is seen in rare cases of patients with AIDS and leukemia in America and in Europe. Once fatal, the disease is now better understood and today the repair of its terrible sequels is looked upon as a great surgical challenge. This paper reports a case of noma in a 3-year-old black African female admitted to this Service. In an already advanced stage of this illness with severe sequelae, she presented with partial amputation of the lips (upper and lower), right cheek, right side of the nose and maxilla. The choice of treatment of the infected area and eventual reconstruction is discussed.

Child, Preschool

Bacterial bone resorption in noma (gangrenous stomatitis).

Bone resorption (gangrenous stomatitis) was found to be associated with extensive bacterial colonization of mandibular bone in three separate cases of noma. Light and electron microscopy revealed a heterogeneous bacterial population with a long, filamentous, Gram-positive organism predominantly in direct contact with the resorbing bone front. The bone was completely denuded and no osteoclastic activity was observed. The highly regular arrangement of the filamentous organism along the resorbing bone and the absence of osteoclastic activity suggested that bone resorption in noma is mediated by bacterial action.

Bacteria

[Preventive surgery of the sequellae of noma].

Preventive surgery for the sequelae of noma aims to accelerate the healing phase of the progressive period of noma (basal stage) and also prepares a good bed for future reconstructive surgery for loss of substance. Certainly such surgery has its limits, however, excellent results are obtained when it is correctly employed. In fact it allows the patient to avoid certain serious complications such as loss of mandibular continuity due to infection. This is confirmed by the two cases presented.

Child, Preschool

Noma. Report of two cases.

Noma is an infectious process involving the oral cavity and occasionally other structures. It is characteristically a childhood affliction, and it never occurs in healthy persons. Conservative treatment in debriding facial lesions greatly enhances reconstructive efforts.

Child, Preschool

Noma in children with severe combined immunodeficiency.

Three Native American children with severe combined immunodeficiency developed noma, a necrotizing gingivostomatitis not previously reported in this country. The similarity between the clinical findings and those observed in monkeys with simian AIDS prompted us to evaluate our patients and their families for human retroviral infection. Antibodies to HTLV-I or HTLV-III/LAV proteins were not identified in patients nor in their family members. Standard bacterial and viral cultures similarly failed to identify a suspect pathogen.

Antibodies, Viral

Neonatal noma.

Neonatal noma is a rare clinical syndrome affecting term and preterm infants. It causes gangrene of the orofacial tissues accompanied by sepsis and a high mortality rate. A preterm 35-week infant, severely growth retarded in utero, developed clinical signs of sepsis with ulcers all over the oral mucosa caused by local infection with Pseudomonas aeruginosa. The lesions healed with cicatricial sequelae of the mouth, sequestration of teeth, and retraction of perimandibular soft tissues.

Female