Noma in an 18 months Toddler: A Rare Case Report with Review of Literature

Abstract

Introduction

Noma is a polymicrobial necrotizing infection of the mouth and face which destroys the soft and hard tissues of the oral and paraoral structures. Malnutrition, poverty, lack of basic hygiene, and chronic disease state etc., possess a risk of developing noma. Noma neonatorum is a progressive gangrenous disease affecting the premature infants.

Aim

We present a rare case of noma in an 18 months toddler with a chronic debilitating condition predisposing to the disease progression.

Results

The necrotizing infection of the face developed after six months of multiple episodes of chronic blood-tinged diarrhea. The facial defect was managed with release of fibrosis and reconstruction with a superiorly based nasolabial flap.

Discussion

Noma reflects extreme malnutrition and poverty with a vast range of etiopathological agents. This case emphasizes the need to understand the host risk factors and etiopathologic agent predisposing to this rare opportunistic and dormant but devastating disease, the morbidities associated with it and measures to prevent it.

Introduction

“Noma” (from Greek word: to “devour”) is a necrotizing disease of the mouth and face with 90% mortality rate if left untreated [1]. In 1848, Tourdes described it as a “gangrenous affection of the mouth infecting children with bad hygiene and serious illness, beginning as an ulcer of the mucous membrane with edema of the face, extending from within out, rapidly destroying the soft parts and the bone, and almost always quickly fatal.” The latest WHO ICD 11 classifies Noma under Necrotizing periodontal diseases, with a code of DA0C.31, defining it as a devastating infectious disease which destroys the soft and hard tissues of the oral and paraoral structures [2]. Its pathogenesis is poorly understood although it is described as a polymicrobial infection. The risk factors include malnutrition, poverty, poor living conditions, lack of basic hygiene and sanitation, chronic disease, and immunocompromised state [1, 3]. In children, it has been associated with Fusobacterium spp., Prevotella spp., Corynebacterium pyogenes, Bacteroides fragilis and Bacillus cereus [2].

Noma neonatorum, (term coined by Ghosal et al., [5]) is a similar gangrenous disease-causing progressive mutilating tissue destruction of facial soft tissue and bone affecting low birth weight premature infants presenting with severe comorbid pathologies [4, 5]. The pathogenesis is similar to Noma, including malnutrition, polymicrobial infection (Pseudomonas being the most frequent bacteria), and immune dysfunction in newborns. It is a preventable disease and prompt diagnosis with aggressive antibiotic treatment in the early stages are critical in halting the disease progression and further morbidity. Once it progresses, the sequelae may range from functional disability in oral intake, speech, facial disfigurement, with permanent loss of facial structures to even death.

The aim of this paper is to present a rare case report of acute noma in an 18 months old toddler which appeared as a consequence of a chronic systemic ailment and rapidly progressed to involve the facial tissues. To the best of our knowledge, this is the first case of noma reported in the eastern region of India in more than 45 years.

Materials & Methods

A comprehensive 10-year search of scientific literature was done in PubMed and Scopus using MeSH terms for understanding the host risk factors, etiopathological agent/s, clinical features and morbidities associated with noma neonatorum and noma in children and adults. The MeSH terms included noma, orofacial gangrene, noma neonatorum, gangrene, cancrum oris. After removing duplicates, and articles in other language, twenty articles were reviewed. Written informed consent was obtained from the parents for publishing the data and photographs (Figs. 1, 2 and 3).

Fig. 1.

Pre-operative picture demonstrating ulceration with loss of lip commissure

Fig. 2.

Extensive intraoral ulceration, induration of cheek with rolled out margin post debridement

Fig. 3.

Release of the fibrosis followed by reconstruction with superiorly based nasolabial flap to cover lip and cheek defects. Mouth opening 4 mm

Results

Patient information

An 18 months old toddler was referred from Dept. of Pediatrics to the Dept. of OMFS, with extensive oral ulceration and loss of tissues at right commissure of lip. The onset of symptomatology dated 6 months back with multiple episodes of mucoid, blood-tinged diarrhea (10–12 times/day for 6 months). It was associated with abdominal cramps, progressive pallor, and appearance of a non-healing ulcer at the right corner of mouth. There was no relevant past medical, family or birth history. He had been receiving symptomatic treatment at a local primary health centre for the same.

Clinical Findings

On examination, an irregular ulcer approx. 5 × 4 cm² was noted at right commissure involving both upper and lower lip, with tissue loss of approx. One-third of both lips, the margins were rolled out. Ulcer bed was covered with foul smelling necrotic debris, and slough. Mouth opening was 15 mm.

On palpation, there was local rise of temperature and tenderness, surrounding skin was erythematous and indurated. On intraoral examination, extensive mucosal ulceration was found bilaterally involving the labial, buccal sulcus, and right-side palatal surface, covered with necrotic slough and debris, severely tender on manipulation. Teeth were non mobile and tongue was spared of the disease. Underlying bone appeared to be uninvolved (Table 1).

Table 1.

Summary of reports of cancrum oris /noma and noma neonatorum [4, 6–23]

Author	Age of patient	Stage of noma	Host risk factor	Clinical features	Etiopathological agent	Morbidities
Present case	18 mo	Stage III, IV, V	Chronic diarrhea, anemia, ulcerative colitis activity 1	Extensive oral ulceration with loss of tissues at Right commissure of lip, bilateral labial, buccal sulcus and Right-side palatal surface	Not isolated	Trismus, U/L lip defect with scarring
Nayak PA et al. [6]	25 days	Noma neonatorum	Malnutrition, septicaemia	Small boil over upper lip- progressed to gangrenous blackish lesion involving upper lip, alveolar mucosa, eyelids, lateral nose	Pseudomonas aeruginosa	Inability to suck, facial disfigurement, permanent loss of nose, deformation of upper lip
Raimondi F et al. [7]	28 days	Noma neonatorum	Premature delivery, nosocomial infection	White linear lesion at lower gingival mucosa rapidly progressing to upper gingiva with ulceration	MDR resistant Pseudomonas aeruginosa	Not reported
Niekerk C et al. [8]	32 years	Stage III, IV, V	Immunodeficiency, HIV seropositive, debilitated, anemic, anorexia, diarrhea	Well defined full thickness defect of lip below lower vermilion border with necrotizing periodontitis & necrotizing stomatitis	Streptococci, Klebsiella pneumoniae	Facial disfigurement
Nipun A et al. [9]	Variable	Not reported	Extreme poverty, severe malnutrition, poor sanitation, poor oral health	Not reported	Not reported	Not reported
Singh A et al. [10],	2.5 years	Stage III	Acute leukemia,	Recurrent fever, progressive pallor, lymphadenopathy and a raw area on the right cheek, with discharging sinus	Not reported	Not reported
LeeYJ et al. [11],	11 years	Stage III, IV, V	Crohn’s disease	Were 3–4 cm necrotic ulcers with crusting & yellowish discharge at B/L corner of lips	Klebsiella pneumonia & Candida albicans	Buccal stricture
Madsen T et al. [12],	32 years	Stage V	Immunodeficiency, HIV seropositive	Loss of tooth, perforation in right cheek	Could not be identified	Speech difficulty, facial disfigurement, occasional eating difficulty
Tungotyo M[13]	16 months	Stage III, IV, V	Severe malnutrition	8X5cm dark necrotic patch on left cheek involving upper lip, nose, upper gingiva	Ebiino (extraction of false tooth buds)	Facial disfigurement
Hatcher J, Williamson L[14]	43 years	Stage III, IV, V	Immunodeficiency, HIV seropositive	Ulcerated oral lesion with surrounding pus and facial soft tissue destruction, fetid oral odor, seborrheic dermatitis, cachexia	Not reported	Facial disfigurement,
Gupta et al. [15],	2.5 years	Stage III, IV	Fever	Fever, swelling and ulceration of his lower lip associated with drooling and purulent discharge	Klebsiella pneumonia	Lower lip defect with scarring
Osovsky M et al. [4],	6 days	Noma neonatorum	Premature delivery, respiratory distress syndrome	Decubitus ulcer right cheek, pulmonary hypertension, left pneumothorax, intraventricular hemorrhage,	Citrobacter koseri	Facial disfigurement, death on 15th day of life
Madabhavi I[16]	27 years	Stage III, IV	Immunocompromised state (H/o acute lymphoblastic leukemia)	Gangrenous tissue in infranasal, intranasal, upper lip	Infiltration of soft tissue by lymphoblasts	Not reported
Adeniyi SA, Awosan KJ[17]	3.0 ± 1.4 years	All stages	Measles, Protein energy malnutrition	Soft tissue lesions mostly involved multiple sites involving cheek 84.3%, U/L lip 39.3%, nose 25.2%	Not reported	Palatal & osseous defects, trismus
Brotherton BJ[18]	13 years	Stage III, IV, V	Severe malnutrition	2.5X 2.5 cm necrotic eschar over mandible below lower lip	Not reported	Trismus, facial disfigurement
Xu et al. [19],	6 years	Stage II, III	Septic shock	3X4cm swelling right cheek, necrosis crusting, tissue defect, septic shock	Not reported	Not reported
Ganju and kumar[20]	14 days	Noma neonatorum	Lung consolidation, LRTI	Black gangrenous discoloration & induration of lips, anterior tongue, palate, respiratory distress, subcostal, intercostal retraction, B/L crackle	Acinetobacter spp	Partial loss of lip and perforation of palate
Ding et al. [21],	66 years	Stage III, IV, V	Chronic facial tissue deformity	Left lower facial tissue defect with destruction of left lip, cleft of mouth, left maxilla defect, nasal septum, left lower eyelid droop, highly differentiated SCC post initial treatment	Not reported	Facial disfigurement
Traore et al. [22]	32 years	Stage III, IV, V	Anemia (Hb 7 g/dL)	Pain & swelling left cheek progressing to 6X4cm gangrenous loss of soft tissue with foul odor creating orostoma	Polymicrobial: Escherichia coli extended-spectrum beta lactamase (ESBL), Kluyveromyces lactis, Candida tropicalis, Clavispora lusitaniae, Paenibacillus alvei Candida tropicalis, Clavispora lusitaniae, Paenibacillus alvei	Facial disfigurement, death after 1 month
Park et al. [23],	12 years	Stage II, III, IV, V	Patent ductus arteriosus, acute myeloid leukemia since 1-year, Neutropenic fever, Vancomycin resistant enterococci sepsis	Ecchymosis of chin with necrotizing gingivitis progressing to gangrenous necrosis and loss of anterior portion of mandible, loss of half of lower lip	Not reported	Loss of oral competence, drooling of saliva, difficulty in oral intake, facial disfigurement

Diagnostic assessment & Interpretation

On routine examinations, the patient was found to be anemic (hemoglobin 4.4 g/dL), with a TLC of 25,500 /µL with 80.1% neutrophils, 16.8% lymphocytes. Quantitative c-reactive protein was elevated at 220.9 mg/L, with hypoproteinaemia, hypoalbuminemia (serum protein 5.0 gm/dL, albumin 2.6 g/dL). Total RBC count was 2.09 × 10⁶/µL, and peripheral smear showed microcytosis, hypochromia with fragments. An impression of hemolytic anemia along with iron deficiency was given. Sickling test was negative. Gram staining showed occasional pus cells. No growth was found on bacterial culture, fungal KOH mount and culture reports.

USG of abdomen and pelvis showed bowel within bowel appearance involving a small bowel loop in left hypochondrium suggesting intussusception with minimal ascites, and grade 1 fatty changes in liver. On colonoscopy, presence of multiple ulcers with minimal bleeding starting from rectum till the splenic flexure was noted with obliteration of the vascular pattern, and an impression of ulcerative colitis was given. Histopathological examination confirmed Ulcerative colitis Activity 1 (Mayo Endoscopic Score).

A diagnosis of Cancrum Oris (Noma) WHO Stage 3 was made based on the clinical presentation. (Table 2).

Table 2.

WHO stages of Noma [1]

Stage	Description
Stage 1	Acute Necrotizing gingivitis
Stage 2	Edema
Stage 3	Gangrenous stage
Stage 4	Scarring stage
Stage 5	Sequelae stage

Intervention

Under multidisciplinary consultation, medical management and hemodynamic stabilization of his systemic condition was done which comprised of adequate antibiotics and analgesic coverage, using intravenous (IV) piperacillin 1 gm + tazobactam 125 mg TDS, IV fluconazole 36 mg OD, IV metronidazole for 7 days, IV ondansetron SOS and ranitidine IV BD, syrup Zinc (5 ml/20 mg), Tablet mesalamine 400 mg ½ tablet OD, fluid management with oral and IV fluids, 2 units of whole blood transfusions and wound management.

Ulcer debridement was done using diluted hydrogen peroxide solution, necrotic debris and slough was completely removed and sent for bacterial culture and sensitivity, fungal KOH mount and fungal culture and sensitivity. To induce epithelialization of the ulcer bed, regular dressing using diluted peroxide was done followed by local application of 2% metronidazole gel.

Follow-Up

On subsequent follow ups, improvement in healing of the ulcer was noted with marked fibrosis (WHO Stage 4 NOMA) and trismus, patient’s attendant was advised to continue regular dressing with diluted peroxide, chlorhexidine mouthwash 0.2% and 2% metronidazole gel and continue mouth opening exercises for the patient.

On 3rd week of follow-up, the patient presented with healing of ulcer by secondary intention leading to marked fibrosis and trismus. The patient had inability to eat with a mouth opening of 1 mm. Oral hygiene and physiotherapy instructions were reinforced and the patient was discharged on request after hemodynamic stabilization.

Six months later, and after stabilization of the patient’s general conditions, the patient underwent release of the fibrosis followed by reconstruction with superiorly based nasolabial flap to cover lip and cheek defects. Due to post op edema, the immediate post op Interincisal opening improved from 1 to 4 mm. Patient was advised for subsequent physiotherapy to improve oral opening.

Discussion

Noma has been described as the “Face of Poverty.” It is a preventable disease that mainly affects the underprivileged and the immunocompromised. The disease has almost disappeared from developed countries with improvements in the standard of living, but has remained a health problem in sub-Saharan Africa [2]. The peak incidence of acute noma is between 1 and 4 years of age, which coincides with the period of linear growth retardation in socioeconomically deprived children. The disease mainly affects the younger age groups with severe malnutrition and adults with immune dysfunction which makes them more susceptible to infections. Current prevalence is not known due to lack of adequate data and reporting from these regions. According to UNICEF, WHO, World Bank group Malnutrition Estimates, globally 45.4 million children under the age of 5 years are moderate and severe acutely malnourished out of which 13.6 million children are severely acutely malnourished [24]. WHO classifies Noma into various stages (Table 2) [1, 2]. Enwonwu and colleagues suggested a three way relationship between malnutrition, host immune dysfunction, and increased susceptibility to infections under deprived socioeconomic state for understanding the pathogenesis of Noma [2].

The review of past published literature (summarized in Table 1) gave an insight of the multifactorial nature of the disease and various host risk factors in the different age groups making them susceptible [4, 6–23]. The vast bacterial diversity includes organisms such as Pseudomonas spp, Acinetobacter, and Citrobacter spp causing Noma neonatorum in premature neonates, with respiratory distress syndrome and deprived immunity, Klebsiella causing disease in children, and polymicrobial infections of Klebsiella, Streptococcus spp, Candida spp, E. coli and Clavispora in adults. Falker and colleagues in their study of 62 Nigerian noma patients aged between 3 and 14 years conducted a study of the predominant microflora in active sites of noma lesions in 8 patients and found that, with other microbes, Fusobacterium necrophorum was isolated from 87.5% of noma lesions which suggested its important association and possibility of animal transmission [25].

Our patient had a long-standing history of chronic diarrhea, with pallor, wasting of muscles. Farley in his study has reported chronic systemic comorbidity being one of the risk factors predisposing to noma [26]. The laboratory results showed microcytic, hypochromic anemia with iron deficiency, with hypoproteinaemia, hypoalbuminemia. The child’s condition was attributed to Ulcerative Colitis which led to malnutrition, delayed development of the child and subsequently Noma. It needs to be identified and differentiated from other similar lesions like myocutaneous leishmaniasis, lupus erythematosus, leprosy, syphilitic lesion, physical trauma like burns, oral cancer, agranulocytic angina, midline granuloma of face, and yaws. Warning signs include recent history of fever or debilitating disease, oral mucosal ulcer with necrosis, oral malodor, severe wasting in a deprived child. HIV serological testing should be done in all suspected patients [2, 5, 10].

The disease starts with eruption over gingiva or ulcer and rapidly progresses to involve adjacent and deeper structures leading to extensive necrosis and gangrene resulting in loss of structure. The patient suffers loss of function, difficulty in speech, eating and drinking, loss of oral competence, and neonates may have difficulty feeding. The disease in its advanced stage can cause extensive scarring and fibrosis leading to complete trismus. Taking utmost care of oral hygiene can prevent onset of noma in the susceptible population.

A multidisciplinary team including Intensivist (if critically ill), pediatricians, maxillofacial surgeon, reconstructive surgeon, speech therapist, and physiotherapist is important in the treatment of noma. The treatment includes specific drug therapy for underlying cause and broad-spectrum antimicrobial and analgesics coverage, surgical debridement of the wound, hydrogen peroxide or saline wash, regular dressings. Correction of underlying systemic illness, with fluid and electrolyte therapy, if required, blood transfusions, protein rich diet, and maintenance of proper hygiene and sanitation are of utmost importance. With the introduction of antibiotics, the mortality rate of noma has significantly reduced. Proper immunization schedule should also be ensured in this age group. The patients with morbidities and sequelae of noma may be benefited by reconstruction surgeries using skin grafts, pedicled and free vascularized flaps, prosthetic rehabilitation, speech therapy and physiotherapy.

Malnutrition is described as India’s silent emergency [10]. In India about 21 per cent of children under age 5 are wasted (moderate and severe malnutrition) [27]. Since 2005, severe wasting in children declined from six per cent to five per cent in India, while wasting levels remained stagnant (19.8 per cent in 2006, 17.3 per cent in 2018) [28]. This decline may be contributed by the Mid-day meal scheme launched by the Ministry of education in 1995 and the Swachh Bharat Mission launched by the Prime Minister of India in 2014 which aims to achieve universal sanitation coverage.

The case highlights the need for surveillance of noma as very few cases have been reported from India. Our case represents it as a “dormant but potentially dangerous silently lurking entity” devastating the life of many children. Maxillofacial surgeons, and Pediatricians should be aware of the clinical picture of noma and its possible association with host risk factors. Early diagnosis and aggressive treatment may reduce the risks of severe morbidity and mortality.

Declarations

Conflict of interest

The authors declare that they have no conflicts of interest.