idiopathic intracranial hypertension ( iih ) is a condition characterized by headache , papilledema , and a raised cerebrospinal fluid pressure with normal brain imaging . it commonly occurs in obese women , but is occasionally reported among pediatric chronic renal failure and renal transplant patients . we present a case of adult renal transplant recipient diagnosed with iih , who manifested rhinocerebral zygomycosis with extraocular involvement due to escalated steroid therapy for iih . a 20-year - old woman , a case of live - related post - renal transplant ( haplomatch ) , of 5-year duration , with underlying chronic allograft nephropathy ( on prednisolone 7.5 mg and azathioprine 50 mg ) presented with persistent non - throbbing headache , double vision , and inability to read for 3 weeks . examination revealed blood pressure of 140/90 mm and bilateral papilledema with restriction of left extraocular muscle movements ( lateral rectus paresis ) . investigations revealed anemia ( hb 8.8 g / dl ) , mild azotemia ( serum creatinine 2.2 mg / dl and urea 57 mg / dl ) , normal magnetic resonance imaging ( mri ) , mr venogram of brain , and normal tsh . a diagnosis of iih was made and was managed with acetazolamide , oral glycerol , escalated prednisolone dose , and antihypertensive , with continuation of basic immunosuppressive drugs . one month later , she reported with pain and swelling in the left half of face and inability to open the left eye . examination revealed redness and swelling of the left half of her face with eyelid edema , partial restriction of extraocular muscles of the left side , resolving papilledema , and left maxillary sinus tenderness . computer tomography ( ct ) scan of paranasal sinuses showed left - sided pan - sinusitis with focal calcification [ figure 1 ] , and mri brain confirmed the same in addition to normal neurological structures and cavernous sinus . she underwent fiber optic endoscopic surgery of maxillary , ethmoid , and frontal sinus of the left side . histopathologic examination of sinus material and culture confirmed zygomycosis [ figure 2 ] . she was started on amphotericin - b ( liposomal ) along with continuation of immunosuppressive drugs for next 2 months . during the course of treatment , she developed nodular swelling at inner canthus of left eye , aspirate of which showed broad branches ( aseptate hyphae ) of mucor for which exenteration of the eye was suggested but declined by the patient . she was continued on antifungal therapy and received a total of 1.9 g of amphotericin - b over 2 months along with dual immunosuppression . she continued to remain asymptomatic during the last follow - up at 5 years , with regression of the nodular swelling , improvement in vision , resolved papilledema , and stable renal parameters . computer tomography of paranasal sinuses showing involvement of the maxillary and ethmoidal sinuses by mucor methenamine silver stain showing mycelia ( aseptate hyphae ) and spores of mucor the exact incidence of iih in renal transplant patients is unknown , although a case series has reported it as 4.4% . several risk factors operational in the general population such as drugs , obesity , and anemia may apply in renal transplant patients as well . the presentation , as illustrated in our patient , includes persistent diffuse headache ( 92% ) , transient visual obscurations ( 72% ) , intracranial noises ( 60% ) , and diplopia ( 38% ) , which in turn is due to unilateral or bilateral sixth nerve palsy . these symptoms demand extensive exploration , especially in transplant patients . moreover , causes such as drugs in posttransplant ( tetracyclines , hypervitaminosis - a , lithium , nitrofurantoin , human growth hormone , thyroxine , cyclosporine , and tacrolimus),[35 ] accelerated hypertension , and anemia are all too frequent in post - renal transplant course incriminated as a causative factor for iih . visual impairment can be often mistaken for retinal or optic nerve disorder , hypertensive or diabetic changes , cataracts or calcification of the cornea , or choroidoretinal infections . also , papilledema , the hallmark of iih , is difficult to diagnose in a setting of hypertension as hypertensive retinopathy can mimic it ( as depicted in our patient ) . thus , iih in renal patients can easily be missed unless looked for , and a high index of suspicion helps to prevent optic atrophy . zygomycosis is seldom seen in such a setting of iih and is usually a fatal , opportunistic fungal infection caused by fungi ( commonly by rhizopus oryzae , rhizomucor , mycocladus , and mucor ) encountered in patients with diabetes mellitus , malignancy , or those under immunosuppressive therapy . although there are many case reports of zygomycosis in association with renal transplant , rhinocerebral zygomycosis occurring in a setting of iih in renal transplant has not been reported thus far . many classical diagnostic signs have been proposed such as epistaxis , partial or total ophthalmoplegia , chemosis , and periorbital edema , ptosis , exophthalmos ; other signs include grayish black discoloration of the nasal turbinates , palatal ulcers , and frontal lobe signs ( all seen in our patient except the latter two ) . radiologically , ct is superior to mri in demonstrating orbital involvement , mainly the lateral displacement of a thickened medial rectus muscle , increased density of the orbital apex , and enlargement of the optic nerve . is associated with early diagnosis , a high index of suspicion and prompt diagnosis by aggressive investigation such as tissue biopsy significantly affect the treatment outcome . progression of the disease is usually rapid and it warrants prompt and aggressive combined surgical intervention with antifungal agents . with the introduction of combined therapy with amphotericin - b and surgery , more than 80% of the patients can be expected to survive . our patient underwent early and repeated surgical debridement of all grossly involved tissues as emphasized in the literature on its importance for local control , especially in rhinocerebral , pulmonary , and cutaneous zygomycosis . administration of liposomal amphotericin - b is associated with adverse effects that are less pronounced , even with administration of larger doses . we administered liposomal amphotericin - b ( a total of 1.9 g ) and obtained satisfactory resolution of the disease ( without resorting to extirpation of the eye ) . although universal recommendations on the optimal duration of liposomal amphotericin - b are lacking , factors such as the response of infection to treatment and success in resolving the underlying predisposing condition govern the duration of therapy . the unusual presentation of iih in our adult patient mandating escalated immunosuppression which in turn led to the life - threatening complication of rhinocerebral zygomycosis is a wake - up call for clinicians dispensing high - dose immunosuppression . we also highlight the importance of early identification , and aggressive medical and surgical management in tackling zygomycosis which is life - saving despite pessimistic outcomes at times in transplant setting .
idiopathic intracranial hypertension ( iih ) , once called pseudotumor cerebri , presents with nonspecific signs and symptoms of increased intracranial pressure and papilledema , and is associated with high risk of loss of vision . 
 zygomycosis is a rare but serious fungal infection seen occasionally among renal transplant recipients in the late transplant period with high mortality risk . 
 early diagnosis coupled with multidisciplinary care can salvage the patient from the risk of death . 
 we present an unusual case of adult renal transplant recipient with iih followed by rhinocerebral zygomycosis secondary to amplified immunosuppression that was managed successfully .