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Year : 2015  |  Volume : 4  |  Issue : 4  |  Page : 260-261

Neonatal intestinal mucormycosis: mimicking as necrotizing enterocolitis

Department of Paediatrics and Neonatology, PGIMER and Dr. Ram Manohar Lohia, New Delhi, India

Date of Web Publication16-Oct-2015

Correspondence Address:
Pradeep Kumar
Room No. 303, Doctor's Hostel, Dr. Ram Manohar Lohia Hospital, New Delhi - 110  001, India.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4847.161710

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Neonatal intestinal mucormycosis may mimic and is mistaken as NEC. Diagnosis remains difficult and mortality is high. Unlike NEC where there may be a place for watchful conservative management, urgent aggressive surgical debridement and resection along with systemic antifungal are necessary in GIM. This case is reported to illustrate lessons learnt to improve survival in an otherwise almost universally fatal condition due to missed and delayed diagnosis.

Keywords: Fungal infection, NEC mimics, necrotizing enterocolitis, neonatal intestinal mucormycosis

How to cite this article:
Gorakh R, Kumar P, Maria A. Neonatal intestinal mucormycosis: mimicking as necrotizing enterocolitis. J Clin Neonatol 2015;4:260-1

How to cite this URL:
Gorakh R, Kumar P, Maria A. Neonatal intestinal mucormycosis: mimicking as necrotizing enterocolitis. J Clin Neonatol [serial online] 2015 [cited 2020 Apr 3];4:260-1. Available from: http://www.jcnonweb.com/text.asp?2015/4/4/260/161710

  Introduction Top

Mucormycosis is an opportunistic infection by fungi (saprophytes). Mucormycosis infections occur in the rhino cerebral, respiratory, gastrointestinal, or cutaneous regions, depending on whether the spores are inhaled, ingested, or injected.[1] Neonatal intestinal mucormycosis (NIM) is rare and definitive diagnosis made by biopsy.[2],[3],[4] Thefirst review of neonatal gastrointestinal mucormycosis (GIM) was published in 1994,[2] 15 cases have been reported from India.[1],[4],[5] The incidence of necrotizing enterocolitis (NEC) in the premature infant is high and associated with mortality of 20–40%.[2],[11] We report a neonate with NIM in whom antifungals were started in preoperative period. The patient had a stormy course but survived. The importance of early institution of specific therapy has been highlighted.

  Case Report Top

A 2.6 kg term male neonate was vaginally delivered to mother with 7 days h/o leaking. With severe perinatal asphyxia and features of hypoxic-ischemic encephalopathy II, he was referred to us with cyanosis and seizures at 2 h of life. On day 5, baby developed hematuria and urine examination revealed fungal hyphae. With a provisional diagnosis of sepsis/invasive fungal infection, antibiotics were upgraded, and amphotericin B was started. On day 7, he developed abdominal distension, dark brown aspirates. X-ray abdomen revealed no pneumatosis but free gas under diaphragm. With diagnosis of NEC, stage III with bowel perforation, emergency laparotomy with resection of grossly gangrenous large bowel from cecum up to mid-transverse colon and ileostomy and colostomy was done. Histopathology was consistent with GIM. Baby was discharged after 6 weeks of medical therapy and is doing well on follow-up.

  Discussion Top

Gastrointestinal mucormycosis is a very rare form of mucormycosis and accounts for 7% of all forms of mucormycosis. The NIM is still rare as it is very difficult to suspect and confirm the diagnosis and hence the very high mortality rate.[3] It is not be wrong to speculate that this is an underreported diagnosis since many of the patients present with NEC and not all of them go through histopathological examination of their gut.[3],[11]

Mucosal and skin defense systems of neonates are not well developed. Broad-spectrum intravenous antibacterial therapy and corticosteroids are important risk factors. The ambient humidity of Neonatal Intensive Care Unit may also be a factor for the propensity of the fungus to proliferate and invade the susceptible host by an appropriate route.[5],[8] Similar risk factors existed in our case also although he was a term baby.

Various authors have described NIM as a variant of NEC; however, there is an overlap and most cases present with increasing nasogastric aspirates, abdominal distension, and hematochezia.[1],[7],[9] It is, therefore, possible that fungal invasion in premature neonates could be secondary to the gastrointestinal tract vascular compromise, mucosal injury or even NEC itself since devitalized tissue is more prone to fungal invasion.[11] NIM is suspected on a background of NEC whenever there is deterioration despite appropriate management with absent pneumatosis intestinalis.[7],[11] It is however easier said than done clinically; especially when NEC can have nonspecific presentation. Hence, there is probably a role of prophylactic antifungals. Since early diagnosis and initiating specific medication is the key to survival. Patra et al. described their policy of starting antifungals in high-risk cases.[10] This philosophy has been described earlier by Siu and Lee and further endorsed by our case. In our case, on day 5, we started amphotericin B when we evaluated the baby for hematuria and it revealed fungal hyphae.[10] This was probably one of the reasons for a favorable outcome. The differentiation of NEC from NIM by observing widespread thrombosis of the small vessels of the gut and the characteristic right-angle branching pattern with variable sized, broad, irregularly shaped, and aseptate to pauciseptate hyphae [Figure 1]a and [Figure 1]b is important but should not be the sole criteria to initiate specific treatment.[1],[3],[6],[11]
Figure 1: Broad branching aseptate fungal profiles (arrow) are seen in a background of gangrenous tissue (a) H and E stain, ×40 (b) PAS stain, ×40

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  Acknowledgment Top

We acknowledged Pediatrics Surgery and Pathology Department for their support.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Agarwal K, Sharma M, Singh S, Jain M. Antemortem diagnosis of gastrointestinal mucormycosis in neonates: Report of two cases and review of literature. Indian J Pathol Microbiol 2006;49:430-2.  Back to cited text no. 1
Reimund E, Ramos A. Disseminated neonatal gastrointestinal mucormycosis: A case report and review of the literature. Pediatr Pathol 1994;14:385-9.  Back to cited text no. 2
Sarin YK. Intestinal mucormycosis in a neonate: A case report and review. J Indian Assoc Pediatr Surg 2010;15:98-100.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
Choudhury M, Kahkashan E, Choudhury SR. Neonatal gastrointestinal mucormycosis – A case report. Trop Gastroenterol 2007;28:81-2.  Back to cited text no. 4
Jain D, Kohli K. Neonatal gastrointestinal mucormycosis clinically mimicking necrotizing enterocolitis. Eur J Pediatr Surg 2009;19:405-7.  Back to cited text no. 5
Kecskes S, Reynolds G, Bennett G. Survival after gastrointestinal mucormycosis in a neonate. J Paediatr Child Health 1997;33:356-9.  Back to cited text no. 6
Woodward A, McTigue C, Hogg G, Watkins A, Tan H. Mucormycosis of the neonatal gut: A "new" disease or a variant of necrotizing enterocolitis? J Pediatr Surg 1992;27:737-40.  Back to cited text no. 7
Budhiraja S, Sood N, Singla S, Gupta V. Gastrointestinal mucormycosis in a neonate: Survival without antifungal therapy. Indian J Gastroenterol 2007;26:198.  Back to cited text no. 8
Michalak DM, Cooney DR, Rhodes KH, Telander RL, Kleinberg F. Gastrointestinal mucormycoses in infants and children: A cause of gangrenous intestinal cellulitis and perforation. J Pediatr Surg 1980;15:320-4.  Back to cited text no. 9
Patra S, Vij M, Chirla DK, Kumar N, Samal SC. Unsuspected invasive neonatal gastrointestinal mucormycosis: A clinicopathological study of six cases from a tertiary care hospital. J Indian Assoc Pediatr Surg 2012;17:153-6.  Back to cited text no. 10
[PUBMED]  Medknow Journal  
Parfrey NA. Improved diagnosis and prognosis of mucormycosis. A clinicopathologic study of 33 cases. Medicine (Baltimore) 1986;65:113-23.  Back to cited text no. 11


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