Seminar Noma (cancrum Oris)

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SeminarNoma (cancrum oris)Cyril O Enwonwu, William A Falkler Jr, Reshma S PhillipsNoma is an opportunistic infection promoted by extreme poverty. It evolves rapidly from a gingival inflammation togrotesque orofacial gangrene. It occurs worldwide, but is most common in sub-Saharan Africa. The peak incidence ofacute noma is at ages 1–4 years, coinciding with the period of linear growth retardation in deprived children. Noma isa scourge in communities with poor environmental sanitation. It results from complex interactions betweenmalnutrition, infections, and compromised immunity. Diseases that commonly precede noma include measles,malaria, severe diarrhoea, and necrotising ulcerative gingivitis. The acute stage responds readily to antibiotictreatment. The sequelae after healing include variable functional and aesthetic impairments, which requirereconstructive surgery. Noma can be prevented through promotion of national awareness of the disease, povertyreduction, improved nutrition, promotion of exclusive breastfeeding in the first 3–6 months of life, optimum prenatalcare, and timely immunisations against the common childhood diseases.The WHO International Statistical Classification ofDiseases, code A69.0 lists necrotising ulcerative stomatitis,which includes noma, cancrum oris, and fusospirochaetalgangrene. Noma is derived from the Greek voμη , whichmeans to graze or to devour.1,2 Orofacial noma is aninfectious disease that starts as gingival ulceration andspreads rapidly through the orofacial tissues, establishingitself with a well-demarcated perimeter surrounding ablackened necrotic centre (figure 1).1,3,4 The gangrene caninvolve not only the mandible and maxilla but also thenose and infraorbital margins (figure 2). Unlike otherinfectious processes of the face, which mostly expandalong cellular spaces of the head and neck, the nomalesion spreads through anatomical barriers such asmuscles.5 Names for the disease include cam-tan-ma(oral inflammation like a galloping horse) in Vietnam2and ciwon iska (the wind disease), which metaphoricallyunderscores its rapid development, among the Hausatribe in Nigeria. In 1848, Tourdes6 described orofacialnoma as a “gangrenous affection of the mouth, especiallyattacking children in whom the constitution is altered bybad hygiene and serious illness, especially from theeruptive fevers, beginning as an ulcer of the mucousmembrane with oedema of the face, extending fromwithin out, rapidly destroying the soft parts and thebone, and almost always quickly fatal”. This descriptionis still accurate, except for the reduction in mortality ratewith prompt treatment.1,7–9Acute [A: OK?] noma is seen predominantly in childrenaged 1–4 years, although late stages can occur inadolescents and adults.1,2,10,11 The WHO designates nomaa health priority.12 There have been several recent reviewson noma,2,13–16 and some2,14 provide detailed informationon the history of the disease dating back to classical andmedieval civilisations in Europe. Surgical repairprocedures for the sequelae of noma have also beenreviewed lately.2,14,17 This Seminar therefore focusesprimarily on acute noma.Lancet 2006; 367: pg–pgDepartment of BiomedicalSciences, School of Dentistry(Prof C O Enwonwu ScD,W A Falkler Jr PhD,R S Phillips PhD), andDepartment of Biochemistryand Molecular Biology, Schoolof Medicine(Prof C O Enwonwu), Universityof Maryland, Baltimore, MD,USA [A: we give only onedegree per author; are thoseselected OK? Are any of theother authors full prfoessors?]Correspondence to:Prof Cyril O Enwonwu,Department of BiomedicalSciences, University of Maryland,666 West Baltimore Street,Baltimore, MD 21201, USAcenwonwu@umaryland.eduSearch strategy and selection criteriaFigure 1: A 3-year-old malnourished girl with acute orofacial noma beforeremoval of the tissue sloughThe lesion has a well-demarcated perimeter surrounding a blackened necroticcenter. Courtesy Noma Children Hospital, Sokoto, Vol 367 Month xx, 2006We did a comprehensive search of scientific publications including databases OLDMEDLINE via OVID (1951–65), MEDLINE via PubMed (1950–2005), and ISI web ofscience. The search terms used were “noma”, “cancrum oris”, “oral gangrene”, “orofacialnecrosis”, “necrotiz[s]ing ulcerative gingivitis”, and “noma, malnutrition AND oralhealth”, “noma AND measles”, “noma AND viral infections”, and “noma AND HIV/AIDS”.Many published reports on noma were also identified through searches of COE’sextensive records collected over the past four decades. The historical references werelimited to those retrievable by a MEDLINE search, with noma, cancrum oris, or both asthe key words. Reviews by Tempest1 and Marck2 were guides to historical descriptions ofnoma in earlier centuries. Only reports published in English, French, or German werereviewed.1

SeminarFigure 2: A malnourished boy aged 2·2 years with a noma lesion involving the orofacial tissues, lips, and nose,and extending to the infraorbital marginGlobal burden of nomaNomawas not always restricted to tropical or Africancountries.2,10 It was common in Europe until the end ofthe 19th century.1,2 Noma disappeared from moredeveloped countries in the 20th century, except for casesreported in the concentration camps of Bergen-Belsenand Auschwitz18,19 and, more recently, in association withintense immunosuppressive therapy20 in patients withHIV infection or AIDS,8,9 as well as in native Americanchildren with severe combined immunodeficiencysyndrome.21 The disease almost disappeared in moredeveloped countries with improvements in the standardof living, even before the discovery of penicillin.2,3,13 Bycontrast, noma has remained an important healthproblem of deprived children in sub-Saharan Africa.10,22,23Most of the cases in Africa occur in a belt stretchingacross western and central Africa towards Sudan.10,12,14Several countries in this hot, arid zone are characterisedby mass poverty and frequent famines. In Nigeria1,3,24and Senegal,23 for example, a few specific regionsaccount for most of the cases of noma in those countries.WHO has prepared a global map of reported casesduring the period before 1980 and up to 2000 (figure 3),showing that although most of the countries affectedare in Africa, Asia and Latin America are alsoinvolved.2,10There is a severe lack of global data on acute noma inchildren. The major obstacles have been extensivelydocumented.10,13,23 In 1998, WHO estimated thatworldwide 140 000 children contract noma each year,and 79% of them die from the disease and associatedcomplications.25 In 2003, Fieger and colleagues26estimated an annual incidence of 6·4 cases per1000 children in north-west Nigeria and, by extrapolation,an incidence of 25 600 for the countries bordering theSahara. In specific African countries such as Senegal,23Gambia,27 and Niger,10 the annual incidences derivedfrom hospital records are 0·28–0·84, 1·9, and0·7–1·4 per 1000 children, respectively. These ratesCases reported before 1980Cases reported 1981–1993Cases reported 1994–2000Sporadic cases recently reportedFigure 3: Worldwide distribution of reported cases of nomaAdapted from WHO. Before 1980, many less developed countries, particularly in sub-Saharan Africa, had poor noma reporting systems. Since the 1990s, awarenessof noma has increased in these countries, and some have established control Vol 367 Month xx, 2006

Seminarrepresent the tip of the iceberg, because no more than10% of affected children seek medical care during theacute stage.7,16Over the years, acute noma was known as a disease ofdeprived children [A: since we explain in the nextsentence that the disease has now appeared in HIVpositive children, we think the part of the sentence aboutnon-immunocompromised was superfluous andpotentially confusing.].1,3,11,24 More recently, there havebeen sporadic reports of acute noma-like diseaseoccurring in HIV-positive individuals.8,9,28,29 Between 1989and 1993, the HIV status of 26 of the 45 children youngerthan 3 years admitted to the University of ZambiaHospital for treatment of noma was ascertained; nine(35% [A: we round percentages up or down if thedenominator is less than 100 and especially when talkingabout people]) were seropositive.9 Noma is not listedamong the clinical syndromes associated with HIV/AIDSin children.30,31 Costini and colleagues32 expressed concernthat the AIDS epidemic could increase the number ofnoma cases. There are large subregional differences inthe prevalence of HIV infection in sub-Saharan Africa;southern and eastern Africa have much higher rates thanwestern Africa.33 Nonetheless, the reported incidence ofnoma is higher in western Africa.2,4,34,35Neighbourhood villagechildren (n 55)Mean (SD) age, years2·40 (1·28)Noma group(n 58)p2·58 (1·02)Height for age Z scoreMean (SD)–1·43 (2·22)–3·82 (2·16) 0·001Number (%) –2·0 SD or more20 (37%)53 (91%) 0·001Number (%) –3·0 SD or more7 (13%)41 (70%) 0·001Number (%) –4·0 SD or more4 (8%)25 (43%) 0·001Weight for age Z scoreMean (SD)–1·87 (1·67)–3·65 (1·82) 0·001Number (%) –2·0 SD or more26 (47%)51 (88%) 0·001Number (%) –3·0 SD or more12 (21%)47 (81%) 0·005Number (%) –4·0 SD or more5 (10%)27 (47%) 0·05Adapted from Enwonwu and colleagues with permission. The neighbourhood village children without noma include siblings ofthose with noma.11Table 1: Anthropometric data for Nigerian children [A: we prefer to give exact p values rather than 0·?;please can you give these? Also, we like to give actual numbers with percentages; please check.]Clinical presentation, progression, and sequelaeMany patients with acute noma present with a range offeatures reflecting pre-existing, debilitating –40·5 C), tachycardia, high respiratory rate, andanorexia. The medical history generally shows recurrentfevers, diarrhoea, infections with parasites (eg, malaria)and viruses (eg, measles, herpes) in the recent past.1,2,3,5Severe anaemia, with haemoglobin concentrations as lowas 50–60 g/L, white-blood-cell counts of 20–30 10⁹ per L[A: we give cell counts per litre; please check conversions],and hypoalbuminaemia are common.3,24,36,37 Serumconcentrations of antioxidant micronutrients are verylow, consistent with severe malnutrition and presence ofinfections.3,37 Studies in Nigeria have shown that serumconcentrations of the proinflammatory and antiinflammatory cytokines are higher in children with acutenoma than in healthy urban children of similar age, butthere is less difference between affected children andtheir malnourished neighbourhood counterparts withoutnoma.37 The most prominent feature of children withacute noma is growth retardation (table 1), and many areseverely or critically affected.11,38Orofacial featuresThe orofacial lesion can occur unilaterally or bilaterally,but it is unilateral in many cases. Descriptions of theinitial stages are inconsistent because the disease isgenerally well established before the victim seeks medicalhelp.1,2,16 The early features include soreness of the mouth,pronounced halitosis, foetid taste, tenderness of the Vol 367 Month xx, 2006Figure 4: A 2-year-old deprived girl of 5 kg in weight and 73 cm in heightThe child presented with facial swelling, anaemia, angular cheilosis, skin lesions particularly around the nose, andprominent malodorous breath. Intraoral examination revealed necrotising ulcerative gingivitis involving theposterior right quadrant of the maxillaor cheek, cervical lymphadenopathy, a foul-smellingpurulent oral discharge, and a blue-black discoloration ofthe skin in the affected area.1,3,5,7 The face on the affectedside is swollen in most cases (figure 4). There is generalconsensus that noma starts as gingivitis, most commonlyin the premolar to molar and mandibular incisor regions,extends to the labiogingival fold and on to the mucosalsurface of the cheek and lip.1,3,14,23 Necrotising ulcerativegingivitis, a painful inflammation of the marginalinterdental papillae, has long been thought to be theprecursor of noma,3,39–41 but this view is now disputed.4,22,28This disorder predominantly affects deprived Africanchildren, and has a peak age incidence corresponding tothat of acute noma.3,41,42 Emslie40 stated that noma is anextension of necrotising ulcerative gingivitis; to3

SeminarFigure 5: A Nigerian child with necrotising ulcerative gingivitis involving themaxillary right quadrant with simultaneous ulceration of the adjacent cheekReprinted with permission from Enwonwu.3differentiate between the two, he chose alveolar boneexposure as the transition point, a view shared byothers.43,44 However, infection with the measles virus1,3,36and other viruses might initiate noma.8,45 Postmeaslesnecrotic lesions of the mouth occur in malnourishedchildren.36,39,46 When the inflammation simultaneouslyinvolves the gingivae and the mucosal surface of theadjacent cheek (figure 5), further progression leading toperforation of the cheek is rapid, in a matter of days inmany cases.1,3,5,7 Generally, the external tissue loss is notclosely related to the more extensive intraoraldestruction.7,9 Sequestration of the exposed bone andteeth occurs spontaneously after separation of the softtissue slough. In some cases, debridement of the woundis necessary to prevent secondary infection and promotethe healing process.9 The loss of orofacial tissues isdiverse, varying from a small area (figure 6) to moreextensive destruction (figure 7) of the nose, upper lip,and premaxilla, and the infraorbital margin.2,9The sequelae of acute noma depend largely on the sitesaffected, the extent and severity of tissue destruction, andthe stage of development of the orofacial complex beforethe onset.1,7 They can include displacement of the teeth,disfiguring, intense scarring, bony fusion between themaxilla and mandible, trismus, defective speech, andnasal regurgitation if the maxilla is lost. Thus, survivorsof the acute phase have the two-fold problems ofdisfigurement and functional impairment, as well as theattendant psychological trauma. Details of these andtheir surgical management are described elsewhere.2,14,17Differential diagnosis of nomaFigure 6: Small orofacial noma destruction involving the mandible in amalnourished child aged 2·5 yearsCourtesy Noma Children Hospital, Sokoto, Nigeria.Figure 7: A 2-year-old child after removal of tissue slough and bonesequestration, showing extensive intraoral destruction4The disorder known as noma neonatorum affectsnewborn and preterm infants and clinically resemblesnoma in children.47–49 The necrotic lesion, generally in theoronasal region, develops during the first month of life;in most cases, there is evidence of infection withPseudomonas aeruginosa, Escherichia coli, klebsiella, orstaphylococci.47,48 Except for one case in a preterm baby inthe USA,50 virtually all the reported cases hav been ininfants born in India, China, Lebanon, or Israel.51–53Preterm birth and severe intrauterine growth retardation(IUGR) are important predisposing factors.48 Otherulcerative lesions to be considered in the differentialdiagnosis of noma include leishmaniasis, agranulocyticangina, malignant oral lesions, midline granuloma of theface, and syphilis, but most of these are rare in childrenaged 2–5 years.1 Skin lesions associated with ecthymagangrenosum occur predominantly in the perineal areaand the limbs, with rare facial involvement.54 Extensiveand disfiguring destruction of the mucous membranesof the nose, mouth, and throat can occur inmucocutaneous leishmaniasis, but 90% of cases occur inBolivia, Brazil, and Peru.64 [A: reference numbering goesfrom 54 to 64; please indicate where references 55 to 63should be cited. Some of them are cited later, but theorder is incorrect if those are the first citations.] Vol 367 Month xx, 2006

Seminarulcer resulting from infection with Mycobacteriumulcerans, preferentially affects the limbs, particularly thelegs.65 Necrotising diseases of the periodontiumassociated with HIV infection66 can resemble the earlyintraoral signs of noma. Serological testing for HIVinfection should be done in these patients. In thediagnosis of the early stages of noma, history of a recentexanthematous fever or debilitating disease, oral mucosalulcer with bone exposure, excessive salivation,malodorous breath, and severe stunting or wasting in adeprived child are important warning signs. Diagnosis ofa well-established case of noma in children is notdifficult.1,3Isolates/number of patients sampledFusobacterium necrophorum7/8Prevotella intermedia6/8α streptococci4/8Actinomyces spp3/8Peptostreptococcus micros1/8Veillonella parvula1/8Pseudomonas spp1/8Staphylococcus aureus1/8Details of the sampling and culture procedures are given in Falkler and colleagues.65Table 2: Microorganisms recovered from noma lesions in NigerianchildrenMicrobiologyThe earliest bacteriological studies of noma weredescribed by Weaver and Tunnicliff in 1907.57 An importantmajor microscopic observation is the presence of largenumbers of fusiform bacilli and spirochaetes.1,16,57 Hickenand Eldredge58 suggested that a symbiotic association offusiform bacilli with a non-haemolytic streptococcus andStaphylococcus aureus was needed to produce noma.Emslie40 observed that these organisms were predominantin smears from patients with acute noma but alsoreported presence of other organisms. Macdonald59studied cultures from patients with noma in Nigeria andreported that Bacteroides melaninogenicus might be animportant associated microorganism in mixed infectionsof mucous membranes.Early studies of necrotising ulcerative gingivitis, aputative precursor of noma,3,40,41 incriminated spirochaetes,fusiform bacteria and Prevotella intermedia as potentialcausative agents.60,61 Some reports have associatednecrotising ulcerative gingivitis, noma, or both withhuman cytomegalovirus,62 measles virus,3,40 herpessimplex virus,40,46 and other unspecified viruses. PCRstudies of herpesviruses in 22 Nigerian children withnecrotising ulcerative gingivitis showed that 15 (68%)had viral infection and eight (36%) had viral coinfection[A: please check calculations of absolute numbers.].45Human cytomegalovirus infection (59%) was the mostcommon. The findings suggested that this virus andpossibly other herpesviruses contribute to the onset orprogression of necrotising ulcerative gingivitis inmalnourished Nigerian children. Microbiologicalsamples from the same children were culturedanaerobically on selective media.63 The a,Fusobacterium nucleatum, Peptostreptococcus micros,campylobacter, streptococci, and enteric gram-negativerods; these findings did not differ from those in thehealthy sites of malnourished children withoutnecrotising ulcerative gingivitis. More anaerobes,particularly Prevotella intermedia, were present in themouths of the malnourished than of the healthy children.These findings suggested that malnourished childrenhave a different flora from healthy children, as Vol 367 Month xx, 2006reported by Sawyer and colleagues.64 The presence ofsimilar organisms in malnourished children with orwithout necrotising ulcerative gingivitis also suggestedthat some other organisms or factors might lead to thedevelopment of noma from this putative precursorlesion.Table 2 summarises the microorganisms recoveredfrom the active sites of noma lesions in Nigerian children;Fusobacterium necrophorum was the most commonlyisolated.65 [A: no need to repeat data from table; thepercentages aren’t really necessary with a denominatorof 8.] Actinomyces, veillonella, and α-streptococci arenormal oral flora. Most of the other microorganisms(staphylococci, pseudomonas) isolated from one or a fewcases have previously been associated with nomalesions.40 Prevotella intermedia is involved in periodontaldiseases66,67 and has also been identified as a putativepathogen in necrotising ulcerative gingivitis in youngadults.61 This microorgan

which includes noma, cancrum oris, and fusospirochaetal gangrene. Noma is derived from the Greek voμη , which means to graze or to devour.1,2 Orofacial noma is an infectious disease that starts as gingival ulceration and spreads rapidly through the orofacial tissues, establish

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