Atypical Orofacial Necrosis of Unknown Aetiology: A Case Report with Features Similar to NOMA
An interesting case of atypical orofacial necrosis with no confirmed diagnosis was identified in Glasgow. A patient presenting to Accident & Emergency with septic shock and signs of end-organ dysfunction was noted to have a dark, well demarcated necrotic area of her cheek, chin, tongue, soft and hard palate. Severe malnutrition and alcohol dependence were recorded. Clinical and microbiological findings excluded a group A streptococcal necrotising fasciitis. An idiopathic oro-facial necrosis clinically similar to both Noma (cancrum oris) and necrotising fasciitis was identified in a case in Glasgow.
Key Words: Cancrum Oris; Debilitated & Malnourished; Noma; Necrotising Fasciitis, Orofacial Necrosis; Stomatitis Gangrenous.
Necrotising Fasciitis (NF) is an acute, rapidly spreading and life-threatening infection of the fascia associated with severe sepsis.1 Its prevalence in the human body is 3.5-4 cases occurring per 100,000 people. Of this, only 5% relate to head and neck tissues. Incidence is thought to increase with age and the presence of associated comorbidities. NF is associated with a fairly low incidence, but a high morbidity and mortality and therefore requires urgent surgical debridement and pharmacological treatment.1
Noma (cancrum oris) is an opportunistic condition which primarily affects malnourished children in developing countries.2 It is a polymicrobial infection associated predominantly with anaerobic organisms. Aetiology of Noma is multifactorial: malnutrition, immunodeficiency and poor oral hygiene.3 Global incidences is estimated to be approximately 140,000 cases per year with mortality rate of around 85% without treatment.4,5
A 45-year-old female presented to accident & emergency (A&E) with foetor oris and dark, well demarcated necrotic lesions. (Figure 1) No pain was reported on presentation. The patient reported visiting a dentist for wisdom teeth extraction three weeks prior to admission. She was intoxicated on presentation and was not able to recall the events of the past few months therefore we were unable to obtain a comprehensive history. Patient’s son visited her earlier that day and noticed the smell and the extra-oral lesions. Because of that he called an ambulance which the patient was reluctant to take. The patients’ alcohol consumption was estimated to be 280 units/week and she was a current smoker. (10 pack years)
On presentation the patient was suffering from an extremely malodorous, widespread, but well demarcated necrosis of both intra and extra-oral structures, involving the left cheek, buccal sulcus, tongue, soft palate, buccal fat pad, lateral pharyngeal wall, lower lip, left mandible as well as left and anterior floor of the mouth. Both mandible and maxilla were edentulous and no healing sockets were identified. The patient was clinically septic with haematological evidence of hepatic impairment and severe renal failure requiring supportive renal therapy and was admitted to the intensive care unit (ICU). Her white cell count (WCC) was 19.8x109/L and C-reactive protein (CRP) was 98 mg/L. Initially this case was treated as NF due to the dark demarcated lesions as well as signs of sepsis and the patient was taken for the initial surgery just a few hours after her presentation to A&E. She was started on multiple broad spectrum antibiotics and stayed in ICU for 12 days after admission.
During the initial surgery we noticed that the areas were well demarcated and defined which is not a common presentation of NF. This led us to consider other diseases with similar presentation which include well demarcated lesions involving intra and extra-oral tissues, mainly cheek and buccal mucosa. Due to this we considered Noma as part of the differential diagnoses. Treatment was mainly focused on repeated debridement and broad spectrum antibiotic therapy as either of those conditions would benefit from both of these.
Day 1 showed immediate debridement under general anaesthesia (Figure 2) following presentation and post-operative admission to ICU. Days 2, 4, 7, 8, 14 and 21 showed serial examination with minimal further debridement. (Figure 3 & 4) However, Day 29 revealed reconstruction with radial forearm free flap. (Figure 5)
Therapeutic intervention included Benzyl Penicillin, Metronidazole, Gentamycin, Clindamycin, Vancomycin and Cephtriaxone. The patient was transferred to High Dependency Unit for days 13-16 post admission, after which she remained on a Maxillofacial ward for the rest of her hospital stay. On day 29 she underwent reconstructive surgery with a radial forearm free flap. Once this was stable patient was discharged home on day 42. She returned for her one week follow up but unfortunately failed to return for subsequent appointments.
Diagnostic Assessment and Therapeutic Intervention:
During the first surgery samples were sent to microbiology for immediate assessment. Group B streptococci, Peptostreptococcus anaerobius, Raoultella planticola, Clostridium ramosum, Bacteroides ovatus and Streptococcus salivarius were isolated. Further 13 samples were sent during the subsequent surgeries.
Figure 1: The clinical presentation of the patient prior to admission.
Figure 2: The clinical picture during initial surgery (Day 1).
Figure 3: The clinical picture during further debridement surgery
Figure 4: The clinical picture of last debridement surgery
Figure 5: The 12-day post reconstruction.
NF presents as an extremely rapid, unremitting infection of superficial fascial planes. In this case the necrotic areas were well defined, localised and deep to the superficial fascia. Marked leucocytosis, a feature often reported in NF, was not observed.6 Therefore, Noma was considered as part of our differential diagnoses despite the absence of the archetypal fusospirochaetal organisms; the bacterium often associated with the condition. Nevertheless, other cases of reported Noma have only cultured several anaerobic non-commensal microorganisms as were identified in this case.7 Although Noma is typically associated with developing countries, one case has been reported in Italy in 2009.8 The authors were unable to find other similar cases reported in developed countries.
This report identifies a patient with a diagnosis not clearly fitting into either Noma or NF description and appearance. The presentation of this case suggests that areas in the United Kingdom still suffer from serious deprivation thus resulting in reduced health outcomes, particularly for poor socio-economic regions. This identification of an idiopathic orofacial necrosis with features similar to Noma suggests that areas of the UK may be susceptible to conditions normally associated with third world developing nations and these should be considered in the differential diagnosis of rare necrotic facial infections.
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
1.Dr. Gabriele Baniulyte, SHO in Maxillofacial surgery, 2.Dr. Peter McAllister, Registrar, 3.Dr. Ahad Shafi, Registrar, 4.Dr. Mark Ansell, Consultant 5.Dr. Ewen Thomson, Consultant in Maxillofacial Head Neck Surgery, Queen Elizabeth University Hospital, 1345 Govan Road, Glasgow.
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Dr. Gabriele Baniulyte,
SHO in Maxillofacial surgery, Ward 62,
Queen Elizabeth University Hospital,
1345 Govan Road,
Glasgow - G51 4TF.
Source of Support: Nil, Conflict of Interest: None Declared.
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