mucormycosis , also known as zygomycosis , is an opportunistic and lethal mycological infection caused by a fungus of the order mucorales .
it is mostly observed in immunocompromised patients such as those with hematological malignancy or those who have solid organ transplantation ( sot ) or bone marrow transplants .
the infection usually starts in the middle nasal meatus and spreads to the paranasal sinuses , the orbit and the intracranial structures by direct extension or through blood vessels .
early diagnosis , initiating prompt surgical and medical therapy and correcting the underlying conditions play a vital role in eliminating this infection , but mortality remains high .
mucormycosis is uncommon in sot recipients and most cases have been reported after liver or renal transplantation .
a 52-year - old male patient was transferred to our department from another center for retroorbital pain , headache and decreased vision accuracy in the left eye for the past 36 h. five months earlier , he had undergone orthotopic heart transplantation under the bicaval technique due to ischemic heart failure .
his medical history was significant for type ii diabetes mellitus , poorly controlled by insulin .
he also had hypertension and dyslipidemia ( both under treatment ) and nondialysis - dependent chronic kidney disease .
his baseline immunosuppressive regimen consisted of prednisone , tacrolimus and mycophenolic acid and he was also taking prophylactic antibiotics : cotrimoxazole , valganciclovir and isoniazid with pyridoxine . physical examination on
admission showed that the patient was alert , orientated , afebrile and his vital signs were stable .
anterior rhinoscopy , endoscopy with 0 rigid endoscope and examination of the oral cavity were unremarkable .
although the nasal exploration was normal , in view of the symptoms and the previous transplant , two samples endoscopically directed , one of the nasal fossa by swabbing and the second of the middle meatus were collected .
blood tests showed glucose levels of 248 mg / dl ( 13.8 mmol / l ) and creatinine of 3.63 mg / dl . computed tomography ( ct )
scan revealed soft tissue opacification of left anterior ethmoid sinus and inflammatory changes in the apex of the left orbit [ figure 1a ] .
( a ) computed tomography scan showed soft tissue opacification of left ethmoid sinus ( white arrows ) and inflammatory changes in the apex of the left orbit ( black arrows ) .
( b ) fundus of the left eye with edema and papillary pallor ( * ) , posterior pole retinal edema ( white arrows ) and arteriolar sclerosis ( black arrows ) .
( c ) left nasal endoscopy with 0 rigid endoscope , showing black necrotic eschar ( white arrows ) through the left middle meatus and the middle turbinate ( black arrows ) .
nasal septum ( * ) , ( d ) lactophenol cotton blue culture showing rhizopus fungus .
sporangiophore ( black arrow ) ( lactophenol cotton blue stain , x1000 ) the metabolic derangement was treated , intravenous antibiotics ( piperacillin / tazobactam 2000/250 mg 8 hourly ) was started and insulin was administered on a sliding scale . on the second day of hospitalization , the patient 's clinical status worsened dramatically . left eye movements were limited in all directions ( frozen eye ) and the left pupil was mydriatic .
signs of central retinal artery occlusion were found on the left fundoscopy : edema and papillary pallor , posterior pole retinal edema and arteriolar sclerosis [ figure 1b ] .
a second nasal 0 rigid endoscope showed necrotic tissue in the left middle meatus and the middle turbinate [ figure 1c ] .
the lactophenol cotton blue preparation revealed a sporangium located on the columella at the apical end of the sporangiophore [ figure 1d ] . following the confirmation of rm , intravenous liposomal amphotericin b
the magnetic resonance imaging showed that infection had progressed in the apex of the left orbit , involving the cavernous sinus .
we proposed extensive surgical treatment via left paralateral rhinotomy with orbital exenteration and total ethmoidectomy .
its incidence has increased over the last 20 years due to increasing use of immunosuppressive drugs .
it appears most frequently in the first 6 months after transplantation due to the higher immunosuppression .
the incidence ranges from 0.4% to 16% depending on the sot type and is 0%0.6% in heart transplant recipients .
other significant predisposing conditions to mucormycosis in sot recipients are poorly controlled diabetes mellitus and renal failure .
the fungus spreads quickly in a medium of elevated glucose and acid ph and it is unusual in patients with metabolically controlled diabetes . the main protection against hyphae is neutrophils , but low serum ph decreases their pathogenic and chemotactic capacity .
our patient had three predisposing conditions : immunosuppression for sot , poorly controlled diabetes mellitus and renal failure .
three main ways of transmission of mucormycosis in transplant recipients have been reported : inhalation in approximately 70% , ingestion in 2% and percutaneous introduction in 13% .
it then extends to the orbit and periorbital area where it can produce the orbital apex syndrome with ophthalmoplegia , ptosis , mydriasis and decreased visual accuracy .
finally , it disseminates to the cavernous sinus and then to the meninges and brain .
clinical presentation of the disease usually involves symptoms of sinusitis and periorbital cellulitis with facial pain and decreased vision .
a nasal endoscopy and a biopsy or a culture of the infected area are essential for the definitive diagnosis .
a culture gives us more information about the species of mucorales , but it has a higher rate of false negatives than the biopsy .
yohai et al . reported nasal ulceration or necrosis in 48% and 78% respectively in rm . in our case , the initial nasal endoscopy was unremarkable , but we performed a middle meatus culture in view of the high suspicion of rm and the culture revealed a fungus of the order mucorales .
the contrast - enhanced sinus ct is helpful to delimit the extension of rm and reveal signs of invasion .
magnetic resonance is more sensitive than ct to evaluate intradural and intracranial involvement , the cavernous sinus thrombosis and thrombosis of the cavernous portions of the internal carotid artery .
radical resection removing all necrotic tissue should be done and various debridements are usually required .
lateral rhinotomy , orbital exenteration and occasionally intracranial surgery are performed depending on the extension of rm .
orbital exenteration is necessary when the orbit is infected with limited eye movements in all directions ( frozen eye ) and loss of vision .
the surgery that we proposed to our patient was a left paralateral rhinotomy with orbital exenteration and total ethmoidectomy removing all the unviable tissue .
amphotericin b is the drug of selection , but it is limited because of its renal and systemic toxic effect .
this lipid formulation improves circulation time and concentration in the infected area of the associated amphotericin b. the duration of the treatment is not explicitly established .
survival rates in diabetic patients with rm are higher than those in patients with other predisposing factors such as renal failure , hematologic malignancies or autoimmune diseases .
a delay in diagnosis and treatment , brain involvement , hemiparesis , bilateral sinus involvement and facial necrosis is also associated with poor survival .
rm is a rare and aggressive fungal infection in heart transplant recipients and carries a high mortality rate .
 mucormycosis is an opportunistic acute fungal infection with a high mortality rate seen in immunocompromised patients . 
 it is extremely rare in heart transplant recipients . 
 rhinocerebral mucormycosis ( rm ) is the most frequently observed presentation . 
 we report a case of rm in a heart transplant recipient 5-month after the procedure , with a fatal outcome . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION
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