A 35-year-old-male patient presented with sudden loss of vision associated with periorbital swelling in both eyes (OU). He had systemic complaints of fever and pedal edema. The patient was already undergoing treatment for membranoproliferative glomerulnephritis, and for the previous seven months was taking oral prednisone (pg. mg/day) and Angiotensin converting enzyme inhibitor. Ocular examination revealed visual acuity of light perception (LP) with inaccurate ray projection in OU.
Slit lamp biomicroscopic examination revealed 2+ cells in anterior chamber and pigments over the anterior lens surface in OU. Fundus examination revealed hazy media along with retinal detachment in the inferotemporal area in OU. Sub-retinal exudates, intraretinal hemorrhages along with dilated veins and narrowed arteries were visible. Intraocular pressure (IOP) was 16 mmHg in OU. The patient had blood pressure of 160/100 mmHg and bradycardia. Hence, the initial diagnosis was hypertension related exudative retinal detachment, although both atypical central serous chorioretinopathy and Vogt-Koyanagi Harada syndrome were also considered in differential diagnosis. After receiving a nephrologist's opinion, the patient was stabilized with an injection of hydrocortisone 200 mg OD, frusemide 40 mg OD, cefixime 200 mg BD along with amlodipine 5 mg OD as a hypertensive drug.
After two days, the patient developed generalized hypotony, a staggering gait, truncal ataxia, and buccal and proximal weakness with decreased plantar reflexes. After neurology consultation, a pontomedullary lesion or cytomegalovirus meningitis was considered as an initial cause. A cerebrospinal fluid tap was performed to rule out meningitis; the results were normal. Magnetic resonance imaging (MRI) of the brain [Figure 1a and b] showed multiple cystic-enhancing lesions over both cerebral hemispheres, suggestive of tuberculomas or neurocysticercosis.
The patient was started on anti-tubercular treatment (ATT) along with oral steroids. Four days after starting ATT, the patient had a generalized tonic-clonic seizure. A contrast MRI showed disseminated parenchymal lesions. At the beginning of anti-epileptic treatment, patient showed signs of improvement over the next two weeks; however after this period the patient started worsening. The patient had restricted abduction, sluggishly reacting pupils, 3+ cells in the anterior chamber OU with IOP of 66 and 52 mmHg in the right and left eyes, respectively. Fundus examination revealed increased exudates, increased height of retinal detachment and vitreous cells OU. A diagnosis of disseminated toxoplasma or malignancy was considered. Analysis of an aqueous tap via polymerase chain reaction (PCR) was negative for malignant cells, tuberculosis, cytomegalovirus (CMV), herpes simplex virus (HSV), varicella zoster virus (VZV), and toxoplasma.
An Ahmed glaucoma valve was implanted in the left eye subsequent to failure of IOP control with maximum medical therapy. Postoperatively, the left eye was stony hard with severe conjunctival chemosis and a shallow anterior chamber [Figure 2].
Due to suspicion of metastatic endophthalmitis, five weeks after initial presentation, a vitreous biopsy was performed through pars plana vitrectomy. Intravitreal vancomycin (1 mg/0.1 ml), ceftazidime (2.5 mg/0.1 ml), and voriconazole (1 mg/0.1 ml) were instilled at the end of the procedure and the patient was started on fortified cefazolin 5% and tobramycin 1.4% every hour and Natamycin 5% (every six hours) for presumptive fungal endophthalmitis. All tissue sample smears and cultures that were tested for bacterial and fungal organisms were negative.
Subsequently, the patient had no LP along with severe conjunctival chemosis, exposure keratopathy, and conjunctival abscesses. Cerebrospinal fluid was negative for CMV and Cryptococcus sp. Blood tests were negative for HIV and TORCH (Toxoplasma gondii, other viruses [HIV, measles, and so on), rubella [German measles]), CMV, and herpes simplex. Blood culture showed no evidence of bacterial and fungal organism growth. A bone marrow biopsy was performed to rule out blood cell malignancy but results were normal.
Due to the progressive worsening of patients' systemic status and severe cachexia, a repeat MRI of the brain was done, and revealed a large right parieto-occipital mass lesion [Figure 1c and d]. The orbital region showed bilateral globe deformities, intraocular hemorrhages, and extraocular soft-tissue inflammation [Figure 1e and f].
A multiloculated intracerebral abscess was drained, and a brain biopsy was performed, revealing the presence of the Nocardia organism along with granulomatous reactions [Figure 3a-d]. The patient was administered intravenous imipenem for two weeks, amikacin for four weeks, and later, oral cotrimoxazole and trimethoprim. However, the patient had no LP in both eyes despite anti-nocardial treatment, probably because of delayed diagnosis and severity of involvement. Although the patient improved systemically over next two weeks, he subsequently developed phthisis of both eyes.