Gastrointestinal mucormycosis is a rare but nearly always fatal disease and accounts for more than half of all mucormycosis infections among neonates. Premature birth is the primary risk factor, though malnutrition, hyperglycemia, acidosis, asphyxia, corticosteroid use, recent surgery, instrumentation with orogastric or nasogastric tubes, and exposure to contaminated products have also been implicated. A combination of impaired mucosal integrity and an immature immune system likely contribute to development of infection. Clinical manifestations can mimic necrotizing enterocolitis and can include abdominal pain and distention, vomiting, hematochezia, fever, peritonitis, and sepsis. Diagnosis is challenging because there are no pathognomonic clinical or radiographic signs, and organisms may not be found on culture; diagnosis is often based on evidence of organisms in tissue and signs of angioinvasion on histopathology. Treatment involves a combination of surgery and antifungal therapy. Mortality is extremely high at nearly 80 % but may be reduced with early surgical and medical intervention.
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Prabhu RM, Patel R. Mucormycosis and entomophthoramycosis: a review of the clinical manifestations, diagnosis and treatment. Clin Microbiol Infect. 2004;10 Suppl 1:31–47.
Petrikkos G, Skiada A, Lortholary O, et al. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis. 2012;54 Suppl 1:S23–34.
Roilides E, Zaoutis TE, Walsh TJ. Invasive zygomycosis in neonates and children. Clin Microbiol Infect. 2009;15 Suppl 5:50–4. This is an excellent article that reviews nearly 200 cases of mucormycosis in children and neonates, and specifically discusses how clinical manifestations and mortality of mucormycosis in children and neonates differs from adults.
Roden MM, Zaoutis TE, Buchanan WL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis. 2005;41(5):634–53. This article is the most comprehensive review of mucormycosis (includes nearly 1000 cases in adults and children) in the English-language literature and provides insight into the most common manifestation of mucormycosis, underlying risk factors, and mortality.
Roilides E, Zaoutis TE, Katragkou A, et al. Zygomycosis in neonates: an uncommon but life-threatening infection. Am J Perinatol. 2009;26(8):565–73. This article is most closely related to the topic of this review because it specifically discusses mucormycosis in neonates. All forms of mucormycosis are discussed, including gastrointestinal and cutaneous, which are two of the most common forms in neonates.
Mathur NB, Gupta A. Neonatal zygomycosis with gastric perforation. Indian Pediatr. 2013;50(7):699–701.
Agrawal P, Saikia U, Ramanaathan S, et al. Neonatal small intestinal zygomyocosis misdiagnosed as intussusception in a Two-Day-Old child with a review of the literature. Fetal Pediatr Pathol. 2013;32(6):418–21.
Ozturk MA, Akin MA, Deniz K, et al. Neonatal gastrointestinal mucormycosis in an asphyxiated premature newborn. Turk J Pediatr. 2011;53(6):705–8.
Inoue, S Odaka A, Hashimoto D, et al. Rare case of disseminated neonatal zygomycosis mimicking necrotizing enterocolitis with necrotizing fasciitis. J Pediatr Surg. 2011;46(10):E29–32.
Gupta R, Parelkar SV, Oak S, et al. Neonatal lingual and gastrointestinal mucormycosis in a case of low anorectal malformation-a rare presentation. J Pediatr Surg. 2011;46(4):745–8.
Jain D, Kohli K. Neonatal gastrointestinal mucormycosis clinically mimicking necrotizing enterocolitis. Eur J Pediatr Surg. 2009;19(6):405–7.
Veleminsky Sr M, Noll P, Hanzl M, et al. Necrotizing enterocolitis in children with low birth-weight induced with mucormycose strains. Neuro Endocrinol Lett. 2008;29(6):1021–5.
Dhingra KK, Mandal S, Khurana N. Unsuspected intestinal mucormycosis in a neonate presenting as necrotizing enterocolitis (NEC). Eur J Pediatr Surg. 2008;18(2):119–20.
Patra S, Vij M, Chirla DK, et al. Unsuspected invasive neonatal gastrointestinal mucormycosis: a clinicopathological study of six cases from a tertiary care hospital. J Indian Assoc Pediatr Surg. 2012;17(4):153–6.
Field DJ, Dorling JS, Manktelow BN, et al. Survival of extremely premature babies in a geographically defined population: prospective cohort study of 1994-9 compared with 2000-5. BMJ. 2008;336(7655):1221–3.
Pena CE. Deep mycotic infections in Colombia. A clinicopathologic study of 162 cases. Am J Clin Pathol. 1967;47(4):505–20.
Isaacson C, Levin SE. Gastro-intestinal mucormycosis in infancy. S Afr Med J. 1961;35:581–4.
Hadley GP. Neonatal mucormycosis. P N G Med J. 1981;24(1):54–6.
Siu KL, Lee WH. A rare cause of intestinal perforation in an extreme low birth weight infant — gastrointestinal mucormycosis: a case report. J Perinatol. 2004;24(5):319–21.
Michalak DM, Cooney DR, Rhodes KH, et al. Gastrointestinal mucormycoses in infants and children: a cause of gangrenous intestinal cellulitis and perforation. J Pediatr Surg. 1980;15(3):320–4.
Nissen MD, Jana AK, Jana MJ, et al. Neonatal gastrointestinal mucormycosis mimicking necrotizing enterocolitis. Acta Paediatr. 1999;88(11):1290–3.
Vallabhaneni S, Walker TA, Lockhart SR, et al. Notes from the field: fatal gastrointestinal mucormycosis in a premature infant associated with a contaminated dietary supplement--Connecticut, 2014. MMWR Morb Mortal Wkly Rep. 2015;64(6):155–6. This article describes a fata case of GI mucormycosis in a neonate that resulted from contaminated probiotic products. This article is important because it highlights the public health implications of using unregulated dietary products, especially in immunocompromised neonates.
Alfaleh K, Bassler D. Probiotics for prevention of necrotizing enterocolitis in preterm infants. Cochrane Database Syst Rev. 2008;1:CD005496.
Oliver MR, Van Voorhis WC, Boeckh M, et al. Hepatic mucormycosis in a bone marrow transplant recipient who ingested naturopathic medicine. Clin Infect Dis. 1996;22(3):521–4.
Mead JH, Lupton GP, Dillavou CL, et al. Cutaneous Rhizopus infection. Occurrence as a postoperative complication associated with an elasticized adhesive dressing. JAMA. 1979;242(3):272–4.
Mitchell SJ, Gray J, Morgan ME, et al. Nosocomial infection with Rhizopus microsporus in preterm infants: association with wooden tongue depressors. Lancet. 1996;348(9025):441–3.
Waldorf AR, Ruderman N, Diamond RD. Specific susceptibility to mucormycosis in murine diabetes and bronchoalveolar macrophage defense against Rhizopus. J Clin Invest. 1984;74(1):150–60.
Diamond RD, Haudenschild CC, Erickson 3rd NF. Monocyte-mediated damage to Rhizopus oryzae hyphae in vitro. Infect Immun. 1982;38(1):292–7.
Ibrahim AS, Spellberg B, Walsh TJ, et al. Pathogenesis of mucormycosis. Clin Infect Dis. 2012;54 Suppl 1:S16–22. This article outlines the pathogenesis of mucormycosis and is important mostly because of the receny of its publication.
Sugar AM. Agents of mucormycosis and related species. Principles and practice of infectious diseases. 3rd ed. 1990. p. 1962–72.
Nittala S, Subbarao GC, Maheshwari A. Evaluation of neutropenia and neutrophilia in preterm infants. J Matern Fetal Neonatal Med. 2012;25 Suppl 5:100–3.
Power Coombs MR, Kronforst K, Levy O. Neonatal host defense against Staphylococcal infections. Clin Dev Immunol. 2013;2013:826303.
Filias A, Theodorou GL, Mouzopoulou S, et al. Phagocytic ability of neutrophils and monocytes in neonates. BMC Pediatr. 2011;11:29.
Bjorkqvist M, Jurstrand M, Bodin L, et al. Defective neutrophil oxidative burst in preterm newborns on exposure to coagulase-negative staphylococci. Pediatr Res. 2004;55(6):966–71.
Tekin R, Yalcin O, Yilmaz SM, et al. Gastrointestinal mucormycosis causing an acute intestinal obstruction in neonate patient. J Microbiol Infect Dis. 2011;1:35–7.
Cherney CL, Chutuape A, Fikrig MK. Fatal invasive gastric mucormycosis occurring with emphysematous gastritis: case report and literature review. Am J Gastroenterol. 1999;94(1):252–6.
Anand J, Ghazala K, Chong VH. Massive lower gastrointestinal bleeding secondary to colonic mucormycosis. Med J Malays. 2011;66(3):266–7.
Kosloske AM. Epidemiology of necrotizing enterocolitis. Acta Paediatr Suppl. 1994;396:2–7.
Horbar JD, Carpenter JH, Badger GJ, et al. Mortality and neonatal morbidity among infants 501 to 1500 grams from 2000 to 2009. Pediatrics. 2012;129(6):1019–26.
Woodward A, McTigue C, Hogg G, et al. Mucormycosis of the neonatal gut: a “new” disease or a variant of necrotizing enterocolitis? J Pediatr Surg. 1992;27(6):737–40.
Spellberg B. Gastrointestinal mucormycosis: an evolving disease. Gastroenterol Hepatol. 2012;8(2):140–2. This article reviews cases of GI mucormycosis in the setting of inflammatory bowel disease. Though not speicifically about GI mucormycosis in neonates, it contains a useful section on of the pathogenesis of GI mucormycosis and risk factors for this disease.
Walsh TJ, Skiada A, Cornely OA, et al. Development of new strategies for early diagnosis of mucormycosis from bench to bedside. Mycoses. 2014;57 Suppl 3:2–7. Early diagnosis and treatment is the key to lowering mortality associated with mucormycosis This article discusses the challenges with diagnosis and outlines various strategies being used to develop new approaches to diagnosing mucormycosis.
Guarner J, Brandt ME. Histopathologic diagnosis of fungal infections in the 21st century. Clin Microbiol Rev. 2011;24(2):247–80.
Walsh TJ, Gamaletsou MN, McGinnis MR, et al. Early clinical and laboratory diagnosis of invasive pulmonary, extrapulmonary, and disseminated mucormycosis (zygomycosis). Clin Infect Dis. 2012;54 suppl 1:S55–60. This ia very comprehensive review of all the available diagnostic modalities for mucormycosis including direct visualization of the organism in pathology, culture, and molecular techniques.
Hayden RT, Qian X, Procop GW, et al. In situ hybridization for the identification of filamentous fungi in tissue section. Diagn Mol Pathol. 2002;11(2):119–26.
Kasai M, Harrington SM, Francesconi A, et al. Detection of a molecular biomarker for zygomycetes by quantitative PCR assays of plasma, bronchoalveolar lavage, and lung tissue in a rabbit model of experimental pulmonary zygomycosis. J Clin Microbiol. 2008;46(11):3690–702.
Chamilos G, Lewis RE, Kontoyiannis DP. Delaying amphotericin B-based frontline therapy significantly increases mortality among patients with hematologic malignancy who have zygomycosis. Clin Infect Dis. 2008;47(4):503–9.
Zaoutis TE, Roilides E, Chiou CC, et al. Zygomycosis in children: a systematic review and analysis of reported cases. Pediatr Infect Dis J. 2007;26(8):723–7. This article reviews 157 cases of all types of mucormycosis, specifically in children. It contains useful information on clinical manifestation, treatment, and outcomes.
Ananda-Rajah MR, Kontoyiannis D. Isavuconazole: a new extended spectrum triazole for invasive mold diseases. Future Microbiol. 2015;10:693–708.
Spellberg B, Ibrahim A, Roilides E, et al. Combination therapy for mucormycosis: why, what, and how? Clin Infect Dis. 2012;54 suppl 1:S73–8. This is a comprehensive review of the various treatment options for mucormycosis. It provides in information on which treatment combinations have clinical data and which combinations only have in vitro or animal data.
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Snigdha Vallabhaneni and Rajal K. Mody declare that they have no conflict of interest.
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This article does not contain any studies with human or animal subjects performed by any of the authors.
The findings and conclusions in this paper are those of the authors alone and do not necessarily represent the official views of the Centers for Disease Control and Prevention.
This article is part of the Topical Collection on Epidemiology of Fungal Infections
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Vallabhaneni, S., Mody, R.K. Gastrointestinal Mucormycosis in Neonates: a Review. Curr Fungal Infect Rep 9, 269–274 (2015). https://doi.org/10.1007/s12281-015-0239-9