Home            Past Issues            About IP            About IAP           Author Information            Subscription            Advertisement              Search  

   
case reports

Indian Pediatr 2013;50: 699-701

Neonatal Zygomycosis with Gastric Perforation

NB Mathur and Aashima Gupta

From Referral Neonatal Unit, Department of Pediatrics, Maulana Azad Medical College, New Delhi, India.

Correspondence to: Dr NB Mathur, Dir Prof (Pediatrics),  MAM College, New Delhi 110 002, India.
Email: [email protected] 

Received: October 23, 2012;
Initial review: November 08, 2012;
Accepted: March 14, 2013.

 


Zygomycosis is a rare infection in neonates. The clinical presentation is non-specific and diagnosis most often is made at autopsy. Surgical debridement performed early improves survival. We report a case of neonatal zygomycosis with gastric perforation.

Keywords: Neonate, Zygomycosis, Intestinal perforation.



Neonatal zygomycosis occurs rarely with only 59 cases described in English literature till 2007 [1,2]. Skin is the most common point of entry for these patients. Premature infants represent 72% of cases and the overall mortality rate varies from 64-75% [1,2]. Gastrointestinal tract (GIT) involvement results from ingestion of fungal spores and prematurity is an important predisposing factor [1,3]. Early initiation of therapy is crucial in maximising outcomes and optimal management strategies have not been defined [4]. We present a case of neonatal zygomycosis with gastric perforation.

Case Report

A four-day-old male baby weighing 1880 grams presented with abdominal distension for 30 hours. He was born vaginally at 34 weeks in a private nursing home and had cried immediately after birth. He received oxygen, intravenous fluids and intravenous amikacin and piperacillin-tazobactum for tachypnea, and formula feeds by nasogastric tube by day 2 of life. The baby developed abdominal distension on day 3 and feeds were stopped. The baby was referred to us in view of no improvement. At admission, he was hypothermic (axillary temperature 35.5o C), heart rate was 160/min, respiratory rate was 62/min and capillary refill time was normal. The oxygen saturation on room air was 84%. Examination revealed tense distended abdomen with absent bowel sounds. Rest of systemic examination was normal. Investigations revealed hemoglobin 17.9 g%, total leucocyte count (TLC) 5050 cells/mm3 (Polymorphs 79%, Lymphocytes 17%), and platelet count 1.83 lac/mm3. The immature: total neutrophil ratio was 20% and micro erythrocyte sedimentation rate was 10 mm at the end of first hour. Cerebrospinal fluid examination was normal. Baby was administered oxygen by nasal prongs, intravenous fluids and antibiotics (piperacillin- tazobactum and amikacin) were continued. X- ray abdomen revealed free gas under diaphragm. At 12 hours of admission, exploratory laparotomy was performed, which revealed gangrenous perforation (1 × 2 cm) of anterior wall of stomach with loculated fluid collection in peritoneal cavity. Excision of the gangrenous portion and feeding jejunostomy was done. Baby continued to have bilious nasogastric tube aspirates in the post operative period. He developed leucopenia (TLC 4500/mm3) and thrombocytopenia (platelet count- 78,000/mm3) by fifth post-operative day. Blood urea was high (58mg/dL). Blood cultures were negative. Gastric wall biopsy received on eighth post-operative day revealed gangrenous segment with fungal colonies of zygomycetes with angioinvasion (Fig. 1). Zygomycetes was also isolated from gastric aspirate samples on eight post-operative day. The baby was started on intravenous fluconazole on day 8 of hospitalisation. Jejeunostomy feeds (expressed breast milk) were initiated by day 8 of antifungal therapy. Repeat gastric aspirates were negative. Both leucopenia and thrombocytopenia began to normalise by day 14 of therapy. The baby started gaining weight and was transited to full jejunostomy feeds by day 16 of therapy. However, parents took the baby home against medical advice for personal reasons on day 21 of treatment.

Fig.1 Ulceration of the gastric mucosa with ulcer bed showing broad branching fungal hyphae (arrow) entrapped in the inflammatory granulation tissue (HCE×40). Inset shows hyphae stained black.

Discussion

Zygomycosis is the new taxonomic classification which includes organisms in the order Entomophthorales and Mucorales [1]. The fungi in the order Mucorales have been associated with human disease; and are collectively referred to as zygomycetes [5]. The predisposing factors for zygomycosis include prematurity, asphyxia, umbilical catheterization and assisted ventilation [1,6]. Gastrointestinal tract is the most commonly involved site in neonates followed by cutaneous involvement [2]. Skin is the most common portal of entry in neonates with adhesive tape, monitor leads or central venous access sites predominating. Gastrointestinal infection results from ingestion of fungal spores either from environment or from colonised upper airways. Occasionally hematogenous or direct extension route can result in intestinal involvement [1]. All sites in GIT can be involved with stomach and colon being most preferred sites [1]. Premature infants with gastrointestinal mucormycosis may experience necrotising enterocolitis with or without pneumatosis intestinalis. Perforation after bowel wall necrosis is a common occurrence [1,3]. The baby is generally sick with low counts and may show a poor response to conventional chemotherapy [2]. The diagnosis is made on histological examination which may reveal thrombosis of the small gut vessels with angio-invasion [1,2,5]. Microbiological cultures are generally negative for fungi. 56% neonates were diagnosed as zygomycosis on histology and 44% were diagnosed on histology and culture [2]. The identification of the zygomycete species is generally not performed in most clinical laboratories due to inexperience in the recognition of microscopic differences among species, unavailability of tester strains to perform mating studies, or a lack of access to materials needed to perform molecular diagnostics. Microscopic evidence of a zygomycete in clinical material from a deep tissue biopsy is sufficient for the confirmed diagnosis of zygomycosis.

The case fatality is high, especially in premature neonates [1]. In an in vitro study of 37 isolates of zygomycosis, the mean inhibitory concentration of various antifungals were reported [7]. Amphotericin B has been used in most trials, though occurrence of side effects limits its use [4, 8]. Liposomal amphotericin is less toxic but often not affordable due to its high cost. Azoles have been tested and have proved efficacious. Funada, et al. [9] reported improvement in a patient with pulmonary mucormycosis with fluconazole. Similarly, Selcen, et al. [10] reported complete recovery with fluconazole in a diabetic child with mucormycosis. There is a role of newer therapies like hyperbaric oxygen, immunotherapy and iron chelation [4,8].

Mucor is angioinvasive hence surgical debridement is necessary for radical cure [1,2,4]. Most patients need repeated surgical debridement. Outcome was seen to be adversely affected in neonates where surgical debridement could not be performed [4,8].

To conclude, zygomycosis is a rare life threatening infection in neonates. It should be suspected in neonates presenting as acute abdomen. Early diagnosis combined with medical and aggressive surgical approach is the mainstay of therapy.

Contributors: NBM: case management, finalisation of the manuscript and will act as guarantor; AG: case management and drafting of manuscript.

Funding : None; Competing interest : None stated.

References

1. Wiedermann BL. Zygomycosis. In: Feigin RD, Cherry JD, Demmler GJ, Kaplan SL, eds. Textbook of Pediatric Infectious Diseases. 5th ed. Philadelphia: Saunders; 2004. p. 2633-40.

2. Roilides E, Zaoutis TE, Katraqkuo A, Benjamin DK Jr, Walsh TJ. Zygomycosis in neonates: an uncommon but life threatening infection. Am J Perinatol. 2009;26: 565-73.

3. Malclonalo YA, Baker CJ, Miller MJ. Phycomycosis. In: Remington JS, Klein JO, Wilson CB, Baker CJ, eds. Infectious Diseases of the Fetus and Newborn Infant. 6th ed. Philadelphia: Elsevier; 2006. P. 1157-9.

4. Spellberg B, Walsh TJ, Kontoyiannis DP, Edwards Jr J, Ibrahim AS. Recent advances in the management of mucormycosis:from bench to bedside. Clin Infect Dis. 2009;48:1743-51.

5. Iwen PC, Thapa I, Bastola D. Review of methods for the identification of zygomycetes with an emphasis on advances in molecular diagnostics. Lab Medicine. 2011;42:260-6.

6. Veleminsky M Sr, Hanzl M, Veleminsky M Jr. Necrotising enterocolitis in children with low birth weight induced with mucormycosis strains. Neuroendocrinol Lett. 2008;29:1021-5.

7. Sun QN, Fothergill AW, McCarthy DI, Rinaldi MG, Graybill JR. In Vitro activities of posaconazole, itraconazole, voriconazole, amphotericin B and fluconazole against 37 clinical isolates of zygomycetes. Antimicr Agents Chemother. 2002;46:1581-2.

8. Rogers TA. Treatment of zygomycosis: current and new options. J Antimicr Chemother. 2008;61:i35-i40.

9. Funada H, Miyake Y, Kanamori K, Okafuji K, Machi T, Matsuda T. Fluconazole therapy for pulmonary mucormycosis complicating acute leukemia. Jpn J Med. 1989;28:228-31.

10. Selcen D, Secmeer G, Aysun S, Kanra G, Onerci M, Gokoz A, et al. Mucormycosis in a diabetic child and its treatment with fluconazole: a case report. Turk J Pediatr. 1995;37:165-8.

 

Copyright 1999-2012 Indian Pediatrics