|Year : 2008 | Volume
| Issue : 1 | Page : 45-47
Sino-orbital infection by syncephalastrum racemosum in chronic hepatorenal disease
VP Baradkar, Meenakshi Mathur, Monalisa Panda, Simit Kumar
Department of Microbiology, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai - 400 022, India
V P Baradkar
Department of Microbiology, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai - 400 022
Source of Support: None, Conflict of Interest: None
| Abstract|| |
We describe the first recorded case of invasive rhino-orbital infection with the zygomycete Syncephalastrum racemosum in a 45-year-old male patient with chronic hepatitis B infection, along with cirrhosis of liver. The patient was successfully treated with partial surgical debridement and liposomal amphotericin B.
Keywords: Hepatic disease, mulormycosis, sino-orbital infection, Syncephalastrum racemosum
|How to cite this article:|
Baradkar V P, Mathur M, Panda M, Kumar S. Sino-orbital infection by syncephalastrum racemosum in chronic hepatorenal disease. J Oral Maxillofac Pathol 2008;12:45-7
|How to cite this URL:|
Baradkar V P, Mathur M, Panda M, Kumar S. Sino-orbital infection by syncephalastrum racemosum in chronic hepatorenal disease. J Oral Maxillofac Pathol [serial online] 2008 [cited 2020 Sep 21];12:45-7. Available from: http://www.jomfp.in/text.asp?2008/12/1/45/42199
| Introduction|| |
Mucormycosis, though uncommon, is often a fatal infection caused by fungi of family, Mucoraceae.  An underlying disease most frequently is diabetes mellitus. Other predisposing factors include hematological malignancy, treatment with cytotoxic drugs, long-term treatment with corticosteriods, AIDS, renal disease, or liver disease (cirrhosis). ,,
Mucormycosis is usually seen in different anatomical sites, such as paranasal, rhino-orbital, rhino-orbitocerebral, cerebral, pulmonary, gastrointestinal, and rarely as a disseminated disease. ,,
The most common agent causing human disease is Rhizopus spp., followed by Rhizomucor.  Other agents which have been reported to cause mucormycosis are Absidia, Mucor, Motirella, Cunninghamella, Saksenia,  and Syncephalastrum. ,
Response to treatment depends upon early diagnosis and initiation of antifungal therapy augmented by surgical debridement. ,,
Here, we have reported a rare case of invasive rhino-orbital mucormycosis caused by the least pathogenic agent, Syncephalastrum racemosum , in a patient suffering from chronic hepatitis leading to cirrhosis of liver.
| Case History|| |
A 45-year-old male patient with a 12-year history of chronic hepatitis B infection and hepatic cirrhosis was admitted for fever and chills; along with puffiness of face; and edema of feet, which had begun 15 days earlier. He also had a 1-month history of episodes of hematemesis and epistaxis.
On general examination, the patient was found to have icterus and pallor. On abdominal examination, there was no guarding, rigidity, free fluid, or organomegaly. Findings from examinations of cardiovascular and respiratory systems were within normal limits.
The laboratory reports showed a WBC count of 8600/mm 3 , normal platelet and differential count (no neutropenia). The patient was however anemic, with Hb of 9 g/dL. His renal output was within normal range, but the hepatic parameters were deranged. The SGOT value was 104 U/L (0-35 U/L); SGPT, 40 U/L (0-35 U/L); serum alkaline phosphatase, 12U/L (44-147 U/L); total bilirubin, 4 mg/dL; serum albumin, 2.8 g/dL (3.5-5.5 g/dl); and prothrombin time, 13 s (11.1-13.1 s). The serum creatinine was 8 mg/dL.
The patient was HbsAg positive and HbeAg negative. The patient was nondiabetic. He was seronegative for HIV 1 and HIV 2 antibodies. There was no history of long-term steroid administration or any other immunosuppressant. Sonography of abdomen showed findings suggestive of fatty degeneration of liver. The patient improved initially after hospitalization, and the edema of the feet reduced. But few days later, he developed nasal pain; swelling of the left eye, along with watering and congestion; and bilateral bleeding from the nose. CT scan of paranasal sinuses showed mucosal thickening, enhancing soft tissue density in the left maxillary sinus, both ethmoidal and sphenoid sinuses, with mass extending slightly towards the left eye [Figure 1]. The orbit and its contents were normal. Due to the nasal bleeding, detection of mass in the nose, progressive swelling towards the left eye, and the CT scan findings, the treating physician biopsied the lesion and sent specimens for histopathology, microscopy, and culture.
KOH examination revealed thick-walled aseptate distorted hyphae with irregular spacing. Gomori's methanamine silver (GMS) staining also revealed broad aseptate hyphae with collapsed walls and fruiting head [Figure 2]. Hematoxylin and eosin (H and E) staining of biopsy material showed aseptate hyphae [Figure 3]. The sample material was inoculated on Sabouraud's dextrose agar with and without antibiotics respectively. Cultures were incubated at 25°C to 30°C. Whitish fluffy growth appeared in 2 days, characteristic of agents causing mucormycosis [Figure 3]. Lactophenol cotton blue (LPCB) preparation was done from the colony. It showed sporangiophores branching at right angles and terminating into spherical collumella with cylindrical mesosporangia around entire circumference giving a 'daisy head' appearance [Figure 4]. It was differentiated from Aspergillus species on the basis that spores were confined within mesosporangia and hyphae were broad, aseptate. The morphology was confirmed by putting slide culture on potato dextrose agar (PDA).
Repeat biopsy from mucosa of nasal and sinus wall was taken, and the diagnosis of mucormycosis due to Syncephalastrum was confirmed. Histopathological findings were also suggestive of mucormycosis with fragmented hyphae.
Surgical debridement was done, and a short course of intravenous liposomal amphotericin B was given. The patient responded to the treatment, with reduction in the chemosis and swelling of the eye.
| Discussion|| |
Mucormycosis can manifest itself in different morphological forms. The most common form is the 'rhino-orbital' form, which may progress further to 'rhino-orbitocerebral' form. , The fungi are usually harmless commensals, and spores can be seen in the nose, throat, and stool of healthy subjects; but in extraordinary circumstances, they can cause invasive disease. , The infection may start with inhalation and deposition of spores in nasal turbinates. The infection extends into adjacent paranasal sinuses. It may then progress through the ethmoids and retro-orbital tissues to eventually reach the brain through the orbital apex.  Humans are frequently exposed to the spores of these fungi, which ordinarily have little intrinsic pathogenicity. Sporulation and growth require the host defenses to be compromised, and some debilitating illness is identified in over 95% of cases.  The commonest predisposing factor for invasive mucormycosis is uncontrolled diabetes mellitus. ,, Other reported predisposing factors are hematological malignancy, intravenous drug addiction, treatment with cytotoxic drugs, iatrogenic immunosuppression after organ transplantation, severe burns, AIDS, long-term treatment with steroids, kwashiorkor pellagra, and usage of desferrioxamine in dialysis patients. ,, Miscellaneous underlying conditions reported are amebiasis, typhoid fever, gastroenteritis, gastrointestinal or liver disease.  The present case presented with chronic hepatitis B infection and cirrhosis of the liver. Cirrhosis of the liver is a known predisposing factor for mucormycosis.  Apart from the hepatic involvement and cirrhosis of the liver, there was no other predisposing factor in the present case.
The most common agents causing human disease are Rhizopus spp., Rhizomucor, and Absidia. Other agents such as Saksenaea vasiformis, Apophysis elegans, Cunninghamella, Mortirella , and Syncephalastrum racemosum have also been reported to cause mucormycosis. 
Syncephalastrum racemosum is a ubiquitous saprophyte. It is usually described as having low pathogenic potential. It was also debated initially whether this fungus can cause human disease, but later it was considered as an opportunistic pathogen.  A case of great toenail onychomycosis has been described in a 45-year-old man. It was treated successfully with surgical nail plate avulsion, along with nystatin ointment, b.i.d., for 2 weeks. 
An invasive case of intra-abdominal zygomycosis caused by Syncephalastrum racemosum was described by Schelbusch et al.  It was the first recorded case of invasive infection of anterior abdominal wall and omentum, which was successfully treated with partial surgical debridement and amphotericin B lipid complex.
In this case report, there was invasive rhino-orbital infection caused by Syncephalastrum racemosum .
Searching the literature with Pub Med. Medicine, Cinatil proquest, Sciencedirect, Embase, and web of science, no previous case of rhino-orbital mucormycosis by Syncephalastrum racemosum was found.
At present, amphotericin B is the drug of choice for mucormycosis.  This drug is effective in eradicating the primary lesion and also can control micrometastasis. Incorporation of these drugs into liposomes results in reduced nephrotoxicity due to altered physiological distribution. Reversible toxic effects of amphotericin B include nephrotoxcity, bone marrow depression, fever, chills in rare cases.  Its dose is 0.7 to 1 mg/kg/day. In the present case, the patient received a short course of liposomal amphotericin B for 1 week with surgical debridement, to which he responded. Afterwards, he was given oral fluconazole for 3 weeks.
Surgery remains the primary mode of treatment.  An effort should be made to remove as much devitalized and necrotic tissue as possible. The prognosis of mucormycosis has improved markedly in the last 30 years ,, with the use of Amphotericin B. Early institution of medical and surgical therapy is an important factor, which favors good outcome.
With rapidly expanding population of immunocompromised patients, mucormycosis and other fungal infections of nose and paranasal sinuses are likely to increase. Therefore, a high index of suspicion and proper cooperation between clinicians and microbiologists are needed for successful outcome of the treatment.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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