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 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 3  |  Issue : 3  |  Page : 158-161

Simultaneous occurrence of noma in identical twin brothers: Possibility of a genetic basis


1 Department of Dental and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
2 Department of Surgery, Noma Children Hospital, Sokoto, Nigeria
3 Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Usmanu Danfodiyo University, Sokoto, Nigeria
4 Department of Oral and Maxillofacial Surgery, University of Maiduguri/University of Maiduguri Teaching Hospital, Maiduguri, Nigeria

Date of Submission10-Feb-2023
Date of Decision06-May-2023
Date of Acceptance12-May-2023
Date of Web Publication14-Aug-2023

Correspondence Address:
Dr. Mujtaba Bala
Department of Dental and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, PMB 2370, Sokoto
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_34_23

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  Abstract 


Background: Noma is a devastating orofacial gangrenous condition that may cause significant mutilation of the facial anatomy. The pathogenesis is poorly understood and has been reported in a setting of low socioeconomic and unhygienic conditions with associated malnutrition. We present this condition in identical twin brothers. Clinical Description: Two identical twin brothers presented with painful swelling of the cheek with ulcerative defects in their upper lips. Both belonged to an impoverished family, and were malnourished, unimmunized, with poor oral hygiene. The typical anatomical presentation of the lesions with the characteristic family setting made the diagnosis consistent with noma (cancrum oris). Other siblings living in the same family with similar background conditions were unaffected. Management: After clinical stabilization, definitive surgical care, including debridement and regular dressings was done under the cover of intravenous antibiotics. With aggressive management along with nutritional rehabilitation and oro-muscular physiotherapy, the acute phase subsided and the patients were discharged in the scarring phase, with a plan for surgical reconstruction of the defect at a later date. Conclusions: The simultaneous presentation of noma in identical twins, with the sparing of other siblings living in the same contributing circumstances, raises suspicion of a genetic basis for this condition, which is as yet unknown. As the etiopathogenesis of noma is evolving, further evidence is needed to establish a genetic predisposition.

Keywords: Children, necrotic, oro-facial gangrene, siblings


How to cite this article:
Bala M, Braimah RO, Bello AA, Taiwo AO, Suleiman IK. Simultaneous occurrence of noma in identical twin brothers: Possibility of a genetic basis. Indian Pediatr Case Rep 2023;3:158-61

How to cite this URL:
Bala M, Braimah RO, Bello AA, Taiwo AO, Suleiman IK. Simultaneous occurrence of noma in identical twin brothers: Possibility of a genetic basis. Indian Pediatr Case Rep [serial online] 2023 [cited 2023 Sep 26];3:158-61. Available from: http://www.ipcares.org/text.asp?2023/3/3/158/383626



Noma is a gangrenous infection, characterized by progressive necrotic ulceration and destruction of the oral and perioral tissue, involving the maxilla, mandible, and may extend to the nose and infraorbital margins, causing significant disfiguration of the facial anatomy.[1],[2],[3] It has been reported mainly from underdeveloped countries, mostly in children 2–5 years of age. Chronic malnutrition has been reported to be the most common predisposing factor,[4] with other factors being poor oral hygiene, comorbidities, poverty, and immunodeficiency.[2] The pathogenesis of noma is not fully understood.[5] There is limited information regarding the molecular and genetic basis of the condition. We present an interesting report of noma occurring simultaneously in two siblings who were identical twins.


  Clinical Description Top


Two 3-year-old boys, who were identical twins, were referred from a rural comprehensive health-care center to the Noma Childrens' Hospital, Sokoto, with chief complaints of painful swelling on the face for 12 days and 8 days, for Twin A and B, respectively. Both boys had been referred to our center after initial stabilization, intravenous (IV) antibiotics, and wound debridement.

The elder of the twins (Twin A) also had associated fever, difficulty in feeding, and bleeding from the gums. There was neither diarrhea nor any other boils/pyoderma elsewhere in the body. The younger twin (Twin B) presented with pain, cheek swelling, fever, and loss of appetite for the past 8 days, without any associated vomiting, bleeding gums, or any other boils/pyoderma anywhere else in the body.

In both children, there was no history of measles, varicella, or any other medical illness requiring hospitalization. The twins were third in birth order, born to a nonconsanguineously married couple. Delivery had been assisted by local birth attendants, with neither proper antenatal care nor any immediate natal examination, and hence no records of birth weight. As per the mother, the delivery was uneventful. Both twins had been breastfed from birth. As the mother had felt breast milk to be inadequate for two babies, so babies were supplemented with mainly millet and guinea corn drinks. The children had not been immunized. At presentation to us, both siblings were taking a diet consisting mainly of carbohydrates, with little or no protein.

The patients came from a family of eight individuals in a monogamous setting. They belonged to a low socioeconomic background, with the father being a small-scale farmer and the mother being a home maker. They lived in a house made from clay in a remote village. The eldest among the children was 7 years old and the youngest was 1 year and 3 months. There was neither history of skin diseases nor any dental problems among any of the siblings. The mother cleaned the children's teeth with charcoal. There was no history of tuberculosis or any other medical illness in the family or among the relatives.

On examination, Twin A was conscious and afebrile, with normal volume pulses of 114/min, temperature of 36.2°C, respiratory rate of 22/min, and SPO2 of 100%. He had a body weight of 7.2 kg (weight for age Z-score −3.9), a height of 75.6 cm (height for age Z-score −3.1), and weight for height z-score of −3.7. Mid-upper arm circumference was 10.5 cm. There was pallor but no cyanosis, icterus, or lymphadenopathy. There was pedal edema. The younger Twin B, on examination, was also found to be conscious and alert with a pulse of 106/min, temperature of 35.5°C, though the mother had given a history of previous fever, respiratory rate of 22/min, and SPO2 of 100%. He had a body weight of 8.5 kg (weight for age Z-score −3.0), a height of 78.7 cm (height for age Z-score −2.8), and a weight for height z-score of −2.2. Mid-upper arm circumference was 10.5 cm. There was pallor with signs of wasting but no cyanosis, icterus, lymphadenopathy, or edema.

While the elder twin had a small ulcerative defect involving the left side of the upper lip and mucosa, measuring 0.5 cm × 1 cm [Figure 1], the younger one showed a large ovoid ulcerative defect involving the right side of the upper lip and cheek measuring 3 cm × 4 cm with sequestration of maxillary teeth and alveolar bone [Figure 2]. In both children, all deciduous teeth were present, with poor oral hygiene and gingivitis, but there was no glossitis or cheilitis. Systemic examination did not reveal any abnormality in either of the twins.
Figure 1: Noma lesion in Twin A with a small ulcerative defect involving the left side of the upper lip and mucosa (0.5 cm × 1 cm)

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Figure 2: Noma lesion in Twin B with large ovoid ulcerative defect involving the right side of the upper lip and cheek (3 cm × 4 cm) with sequestration of maxillary teeth and alveolar bone

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Laboratory investigations [Table 1] showed anemia and hypoalbuminemia in both children. Chest X-ray in both twins were normal. Sputum samples were taken in both patients for cartridge-based nucleic acid amplification test, which was negative for mycobacterium tuberculosis. The blood group was B+ in both twins. Both twins were seronegative for human immunodeficiency virus, hepatitis B, and hepatitis C, VDRL and malaria. No organism was isolated from cultures taken from the ulcerative lesions.
Table 1: Laboratory investigations results of the twins

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Thus, taking into account the background of poverty, malnutrition, lack of antenatal, natal, or postnatal medical care, unimmunized status, the orofacial lesions presented by the twins were diagnosed as noma or cancrum oris.


  Management and Outcome Top


Patients were treated with IV antibiotics (amoxicillin–clavulanate 30 mg/kg every eight hourly for 7 days), high calories and high-protein diet consisting of blended peanuts in paste form eeZeePaste™ NUT manufactured by Compact India Pvt. Ltd. The children were encouraged to consume a minimum of three sachets of the eeZeePaste™ NUT per day. The children were also administered IV fluid and analgesics (IV paracetamol 10 mg/kg, eight hourly for 3 days). In addition, wound debridement and dressing were done. Serial alternate-day closed dressings were done using diluted hydrogen peroxide with topical antibiotics. When the healing improved, open dressing was carried out with the application of topical antibiotics. During the period of wound dressing, regular jaw exercise was carried out by physiotherapists to prevent limitation in mouth opening and temporomandibular joint ankylosis. After the ulcers had healed and the lesions had started scarring, (after 2 weeks in Twin A and 5 weeks in Twin B), the patients were discharged home. Nutritional rehabilitation was carried out during the entire period of hospitalization. The children responded well, without any complications. They were followed up for the surgical reconstruction of the defect at a later date.


  Discussion Top


The condition described above, in the twin brothers, is consistent with a classical clinical scenario of noma or cancrum oris. Although the risk factors for noma are well known, such a presentation simultaneously in twin brothers while other siblings were spared, creates interest regarding the genetic predisposition in identical twins. Overall, the etiology of noma is poorly understood, and as yet, there is no information regarding genetic predisposition.

Noma is a condition described since ages, with one of the earliest definitions being given by Tourdes, who described it as a gangrenous ulcer affecting the mouth and face of children rapidly spreading and destroying the surrounding tissues.[6] Unlike other infectious conditions of the face, which may progress along anatomic spaces of the head and neck, noma disrupts these anatomic barriers and spreads through muscle and bone.[2] While in the 1800s, noma was seen in Europe and India, after 2000, the condition became more prevalent in Africa, South America, and Asia.[1],[7] The “noma belt” is located in sub-Saharan Africa, spanning from Senegal to Ethiopia.[1] Children between 2 and 6 years are most commonly affected.[3]

The exact pathogenesis of noma is unclear and is believed to be multifactorial in nature. Chronic malnutrition, comorbidities such as pneumonia, diarrhea, measles, HIV, lack of breastfeeding, lack of access to basic health care, unimmunized status, poor oral hygiene, unmarried mother, or neglectful rearing by a caretaker are the contributing backgrounds.[7] Some studies have hypothesized the proximity of livestock to living areas and poor sanitation[8] as risk factors for noma. Another theory postulated to explain the occurrence of noma in 2–6 years of age, is that this is the teething age when the formation of deciduous teeth results in slowing of the circulatory flow to the gums, making the oral cavity susceptible to infections.[7] In our report, the age of the twins, as well as the socioeconomic and nutritional status, corroborates with these risk factors.

We encountered a similar presentation of noma simultaneously in twin brothers, while other siblings were unaffected. Although there is no direct evidence regarding genetic predisposition for noma, it is well known that up to a third of the variance of periodontitis in the population is due to genetic factors, with higher heritability for more severe disease.[9] Further, a recent study on microbiome similarity analysis in 198 children (49 monozygotic and 50 dizygotic twins) with a mean age of 9.7 ± 2.7 years, showed that monozygotic twins share more bacterial microbial content compared to dizygotic twins.[10] These reasonings could justify the occurrence of noma in the identical twins, reported in the current case.

Noma is categorized by the World Health Organization as a necrotizing ulcerative stomatitis that shares many features with acute necrotizing ulcerative gingivitis.[11] Furthermore, both are caused by similar organisms, require a breach in host defense mechanisms, and both result in tissue damage and necrosis. Based on these similarities, there is a high index of suspicion that noma could also have a genetic basis in its etiopathogenesis.

In addition to surgical correction and reconstruction, management of noma should focus on correcting the nutritional status, along with broad-spectrum antibiotic coverage and supportive care. In the current report, the twins were managed from the acute phase of noma to the scarring phase. They are currently being rehabilitated nutritionally for the plastic reconstruction of the residual defect using local flaps.


  Conclusions Top


As the etiopathogenesis of noma is largely unknown and theories are evolving overtime, our report of the simultaneous occurrence of noma in twin brothers will create awareness of the possible genetic basis for the condition, which needs to be evaluated further.



Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for their images and other clinical information to be reported in the journal. The patient's parents understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chukwuma BC, Mujtaba B, Ibikunle AA, et al. Nutritional status and anemia in persons with cancrum oris. Niger J Med 2021;30:670-4.  Back to cited text no. 1
  [Full text]  
2.
Evrard L, Laroque G, Glineur R, et al. Noma: Clinical and evolutive aspect. Acta Stomatol Belg 1996;93:17-20.  Back to cited text no. 2
    
3.
Mujtaba B, Chimezie CB, Adebayo AI, et al. Clinico-pathological analysis of osteomyelitis in cancrum oris (noma) patients seen in noma children hospital, Northwest Nigeria. Niger J Dent Res 2022;7:29-34.  Back to cited text no. 3
    
4.
Braimah RO, Adeniyi AS, Taiwo AO, et al. Risk factors and mortality rate of acute cancrum oris (noma) in Sokoto North-West Nigeria: A 13-year survey. J Pediatr Dent 2017;5:1-5.  Back to cited text no. 4
  [Full text]  
5.
Enwonwu CO, Falkler WA Jr., Idigbe EO, et al. Pathogenesis of cancrum oris (noma): Confounding interactions of malnutrition with infection. Am J Trop Med Hyg 1999;60:223-32.  Back to cited text no. 5
    
6.
Tourdes J. Noma or Sphacele of the Mouth in Children. Strasbourg: Thesis; 1848.  Back to cited text no. 6
    
7.
Farley E, Mehta U, Srour ML, et al. Noma (cancrum oris): A scoping literature review of a neglected disease (1843 to 2021). PLoS Negl Trop Dis 2021;15:e0009844.  Back to cited text no. 7
    
8.
Barrera J, Connor MP. Noma in an Afghani child: A case report. Int J Pediatr Otorhinolaryngol 2012;76:742-4.  Back to cited text no. 8
    
9.
Nibali L, Bayliss-Chapman J, Almofareh SA, et al. What is the heritability of periodontitis? A systematic review. J Dent Res 2019;98:632-41.  Back to cited text no. 9
    
10.
Kasimoglu Y, Koruyucu M, Birant S, et al. Oral microbiota and dental caries data from monozygotic and dizygotic twin children. Sci Data 2020;7:348.  Back to cited text no. 10
    
11.
World Health Organization (WHO). Regional Office for Africa Noncommunicable Diseases Cluster (NCD). Cité du Djoué, P.O. Box 06 Brazzaville: Republic of the Congo; 2017. URL. Available from: http://www.who.int/iris/handle/10665/254579. [Last accessed on 2023 Feb 10, Last updated on 2023 Jan 27].  Back to cited text no. 11
    


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