Retrospective Characterization of Noma Cases Found Incidentally across Nigeria during Outreach Programs for Cleft Lip from 2011–2020

ABSTRACT.

Reports of cases of noma in Nigeria remain scarce despite its known and devastating effects on victims. This report presents a retrospective cross-sectional study based on data regarding on patients with noma encountered incidentally during Oral Health Advocacy Initiative outreach on orofacial diseases across 34 states and the Federal Capital Territory in Nigeria over 10 years (2011–2020), which was aimed at contributing to an understanding of the epidemiology of noma in Nigeria. The data were collated and analyzed, and are presented in frequency distribution tables and charts. A total of 7,195 patients with noma were encountered. The northeastern region had the greatest number of patients (n = 1,785, 24.8%) whereas the southwestern region had the least (n = 196, 2.7%). When aggregated by state, Ondo State had the least number of patients (n = 31, 0.4%) whereas Kano State had the greatest (n = 623, 8.7%). Patient age ranged from 3 to 70 years, with a slight male preponderance (56.9%). This report highlights the fact that noma is prevalent in Nigeria but remains neglected, with extensive but preventable physical, emotional, and social debilitation and devastation of the victims across all age groups. There is a need for a more robust survey to determine the true burden of the disease. There is also an urgent need for collaboration between governments and nongovernmental organizations to institute appropriate interventions by way of public education and enlightenment, as well as case detection and early treatment to mitigate the devastating consequences of delayed or poorly managed cases.

INTRODUCTION

Noma, otherwise called stomatitis gangrenosa or cancrum oris, is a debilitating infectious disease that attacks the orofacial complex, destroying the oral and para-oral structures, and resulting in dehumanizing orofacial gangrene, which may involve the mandible, maxilla, and occasionally the nose, extending to the infraorbital margins.^1,2 Etymologically, noma has its origin from the Greek word “nomein,” meaning “to devour,” and was first reported by Hippocrates in about 5th century bc.³ Although noma is considered an infectious disease, the exact causative microorganisms remain unclear.^2–4

The disease affects all ages, but mostly children 2 to 6 years of age. Those who survive have severe facial disfigurements and multiple functional impairments, including difficulties eating, seeing, and breathing, which contributes to stigmatization.⁵ The disease progresses through unique clinical stages, beginning at the reversible and seemingly inconsequential necrotizing gingivitis/edema stages to the grotesque gangrenous stage associated with extensive soft and hard tissue necrosis, often within a week.^1,2 Noma is classified into six stages: stage 0, simple gingivitis; stage 1, acute necrotizing ulcerative gingivitis; stage 2, edema; stage 3, gangrene; stage 4, scarring; and stage 5, sequelae.⁶

Noma is an opportunistic infection associated with an imbalance of microorganisms of the oral flora.⁷ Studies have associated noma with poverty and the impoverished regions of the world, especially sub-Saharan Africa, informing the name noma belt region.² Although this disease has been present in Europe since ancient times, it is now reported mainly in Africa and occasionally in Latin America and Asia.^1,3 With improvements in hygiene and nutritional status, and the decline in measles outbreaks and other eruptive fevers, noma disappeared from more developed countries,^8,9 but reappeared during World War II in concentration camps and in the Netherlands, where the population suffered from real famine in the winter of 1944 and 1945.⁹

Without treatment, approximately 90% of patients with noma die within 1 or 2 weeks because of sepsis.¹⁰ The small percentage of survivors generally suffers from severe sequelae, such as facial mutilation, trismus, oral incontinence, and speech problems.¹¹ Treatment with antibiotics, wound debridement, and nutritional support in patients seen early will greatly reduce mortality and morbidity.⁵ Noma can become inactive with or without treatment, and patients can survive into adulthood, but often require extensive reconstructive surgery and physiotherapy to ameliorate the resulting defects and improve function.^6,12

Epidemiologically, the true global incidence of noma is unknown. However, alarming reports have been published in extant literature by Bourgeois and Leclercq¹⁰ and Farley et al.,¹² indicating that the magnitude of the noma problem in the world may not yet have been recognized sufficiently. This challenge is still unabated. The explanation is that noma occurs among the poorest in the poorest societies, who do not have access to proper health care and adequate nutrition.

Research targeted at determining the incidence and prevalence of noma constitutes a central feature of public health action against the disease.² Reports estimate a range from 30,000 to 140,000 individuals as the annual global incidence.^7,11,13 A few studies and reports in Nigeria have shown varied incidence and prevalence of noma in the past two decades, including the three patients in 10 years reported by Oji¹⁴ in Enugu, a prevalence of 6.4 per 1,000 population reported from a study in the northeast by Fieger et al.,¹¹ and a prevalence of 7 per 1,000 from a hospital-based study in Ibadan by Denloye et al.¹⁵ Bello et al.² reported a prevalence rate of 8.3 to 100,000 (0.083 per 1,000 population) from their outreach in the north–central zone of the country, whereas Farley et al.¹² documented a 3.3% prevalence rate from another study in northeastern Nigeria.

The Oral Health Advocacy Initiative (OHAI) has been carrying out outreach programs across the nation from 2011 to date to identify and correct cleft lip and palate malformations surgically. Advertisements, community mobilization, and invitations for free surgical correction for individuals with cleft lip and palate attracted patients with noma for treatment. This report describes the findings from such patients with noma, spanning a period of 10 years (2011–2020).

METHODS

This is a retrospective cross-sectional study based on data on patients with noma encountered incidentally during OHAI outreach on orofacial disease across 34 states and the Federal Capital Territory in Nigeria for 10 years (2011–2020). These patients presented at OHAI outreach camps across the country. The data were collected at the state level and pooled to form the subnational data set used.

Inclusion and exclusion criteria were used to define patients used for the report. The inclusion criteria included patients with orofacial infection with orofacial deformity that was not present at birth and patients with established orofacial deformities (sequelae) that started with an infective process. Exclusion criteria were as follows: orofacial deformities present at birth or a result of other noninfective causes, such as trauma and cleft lip and palate, and orofacial infections without any deformities.

For clinical diagnosis, basically, patients must not have been born with the facial manifestation that would have started as an infection. Patients were categorized based on the location of the lesion: the left cheek, left angle, left cheek and angle, right cheek, right cheek and angle, right angle, upper lip, and lower lip. Other information recorded include patients’ biographical data, whether they were able to open their mouth, immunization history, and tooth brushing/cleansing habits. Patients’ level of oral health education, knowledge of nutrition, information on feeding habits, onset of lesion, and experiences preceding and succeeding the lesion occurrence were also documented.

Data were analyzed using the IBM Statistical Package for the Social Sciences (version 25; (IBM Corp., Armonk, NY). The results are presented as frequency distribution tables and charts.

Informed consent was obtained from participants and ethical clearance was obtained from the National Hospital Abuja Ethics Committee (reference no. NHA/EC/084/2021).

RESULTS

Demographic characteristics of subjects.

A total of 7,195 patients with noma were encountered across 34 states and the Federal Capital Territory of Nigeria (see Supplemental Material Appendix S1). As shown in Table 1, patient age ranged from 3 to 70 years; 1,186 (16.5%) were younger than 10 years, 1,796 (25%) were 10 to 30 years, 2,853 (39.7%) were 31 to 50 years, and 1,360 (18.9%) were 50 years or older. There was a slight male predominance (n = 4,094, 56·9%), 4,460 (62%) were farmers, 3,488 (48.5%) had no education at all, 2,408 (33.4%) had completed the primary level of education, and 131 (1.8%) attained the tertiary level of education.

Table 1.

Demographic characteristics of patients with noma

Characteristic	n	%
Gender
Male	4,094	56.9
Female	3,101	43.1
Occupation
Farming	4,460	62.0
Civil servant	140	1.9
Student	605	8.4
Housewife	917	12.7
Pupil	856	11.9
Other	217	3.0
Education
None	3,488	48.5
Koranic	319	4.4
Primary	2,408	33.5
Secondary	849	11.8
Tertiary	131	1.8
Age, years
< 10	1,186	16.5
10–30	1,796	25.0
31–50	2,853	39.7
> 50	1,360	18.9
Total	7,195	100.0

Sites of tissue lesions.

The soft tissue lesions involved multiple sites. As shown in Figure 1, the right cheek and angle were affected in 1,909 patients (26.5%), the left angle in 1,052 (15.6%), and the left cheek in 1,045 (14.5%). The upper lip was involved in 784 patients (10.9%) and the lower lip in 721 (10%). A total of 5,979 (83.1%) patients were still able to open their mouth despite the lesions, whereas 1,216 (16.9%) were affected by trismus.

Figure 1.

Frequency of location of lesions in the orofacial region.

Table 2 shows that 5,723 patients (79.5%) reported not having any oral health education; 2,867 patients (39.8%) practice oral hygiene by rinsing their mouth with water and using their fingers; 1,279 patients (17.8%) rinse their mouth with just water; 1,352 patients (18.8%) use a toothbrush and toothpaste; 1,348 patients (18.8%) use a chewing stick, charcoal, or salt; and 349 patients (4.9%) reported no form of mouth cleaning. With regard to regularity or oral care, 3,659 patients (50.9%) cleaned their mouth occasionally, 1,338 (18.6%) cleaned their mouth once a day, and 2,198 (30.5%) cleaned their mouth twice a day.

Table 2.

Oral health hygiene and education

Oral health hygiene and education	n	%
Any oral health education
Yes	1,412	19.6
No	5,723	79.5
Prefer not to say	60	0.8
Mouth-cleaning habits
Rinses mouth using water	1,279	17.8
Rinses mouth using water and fingers	2,867	39.8
Cleans mouth using chewing stick	753	10.5
Uses toothbrush and toothpaste	1,352	18.8
Uses charcoal	394	5.5
Uses salt	201	2.8
Does not clean mouth	349	4.9
Regularity of mouth cleaning
Occasional	3,659	50.9
Once a day	1,338	18.6
Twice a day	1,849	25.6
Does not clean mouth	349	4.9
Total	7,195	100.0

A total of 6,585 patients (91.5%) have had no education in feeding and nutrition. A total of 6,537 (90.9%) consume carbohydrate-based foods more often (Table 3), and 322 (4.5%) and 336 (4.7%) reported consuming proteins and fats, respectively. Although 3,791 patients (52.7%) eat as often as food is available, 919 (12.8%) could afford only one or two meals a day.

Table 3.

Feeding and nutritional behavioral pattern of patients with noma

Behavioral Pattern	n	%
Any education in feeding and nutrition
Yes	610	8.5
No	6,585	91.5
Food mostly eaten
Carbohydrates	6,537	90.9
Proteins	322	4.5
Fats	336	4.7
How often food is eaten
Once a day	119	1.7
Twice a day	800	11.1
Thrice a day	2,457	34.1
As often as food is available	3,791	52.7
Prefer not to say	28	0.4
Total	7,195	100.0

Reactions after lesion occurrence.

Approximately 70% of the lesions started before the age of 8 years, as shown in Figure 2, whereas 10% started after the age of 16.

Figure 2.

Age when lesion started.

Table 4 shows that 2,832 complications (39.4%) started as a boil, 1,809 (25.1%) as swelling, 1,712 (23.8%) as itching around the area of the lesion, and 748 (10.4%) as bleeding from the gums. Most patients embarked on self-treatment; 3,011 (41.8%) resorted to home treatment with charcoal, 951 (13.2%) opted for traditional treatment with a sacrifice, 536 (7.4%) visited patent medicine stores, 1,364 (19.0%) visited a hospital, and 1,333 (18.5%) sought no treatment. Of the 1,364 patients who sought care at a hospital, 649 (47.6%) did so within the first 3 weeks of the lesion, 239 (17.5%) within a month or two, 53 (3.9%) waited for as long as 3 months before visiting the hospital, and 253 (18.5%) waited up to 1 year before visiting a hospital.

Table 4.

Circumstances surrounding lesion occurrence

Circumstance	n	%
How lesion started
Boil	2,832	39.4
Itching	1,712	23.8
Swelling	1,809	25.1
Bleeding from gums	748	10.4
Measles	94	1.3
First reaction after lesion discovery
Home treatment with charcoal	3,011	41.8
Traditional treatment with sacrifice	951	13.2
Went to hospital	1,364	19.0
Visited patent medicine store for medication	536	7.4
No treatment	1,333	18.5
How long it took to visit a hospital
1–3 weeks	649	47.6
1–2 months	239	17.5
3 months	53	3.9
> 3 months	130	9.5
1 year	253	18.5
> 1 year	40	2.9
What did the hospital do for you?
I was treated	942	70.6
I was referred to another hospital	343	25.9
Nothing	41	3.5
Immunization history
Incomplete immunization	3,007	41.8
Doesn’t know	3,074	42.7
Completed immunization schedule	611	8.5
Refused it	503	7.0
Total	7,195	100.0

DISCUSSIONS

The total number of patients with noma found incidentally suggests the existence of a high burden of the disease across the country. This is so because the report focused only on complicated cases that were easily diagnosed clinically. When the uncomplicated or early and fatal manifestations are considered, the burden could indeed be worrisome. Noma is highly preventable, and the progression to complications and death is reversible, making the disease easily amenable to remedy. An early intervention could have prevented the numerous manifestation of trismus, and respiratory and feeding/nutritional challenges faced by some of the patients.

The majority of cases of noma began in children younger than 8 years old. This places the burden of care on parents. The low level of education of the patients coupled with extreme poverty, poor nutrition, and poor personal and environmental hygiene magnifies the risk of acquisition of the disease.^2,5,16–19 The settings in which the patients with noma were found demonstrates the association of the disease with poor nutritional status, poverty, low levels of education, and poor oral and environmental hygiene, as established in previous studies.^2,5,16–19 Most of the patients affected consumed predominantly carbohydrate-based food, suggesting that most experience protein malnutrition, which ultimately lowers the immune status.^5,7,16,20,21 In the context of poor oral hygiene practices, there could be proliferation of oral flora, which may capitalize on the opportunities of likely mucosal abrasions resulting from inflammation and use of such materials as coal to invade the tissues of the mouth and set up rapidly spreading necrotic lesions. Although the true pathogens associated with noma remain unknown, it is most likely that members of the oral flora play significant roles in the pathogenesis of the disease. A properly designed study is needed to decipher the true pathogens.

The poor health-seeking behavior of the communities accentuates the impact of the disease. Inappropriate remedies were tried, and many of them were the result of poverty and lack of access to health-care facilities. Many of the early manifestations of noma, as seen in our study, are as common as many other local ailments that are often ignored or amenable to local remedies, and may explain in part the delay in seeking appropriate health-care services.

This study has revealed the existence and neglect of a highly preventable but debilitating and often fatal disease. It has added to the stock of existing but scarce data on noma, which were mainly from hospital-based or subregional studies. There is need for policymakers, nongovernmental organizations and governments to give attention to this disease by carrying out further studies to determine the actual burden and etiology of the disease, as well as to institute preventive measures such as education, nutritional support, and access to health care for early detection and treatment.

CONCLUSION

This is the first near-countrywide epidemiological report on noma, and it has revealed that noma is indeed very prevalent in Nigeria. We recommend a multifaceted approach toward tackling the scourge of this disease in our country by addressing the multiple problems of poor oral health hygiene, malnutrition, poverty, lack of access to health-care services, and poor personal and environment hygiene. Awareness should be created regarding the early manifestations of the disease and the appropriate healthy actions to be taken. Education of health workers in existing health facilities on disease identification is critical. This will facilitate proper diagnosis and referral to appropriate centers that specialize in noma treatment.

Note: Supplemental Appendix S1 appears at www.ajtmh.org.