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Noma neonatorum
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     Department of Neonatology, King Edward Memorial Hospital and Seth G S Medical College, Mumbai, India,

    Abstract

    Noma Neonatorum is characterized by a gangrenous process involving mucocutaneous junctions of oral, nasal and anal area and occasionally, the eyelids and scrotum. It is seen during the first few weeks of neonatal life in premature and low birth weight babies. Noma Neonatorum is commonly described with pseudomonas aeruginosa septicemia. A case of Noma Neonatorum associated with E.coli sepsis is described for the first time.

    Keywords: Neonate; E. coli; Noma; Pseudomonas aeruginosa

    Noma Neonatorum is characterized by a gangrenous process involving mucocutaneous junctions of oral, nasal and anal area and occasionally, the eyelids and scrotum. It is seen during the first few weeks of neonatal life in premature and low birth weight babies.[1] It is usually associated with Pseudomonas aeruginosa pticemia.[2] Barring one large series of 48 babies from Calcutta in 1977, cases of Noma Neonatorum are reported sporadically.[2]

    Case Report

    34wks male preterm, with birth weight 1800 gm, second of diamniotic dichorionic twins, born to a primigravida with uneventful antenatal period was transferred to our institution for respiratory distress and jaundice after eight days of birth. The referring doctor noticed erythema of the nasal region after seven days of birth. There was no history of intubation or nasogastric tube placement before onset of erythema. Examination revealed sick low birth weight infant with hypothermia, shock, sclerema and necrosed anterior end of nasal septum forming a black slough. Face was erythematous Figure1. The condition was diagnosed as Noma Neonatorum. Baby had icterus till soles and had clinical features of bilirubin encephalopathy.

    The baby was started on intravenous ceftazidime, amikacin, intravenous fluids and phototherapy. Double volume exchange transfusion was performed. The baby's general condition deteriorated and facial erythema increased over the next 24 hours. The baby expired within 48 hours of admission. The blood cultures sent on the day of admission grew E.coli and the culture of exudate from the site of Noma grew acinetobacter.

    Discussion

    Noma (cancrum oris) is a devastating gangrenous disease that leads to severe tissue destruction in the face and is associated with high morbidity and mortality. The term Noma was first described by Tourdes in 1848 which originates from the Greek verb numein meaning to 'devour'.[3] The term Noma is used to describe the condition in children younger than 12 years, now seen mainly in Africa and occasionally in Latin America and Asia. Malaria, malnutrition, measles, and poor oral hygiene have role in the pathogenesis of Noma disease in children. Noma in children is caused by corynebacterium, fusobacteria and bacteroids.[3] Noma Neonatorum is completely different disease than Noma and is exclusively seen in neonates. It is so called because of the similarity of the facial lesions. However distinct age group, clinical course, microbiology and prognosis make them two separate disease entities.[4]

    The term Noma Neonatorum was coined by Ghosal et al in 1977 from Calcutta, India, when they described forty eight cases of Noma Neonatorum.[1] Neonatal Noma affects mostly preterm, low birth weight babies, though three case reports of Noma Neonatorum in full term babies exist.[5] Noma Neonatorum occurs after third day, usually within first two weeks of birth and before 27 days of birth. Characteristically the general condition of the baby is very poor before the onset of the gangrenous process. Setting up of Noma is followed by rapid spread of sclerema and mostly death within next 1 -3 days. It is postulated that infection may be the precipitating factor in the causation of gangrene in very sick premature infants with the general and local circulatory inadequacy and poorly developed immune mechanism.[1],[2]

    Most of the cases of Noma Neonatorum have been described with Pseudomonas aeruginosa sepsis. Ghosal et al reported 86% blood culture positivity for Pseudomonas in Noma Neonatorum cases. Out of 38 isolates, 2 isolates had mixed growth of Pseudomonas aeruginosa and E. coli.[2] Noma Neonatorum has been linked so much to Pseudomonas infection that some researchers question the distinction between Noma Neonatorum and a neonatal presentation of ecthyma gangrenosum.[6] The present case had growth of E.coli from blood and Acinetobacter from the Noma site. Eisele et al from their series had reported isolation of Klebsiella and Staphylococcus species from cases of Noma neonatorum. (7) These reports suggests possible role of common inflammatory mechanism and or bacterial virulence factors, common to certain bacteria, in causation of noma in a critically sick neonate.

    Noma Neonatorum needs early identification and specific antimicrobial therapy. Despite aggressive treatment most cases of Noma Neonatorum die. Very few cases have reported survival.[6],[8] Extensive surgical debridement is contraindicated in these cases and reconstructive surgery is advocated after first year of life.[8]

    Acknowledgements

    Dr. Nilima Kshirsagar, Dean, Seth G.S. Medical College and K. E. M. Hospital, for permitting to publish this article

    Contributors

    TBP was involved in management of patient, data collection, review of literature and drafting the manuscript, RHU and RNN were involved in management of patient, concept and design of the article and critical revision of the manuscript for important intellectual content.

    References

    1. Ghosal SP, Chaudhuri M, Dutta N, Sarkar AK, Mukherjee AK, Sen Gupta PC. Noma Neonatorum. Indian Pediatr 1977; 14(9): 709-714.

    2. Sen Gupta PC, Ghosal SP, Mukherjee AK, Maity TR. Noma Neonatorum: Its aetiopathogenesis. Lancet 1978; 2(8084) : 289-291.

    3. Baratti-Mayer D, Pittet B, Montandon D, Bolivar I, Bornand JE, Hugonnet S et.al. for Geneva Study Group on Noma. Noma: an "infectious" disease of unknown aetiology. Lancet Infect Dis 2003; 3(7) : 419-431.

    4. Borle RM, Agrawal M. Noma Neonatorum. Int J Oral Maxillofac Surg 1987; 16(5): 626-629.

    5. Atiyeh BS, Hashim HA, Rubeiz MT, Hamdan AM, Bitar FF, Serhal HM. Necrotising infection of the orofacial tissues in neonates (Noma Neonatorum). Case report. Scand J Plast Reconstr Surg Hand Surg 1998; 32(3) : 343-345.

    6. Freeman AF, Mancini AJ, Yogev R. Is Noma Neonatorum a presentation of ecthyma gangrenosum in the newborn Pediatr Infect Dis J 2002; 21(1) : 83-85.

    7. Eisele DW, Inglis AF Jr, Richardson MA. Noma and noma neonatorum Ear. Nose and throat J 1990; 69: 119.

    8. Prajapati NC, Chaturvedi P, Bhowate RR, Mishra S. Noma Neonatorum. Indian Pediatr 1995; 32(9) : 1019-1021.(Parikh Tushar B, Nanavati)