Case Report: Gastrointestinal Basidiobolomycosis in a Young Girl with Cystic Fibrosis and Celiac Disease.

Basidiobolomycosis is an uncommon fungal infection that has been reported in the literature mainly as a cause of infection in the skin and subcutaneous tissue. Intraabdominal infections have been reported in tropical and subtropical areas in the Middle East, such as Iran and Saudi Arabia, and in the United States. Our patient was a 6-year-old girl with cystic fibrosis and celiac disease who was referred to our department with a history of chronic abdominal pain. In the imaging studies of the abdomen and pelvis, a large retroperitoneal mass was reported in the right upper part of the abdomen with involvement of the duodenum and the mesentery of the small and large intestines, as well as the superior mesenteric vessels, and was diagnosed as basidiobolomycosis through biopsy. Because of the large unresectable mass, the patient was first treated with antifungal drugs for 2 months and then surgical resection was performed. The main point in the management of these patients is a combination of antifungal therapy and surgical resection. In some patients, complex surgeries such as the Whipple procedure may be performed to appropriately manage intraabdominal infections.

Colonic basidiobolomycosis in a patient with systemic lupus erythematosus (SLE).

BACKGROUND: Basidiobolus ranarum belongs to the Entomophthorales order and the Zygomycetes class. This fungus is an environmental saprophyte that can be found in soil and rotting vegetables.Primarily restricted to tropical regions including Asia, Africa, and South America. It might cause chronic inflammatory diseases, mostly affect subcutaneous tissue. Systemic infections involving the gastrointestinal tract are extremely rare. CASE PRESENTATION: Herein, we present a 44-year-old Persian man with the past medical history of lupus erythematosus with colicky abdominal pain started from three months before admission with many vomiting episodes, and a mass on the right lower quadrant, who had been thought initially to have an abdominal malignancy. The patient had vital signs were within normal ranges. His physical examination revealed tenderness and rebound tenderness on the right lower quadrant of the abdomen. A fixed mass 10 × 10 centimeter in diameter was palpated in the same quadrant. Laboratory, radiologic, colonoscopic examination was requested. The patient underwent laparotomy which revealed a mass in the terminal ileum and ascending colon with retroperitoneal adhesion and invasion to the right ureter behind it. Pathologic examination showed basidiobolomycosis infection in the specimen. CONCLUSION: Fungal infection should be among the differential diagnoses for adults present with abdominal mass in endemic regions of the world.

Entomophthoromycosis in a child: Delayed diagnosis and extensive involvement.

Entomophthoromycosis is a rare fungal infection of the skin and subcutaneous tissue occurring predominantly in tropical and subtropical regions. In children, it mostly affects the lower half of the body. With this, we report a case of Entomophthoromycosis in a 6-year-old girl who presented late with extensive involvement of the upper half of the body. She responded well to treatment with potassium iodide and itraconazole. We also reviewed cases of Entomophthoromycosis reported in children.

Conidiobolus pachyzygosporus invasive pulmonary infection in a patient with acute myeloid leukemia: case report and review of the literature.

BACKGROUND: Conidiobolus spp. (mainly C. coronatus) are the causal agents of rhino-facial conidiobolomycosis, a limited soft tissue infection, which is essentially observed in immunocompetent individuals from tropical areas. Rare cases of invasive conidiobolomycosis due to C. coronatus or other species (C.incongruus, C.lamprauges) have been reported in immunocompromised patients. We report here the first case of invasive pulmonary fungal infection due to Conidiobolus pachyzygosporus in a Swiss patient with onco-haematologic malignancy. CASE PRESENTATION: A 71 year-old female was admitted in a Swiss hospital for induction chemotherapy of acute myeloid leukemia. A chest CT performed during the neutropenic phase identified three well-circumscribed lung lesions consistent with invasive fungal infection, along with a positive 1,3-beta-d-glucan assay in serum. A transbronchial biopsy of the lung lesions revealed large occasionally septate hyphae. A Conidiobolus spp. was detected by direct 18S rDNA in the tissue biopsy and subsequently identified at species level as C. pachyzygosporus by 28S rDNA sequencing. The infection was cured after isavuconazole therapy, recovery of the immune system and surgical resection of lung lesions. CONCLUSIONS: This is the first description of C. pachyzygosporus as human pathogen and second case report of invasive conidiobolomycosis from a European country.

Gastrointestinal pythiosis with concurrent presumptive gastrointestinal basidiobolomycosis in a Boxer dog.

A 2-year-old female spayed Boxer dog was presented for a 1-month history of progressive hemorrhagic diarrhea with tenesmus and weight loss despite trial courses of antibiotics and diet change. Abdominal ultrasound revealed severe, focal thickening, and loss of normal architecture of the colonic wall with abdominal lymphadenomegaly. Dry-mount fecal cytology, performed on several consecutive days, consistently revealed numerous, round, 16-20 µm structures with basophilic, granular content, and a thin cell wall. Transmission electron microscopy identified these structures as fungi. Culture, polymerase chain reaction (PCR), and sequencing of the internal transcribed spacer, D1/D2 regions, and DNA-directed RNA polymerase II core subunit (RPB2) confirmed the presence of Basidiobolus microsporus in the feces. Biopsies collected via ileocolonoscopy revealed marked, multifocal, chronic, neutrophilic, and eosinophilic ileitis and colitis with ulceration, granulation tissue, and intralesional hyphae (identified with Gomori methenamine silver stain). A Pythium enzyme-linked immunosorbent assay and Pythium-specific PCR performed on the formalin-fixed paraffin-embedded biopsy specimens were positive while Basidiobolus-specific PCR was negative, thus confirming a diagnosis of pythiosis. This report describes a fatal case of colonic and intestinal pythiosis with the presence of fecal Basidiobolus sp. spores, suggestive of concurrent gastrointestinal basidiobolomycosis.

Disseminated intestinal basidiobolomycosis with mycotic aneurysm mimicking obstructing colon cancer.

Basidiobolomycosis is a rare fungal infection that may affect the gastrointestinal tract. It is caused by Basidiobolus ranarum and less than 80 cases have been reported in the literature. The incidence seems to be higher in the Middle East and in particular Saudi Arabia where most cases are diagnosed in the south-western region. An 18-year-old woman presented to the emergency department with an obstructing caecal mass initially suspected to be malignant. Surgical resection was complicated by bowel perforation, histology and cultures confirmed basidiobolomycosis infection. The postoperative course was complicated by an enterocutaneous fistula, fungal intra-abdominal abscesses, liver and lung abscesses, formation of mycotic hepatic artery aneurysm and meningoencephalitis. The patient eventually expired due to sepsis despite aggressive treatment. Diagnosis and management of such rare cases are very challenging and require a multidisciplinary approach. Complications are common and associated with a high mortality.

Neonatal Acute Liver Failure Associated with Angioinvasive Hepatic Zygomycosis.

BACKGROUND: Neonatal acute liver failure (NALF) is often a fatal condition. Zygomycosis is a fungal infection that is often fatal in both adults and infants. Only a few cases of hepatic zygomycosis are reported in the literature and they are invariably associated with immunosuppression. MATERIALS AND METHODS: Post-mortem liver biopsy from a 14-day old neonate demonstrated confluent panacinar necrosis with angioinvasive zygomycosis. The limited work-up could not rule out an underlying immunodeficiency. CONCLUSION: Angioinvasive hepatic zygomycosis can present in the neonatal period as NALF.

Basidiobolomycosis in Togo: clinico-pathological study of a series of 12 presumed cases.

OBJECTIVE: The purpose of our study was to describe the histological diagnosed of the Basidiobolomycosis cases from 1990 to 2017 (28 years) in the only Pathology Anatomy Laboratory in Togo. RESULTS: A total of 12 cases of suspected Basidiobolomycosis have been identified. The sex ratio (M/F) was 2. The average age of the patients was 24.8 ± 1.6 years. Six patients (6/12) had a pathological history: HIV infection (n = 4 cases) and tuberculosis (n = 2 cases). The clinical manifestations were localized to pure skin (n = 9 cases), skin and mucous digestive (n = 2 cases) and disseminated (n = 1 cases). Direct mycological examination and culture in 4 patients was positive in 3 patients. The samples examined consisted of 11 cutaneous biopsies measuring 1-3 cm and a biopsy of the intestinal mucosa. Histology showed granulomatous inflammation of the dermohypodermal site with numerous giant cells associated with eosinophilic polynuclear cells, in which there are 5-7 mm non-septate, irregular mycelial filaments. Patients were treated with ketoconazole at a dose of 10 mg/kg daily. The progression of the patients' condition was favorable after 4 weeks of treatment with a regression of the closets size. Patients were completely healed after 8 weeks of treatment, without recurrence after 6 months. No deaths have been recorded.

Basidiobolomycosis complicated by hydronephrosis and a perinephric abscess presenting as a hypertensive emergency in a 7-year-old boy.

A 7-year-old boy presented with a chronic, indurated, tender left thigh swelling in association with a hypertensive emergency. He had a bilateral moderate degree of hydronephrosis and a left perinephric abscess, and MRI features of posterior reversible encephalopathy syndrome. Histopathological examination of the biopsy specimen demonstrated eosinophilic fasciitis with filamentous fungi. Basidiobolus ranarum was isolated from the culture. The fungus was also isolated from a perinephric fluid aspirate. Computerised tomography of the abdomen demonstrated features consistent with fungal invasion of the pelvic floor muscles and urinary bladder, leading to bilateral hydronephrosis. He required multiple antihypertensive drug therapy and was treated with intravenous amphotericin B, oral itraconazole and potassium iodide. Antihypertensive agents were discontinued after 2 weeks of antifungal therapy. At 6-months follow-up, the hydronephrosis had resolved completely. Perinephric abscess associated with basidiobolomycosis has not been reported previously.

Cutaneous basidiobolomycosis: Seven cases in southern Benin.

BACKGROUND: Cutaneous basidiobolomycosis is the most common form of entomophthoramycosis. Herein we report seven cases of cutaneous basidiobolomycosis. PATIENTS AND METHODS: A retrospective observational study was conducted at the Buruli ulcer treatment centre in Pobè and at the national teaching hospital in Cotonou from 2010 to 2015. RESULTS: Seven cases of cutaneous basidiobolomycosis were diagnosed. The mean patient age was 9.53 years. There were 4 female and 3 male patients, all from southeast Benin. Clinically, the disease presented in all cases as a hard, well-defined, subcutaneous plaque with little inflammation, and which could easily be lifted from the deep structures but remained attached to the surface structures. The overlying skin was hyperpigmented. Plaques were localized to the buttocks or thighs. All patients had inflammatory anaemia with an accelerated erythrocyte sedimentation rate (30 to 70mm over the first hour), and a low haemoglobin count (8.7 to 11.4g/dL). Blood hypereosinophilia (650 to 3784elements/mm3) was present in six of the seven subjects. Histopathology (performed for 5 of the 7 subjects) showed granulomatous lesions with foreign-body giant cells, and inflammatory cells, with occasional eosinophils surrounding fungal hyphae (Splendore-Hoeppli phenomenon). Mycological analysis revealed Basidiobolus ranarum in three cases. The patients were treated with ketoconazole (5/7) and itraconazole (2/7), with good outcomes after 10 to 24 weeks of therapy. DISCUSSION: Cutaneous basidiobolomycosis is uncommon in southern Benin, with only seven cases being diagnosed over 6 years. The diagnosis of cutaneous basidiobolomycosis is a challenge in the field in Benin due to the non-specific clinical presentation, the lack of technical resources, and the existence of numerous differential diagnoses. CONCLUSION: Cutaneous basidiobolomycosis is an uncommon fungal infection in southern Benin chiefly affecting children.

Zygomycosis Presenting as Acute Bilateral Renal Artery Thrombosis in a Healthy Young Male.

We present a case of acute renal artery thrombosis due to disseminated zygomycosis in a healthy young adult male.The diagnosis of renal artery occlusion was made on contrast-enhanced CT (CECT) and confirmation of etiology was made only on post mortem biopsy. We suggest that the presence of vascular thrombosis on CECT in a patient presenting with febrile illness should be regarded as an indicator of possible infection by angiotropic fungi, such as zygomycosis, which could help clinician to pursue histological diagnosis aggressively.

Severe invasive pulmonary zygomycosis by Rhizomucor pusillus and concomitant severe bacterial endocarditis in acute promyelocytic leukaemia.

We describe a severe pulmonary invasive zygomycosis sustained by Rhizomucor pusillus and concomitant bacterial endocarditis in a woman diagnosed with acute promyelocytic leukaemia.

Diagnostic accuracy of morphologic identification of filamentous fungi in paraffin embedded tissue sections: correlation of histological and culture diagnosis.

AIMS AND OBJECTIVES: The aim was to investigate the correlation between histological and culture diagnosis of filamentous fungi. MATERIALS AND METHODS: Tissue sections from biopsy samples stained with Hematoxylin and Eosin and special stains from samples of chronic invasive/noninvasive sinusitis and intracranial space occupying lesions during 2005-2011 diagnosed to have infection due to filamentous fungi were reviewed. The histopathology and culture diagnoses were analyzed for correlation and discrepancy. RESULTS: There were 125 samples positive for filamentous fungi on biopsy. Of these 76 (60.8%) were submitted for culture and fungi grew in 30 (39.97%) samples. There was a positive correlation between histological and culture diagnosis in 25 (83.33%) samples that included Aspergillus species (16/19), Zygomycetes species (8/10) and dematiaceous fungi (1/1). The negative yield of fungi was more in Zygomycetes species (20/30) when compared to Aspergillus species (25/44). There was a discrepancy in diagnosis in 5/30 (16.67%) samples which included probable dual infection in two, and dematiaceous fungi being interpreted as Aspergillus species in three samples. CONCLUSION: Histopathology plays a major role in the diagnosis of infection due to filamentous fungi, especially when cultures are not submitted or negative. The discrepancy between histological and culture diagnosis was either due to dematiaceous fungi being interpreted as Aspergillus species or probable dual infection.

[Conidiobolus coronatus infections revealed by a facial tumor]. / Infection à Conidiobolus coronatus révélée par une tumeur de la face.

INTRODUCTION: Conidiobolomycoses (Conidiobolus coronatus fungal infections) are rare and potentially severe infections prevalent in the tropics. The disease starts in the facial sinus and evolves as a subcutaneous tumor on the mid face OBSERVATION: A 19-year-old female patient from Burkina Faso presented with an acquired nasal deformation having evolved for a few months, associated to bilateral nasal obstruction. The patient had no medical or surgical history. The pathological analysis of the surgical exeresis allowed diagnosing a C. coronatus infection. DISCUSSION: The C. coronatus lives in decaying vegetation in hot and humid climates. It is a potential human pathogen that infects immunocompetent patients presenting with micro-wounds of the sinus and nasal mucosa. Hundred cases have been reported. The management is specific. The diagnosis should be discussed in case of distorting tumors of the midface.

Fatal-mixed cutaneous zygomycosis-aspergillosis: a case report.

Mucorales and Aspergillus are molds responsible for infections in immunocompromised patients. In this report, we describe a case of a rare extensively mixed cutaneous infection caused by Lichtheimia ramosa, Aspergillus fumigatus and Aspergillus terreus in a neutropenic patient suffering from an acute leukemia. The fatal outcome of this patient can be attributed to its hematologic malignancy, the extensive nature of the lesions and the resistance of the strains to antifungals.

Basidiobolomycosis a mysterious fungal infection mimic small intestinal and colonic tumour with renal insufficiency and ominous outcome.

Basidiobolomycosis is a rare fungal infection caused by Basidiobolus ranarum a worldwide environmental saprophyte fungus that is potentially a pathogen. B ranarum mainly affects the subcutaneous tissues but gastrointestinal and disseminated infections rarely have been reported previously. We report a case of B ranarum infection in a 12-year-old immunocompetent boy presented with abdominal pain from 2 months earlier before admission in the hospital. Abdominal CT scan revealed a lobulated mass-like lesion in the vicinity of right lower quadrant enveloping ascending colon and terminal ileum with involvement of mesentery. Histopathological examination showed severe full-thickness infiltration of inflammatory cells mainly eosinophils admixed with multinucleated giant cells and large areas of necrosis with granulomatous feature. Periodic acid Schiff staining demonstrated thin-walled septate fungal hyphae's surrounded by eosinophilic material (Splendore-Hoppeli phenomenon) identified as a basidiobolomycosis.

Eosinophilic granulomatous gastrointestinal and hepatic abscesses attributable to basidiobolomycosis and fasciolias: a simultaneous emergence in Iraqi Kurdistan.

BACKGROUND: Deep eosinophilic granulomatous abscesses, as distinguished from eosinophilic subcutaneous abscesses, are rare. Most reports are from the Far-East and India where the most commonly attributed cause is Toxocara. Sulaimaniyah in Northeastern Iraq has experienced an outbreak of eosinophilic granulomatous liver and gastrointestinal (GI) abscesses beginning in 2009. The purpose of this study was to determine the etiology and guide treatment. METHODS: The study was an ongoing investigation of patients having a histopathologic diagnosis of eosinophilic granulomatous abdominal abscesses in Sulaimaniyah hospitals from May 2009 to August 2012. Tissues were examined for organisms, and Enzyme Linked Immunoabsorbent Assays (ELISA) were performed for serum antibodies to Fasciola hepatica, Toxocara, and Echinococcus granulosus. RESULTS: Fourteen patients had granulomatous inflammation surrounding a central necrotizing eosinophilic exudate identified in surgical pathology specimens from abdominal surgeries. Two children and four adults had abscesses that formed GI masses. These patients included a 39 year old male with oropharyngeal and transverse colon disease, and a 48 year old male with liver and GI abscesses. All sites demonstrated a Zygomycete fungus surrounded by eosinophilic Splendori-Hoeppli material consistent with basidiobolomycosis. Five of the six patients with fungal disease were treated by surgery and 4 to 7 months of itraconozol. One child died of intestinal perforation while receiving IV amphotericin B; two adults required additional surgery for recurrent GI obstruction. Eight patients had isolated liver abscesses with no organisms identified by histopathology: ELISA results for F. hepatica were positive for five, borderline for one, and negative for two patients. These eight patients were treated for fascioliasis by surgical resection of localized abscesses and albendazol. One patient serologically positive for F. Hepatica was found to have a common duct fluke two years after initial diagnosis. Serological testing for Toxocara and Echinococcus granulosus was negative in all 14 patients. CONCLUSIONS: Basidiobolomycosis and F. hepatica are implicated as the cause of abdominal eosinophilic granulomatous abscesses in 12 of 14 patients identified over a period of 40 months in northern Iraq. Treatment was complicated by chronic biliary tract disease in fascioliasis and perforation and recurrent intestinal obstruction with basidiobolomycosis.

Isolated renal zygomycosis: novel diagnostic and prognostic criteria with experience of a tertiary care center.

BACKGROUND: Isolated renal zygomycosis is a life-threatening infection and difficult to diagnose ante mortem due to varied presentations. Most reports in the literature are case reports. We are presenting our experience of 10 patients. MATERIALS AND METHODS: Retrospective data of 10 consecutive patients with primary renal zygomycosis, including 2 post-transplant patients, in our tertiary care center was analyzed. Epidemiological characteristics, predisposing conditions, clinical presentation, diagnostic findings and treatment outcomes were recorded. Characteristic radiological findings were recorded. Localized disease was managed by supportive treatment or percutaneous drainage and extensive disease with unilateral or bilateral nephrectomy. Renal involvement was confirmed in all patients by histopathology. RESULTS: The mean age of presentation was 35 years. Five patients who had bilateral renal involvement presented with oliguric acute renal failure, hematuria and abdominal pain. Three had unilateral renal disease and presented with flank pain and fever. The two post-transplant patients presented with fever and graft dysfunction. Even after aggressive treatment 5 patients died, accounting for a mortality rate of 50%. CONCLUSION: Isolated renal zygomycosis can be diagnosed with typical radiological findings, combined with clinical, laboratory and histopathological features. This study describes the newer ante mortem radiological diagnostic criteria and prognostic predictors of the disease.

Pulmonary zygomycosis in a non-neutropenic patient with myelodysplastic syndrome on lenalidomide.

Pulmonary zygomycosis is an uncommon infection that occurs mostly in immunocompromised patients. We report the case of a 75-year-old man with myelodysplastic syndrome, treated with lenalidomide for 3 months, who developed respiratory failure and a rapidly progressive left upper lobe consolidation. An extensive workup was unrevealing of the etiology, and the patient expired. A full autopsy was declined, but an in situ post-mortem transbronchial lung biopsy revealed pulmonary zygomycosis. This unique case illustrates the potential risks of lenalidomide therapy in patients with myelodysplastic syndrome and the difficulties in diagnosing pulmonary zygomycosis. To our knowledge this is the first report of a diagnostic in situ post-mortem transbronchial lung biopsy.