Introduction

GIB is a rare fungal infection that causes unusual chronic, skin infections and is increasingly being recognized as a causative agent of gastrointestinal basidiobolomycosis (GIB), especially in pediatric populations from tropical and subtropical regions. Basidiobolomycosis is almost always misdiagnosed as other chronic granulomatous diseases, malignancies or inflammatory bowel diseases.1 GIB is rarely reported in medical literature although sporadic cases have been reported worldwide, predominantly in the United States and Saudi Arabia, with some additional cases observed in Brazil and Iran.2 The first presumed case of Gastrointestinal Basidio-bolomycosis (GIB) was reported in 1964 in a six years old Nigerian boy. Subsequently, two cases were reported in 1979 involving the gastro-intestinal tract in apparently healthy individuals.3 Diagnosis of GIB is difficult as its occurrence in immunocompetent host is a cofounder. In addition, the nonspecific clinical presentations, such as fever, abdominal pain, or abdominal mass, could mimic infectious gastrointestinal tract disease or tumors.

Case Report

A 3yr old boy presented with moderate to high grade fever since 15 days (102 F to 103 F). No history of cough, ear discharge, headache, vomiting, loose stools or burning micturition. There were pain abdomen–10 days (intermittent pain abdomen), history of constipation,passage of hard stools and sometimes blood streaked. Abdominal distension 3-4 day (left side of abdomen) was noticed. No history of pallor, icterus, cyanosis, clubbing, lymphadenopathy and oedma are seen. Liver is palpable 3 cm BCM. Routine blood investigation showed increased TLC-21.3x1000/ul, DLC showed eosinophilia(18%),HB-8.4gm/dl, and PLT-639x1000/cmm.

Histopathological examination

Section shows fibrocollagenous and fibroadipose tissue infiltrated by sheets of dense inflammatory cell infiltrate comprising chiefly of eosinophils, neutrophils, histiocytes, few plasma cells and lymphocytes.There are necrotizing granulomas with histiocytic palisading along with eosinophillic microabscesses. Many scattered multinucleated giant cells are seen (Figure 2C). Several broad, non-septate, fungal elements are seen on CSM stain (Figure 2D). These are focally surrounded by granular eosinophilic material (Splendore- Hoeppli phenomenon as diagnostic histological criteria). Abdominal lump biopsy also showed Fungal inflammation (The morphological features of the fungus is suggestive of Mucorspecies possibility of Gastrointestinal Basidiobolomycosis was considered).Fungal culture study displayed tissue examined in 10% KOH showed broad, hyaline, infrequently septate mycelia. Growth on Sabouraud dextrose agar (SDA) was creamy, waxy and glabrous colonies with radial folds. LPCP from culture showed infrequently septate hyphae, and numerous globose zygospores with conjugation beaks characteristic of Basidiobolus ranarum (Entomophthoromycosis).

Figure 1

A & B: Cytospin smears showed predominantly polymorphonuclear leucocytes admixed with reactive mesothelial cells and histiocytes on a clear background (MGG, 40x magnification). C&D Smears showed several broad based, non

septate, fungal elements (MGG and PAS, 40 magnification).

Figure 2

A & B: Cytospin smear showed several broad based, non septate, fungal elements (CSM, 20 & 40x magnification). C. Section showed several broad based, non-septate, fungal elements and focally surrounded by gramular eosinophilic material known as Splendore Hoeppli phenomenon (h &e, 40x magnification). D: Section showed several broad based, non septate, fungal elements (CSM, 40 magnification).

Discussion

Basidiobolomycosis is a rare fungal infection and it was previously included in the Zygomycete class. Of all the pediatric GIB cases reported, almost 70% are from Saudi Arabia.4 Saudi Arabia also has the second highest overall reported GIB patient pool.5 However, basidiobolomycosis is almost always misdiagnosed as other chronic granulomatous diseases, malignancies or inflammatory bowel diseases. 1

The most common presenting symptom is abdominal pain, followed by fever, weight loss, or abdominal mass. GIB has been increasingly reported in the literature, and it is an emerging disease from arid regions worldwide, in particular, the south-western Saudi Arabia.

In patients presenting with peripheral eosinophilia, fever and abdominal pain with presence of intestinal thickening or mass on imaging, the common differential diagnosis comprises of tuberculosis, malignancy, lymphoma, Crohn’s disease and diverticulitis.6 GIB usually has nonspecific clinical manifestations and its diagnosis is challenging. Missed and delayed diagnosis of GIB increases the risk of morbidity and mortality, especially in pediatric patient. Histopathologic examination showed a necrotizing, granulomatous process, with a prominent Splendore-Hoeppli reaction surrounding spores and aseptate hyphae. It is usually a subcutaneous infection that affects mostly young, male persons, and it is transmitted through traumatic inoculation. Confirm diagnosis of GIB requires culture or demonstration of aseptate hyphae with surrounding eosinophilic projections (Splendore-Hoeppli phenomenon) on histopathological examination. This should be differentiated from mucormycosis which may also rarely involve the gastrointestinal tract and show aseptate hyphae without Splendore-Hoeppli phenomenon. 6

Because of its indolent presentation, it is often misdiagnosed as IBD, tuberculosis or Malignancy. An author reported in a 7 year old Saudi girl with abdominal mass, fever and eosinophilia resembling malignancy on radiological and pathological picture fully recovered with only medical therapy in the form of oral Voriconazole.7 A two month old male child from the deserts of western part of Rajasthan, India presented with the complaint of abdominal swelling associated with vomiting, Computed Tomography of abdomen (day+5) showed large heterogeneously enhancing abdomino-pelvic mass predominantly in pre-sacral and right inguinal fossa (RIF) region with encasement of sigmoid colon and upper part of rectum, with resultant bowel obstruction and disseminated gastrointestinal basidiobolomycosis. 8 Radiologically, most of the cases reported as abdominal masses in the colon, liver mass or small bowel mass. Serious complication such as intestinal perforation, abscess and hydronephrosis were reported as well. 9 Youngest case report of GIB in a 16-month-old infant and highlighted two important points. First, the need of high index of suspicious for diagnosis of GIB and its inclusion in the differential diagnosis of any abdominal mass, fever and eosinophilia. Second, it gives additional supportive evidence that medical management with voriconazole without surgical intervention is curative. 10

Most of the reported cases treated as combined treatment (surgical and medical). There are few reported cases responded to medical treatment alone without surgical intervention. 11, 12

Treament advised as ebulking surgery and itraconazole therapy. Patient continued Itraconazole therapy for 3 to 6 months duration. Dose of Itraconazole, or changing over to oral Voriconazole or combination therapy of Itraconazole and Voriconazole. Child showed dramatic response to IV Voriconazole in the form of being afebrile within 48 hrs of starting IV Itraconazole. Child also demonstrated normalization of blood counts (TLC and eosinophils) within a week of starting antifungal therapy. Basidiobolomycosis has a good prognosis based on the available data. Most of the mortalities are attributable to younger age groups, delays in diagnosis and initiation of appropriate antifungal therapy as well as complications of this illness. Our case highlighted the important points is of either GIB as an increasingly recognized cause of abdominal mass and eosinophilia that can mimic other diseases or it gives additional clinical experience that Itracona-zole/Voriconazole without surgical intervention can be curative without relapses, thus avoiding unnecessary major surgeries.

Conclusion

We presented a rare case of gastrointestinal basidiobolomycosis with abdominal pain and mass as the initial presenting symptoms. Although diagnosis is confirmed by cytology, histopathology after surgical removal of the mass in the gastrointestinal tract, the antifungal treatment using itraconazole/Voriconazole showed significant improvement of the clinical symptoms. The result of this case report could assist clinicians to better manage patients with GIB with the intend of avoiding any complications associated with this rare disease. Due to non-availability of this patient was managed with voriconazole for one year. On follow-up, the child is asymptomatic and doing well

Source of Funding

None.

Conflicts of Interest

None.