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Entomophthoromycosis
Entomophthoromycosis
Entomophthoromycosis
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Article history: Aim: Subcutaneous entomophthoromycosis (EM) is an uncommon fungal infection of childhood. This article is
Received 22 July 2014 intended to draw the attention of pediatric surgeons to the fact that EM can mimic soft-tissue tumor.
Received in revised form 23 September 2014 Methods: It is a retrospective review of 16 children treated for subcutaneous EM between 2000 and 2013.
Accepted 13 November 2014 Results: The median age of patients was 3.5 years. The typical lesion was a discoid subcutaneous mass that can be
easily lifted from deeper tissues (the doughnut lifting sign). Lesions were mostly distributed in the lower half of
Key words:
body. All the patients were immunocompetent. Correct clinical diagnosis was made only in 4 cases while others
Zygomycosis
Phycomycosis
were mistaken for a tumor. All the 8 children who underwent wide excision of the pseudotumor had local recur-
Pseudo-tumor rence. Supersaturated solution of potassium iodide was curative in 11 cases while addition of itraconazole was
Fungal infection needed in one case. One child died of muti-drug resistant infection. The mean treatment duration was 4.7 months
Potassium iodide months (range 2–8 months).
Differential diagnosis Conclusion: Subcutaneous EM can mimic soft-tissue tumor. High index of suspicion is essential to avoid misdiag-
Soft-tissue sarcoma nosis and inappropriate treatment. A newly described “doughnut-lifting sign’ may be helpful in clinical diagnosis.
Basidiobolus Emergence of multi-drug resistant infection is a source of concern.
Conidiobolus
© 2015 Elsevier Inc. All rights reserved.
Entomophthoromycosis (EM) is an uncommon fungal infection EM is predominantly a disease of pediatric age group [2,5]. In
caused by entomophthoromycota [1,2]. The phylum is named so be- Burkitt’s series 63% of patients were under the age of 9 and 30% were
cause the fungi are often used as bio-insecticides (entomo = insect; adolescents while only 7% were adults [2]. The infection is mostly con-
phthoro = destroyer). Basidiobolus ranarum and Conidiobolus coronatus fined to subcutaneous tissue, although, disseminated forms [12–14]
are the two common opportunistic pathogens of this group. Both of and visceral involvement [10,15] are rarely reported. Even in endemic
them are saprophytes commonly found in soil and decaying organic areas, subcutaneous EM is frequently mistaken for soft tissue sarcoma
wastes. Accidental inoculation by inconspicuous injuries such as thorn or lymphoma [6,16,17]. Consequently, inappropriate investigations
pricks, scratches, insect bites and contaminated injection-needles may and delay in treatment are not uncommon. In this paper we narrate
cause human infections [3]. It was first described from Indonesia in our clinical experience with 16 cases of subcutaneous EM and intend
1956 by Lie Kian Joe who reported 2 children with Basidiobolus infection to draw the attention of pediatric surgeons to the deceptive clinical
[4]. Subsequently, Burkitt published a large series of 31 cases from presentation of this uncommon but emerging entity.
Uganda [2]. EM is common in tropical areas such as sub-Saharan
Africa [2], South East Asia [5], and Brazil [6]. However, probably due 1. Methods and materials
to international travels, it is increasingly been reported from non-
endemic areas such as USA [7], Australia [8], Germany [9], Arabian Between 2000 and 2013 the principal author (VR) was involved in
peninsula [10], Portugal [11] and Netherlands [12]. the management of 17 children suffering from EM at 4 different institu-
tions namely Rajah Muthiah Medical College (Chidambaram),
☆ Parts of the manuscript were read at the 12th annual meeting of Tamilnadu and Dhanvantri Medical Center (Chidambaram), Sri Ramasamy Memorial
Pondicherry Pediatric Surgeons (TPPS) on 28–30 July 2013, Kanyakumari, India. It was Medical College (Chennai) and Hindu Mission Hospital (Chennai).
awarded with the “Best Oral Presentation” prize.
⁎ Corresponding author at: 200. Fifth Street Viduthalai Nagar, Sunnambu Kolathur,
Clinical details of the patients were retrospectively reviewed. One case
Chennai 600117, India. Tel.: +91 94433 10182. was excluded as the case record could not be retrieved. One case, that
E-mail address: vrthiran@yahoo.co.in (V. Raveenthiran). has already been reported elsewhere [18], is included in the study.
http://dx.doi.org/10.1016/j.jpedsurg.2014.11.031
0022-3468/© 2015 Elsevier Inc. All rights reserved.
V. Raveenthiran et al. / Journal of Pediatric Surgery 50 (2015) 1150–1155 1151
Fig. 1. Difference in the colony morphology of Basidiobolus (A) and Conidiobolus (B). Flat, furrowed, waxy, yellow colonies are typical of Basidiobolus. In contrast, Conidiobolus colonies are
white, buff and powdery with short aerial mycelium.
Diagnosis of EM was accepted if the fungus is demonstrated in tests, estimation of serum electrolytes and electrocardiogram
either histology or culture. In addition to the routine Hematoxylin- (ECG) were done in patients receiving potassium iodide treatment.
eosin (HAE) stained histological preparation, special fungal stains Orally administered supersaturated solution of potassium io-
such as periodic acid-Schiff (PAS), Gomori’s methenamine silver dide (SSKI) was the treatment of our choice. The solution (not com-
(GMS) were used selectively. Presence of Splendore-Hoeppli mercially available) was prepared by hospital pharmacy by
phenomenon (encasement of fungal hyphae by eosinophilic debris dissolving pure crystals of potassium iodide in warm distilled
and eosinophilic leukocytes), presence of characteristic (broad, water and the solution was dispensed in light-proof glass bottles.
aseptate, empty-looking, twisted-ribbon like) fungal hyphae and The dosage of SSKI was 30 to 50 mg/kg/day [2,21]. SSKI contains ap-
absence of vascular invasion were considered diagnostic of EM proximately 47 mg of potassium iodide in each drop [21] Therefore
[19]. Minced tissue specimens were cultured in Sabouraud’s the rule of thumb for dosage was one drop of SSKI/kg/day. Treat-
dextrose agar medium. Fungal culture became routine only after ment was started with half the estimated dose (20 mg/kg/day)
2008 before which it was done sparingly when histological diagnosis and it was gradually increased over the next 4 to 6 days gauging
was difficult. Lactophenol cotton blue mount was used to study the the child’s tolerance. Patients who received oral SSKI were hospi-
fungal morphology. Basidiobolus and Conidiobolus were distin- talized for the initial 72 hours to monitor serum potassium levels
guished by their characteristic colony morphology (Fig. 1) and and cardiac arrhythmia. Thereafter, home administration of the
zygospores (Fig. 2). Serodiagnosis was not practiced. Routine in- drug was continued until the lesion was cured. Therapeutic end-
vitro antifungal susceptibility testing was established in 2011 when point was arbitrarily set at 8 weeks beyond complete resolution of
a multi-drug resistant case was encountered [20]. All the patients all palpable indurations. Drug resistance was suspected when the
were screened for immunodeficiency. Periodic thyroid function lesion did not show any resolution even after 3 weeks of treatment.
Fig. 2. Morphological difference in the zygospore of Basidiobolus (A) and Conidiobolus (B). Beak-shaped remnants of copulation tubes (arrow) are typical of Basidiobolus. Papilla protruding
from conidia (arrow) is characteristic of Conidiobolus. Lactophenol cotton blue preparation 400×.
1152 V. Raveenthiran et al. / Journal of Pediatric Surgery 50 (2015) 1150–1155
Fig. 4. Clinical appearance of subcutaneous Entomophthoromycosis of abdominal wall. (A) Scar of previous wide excision (arrow) along with local recurrence of the mass (arrow heads) is
evident, (B) Doughnut-lifting sign demonstrated in the same patient.
V. Raveenthiran et al. / Journal of Pediatric Surgery 50 (2015) 1150–1155 1153
Fig. 6. Comparison of old and new classification emphasizing the change of nomenclature (Zygomycosis vs Entomophthoromycosis).
1154 V. Raveenthiran et al. / Journal of Pediatric Surgery 50 (2015) 1150–1155
Table 1
and voriconazole [31] are favored over other antifungals such as
Comparison of Mucormycosis (Zygomycota) and Entomophthoromycosis⁎.
flucytosine and fluconazole [28]. Not infrequently more than one anti-
Mucormycosis Entomophthoromycosis † fungal drug are administered concurrently to achieve cure [22,29,32].
Immune status Immuno-compromised Immuno-competent Recent reports [29,30] suggest that the fungi are increasingly resistant
of patients to a variety of antifungal drugs. In our series, a 15-month-old boy died
Vascular Affinity Yes No
due to extensive abdomino-perineal EM which was multidrug resistant.
of fungus
Tissue Gangrene Frequent & Extensive Nil
SSKI, itraconazole, amphotericin B and co-trimoxazole were tried in se-
Tumor like lesions Never seen Common manifestation quence and combination without any benefit. Posaconazole [30] and
Tissue invasion Usually deep tissues Usually subcutaneous voriconazole [31], which are currently recommended for resistant
Frequently visceral Rarely visceral fungi, was not used because of its non-availability then. We do not
Frequently affected Rhinocerebral Limbs and trunk in
have experience with other rescue drugs, such as dapsone, described
area BB face in CB
Splendore-Hoeppli Absent Present in the literature. Usually, EM has excellent prognosis although fatal in-
phenomenon in fections have been reported especially in immunocompromised pa-
histology tients [12,24]. Mortality is common with visceral [12], facial or
Usefulness of SSKI Nil Yes perineal [2,32] involvement. The child who died in the series had peri-
Use of Amphotericin-B Drug of Choice Frequently Resistant
Role of surgery Aggressive debridement Limited to Biopsy
neal involvement. It leads us to think that superadded bacterial sepsis
is cardinal Extensive excision is rather than the fungus per se could be the cause of death.
counterproductive In conclusion, EM should be considered in the differential
Prognosis Frequently fatal Usually excellent diagnosis of slow growing subcutaneous mass. It should not be
Death is exceptional
mistaken for soft tissue tumors to avoid mismanagements. Increasing
SSKI - Supersaturated solution of potassium iodide. incidence of multidrug resistant strains and clinical fatalities are a
⁎ Until recently the term “zygomycosis” was used to mean both mucormycosis and
source of great concern.
entomophthoromycosis. Older literature should be interpreted with this caution.
†
Entomophthoromycosis includes basidiobolomycosis (BB) and Conidiobolomycosis (CB).
[22] Ramesh V, Ramam M, Capoor MR, et al. Subcutaneous zygomycosis: report of [27] Kaufman L, Mendoza L, Standard PG. Immunodiffusion test for serodiagnosing sub-
10 cases from two institutions in North India. J Eur Acad Dermatol Venereol cutaneous zygomycosis. J Clin Microbiol 1990;28:1887–90.
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[24] Choon SE, Kang J, Neafie RC, et al. Conidiobolo mycosis in a young Malaysian woman lamprauges recovered from sheep to antifungal agents. Vet Microbiol 2013;
showing chronic localized fibrosing leukocytoclastic vasculitis: a case report and 166:690–3.
meta-analysis focusing on clinicopathologic and therapeutic correlations with out- [30] Peel T, Daffy J, Thursky K, et al. Posaconazole as first line treatment for disseminated
come. Am J Dermatopathol 2012;34:511–22. zygomycosis. Mycoses 2008;51:542–5.
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zygomycosis caused by Basidiobolus ranarum at the injection site. Case Rep Infect Dis basidiobolomycosis with voriconazole without surgical intervention. J Trop Pediatr
2013. http://dx.doi.org/10.1155/2013/534192 [Article number 534192]. 2014;60:476–9.
[26] Page RM, Brungard J. Phototropism in Conidiobolus, Some Preliminary Observations. [32] Mendiratta V, Karmakar S, Jain A, et al. Severe cutaneous zygomycosis due to
Science 1961;3481:733–4. Basidiobolus ranarum in a young infant. Pediatr Dermatol 2012;29:121–3.