We report a case of invasive retroperitoneal zygomycotic infection caused by Basidiobolus ranarum in a healthy 8-year-old boy. The youngster responded dramatically to potassium iodide. The clinical and pathological features are reviewed to highlight the problems encountered in the management of this rare infection

Alam, M

Hasan, Rumina

Moazam, Farhat

Nazir, Zafar

Pervez, Shahid

English

eCommons@AKU

eCommons@AKUSection of Paediatric Surgery Department of SurgeryJanuary 1997Invasive retroperitoneal infection due tobasidiobolus ranarum with response to potassiumlodide--case report and review of the literatureZafar NazirAga Khan University, zafar.nazir@aku.eduR HasanAga Khan UniversityS PervaizAga Khan UniversityM AlamAga Khan UniversityF MoazamAga Khan UniversityFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_paediatrPart of the Pediatrics Commons, and the Surgery CommonsRecommended CitationNazir, Z., Hasan, R., Pervaiz, S., Alam, M., Moazam, F. (1997). Invasive retroperitoneal infection due to basidiobolus ranarum withresponse to potassium lodide--case report and review of the literature. Annals of Tropical Paediatrics International Child Health, 17(2),161-164.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_paediatr/16Full Terms & Conditions of access and use can be found athttp://www.tandfonline.com/action/journalInformation?journalCode=ypch20Annals of Tropical PaediatricsInternational Child HealthISSN: 0272-4936 (Print) 1465-3281 (Online) Journal homepage: http://www.tandfonline.com/loi/ypch19Invasive retroperitoneal infection due toBasidiobolus ranarum with response to potassiumiodide—case report and review of the literatureZ. Nazir, R. Hasan, S. Pervaiz, M. Alam & F. MoazamTo cite this article: Z. Nazir, R. Hasan, S. Pervaiz, M. Alam & F. Moazam (1997) Invasiveretroperitoneal infection due to Basidiobolus￿ranarum with response to potassium iodide—casereport and review of the literature, Annals of Tropical Paediatrics, 17:2, 161-164, DOI:10.1080/02724936.1997.11747880To link to this article:  https://doi.org/10.1080/02724936.1997.11747880Published online: 13 Jul 2016.Submit your article to this journal Article views: 5Citing articles: 13 View citing articles Annals of Tropical Paediatrics ( 1997) 17, 161-164 Invasive retroperitoneal infection due to Basidiobolus ranarum with response to potassium iodide-case report and review of the literature Z. NAZIR, R. HASAN*, S. PERVAIZ**, M. ALAM* & F. MOAZAM Departments of Surgery, * Microbiology and **Pathology, The Aga Khan University, Karachi, Pakistan (Accepted 2 August 1996) Summary We report a case of invasive retroperitoneal zygomycotic infection caused by Basidiobolus ranarum in a healthy 8-year-old boy. The youngster responded dramatically to potassium iodide. The clinical and pathological features are reviewed to highlight the problems encountered in the management of this rare infection. Introduction Zygomycotic infections are caused by fungi belonging to the orders Mucorales and Ento-mophthorales. Basidiobolus and eonidiobolus species (Entomophthorales) cause a chronic, inflammatory granulomatous disease collec-tively called entomophthoromycosis, reported in otherwise healthy inhabitants of tropical and subtropical regions. 1 The disease is gener-ally restricted to the subcutaneous tissues of the trunk and extremities or the mucosa of nasal and paranasal regions. 1•2 Deeply invasive zygomycosis due to B. ranarum involving the lungs, liver, muscles and gastro-intestinal tract, either primary or secondary to subcuta-neous disease, is rare and affects mainly im-munocompromized hosts. 2-4 If left untreated, it results in severe disfigurement and can be life-threatening. We report a case of primary invasive retroperitoneal zygomycosis due to B. ranarum in a healthy 8-year-old boy. The ob-jective is to highlight the difficulties encoun-Reprint requests to: Dr Zafar Nazir, Department of Surgery, The Aga Khan University, PO Box 3500, Stadium Road, Karachi 74800, Pakistan. Fax: 92 21 4934294. 0272-4936/97/020161-04 tered in diagnosing and treating this rare infection. Case report In October 1994, a previously healthy 8-year-old boy from a rural province in Pakistan presented with right flank pain, low grade fe-ver and weight loss of 2 months duration. There were no gastro-intestinal or pulmonary symptoms. His past medical history was unre-markable apart from a negative appendicec-tomy at a local hospital 5 months prior to admission. There was no evidence suggestive of immunodeficiency and his immunizations were up to date. At presentation, results of the physical examination were normal, except for evidence of weight loss (<lOth percentile) and mild tenderness in the right flank. A well healed scar was visible in the right lower quad-rant. Laboratory data revealed haemoglobin 11.4 g/dl, haematocrit 36%, wee 17,300/ mm3, neutrophils 57%, lymphocytes 26%, eosinophils 16%, and monocytes 1%. The ESR was 30 mm at the end of 1 hour. The biochemical profile and the results of a © 1997 The Liverpool School of Tropical Medicine 162 Z. Nazir et al. chest X-ray were normal. An ultrasound and eT scan of the abdomen revealed a 5 X 6 cm irregular, solid mass in the right suprarenal area. A 24-hour urine sample for vanillyl man-delic acid (VMA) was normal. At exploratory laparotomy a firm, inflammatory mass adher-ent to the upper pole of the right kidney and the undersurface of the liver was resected. Histopathology revealed a chronic granuloma-tous inflammation together with many fungal hyphae. The hyphae were broad and refractile, surrounded by acidophilic acellular material (Splendore-Hoppeli phenomenon). The infiltrate also contained numerous polymorphs and eosinophils. While awaiting microbiology reports and with a provisional diagnosis of mucormycosis, the boy was started on ampho-tericin B (1.5 mg/kg!day). Over the next 4 weeks he showed no response to the treat-ment. He continued to remain febrile, sus-tained a further weight loss of 3 kg and developed a firm, mildly tender, visible swell-ing in the right thoracolumbar region. Tissues submitted at surgery for routine, acid-fast ba-cillus (AFB) and fungal cultures failed to re-veal any growth of organisms. Laboratory data 4 weeks after surgery revealed a haemoglobin of 8.9 g/dl, haematocrit 25.9%, wee 24 200/ mm3, neutrophils 65%, lymphocytes 26%, eosinophils 7%, monocytes 2%, platelets 797 000/mm3 and ESR 80 mm in the 1st hour. The total bilirubin was 4.3 mg!dl, gamma GT 156 lUll, AL T 286 lUll, and alkaline phos-phatase 358 lUll. Serum creatinine and elec-trolytes were normal. Total proteins were 6.4 g!dl, albumin 2.0 g!dl and globulin 4.4 g! dl. lmmunoelectrophoresis revealed no evi-dence of monoclonal gammopathy. A tuberculin purified protein derivative (PPD) skin test was positive and HIV-1 antibody was not detected by EIA. A bone marrow aspir-ation showed reactive changes. Repeat cul-tures of blood, bone marrow, urine, sputum and tissue obtained from the thoracolumbar mass were again reported to be negative. A repeat chest X-ray and eT scan of the abdo-men and thorax at this stage revealed recur-rence of the suprarenal mass with extension into the muscles of the thoracic wall (Fig. 1). The boy was re-examined in December 1994 with a view to debulk the lesion. Due to wide-spread infiltration of the chest wall and overly-ing muscles, the surgical procedure were abandoned after taking adequate tissue sam-ples for histology, microbiology and mycologi-cal studies. The results of repeat histology were similar to that reported previously and the boy continued to deteriorate, in spite of amphotericin B. Five days after surgery, tissue cultures on Sabouraud-dextrose agar at 37°e and 30°e revealed colonies which appeared flat, waxy and greyish-white and showed radial folding suggestive of B. ranarum. As the colon-ies grew older, short aerial hyphae filled with spores became evident. A small piece of a colony from Sabouraud-dextrose agar mounted in lactophenol cotton blue showed numerous globose smooth-walled zygospores with a beak attached to one side (Fig 2). Sev-eral conidia containing meristospores were also observed. There was no growth on my-cosal agar. After morphological confirmation of B. ranarum, amphotericin B was discontin-ued and a saturated solution of potassium iodide (KI) (30 mg/kg!day) was started orally. The boy became afebrile within a week and there was a noticeable decrease in the thora-columbar swelling. Within 6 weeks of this treatment, the thoracolumbar mass and the suprarenal lesion resolved completely, as doc-umented on a eT scan of the abdomen and FIG. 1. CT scan of the upper abdomen/chest showing an infiltrating Basidiobolus ranarnm lesion extending into the abdominal and thoracic wall. Infections due to Basidiobolus ranarum 163 FIG. 2. Zygospore with 'beak' in culture mount of Basidiobolus ranarum (X 240). thorax. The boy when last seen in February 1996 was asymptomatic and had gained 4.5 kg in weight. Discussion B. ranarum is a normal inhabitant of soil and has been isolated from decaying vegetation, mammalian dung, insects and the gastro-intes-tinal tracts of many amphibians and reptiles.1'2 The mode of transmission of infection to human beings remains unknown. It is gener-ally assumed that infection is acquired by direct contact with the contaminated material. Minor trauma, e.g. intramuscular injections and insect bites, have been implicated.1'2,5-7 In the boy presented here,_ inoculation into the retroperitoneum during appendicectomy may have been the mode of transmission. In spite of its ubiquitous distribution, zygomycosis caused by B. ranarum is rare in humans, suggesting low virulence of the organism. Since the first description of the infection in a patient from Indonesia by Joe et al. in 1956,8 approximately 300 cases have been reported in the world literature, mostly from tropical Asia, Africa and South Amer-ica.1-8 To our knowledge this is the first cul-ture-proven case of this rare infection from Pakistan and probably the first report of one affecting primarily the retroperitoneum. Entomophthoromycosis due to B. ranarum is primarily a disease of children. A majority of the reported cases have been in children under 10 years of age, predominantly boys (the male to female ratio varies from 3:1 to 6:1).1'2' 6 The disease is generally restricted to the subcuta-neous tissues of the trunk and extremities of previously healthy individuals. 1,2,6 The involvement of deeper structures such as the lung, liver, muscle and gastro-intestinal tract, either primarily or secondary to subcutaneous tissue infection, has rarely been described in the absence of predisposing factors such as diabetes mellitus, malnutrition and immuno-suppressive disorders. 2-4,? Liberation of lysolecithin and proteinase by the fungus and transient immunosuppression which may occur during common viral infections and surgery have been postulated as factors responsible for invasive and progressive dis-ease in previously healthy individuals. 1 With the increase in human immuno'deficiency virus (HIV) infections, this infection may be seen more frequently in the future. Diagnosis requires a high index of suspicion among physicians working in tropical areas. Due to the nature of their presentation, these 164 Z. Nazir et al. infections can be confused with malignant lesions such as Burkitt's lymphoma or chronic infections such as tuberculosis. 1•9 Biopsy of the lesion with histological and microbiological evaluations are most effective in making the correct diagnosis. Serological tests are not yet available as diagnostic aids. Histologically, lesions produced by B. ranarum are characterized by an acute and/or chronic inflammatory reaction in association with broad, irregular (8-32 mm in diameter), erratically septate hyphae, each surrounded by a distinctive eosinophilic sheath (Splendore-Hoppeli phenomenon) on eosin-haematoxylin staining. In contrast with mucormycosis, the tendency to vascular invasion, tissue infarction and necrosis is uncommon. 1•2 The colonies of B. ranarum are flat, folded, furrowed, greyish and waxy in consistency, whereas the zygospores are 20-50 mm in diameter and have a prominent 'beak' on one side, which is characteristic of the order Entomophthorales (Fig. 2). No standardized treatment is available for this infection as no single agent has proved to be totally effective against B. ranarum. Evalu-ation of treatment of this rare infection is difficult because of the rarity of its occurrence in humans and occasional reports of spon-taneous resolution. 1 Therapeutic trials using amphotericin B, oral azole antifungals (keto-conazole, itraconazole and fluconazole), rifampicin, Kl and trimethoprim/sulfamethox-azole have revealed variable responses. 1•2 In vitro susceptibility studies by Yangco et al. 10 have shown minimal inhibitory effects of amphotericin B, miconazole and 5-fluoro-cytosine. Kl, although demonstrating no in vitro activity against B. ranarum, proved to be dramatically effective in the patient in this report. The proposed mechanism of action is reported to be resolution of granulomas by enhancement of proteolysis and phagocyto-sis. 10 A direct anti-fungal effect of iodine can-not be discounted. Ketoconazole, an orally administered antifungal drug, has been shown to be effective in both in vitro and in vivo studies and may be considered as an adjuvant or alternative to Kl.7•10 The role of surgery in entomophthoromycosis should be limited to biopsies aimed at establishing a diagnosis as extensive procedures can lead to spread of the infection. References Sugar AM. Agents of mucormycosis and related species. In: Mandell GL, Douglas RG, Bemett ]E, eds. Principals and Practice of Infectious Diseases. New York: Churchill Livingstone, 1990; 1962-72. 2 Rippon JW. Zygomycosis. In: Rippon JW, ed. Medical Mycology-The Pathogenic Fungi and The Pathogenic Actinomycetes, 3rd edn. Philadel-phia: WB Saunders, 1988; 681-713. 3 Dworzack DL, Pollock AS, Hodges GR, Bames WG, Ajello L, Padhye A. Zygomycosis of maxillary sinus and palate caused by Basidiobolus haptosporus. Arch Intern Med 1978; 138:1274-5. 4 Schmidt JH, Howard RJ, Chen JL, Pierson KK. First culture proven gastrointestinal entomoph-thoromycosis in the United States: A case report and review of the literature. Mycopathologia 1986; 95:101-4. 5 Antonelli M, Vignetti P, Dahir M, Mohamed MS, Favah AA. Entomophthoromycosis due to Basi-diobolus in Somalia. Trans R Soc Trop Med Hyg 1987; 81:186-7. 6 Scholtens REM, Harrison SM. Subcutaneous phy-comycosis. Trop Geogr Med 1994; 46:371-3. 7 Kamalam A, Thambiah AS. Muscle invasion by Basidiobolus haptosporus. Sabouraudia: J Med Vet Mycol 1984; 22:273-7. 8 Joe LK, Njo-Imjo TE. Basidiobolus ranarum as a cause of subcutaneous phycomycosis in Indonesia. Arch Dennatol 1956; 7 4:378-83. 9 Bittencourt AL, Serra G, Sadigursky M, Aranjo MDGS, Campos MCS, Sampaio LCM. Subcuta-neous zygomycosis caused by Basidiobolus hap-tosporus: presentation of a case mimicking Burkitt's lymphoma. Am J Trop Med Hyg 1982; 31:370-3. 10 Yangco BG, Okafor Jl, Testrake D. In vitro suscep-tibilities of human and wild type isolates of Basi-diobolus and Conidiobolus species. Antimicrob Agents Chemother 1984; 25:413-16. 

Invasive retroperitoneal infection due to basidiobolus ranarum with response to potassium lodide: Case report and review of the literature

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Invasive retroperitoneal infection due to basidiobolus ranarum with response to potassium lodide: Case report and review of the literature

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